Publikationen von Priv.-Doz. Dr. med. Hans Gerd Kehl

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(Stand 30/05/2016)

Bibliometrie , "zur Anzeige der Zusammenfassung + Statistik können Sie den Link [Abstract] in der nächsten Zeile anklicken"
Abstract:

Summe der Impactfaktoren: 109,594
Zitierungen durch andere Autoren: 567
daraus ermittelter Hirsch-Index (h-index): 13

Die Dokumentation dieser Daten ist auch abrufbar bei ResearcherID.

Publikationen in Zeitschriften mit Peer-Review Verfahren sind, soweit vorhanden, mit öffentlichen Links und den jeweiligen Impactfaktoren angegeben.
Die Impactfaktoren sind den Verzeichnissen des Journal Citation Reports (JCR) entnommen. Publikationen im aktuellen Jahr, die noch keinen neuen Impact-Faktor erhalten haben, wurden mit dem letzten verfügbaren Impactfaktor angegeben.
Zitierungen der eigenen Arbeiten durch andere Autoren und der daraus resultierende Hirsch-Index (h-index) wurden mit der Datenbank "Web of Science" ermittelt. Bei ResearcherID ist dieser Index ebenfalls auffindbar unter dem Link "Citation Metrics".

Die folgende Aufstellung enthält zusätzlich Publikationen in nicht gelisteten Zeitschriften sowie Buchbeiträge und publizierte Vorträge.

BibTeX:

@article{Zusammenfassung,

  author = {Bibliometrie },

  title = {zur Anzeige der Zusammenfassung + Statistik können Sie den Link  [Abstract] in der nächsten Zeile anklicken}

}

2016 Kehl HG, "Kapitel 3.6: Sonografie des Herzens und der herznahen Gefäße. In: Fetoneonatale Lunge", 2016, : 101-4. Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016.
BibTeX:

@book{Kehl_Hentschel-Jorch_3-6_Echo,

  author = {Kehl, H. G. },

  editor = {Hentschel, R. and Jorch, G},

  title = {Kapitel 3.6: Sonografie des Herzens und der herznahen Gefäße. In: Fetoneonatale Lunge},

  publisher = {Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016},

  year = {2016},

  pages = {101-4},

  url = {http://amazon.de/o/ASIN/3131748710/}

}

Kehl HG, "Kapitel 3.7: Herzkatheteruntersuchung. In: Fetoneonatale Lunge", 2016, : 104-6. Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016.
BibTeX:

@book{Kehl_Hentschel-Jorch_3-7_HKU,

  author = {Kehl, H. G. },

  editor = {Hentschel, R. and Jorch, G},

  title = {Kapitel 3.7: Herzkatheteruntersuchung. In: Fetoneonatale Lunge},

  publisher = {Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016},

  year = {2016},

  pages = {104-6},

  url = {http://amazon.de/o/ASIN/3131748710/}

}

Kehl HG, "Kapitel 3.7.6: Angio-Computertomografie in Dual Source-Technik. In: Fetoneonatale Lunge", 2016, : 106-8. Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016.
BibTeX:

@book{Kehl_Hentschel-Jorch_3-7-6_CT,

  author = {Kehl, H. G. },

  editor = {Hentschel, R. and Jorch, G},

  title = {Kapitel 3.7.6: Angio-Computertomografie in Dual Source-Technik. In: Fetoneonatale Lunge},

  publisher = {Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016},

  year = {2016},

  pages = {106-8},

  url = {http://amazon.de/o/ASIN/3131748710/}

}

Kehl HG, "Kapitel 4.2: Interaktion zwischen Herz-Kreislauf-System und Lunge. In: Fetoneonatale Lunge", 2016, : 152-4. Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016.
BibTeX:

@book{Kehl_Hentschel-Jorch_4-2_Herz-Lunge,

  author = {Kehl, H. G. },

  editor = {Hentschel, R. and Jorch, G},

  title = {Kapitel 4.2: Interaktion zwischen Herz-Kreislauf-System und Lunge. In: Fetoneonatale Lunge},

  publisher = {Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016},

  year = {2016},

  pages = {152-4},

  url = {http://amazon.de/o/ASIN/3131748710/}

}

Kehl HG, "Kapitel 4.5: Pulmonale Hypertonie und sekundäre Lungengefäßerkrankungen. In: Fetoneonatale Lunge", 2016, : 161-4. Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016.
BibTeX:

@book{Kehl_Hentschel-Jorch_4-6_pH,

  author = {Kehl, H. G. },

  editor = {Hentschel, R. and Jorch, G},

  title = {Kapitel 4.5: Pulmonale Hypertonie und sekundäre Lungengefäßerkrankungen. In: Fetoneonatale Lunge},

  publisher = {Thieme Verlag Stuttgart, ISBN: 978-3-13-174871-3, erscheint 07/2016},

  year = {2016},

  pages = {161-4},

  url = {http://amazon.de/o/ASIN/3131748710/}

}

2015 Bertram H, Emmel M, Ewert P, Grohmann J, Haas N, Jux C, Kehl HG, Kitzmüller E, Kretschmar O, Müller Gö, Wiebe W and Investigators of the Working Group Interventional Cardiology of the German Society of Pediatric Cardiology, "Stenting of Native Right Ventricular Outflow Tract Obstructions in Symptomatic Infants.", Journal of Interventional Cardiology., 2015, Vol. 28(3) : 279–287.
Ranking: 1,318
Abstract: OBJECTIVE:To assess feasibility, safety and effectiveness of right ventricular outflow tract (RVOT) stenting in symptomatic young infants.

METHODS:Multicentre evaluation of 35 patients intended to undergo RVOT stenting in 11 pediatric cardiac centres from 2009 to August 2011.

RESULTS:Median age and weight at the time of first stent implantation were 8 weeks and 3.3?kg, with 40% of patients <3?kg. A total of 19 patients had suffered from hypoxemic spells, 8 patients were ventilated, 6 on inotropic support and 5 on prostaglandin infusion. Severe concomitant malformations were present in 11 patients, and acute infections in 2. Stenting of the RVOT was successfully performed in 33 patients, improving oxygen saturation from a median of 77 to 90% 2 days after intervention. Besides the 2 patients in whom RVOT stenting was not successful for technical reasons, there were no procedural complications. In 17 of 33 patients, 1-3 reinterventions were performed during follow-up, less than half of those were reinterventions in the RVOT. A total of 27 patients have undergone successful surgical repair 4-162 (median 19.5) weeks after initial RVOT stent implantation, 2 patients are still waiting. There were no perioperative deaths.

CONCLUSIONS:Stenting of the RVOT provides a safe and effective management strategy for initial palliation in symptomatic young infants, including those patients not suitable or at higher risk for surgical therapy.

BibTeX:

@article{Bertram2015,

  author = {Bertram, Harald and Emmel, Mathias and Ewert, Peter and Grohmann, Jochen and Haas, Nikolaus and Jux, C and Kehl, Hans GGerd and Kitzmüller, Erwin and Kretschmar, Oliver and Müller, Götz and Wiebe, Walter and Investigators of the Working Group Interventional Cardiology of the German Society of Pediatric Cardiology},

  title = {Stenting of Native Right Ventricular Outflow Tract Obstructions in Symptomatic Infants.},

  journal = {Journal of Interventional Cardiology},

  year = {2015},

  volume = {28},

  number = {3},

  pages = {279–287},

  url = {http://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=25990981&retmode=ref&cmd=prlinks},

  doi = {10.1111/joic.12198}

}

Dehne J, Kehl HG, Debus V, Frank T, Gehrmann J, Schulze-Bahr E and Jux C, "Novel desmoplakin c.1789 T>C mutation in Carvajal Syndrome, a rare cause of DCM in children", Thorac Cardiovasc Surg., 2015, Vol. 63 (Suppl 3) : S 215.
Abstract: We report on a 13 year-old boy with clinical signs of Carvajal Syndrome in whom a novel c.1789 T>C mutation in the desmoplakin gene could be detected.
A 13 years old patient presented with dyspnea, fatigue, and vertigo under physical stress. On exam he showed remarkable acantholytic palmoplan- tar keratoderma and woolly hair. Chest-X-ray, echocardiography and MRI demonstrated severe dilatative cardiomyopathy of both ventricles with non-compactioning disarray and fibrosis. The ECG revealed negative T-waves in II, III aVF and V1-V4 together with polymorphic ventricular extrasystoles.
Histologic examination of myocardial biopsies showed chronic damage as in dilatative cardiomyopathy without inflammatory reaction or detection of infectious agents.
Genetic investigation revealed a novel heterozygous c.1789 T>C mutati- on in the desmoplakin-gene on chromosome 6p24 with an exchange of amino acid Ser597Pro in the spectrin binding domain 4 of the protein. Carvajal syndrome is defined by characteristic cardiocutaneous lesions: streaky palmplantar hyperkeratosis, woolly hair and early onset of primarily left ventricular dilatative cardiomyopathy with high risk of sudden death caused by arrhythmia or heart failure. Naxos Syndrome is a similar syndrome classified by arrhythmogenic right ventricular cardiomyopathy (ARVC) which summarises diseases with non-functional proteins of the desmosome-complex. Characteristically these diseases show left, right or biventricular cardiomyopathy and are responsible for up to 12,5% of sudden cardiac deaths especially in young people.
BibTeX:

@article{Dehne_Abstract_DGPK_Carvajal_2015,

  author = {Jana Dehne and Hans Gerd Kehl and Volker Debus and Thomas Frank and Josef Gehrmann and Erik Schulze-Bahr and Christian Jux},

  title = {Novel desmoplakin c.1789 T>C mutation in Carvajal Syndrome, a rare cause of DCM in children},

  journal = {Thorac Cardiovasc Surg},

  year = {2015},

  volume = {63 (Suppl 3)},

  pages = {S 215},

  doi = {10.1055/s-0035-1556019}

}

Rutsch F, Macdougall M, Lu C, Buers I, Mamaeva O, Nitschke Y, Rice GI, Erlandsen H, Kehl H-G, Thiele H, Nürnberg P, Höhne W, Crow YJ, Feigenbaum A and Hennekam RC, "A Specific IFIH1 Gain-of-function Mutation Causes Singleton-Merten Syndrome", Am J Hum Genet., 2015, Vol. 96(2) : 275–-282.
Ranking: 10,931
Abstract: Singleton-Merten syndrome (SMS) is an infrequently described autosomal-dominant disorder characterized by early and extreme aortic and valvular calcification, dental anomalies (early-onset periodontitis and root resorption), osteopenia, and acro-osteolysis. To determine the molecular etiology of this disease, we performed whole-exome sequencing and targeted Sanger sequencing. We identified a common missense mutation, c.2465G&gt;A (p.Arg822Gln), in interferon induced with helicase C domain 1 (IFIH1, encoding melanoma differentiation-associated protein 5 [MDA5]) in four SMS subjects from two families and a simplex case. IFIH1 has been linked to a number of autoimmune disorders, including Aicardi-Goutières syndrome. Immunohistochemistry demonstrated the localization of MDA5 in all affected target tissues. In vitro functional analysis revealed that the IFIH1 c.2465G&gt;A mutation enhanced MDA5 function in interferon beta induction. Interferon signature genes were upregulated in SMS individuals’ blood and dental cells. Our data identify a gain-of-function IFIH1 mutation as causing SMS and leading to early arterial calcification and dental inflammation and resorption.
BibTeX:

@article{Rutsch2015,

  author = {Rutsch, Frank and Macdougall, Mary and Lu, Changming and Buers, Insa and Mamaeva, Olga and Nitschke, Yvonne and Rice, Gillian I and Erlandsen, Heidi and Kehl, Hans-Gerd and Thiele, Holger and Nürnberg, Peter and Höhne, Wolfgang and Crow, Yanick J and Feigenbaum, Annette and Hennekam, Raoul C},

  title = {A Specific IFIH1 Gain-of-function Mutation Causes Singleton-Merten Syndrome},

  journal = {Am J Hum Genet},

  year = {2015},

  volume = {96},

  number = {2},

  pages = {275–-282},

  url = {http://www.sciencedirect.com/science/article/pii/S0002929714005205},

  doi = {10.1016/j.ajhg.2014.12.014}

}

2014 Kehl HG, "Dreidimensionale Angiokardiographie", 2014, Books on Demand (BoD), Norderstedt, ISBN: 978-3-8423-4483-9.
Abstract: Obwohl die Angiokardiographie seit Jahrzehnten eine Standarduntersuchungsmethode in der Kinderkardiologie ist, sind valide Messmethoden für Ventrikelvolumina unter pathologischen Kreislaufverhältnissen nicht verfügbar. Diese Arbeit zeigt ein Verfahren für dreidimensionale Rekonstruktionen von Herzen aus angiokardiographischen Sequenzen mit gleichzeitiger Rotation der Aufnahmeebenen.
Die Rekonstruktion basiert auf einem densitometrisches Verfahren, der sog. gefilterten Rückprojektion, sie benötigt keine Annahmen zu den untersuchten Objekten. Der Algorithmus von Feldkamp wurde an die Geometrie biplaner Rotationsangiographien adaptiert und auch für Angulationen erweitert. Das System wurde mit ausführlichen Untersuchungen an statischen und dynamischen Objekten validiert. Es konnten getrennte, sich überkreuzende Strukturen mit einem Abstand vom 1mm unterschieden werden. Die wahren Volumina von linksventrikulären und rechtsventrikulären Ausgusspräparaten wurden mit konventionellen Messverfahren nach der Flächen-Längen Methode und dreidimensionalen Messverfahren verglichen. Der Vergleich mit dem Bland-Altmann-Verfahren zeigt, dass die Messung mit der Voxel-Auffüll-Methode die besten Ergebnisse erbrachte. Die Abweichung betrug bei linken Ventrikeln +0,9% ± 3,3% (Konfidenzintervall -5,5% bis +7,3%), bei rechten Ventrikeln -2,1% ± 3,4% (-8,7% bis +4,5%). Dynamische Messungen mit einem Kunstherzen ergaben mit der Voxel-Auffüll-Methode eine Abweichung von -0,9% ± 28,9% (-55,7% bis +57,5%).
Die dreidimensionale Angiokardiographie bietet die genaueste Methode zur an- giographischen Volumenmessung. Die dreidimensionale Darstellung kardiale Strukturen erscheint für Katheter-Interventionen besonders sinnvoll.
BibTeX:

@book{Kehl:2014vv,

  author = {Kehl, Hans Gerd},

  title = {Dreidimensionale Angiokardiographie},

  publisher = {Books on Demand (BoD), Norderstedt, ISBN: 978-3-8423-4483-9},

  year = {2014},

  url = {http://amazon.de/o/ASIN/384234483X/}

}

Kehl H, Kiski D, Orth A, Jux C, Malec E and Januszewska K, "Bedeutung von Z-Scores bei angeborenen Herzfehlern", Zeitschrift für Herz-,Thorax- und Gefäßchirurgie., 2014, Vol. 28 : 332-336.
Abstract: Hintergrund
Die Beurteilung, ob Wachstums zeitgerecht verläuft, ist eine wichtige Aufgabe bei der Behandlung von herzkranken Kindern, da Therapieentscheidungen davon abhängig sind. Die in der Pädiatrie übliche Dokumentation von Wachstum mit Perzentilenkurven, ist für kinderkardiologische Belange zu wenig sensitiv.
Methoden
Z-Scores beschreiben in standardisierter Form die Abweichung eines Messwertes von dem zu erwartenden Mittelwert in der Vergleichsgruppe (µ). Z-Scores (Z) werden ermittelt aus dem Quotienten der Differenz des gemessenen Wertes (x) von µ und der zugehörigen Standardabweichung (σ); die Formel lautet: Z= (x-µ)/σ.
Ergebnisse
Eine Reihe von Publikationen liefern die Daten zur Kalkulation von Z-Scores vielfältiger kardiologischer Parameter insbesondere für echokardiographische Untersuchungen. Die Kalkulationen werden inzwischen durch Online-Portale und Smartphone Applikationen unterstützt. Trotz Limitationen der zugrunde liegenden Daten für die Kalkulation von sehr niedrigen und sehr hohen Z-Scores, konnten erste Arbeiten diskriminatorische Bedeutung von Z-Scores für das Outcome verschiedener Therapieverfahren aufzeigen.
Beurteilung
Z-Scores beschreiben in standardisierter Form, wie weit ein Messwert vom Erwartungswert abweicht. Z-Scores ermöglichen eine genauere und universellere Darstellung von Messwerten als Perzentilen.
BibTeX:

@article{Kehl2014,

  author = {Kehl, H.G. and Kiski, D. and Orth, A. and Jux, C. and Malec, E. and Januszewska, K.},

  title = {Bedeutung von Z-Scores bei angeborenen Herzfehlern},

  journal = {Zeitschrift für Herz-,Thorax- und Gefäßchirurgie},

  year = {2014},

  volume = {28},

  pages = {332--336},

  url = {http://dx.doi.org/10.1007/s00398-014-1105-8},

  doi = {10.1007/s00398-014-1105-8}

}

2013 Bayer L, Rössig C, Jux C and Kehl H, "Toxische Kardiomyopathie nach Chemotherapie: frühzeitigere Diagnose mit echokardiographischen Strain Imaging?", Thorac cardiovasc Surg., 2013, Vol. 61(S 02) : P 51.
BibTeX:

@article{Bayer2013,

  author = {Bayer, L and Rössig, C and Jux, C and Kehl, H},

  title = {Toxische Kardiomyopathie nach Chemotherapie: frühzeitigere Diagnose mit echokardiographischen Strain Imaging?},

  journal = {Thorac cardiovasc Surg},

  year = {2013},

  volume = {61},

  number = {S 02},

  pages = {P 51},

  url = {http://dx.doi.org/10.1055/s-0033-1354540},

  doi = {10.1055/s-0033-1354540}

}

Feigenbaum A, Müller C, Yale C, Kleinheinz J, Jezewski P, Kehl HG, MacDougall M, Rutsch F and Hennekam RCM, "Singleton-Merten syndrome: an autosomal dominant disorder with variable expression.", Am J Med Genet A., 2013, Vol. 161A(2) : 360-370.
Ranking: 2,391
Abstract: In 1973, Singleton and Merten described two females with abnormal dentition, unique radiographic changes especially of the hands, and severe calcification and intimal weakening of the aortic arch and valve. Since then three additional cases with similar features have been reported and the diagnosis was suggested in another three individuals. We present an update of one case and the detailed clinical phenotype of six other cases with Singleton-Merten syndrome. The occurrence of the disorder in six members of two families and vertical male-to-male transmission indicate an autosomal dominant pattern of inheritance. Variability in phenotype, also within a single family, is significant. Core manifestations are marked aortic calcification, dental anomalies (delayed eruption and immature root formation of primarily the anterior permanent teeth, and early loss of permanent teeth due to short roots, acute root resorption, high caries, and aggressive alveolar bone loss), osteopenia and acro-osteolysis, and to a lesser extend also glaucoma, psoriasis, muscle weakness, and joint laxity. Additional clinical characteristics described here include particular facial characteristics (high anterior hairline, broad forehead, smooth philtrum, thin upper vermillion) and abnormal joint and muscle ligaments. The cause and pathogenesis of this syndrome remain unknown. © 2013 Wiley Periodicals, Inc.
BibTeX:

@article{Feigenbaum_AmJMedGen_2013,

  author = {Feigenbaum, Annette and Müller, Christine and Yale, Christopher and Kleinheinz, Johannes and Jezewski, Peter and Kehl, Hans Gerd and MacDougall, Mary and Rutsch, Frank and Hennekam, Raoul C M.},

  title = {Singleton-Merten syndrome: an autosomal dominant disorder with variable expression.},

  journal = {Am J Med Genet A},

  year = {2013},

  volume = {161A},

  number = {2},

  pages = {360--370},

  url = {http://dx.doi.org/10.1002/ajmg.a.35732},

  doi = {10.1002/ajmg.a.35732}

}

Kehl H and Schülke C, "Update 2013. Kardiale Computertomographie", Thorac cardiovasc Surg., 2013, Vol. 61(S 02) : U 2.
BibTeX:

@article{Kehl2013,

  author = {Kehl, H and Schülke, C},

  title = {Update 2013. Kardiale Computertomographie},

  journal = {Thorac cardiovasc Surg},

  year = {2013},

  volume = {61},

  number = {S 02},

  pages = {U 2},

  url = {http://dx.doi.org/10.1055/s-0033-1354488},

  doi = {10.1055/s-0033-1354488}

}

Kehl H, Malec E, Januszewska K, Jux C, Tjan T, Stege D, Verfürth A, Singer S and Schülke C, "Herzfehler mit komplexen Gefäßmalformationen: bessere Diagnostik bei kleinen Kindern mit Angio-CT?", Thorac cardiovasc Surg., 2013, Vol. 61(S 02) : V 17.
BibTeX:

@article{Kehl2013a,

  author = {Kehl, H and Malec, E and Januszewska, K and Jux, C and Tjan, TDT and Stege, D and Verfürth, A and Singer, S and Schülke, C},

  title = {Herzfehler mit komplexen Gefäßmalformationen: bessere Diagnostik bei kleinen Kindern mit Angio-CT?},

  journal = {Thorac cardiovasc Surg},

  year = {2013},

  volume = {61},

  number = {S 02},

  pages = {V 17},

  url = {http://dx.doi.org/10.1055/s-0033-1354445},

  doi = {10.1055/s-0033-1354445}

}

Kehl H, Stege D, Verfürth A, Niederstadt T and Köhler M, "Zerebrale Thrombembolie nach kardialer Katheterintervention: erfolgreiche interventionelle Thrombektomie", Thorac cardiovasc Surg., 2013, Vol. 61(S 02) : P 56.
BibTeX:

@article{Kehl2013b,

  author = {Kehl, H and Stege, D and Verfürth, A and Niederstadt, T and Köhler, M},

  title = {Zerebrale Thrombembolie nach kardialer Katheterintervention: erfolgreiche interventionelle Thrombektomie},

  journal = {Thorac cardiovasc Surg},

  year = {2013},

  volume = {61},

  number = {S 02},

  pages = {P 56},

  url = {http://dx.doi.org/10.1055/s-0033-1354545},

  doi = {10.1055/s-0033-1354545}

}

Webinger J, Abdul-Khaliq H, Apitz C, Berger F, Dalla-Pozza R, Eichhorn J, Gravenhorst V, v d Hagen M, Kehl H, Lange M and et al., "Evaluation einer Multizentrischen Studie nach dem Arzneimittelgesetz im Kindesalter (Eudra-CT Nummer: 2009-009871-36)", Thorac cardiovasc Surg., 2013, Vol. 61(S 02) : V 51.
BibTeX:

@article{Webinger2013,

  author = {Webinger, J and Abdul-Khaliq, H and Apitz, C and Berger, F and Dalla-Pozza, R and Eichhorn, J and Gravenhorst, V and v d Hagen, M and Kehl, H and Lange, M and et al.},

  title = {Evaluation einer Multizentrischen Studie nach dem Arzneimittelgesetz im Kindesalter (Eudra-CT Nummer: 2009-009871-36)},

  journal = {Thorac cardiovasc Surg},

  year = {2013},

  volume = {61},

  number = {S 02},

  pages = {V 51},

  url = {http://dx.doi.org/10.1055/s-0033-1354479},

  doi = {10.1055/s-0033-1354479}

}

2012 Singer S, Debus V, Scheld HH and Kehl HG, "Perikardtamponade bei isolierter Perikarditis durch Meningokokken.", Klin Padiatr., 2012, Vol. 212(2) : 90-91.
Ranking: 1,772
BibTeX:

@article{Singer_Klin-Padiatr_2011,

  author = {Singer, S. and Debus, V. and Scheld, H. H. and Kehl, H. G.},

  title = {Perikardtamponade bei isolierter Perikarditis durch Meningokokken.},

  journal = {Klin Padiatr},

  year = {2012},

  volume = {212},

  number = {2},

  pages = {90-91},

  url = {http://dx.doi.org/10.1055/s-0031-1284371},

  doi = {10.1055/s-0031-1284371}

}

2011 Kehl HG, Kiski D, Maintz D, Stege D, Debus V, Kotthoff S and Seifarth H, "Reduced Radiation Dose of Thoracic and Cardiac Dual Source Computertomography with High-Pitch Protocol in Infants and Children", Pediatric Research., 2011, Vol. 70 : 249–249.
BibTeX:

@article{Kehl2011,

  author = {Kehl, H G and Kiski, D and Maintz, D and Stege, D and Debus, V and Kotthoff, S and Seifarth, H},

  title = {Reduced Radiation Dose of Thoracic and Cardiac Dual Source Computertomography with High-Pitch Protocol in Infants and Children},

  journal = {Pediatric Research},

  year = {2011},

  volume = {70},

  pages = {249–249},

  url = {http://dx.doi.org/10.1038/pr.2011.474},

  doi = {10.1038/pr.2011.474}

}

2010 Masjosthusmann K, Sandkoetter J, Kotthoff S, Scheid HH, Rellensmann G, Werner C and Kehl HG, "Erstmanifestation einer Mitochondriopathie als Ursache maligner Herzrhythmusstörungen und kardialer Dekompensation.", Klinische Padiatrie., 2010, Vol. 222 (S 01) : S113-S113.
Abstract: Hintergrund: Mitochondriopathien sind eine Gruppe von Enzymdefekten mit gestörter Phosphorylierung und daraus resultierenden Problemen bei der Energiegewinnung der Zellen. Klinisch stehen meist Muskelfunktionsstörungen im Vordergrund, aber auch andere Organsysteme können betroffen sein. Kasuistik: Wir berichten über ein 3 1/2 Jahre altes Zwillingsmädchen mit einer Infektion der oberen Luftwege das zusätzlich mit ventrikulären Tachkardien und einer rasch progredienten Herzinsuffizienz auffiel. Bei beiden Zwillingskindern bestand ein Kleinwuchs, die übrige Anamnese war unauffällig. Echokardiographisch zeigte sich ein stark dilatiertes Herz mit erheblich reduzierter Pumpleistung. Da die ventrikulären Tachykardien pharmakologisch nur passager therapierbar waren und sich eine progredienter Laktatazidose einstellte, wurde bei dem Kind unter der Arbeitshypothese einer dekompensierten dilatativen Kardiomyopathie 8 Stunden nach Aufnahme ein links-ventrikuläres Unterstützungssystem (LVAD) mit dem Ziel der Überbrückung oder einer späteren Herztransplantation implantiert. Im weiteren Verlauf traten bei dem Kind jedoch thrombembolischen Komplikationen hinzu, die zu einer Hirnmassenblutung mit letalem Ausgang führten.

Durch die intraoperativ entnommenen Herzmuskelbiopsien, konnte post mortem molekulargenetisch die Diagnose einer Mitochondriopathie (Kombinierter Atmungskettendefekt Komplex I/III) gestellt werden. Das Geschwisterkind ist nicht betroffen. Schlussfolgerung: Mitochondriopathien sind bei der Abklärung von kardialen Dekompensationen wichtige Differenzialdiagnosen. Maligne Herzrhythmusstörungen sind in diesem Zusammenhang selten klinisch führende Befunde. Mitochondriopathien können auch erst im Kleinkindalter im Rahmen von Infektionen manifest werden und rasch progredient verlaufen.

BibTeX:

@article{Masjosthusmann2010,

  author = {Masjosthusmann, K. and Sandkoetter, J. and Kotthoff, S. and Scheid, H. H. and Rellensmann, G. and Werner, C. and Kehl, H. G.},

  title = {Erstmanifestation einer Mitochondriopathie als Ursache maligner Herzrhythmusstörungen und kardialer Dekompensation.},

  journal = {Klinische Padiatrie},

  year = {2010},

  volume = {222 (S 01)},

  pages = {S113--S113},

  url = {https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0030-1261645},

  doi = {10.1055/s-0030-1261645}

}

Nowacki M, Viemann D, Stege D, Franssen M, Hoernig-Franz I, Burns JC and Kehl HG, "Therapierefraktäres Kawasaki Syndrom bei einem 7 Wochen alten Säugling - Erfolgreiche Behandlung mit anti-TNF-α und Cyclosporin A.", Klinische Padiatrie., 2010, Vol. 222 (S 01) : S109-S109.
Abstract: Hintergrund: Das Kawasaki Syndrom (KS) ist eine der häufigsten systemischen Vaskulitiden des Kindesalters mit einem hohem Risiko für Beteiligung der Koronararterien besonders bei Säuglingen. Vor allem diese Altersgruppe ist sowohl durch atypische Verläufe mit später Diagnose als auch durch fehlendes Ansprechen auf die Standardtherapie in ca. 20% der Fälle gefährdet. Kasusitik: Ein sieben Wochen alter männlicher Säugling entwickelte Fieber bis 40°C ohne Fokus, die Leukozyten lagen über 18000/µl das CRP über 12mg/dl. Im Verlauf von vier Tagen traten hinzu ein großer cervicaler Lymphknoten, ein flüchtiges Exanthem und eine Dilatation an beiden Koronararterien (z-score &gt;3). Unter dem Verdacht eines Kawasaki Syndrom erfolgte eine Therapie mit Immunglobulinen (IVIG, 2mg/kg) und ASS. Fieber und Entzündungsmaker sistierten danach nur passager. Das CRP stieg wieder bis über 30mg/dl, die Leukozyten bis 28000/µl, eine Thrombozytose bis 1,2Mio/µl, bilaterale conjunktivale Gefäßinjektionen und eine dezente palmare Desquamation traten hinzu obwohl erneut IVIG sowie zusätzlich Corticoide bis 30mg/kg gegeben wurden. Erst nach einer anti-TNF-α-Therapie (Infliximab) normalisierte sich das CRP. Leuko- und Thrombozyten erreichten erst unter zusätzlicher Therapie mit Cyclosporin A nach weiteren 3 Wochen Normwerte. Echo und Koronarangio zeigten 3 Monate nach Erkrankungsbeginn keine Auffälligkeiten. Schlussfolgerung: Das KS ist eine wichtige Differenzialdiagnose bei ungeklärtem Fieber, besonders atypische Verläufe stellen im Säuglingsalter klinisch eine große Herausforderung dar. Fehlendes Ansprechen des KS auf eine initiale Standardtherapie mit IVIG, ASS und/oder Corticoide wird durch eine Wiederholung oder Intensivierung der Standardtherapie nicht immer gebessert. Eine Therapie mit anti-TNF-α und Cyclosporin A sind in solchen Fällen möglicherweise eine bessere Therapieoption.
BibTeX:

@article{Nowacki2010,

  author = {Nowacki, M. and Viemann, D. and Stege, D. and Franssen, M. and Hoernig-Franz, I. and Burns, J. C. and Kehl, H. G.},

  title = {Therapierefraktäres Kawasaki Syndrom bei einem 7 Wochen alten Säugling - Erfolgreiche Behandlung mit anti-TNF-α und Cyclosporin A.},

  journal = {Klinische Padiatrie},

  year = {2010},

  volume = {222 (S 01)},

  pages = {S109--S109},

  url = {https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0030-1261634},

  doi = {10.1055/s-0030-1261634}

}

Stege D, Kiski D, Tjan T, Wolf B, Scheld HH and Kehl HG, "1-year left ventricular assist device (LVAD) experience as bridge to heart transplantation in an infant with Bland-White-Garland syndrome.", Thorac Cardiovasc Surg., 2010, Vol. 58(Suppl 2) : S167-S169.
Ranking: 0,753
Abstract: Left ventricular assist device (LVAD) implantation has become an established therapy in adults as well as in children as a bridge to heart transplantation or to aid myocardial recovery. We describe the first case worldwide of an infant suffering from Bland-White-Garland syndrome successfully treated with a left ventricular assist device (Berlin Heart(R); Excor(R) Pediatric) as a bridge to heart transplantation for a period of more than one year.
BibTeX:

@article{Stege_TCS_2010,

  author = {D. Stege and D. Kiski and T. Tjan and B. Wolf and H. H. Scheld and H. G. Kehl},

  title = {1-year left ventricular assist device (LVAD) experience as bridge to heart transplantation in an infant with Bland-White-Garland syndrome.},

  journal = {Thorac Cardiovasc Surg},

  year = {2010},

  volume = {58},

  number = {Suppl 2},

  pages = {S167--S169},

  url = {http://dx.doi.org/10.1055/s-0029-1240708},

  doi = {10.1055/s-0029-1240708}

}

2009 Gerling C, Rukosujew A, Kehl HG, Tjan TDT, Hoffmeier A, Vogt J, Scheld H-H and Krasemann T, "Do the age of patients with tetralogy of fallot at the time of surgery and the applied surgical technique influence the reoperation rate? a single-center experience.", Herz., 2009, Vol. 34(2) : 155-160.
Ranking: 0,894
Abstract: BACKGROUND AND PURPOSE: Primary repair of tetralogy of Fallot (TOF) has been favored in many centers for years now and results and advantages of this management seem to verify this procedure. The authors wanted to know, if the age at the time of surgery and the surgical techniques had an influence on the long-term results. PATIENTS AND METHODS: Between 1992 and 2003, 124 patients underwent complete repair of TOF at the University Hospital Münster, Germany. Patients were subdivided into two groups based on their age (< 1 year and > 1 year of age). Patients in whom a transannular patch (TAP) was used were compared with those without (NTAP), or in whom a conduit was used. RESULTS: Overall mortality was 8 with an average age of death of 9.53 years (range 0.06-19.77 years). The patients' age at the time of surgery affected their survival as only two cases of death were reported among the group of children < 1 year of age (3.2 whereas eight patients were older (12.9 p = 0.0483). Six patients died within the first 30 days post surgery. Reoperation had to be performed in 21 cases, 13 (61.9 of these patients were < 1 year of age at the time of surgery, eight were older (38.1. A TAP, NTAP or conduit treatment did not show significant differences in long-term survival or freedom from reoperation. CONCLUSION: Early repair of TOF within the 1st year of life can be recommended, because mortality is lower than in patients treated at a higher age. There seems no significant difference in the reintervention rate between patients treated within the 1st year of life or later.
BibTeX:

@article{Gerling_TOF_Herz_2009,

  author = {Christoph Gerling and Andreas Rukosujew and Hans Gerd Kehl and Tonny D T Tjan and Andreas Hoffmeier and Johannes Vogt and Hans-Heinrich Scheld and Thomas Krasemann},

  title = {Do the age of patients with tetralogy of fallot at the time of surgery and the applied surgical technique influence the reoperation rate? a single-center experience.},

  journal = {Herz},

  year = {2009},

  volume = {34},

  number = {2},

  pages = {155--160},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/19370333},

  doi = {10.1007/s00059-009-3169-x}

}

Neumann TE, Allanson J, Kavamura I, Kerr B, Neri G, Noonan J, Cordeddu V, Gibson K, Tzschach A, Krüger G, Hoeltzenbein M, Goecke TO, Kehl HG, Albrecht B, Luczak K, Sasiadek MM, Musante L, Laurie R, Peters H, Tartaglia M, Zenker M and Kalscheuer V, "Multiple giant cell lesions in patients with Noonan syndrome and cardio-facio-cutaneous syndrome.", Eur J Hum Genet., 2009, Vol. 17(4) : 420-425.
Ranking: 3,564
Abstract: Noonan syndrome (NS) and cardio-facio-cutaneous syndrome (CFCS) are related developmental disorders caused by mutations in genes encoding various components of the RAS-MAPK signaling cascade. NS is associated with mutations in the genes PTPN11, SOS1, RAF1, or KRAS, whereas CFCS can be caused by mutations in BRAF, MEK1, MEK2, or KRAS. The NS phenotype is rarely accompanied by multiple giant cell lesions (MGCL) of the jaw (Noonan-like/MGCL syndrome (NL/MGCLS)). PTPN11 mutations are the only genetic abnormalities reported so far in some patients with NL/MGCLS and in one individual with LEOPARD syndrome and MGCL. In a cohort of 75 NS patients previously tested negative for mutations in PTPN11 and KRAS, we detected SOS1 mutations in 11 individuals, four of whom had MGCL. To explore further the relevance of aberrant RAS-MAPK signaling in syndromic MGCL, we analyzed the established genes causing CFCS in three subjects with MGCL associated with a phenotype fitting CFCS. Mutations in BRAF or MEK1 were identified in these patients. All mutations detected in these seven patients with syndromic MGCL had previously been described in NS or CFCS without apparent MGCL. This study demonstrates that MGCL may occur in NS and CFCS with various underlying genetic alterations and no obvious genotype-phenotype correlation. This suggests that dysregulation of the RAS-MAPK pathway represents the common and basic molecular event predisposing to giant cell lesion formation in patients with NS and CFCS rather than specific mutation effects.
BibTeX:

@article{Neumann_Noonan_EJHG_2009,

  author = {Thomas E Neumann and Judith Allanson and Ines Kavamura and Bronwyn Kerr and Giovanni Neri and Jacqueline Noonan and Viviana Cordeddu and Kate Gibson and Andreas Tzschach and Gabriele Krüger and Maria Hoeltzenbein and Timm O Goecke and Hans Gerd Kehl and Beate Albrecht and Klaudiusz Luczak and Maria M Sasiadek and Luciana Musante and Rohan Laurie and Hartmut Peters and Marco Tartaglia and Martin Zenker and Vera Kalscheuer},

  title = {Multiple giant cell lesions in patients with Noonan syndrome and cardio-facio-cutaneous syndrome.},

  journal = {Eur J Hum Genet},

  year = {2009},

  volume = {17},

  number = {4},

  pages = {420--425},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/18854871},

  doi = {10.1038/ejhg.2008.188}

}

2008 Debus V, Kotthoff S, Grüber L, Kehl HG, Vogt J, Wedekind H, Breithardt G and Schulze-Bahr E, "Klinischer Verlauf von 83 genotypisierten Kindern mit kongenitalem Long-QT-Syndom", Clinical Research in Cardiology., 2008, Vol. 97 : 681.
Abstract: Einführung: Das Long-QT-Syndom (LQTS) ist eine genetisch determi- nierte, primär elektrisch bedingte, kardiale Erkrankung. Sie prädis- poniert zu Synkopen, ventrikulären Tachyarrhythmien (TdP/VT) und plötzlichem Herztod (SCD).
Ergebnisse: Wir untersuchten 83 Kinder (39 männlich, 44 weiblich) zwischen 0–18 Jahren mit der Erstdiagnose eines LQTS. Die bei allen Kindern durchgeführte molekulargenetische Genotypisierung ergab ein LQT1 bei 42%, LQT2 bei 34%, LQT3 bei 13% und ein Jervell-Lan- ge-Nielsen-Syndrom (JLNS) bei 11%.
59 Kinder (71%) waren zum Zeitpunkt der Diagnose symptoma- tisch, meist mit Synkopen (42), gefolgt von TdP/VT (27) und überleb- tem Herztod (17). Die höchsten Raten symptomatischer Patienten fie- len auf LQT3 (82%) und JLNS (89%), gefolgt von LQT2 (75%) und LQT1 (60%). 9 Kinder (meist LQT2) verstarben initial (SCD) im Alter zwischen 3 und 17 Jahren, dabei hatten 78% eine QTc <500 ms und 33% < 600 ms.
69 von 74 Kindern (93%) erhielten eine Therapie (b-Blocker 60, Mexiletin 4, Schrittmacher 3, ICD 9). Während des mittleren Follow- up von 6±4,8 Jahren entwickelten 31% der Kinder erneut Symptome, meist Synkopen (82%), seltener VT/TdP (45%). Ein überlebter Herz- tod bzw. adäquate ICD-Therapie wurde dreimal (14%), ein SCD ein- mal bei einem Säugling mit LQT3 trotz b-Blockermedikation beobach- tet.
Patienten mit LQT3 und JLNS wiesen deutlich verlängerte QTc-Zei- ten meist < 500 ms auf, wurden früher symptomatisch und hatten die höchste Rate an kardialen Ereignissen im Verlauf.
BibTeX:

@article{Arrythmie_outcome_Debus_CRiC_2008,

  author = {V. Debus and S. Kotthoff and L. Grüber and H. G. Kehl and J. Vogt and H. Wedekind and G. Breithardt and E. Schulze- Bahr},

  title = {Klinischer Verlauf von 83 genotypisierten Kindern mit kongenitalem Long-QT-Syndom},

  journal = {Clinical Research in Cardiology},

  year = {2008},

  volume = {97},

  pages = {681},

  url = {http://www.kinderkardiologie.org/Tagungen/dgpk40/AbstractBuch2008.pdf},

  doi = {10.1007/s00392-008-0702-5}

}

2007 Kehl H, Schmidt C, Tjan T, Stege D, Vogt J and Zahn P, "Fallbeispiel: Akute kardiale Dekompensation 12 Jahre nach Korrektur-Operation eines angeborenen Herzfehlers, S. 99--100 In: Seeberger M (Ed.), Zerkowski HR (Ed.) Die Echokardiographie im perioperativen und intensiv-medizinischen Bereich", 2007, Springer, ISBN: 978-3-7985-1607-6.
BibTeX:

@book{Kehl_2007,

  author = {Kehl, HG and Schmidt, Ch and Tjan, TDT and Stege, D and Vogt, J and Zahn, PK},

  title = {Fallbeispiel: Akute kardiale Dekompensation 12 Jahre nach Korrektur-Operation eines angeborenen Herzfehlers, S. 99--100 In: Seeberger M (Ed.), Zerkowski HR (Ed.) Die Echokardiographie im perioperativen und intensiv-medizinischen Bereich},

  publisher = {Springer, ISBN: 978-3-7985-1607-6},

  year = {2007},

  url = {http://link.springer.com/content/pdf/10.1007%2F978-3-7985-1608-3_13.pdf}

}

Kehl H, Schmidt C, Tjan T, Scheld H, Van Aken H and Zahn P, "Kongenitalen Herzvitien im Erwachsenenalter: Abklärung und perioperatives Management für notfallmässige und elektive nicht-kardiochirurgische Operationen, S. 101-121 n: Seeberger M (Ed.), Zerkowski HR (Ed.) Die Echokardiographie im perioperativen und intensiv-medizinischen Bereich", 2007, Springer, ISBN: 978-3-7985-1607-6.
BibTeX:

@book{Kehl_2008,

  author = {Kehl, HG and Schmidt, C and Tjan, TDT and Scheld, HH and Van Aken, HK and Zahn, PK},

  title = {Kongenitalen Herzvitien im Erwachsenenalter: Abklärung und perioperatives Management für notfallmässige und elektive nicht-kardiochirurgische Operationen, S. 101-121 n: Seeberger M (Ed.), Zerkowski HR (Ed.) Die Echokardiographie im perioperativen und intensiv-medizinischen Bereich},

  publisher = {Springer, ISBN: 978-3-7985-1607-6},

  year = {2007},

  url = {http://link.springer.com/content/pdf/10.1007%2F978-3-7985-1608-3_14.pdf}

}

Kehl HG, "Dreidimensionale Angiocardiographie. Dreidimensionale Rekonstruktionen des Herzens aus Rotations-Cinecardiographien. Entwicklung und Validierung der Methoden sowie erste klinische Anwendungen.". Habilitationsschrift, Westfälische Wilhelms Universität Münster., 2007,
Abstract: Obwohl die Angiokardiographie seit Jahrzehnten eine Standarduntersuchungsmethode in der Kinderkardiologie ist, sind valide Messmethoden für Ventrikelvolumina unter pathologischen Kreislaufverhältnissen nicht verfügbar. Das Ziel dieser Arbeit war, mit angiokardiographischen Sequenzen eine dreidimensionale Darstellung von Herzen zu erreichen und unabhängig von Aufnahmewinkeln und genauere Volumenbestimmungen von linken sowie rechten Ventrikeln zu ermöglichen.
Die Rekonstruktion basiert auf einem densitometrisches Verfahren, der sog. gefilterten Rückprojektion, sie benötigt keine Annahmen zu den untersuchten Objekten. Der Algorithmus von FELDKAMP et al. publizierte Algorithmus [65] wurde am Fraunhofer Institut für graphische Datenverarbeitung, Darmstadt, an die Geometrie biplaner Rotationsangiographien adaptiert und später auch für Angulationen erweitert [108, 203]. Das System wurde mit ausführlichen Untersuchungen an statischen und dynamischen Objekten validiert. Es konnten getrennte, sich überkreuzende Strukturen mit einem Abstand vom 1mm unterschieden werden. Die wahren Volumina von 21 linksventrikulären und 22 rechtsventrikulären Ausgusspräparaten wurden mit konventionellen Messverfahren nach der Flächen-Längen Methode und dreidimensionalen Messverfahren verglichen.
Der Vergleich mit dem BLAND-ALTMANN-Verfahren zeigt, dass die Messung mit der Voxel-Auffüll-Methode die besten Ergebnisse erbrachte. Die Abweichung betrug bei linken Ventrikeln +0,9% +/- 3,3 (Konfidenzintervall [CI] -5,5% bis +7,3%), bei rechten Ventrikeln -2,1% +/- 3,4% (CI -8,7% bis +4,5%). Die beste zweidimensionale Flächen-Längen-Methode bei linken Ventrikeln (monoplan 30RAO) zeigte eine Abweichung von +13,3%  9,4% (CI -5,2% bis +31,8%), bei rechten Ventrikel (monoplan 0RAO) eine Abweichung von +8,5% +/-27,1% (CI -44,5% bis +61,6%). Dynamische Messungen mit MEDOSr Kunstherzen ergaben mit der Voxel-Auffüll-Methode eine Abweichung von -0,9% +/- 28,9% (CI -55,7%
bis +57,5%).
Die dreidimensionale Angiokardiographie bietet die genaueste Methode zur angiographischen Volumenmessung. Kardiale Strukturen dreidimensional darstellen zu können, erscheint für Katheter-Interventionen besonders sinnvoll.
BibTeX:

@mastersthesis{Kehl_Habil,

  author = {Kehl, Hans Gerd},

  title = {Dreidimensionale Angiocardiographie. Dreidimensionale Rekonstruktionen des Herzens aus Rotations-Cinecardiographien. Entwicklung und Validierung der Methoden sowie erste klinische Anwendungen.},

  school = {Habilitationsschrift, Westfälische Wilhelms Universität Münster},

  year = {2007},

  url = {/Users/hgk/Angio_3D/Arbeit/Master1.pdf}

}

Kranz C, Jungeblut C, Denecke J, Erlekotte A, Sohlbach C, Debus V, Kehl HG, Harms E, Reith A, Reichel S, Grobe H, Hammersen G, Schwarzer U and Marquardt T, "A defect in dolichol phosphate biosynthesis causes a new inherited disorder with death in early infancy.", Am J Hum Genet., 2007, Vol. 80(3) : 433-440.
Ranking: 11,092
Abstract: The following study describes the discovery of a new inherited metabolic disorder, dolichol kinase (DK1) deficiency. DK1 is responsible for the final step of the de novo biosynthesis of dolichol phosphate. Dolichol phosphate is involved in several glycosylation reactions, such as N-glycosylation, glycosylphosphatidylinositol (GPI)-anchor biosynthesis, and C- and O-mannosylation. We identified four patients who were homozygous for one of two mutations (c.295T-->A [99Cys-->Ser] or c.1322A-->C [441Tyr-->Ser]) in the corresponding hDK1 gene. The residual activity of mutant DK1 was 24% when compared with control cells. The mutated alleles failed to complement the temperature-sensitive phenotype of DK1-deficient yeast cells, whereas the wild-type allele restored the normal growth phenotype. Affected patients present with a very severe clinical phenotype, with death in early infancy. Two of the patients died from dilative cardiomyopathy.
BibTeX:

@article{Kranz_Dolichol_AmJHumGen2007,

  author = {Christian Kranz and Christoph Jungeblut and Jonas Denecke and Anne Erlekotte and Christina Sohlbach and Volker Debus and Hans Gerd Kehl and Erik Harms and Anna Reith and Sonja Reichel and Helfried Grobe and Gerhard Hammersen and Ulrich Schwarzer and Thorsten Marquardt},

  title = {A defect in dolichol phosphate biosynthesis causes a new inherited disorder with death in early infancy.},

  journal = {Am J Hum Genet},

  year = {2007},

  volume = {80},

  number = {3},

  pages = {433--440},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/17273964},

  doi = {10.1086/512130}

}

Kehl HG, Maintz D, Fischbach R, Kotthoff S, Tjan T, Zahn P and Wichter T, "Cor triatriatum sinistrum combined with supracardiac anomalous pulmonary venous return in an adult causing arrhythmogenic heart failure", Clinical Research in Cardiology., 2007, Vol. 96(10) : 752-754.
Ranking: 1,442
Abstract: A 41-year-old female developed progressing dyspnea on exertion resulting within 6 days in orthopnoea, hepatomegalia, and peripheral edema. Her ECG showed permanent atrial fibrillation, her chest X-ray demostrated cardiomegaly (cardiothoracic index > 0.7). Amiodarone was initiated for rate control and as an attempt for cardioversion. However, this was complicated by QT prolongation with proarrhythmic torsade de pointes, also requiring resuscitation due to cardiac arrest. After this incident the patient was transferred to our institution for implantation of a cardiac defibrillator. The further history of the patient was uneventful including two pregnancies. Echocardiography showed an enlarged right heart with a hypokinetic, hypertrophic right ventricle, a dilated pulmonary artery but normal left ventricular function. A membrane in the left atrium (LA) was suspected but the patient’s adiposis limited a more detailed assessment. Cine magnetic resonance images using steady-state, free-precession and phase-contrast sequences in transverse, short-axis, and oblique planes surprisingly demonstrated a cor triatriatum combined with a supracardiac total anomalous of pulmonary venous return without an atrial septum defect (Figure 1A and 1B). The relation of pulmonary to systemic flow was measured as 2.4:1. These findings and a suspected secondary pulmonary hypertension were confirmed by cardiac catheterization (Figure 2A and 2B) and electrocardiogram-gated multidetector computed tomography (Figure 3). The pulmonary arterial pressure was as high as systemic arterial pressure, the relation of pulmonary to systemic vascular resistance was calculated as 0.44:1, the gradient across the membrane in LA was more than 30 mm Hg. The resection of the LA membrane, ligation of the vena verticalis and a perioperative control of pulmonary hypertension with nitrite oxide and sildenafil enabled to reconvert the patient in NYHA class 1.
BibTeX:

@article{Lungenvenen_Anomalie_OP_Kehl_Cric_2007,

  author = {Hans Gerd Kehl and David Maintz and Roman Fischbach and Stefan Kotthoff and Tonny Tjan and Peter Zahn and Thomas Wichter},

  title = {Cor triatriatum sinistrum combined with supracardiac anomalous pulmonary venous return in an adult causing arrhythmogenic heart failure},

  journal = {Clinical Research in Cardiology},

  year = {2007},

  volume = {96},

  number = {10},

  pages = {752--754},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/17701367},

  doi = {10.1007/s00392-007-0554-4}

}

Spieker T, Krasemann T, Hoffmeier A, Buning A, Debus V, Kehl H, Drees G, Eltze E, Scheld HH and Tjan TDT, "Heart transplantation for isolated noncompaction of the left ventricle in an infant.", Thorac Cardiovasc Surg., 2007, Vol. 55(2) : 127-129.
Ranking: 0,741
Abstract: Isolated noncompaction of the left ventricular myocardium is a rare cardiomyopathy typically showing a "spongy" myocardium on ultrasound. We report on the ultrasonic and pathomorphological characteristics of an infant who, at the age of 40 days, was treated by heart transplantation for isolated noncompaction. Noncompaction should be suspected in newborns with otherwise unexplained cardiomyopathy and a "spongy" left ventricle. However, ultrasonic and pathological findings may be much less pronounced at this age than later in life.
BibTeX:

@article{Spieker_ThoracCardiovascSurg_2007,

  author = {T. Spieker and T. Krasemann and A. Hoffmeier and A. Buning and V. Debus and H. Kehl and G. Drees and E. Eltze and H. H. Scheld and T. D T Tjan},

  title = {Heart transplantation for isolated noncompaction of the left ventricle in an infant.},

  journal = {Thorac Cardiovasc Surg},

  year = {2007},

  volume = {55},

  number = {2},

  pages = {127--129},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/17377870},

  doi = {10.1055/s-2006-924619}

}

2006 Kehl HG, Schmidt C, Tjan T, Stege D, Vogt J and Zahn P, "Akute kardiale Dekompensation 12 Jahre nach Korrektur-Operation eines angeborenen Herzfehlers", Intensivmedizin und Notfallmedizin., 2006, Vol. 43(3) : 241-242.
Abstract: Kasuistik: Ein 17-jähriger Patient erleidet beim Freizeit-Walking eine Synkope. Ein herbeigerufener Notarzt stellt bei dem somnolenten Patienten eine monomorphe ventrikuläre Tachykardie mit HF von ca. 210/min fest. Durch sofortige Kardioversion wird ein Sinusrhythmus mit breiten QRS-Komplexen und häufigen monomorphen ventrikulären Extrasystolen erreicht. Daraufhin erfolgt eine einmalige intravenöse Applikation von 300 mg Amiodaron (4 mg/kg). Trotz verbesserter Bewusstseinslage wird noch vor dem Transport zur Klinik wegen fortbestehender arterieller Hypotonie mit systolischen Werten < 90 mm Hg eine orotracheale Intubation und maschinelle Beatmung vorgenommen (Die Indikation zur Intubation sollte anders dargestellt werden z.B.: Aufgrund einer eingeschränkten Bewusstseinslage und instabiler hämodynamischer Verhältnisse (systolische Werte < 90 mmHg). Eine alte mediane Sternotomienarbe lässt an eine vorangegangene Operation denken. Die begleitende Freundin bestätigt eine mehr als 10 Jahre zurückliegende Herzoperation wegen eines angeborenen Herzfehlers. In der Klinik kann an Hand vorhandener Unterlagen als Vitium cordis eine Pulmonalatresie mit VSD in Erfahrung gebracht werden. Operativ war im Neonatalalter zunächst ein zentraler aorto-pulmonaler Shunt angelegt worden; im Alter von 1 Jahr erfolgte eine erste Korrektur-OP mit VSD-Verschluß und Implantation eines klappenlosen Conduits vom rechten Ventrikel zur Pulmonalbifurkation, der mit 5 Jahren gegen einen größeren klappentragenden Homograft ausgetauscht wurde. Die Untersuchungsqualität der transthorakalen Echokardiographie ist sehr limitiert, die TEE ermöglicht eine bessere Darstellung. Der VSD mit ehemals überreitender Aorta zeigt sich durch den Patch gut verschlossen, die Aortenklappe nur gering insuffizient (s. Abb. 1 und Video 1). Auffallend ist eine ausgeprägte Hypertrophie des rechten Ventrikels (s. Abb. 2 und Video 2) und der nur schlecht einsehbare rechtsventrikuläre Ausflusstrakt (s. Abb. 3) über dem im konventionellen Doppler ein Gradient von 45 mm Hg bestimmbar ist (s. Abb. 4).
BibTeX:

@article{Kehl_Kasuistik_Intensivmed2006,

  author = {H. G. Kehl and Ch. Schmidt and T.D.T. Tjan and D. Stege and J. Vogt and P.K. Zahn},

  title = {Akute kardiale Dekompensation 12 Jahre nach Korrektur-Operation eines angeborenen Herzfehlers},

  journal = {Intensivmedizin und Notfallmedizin},

  year = {2006},

  volume = {43},

  number = {3},

  pages = {241--242},

  url = {http://www.springerlink.com/content/v016h013704x165n/},

  doi = {10.1007/s00390-006-0699-9}

}

Kehl HG, Schmidt C, Tjan T, Scheld H, van Aken H and Zahn P, "Kongenitale Herzvitien im Erwachsenenalter. Perioperative Echokardiographie für notfallmäßige und elektive nicht-kardiochirurgische Operationen", Intensivmedizin und Notfallmedizin., 2006, Vol. 43 : 310-330.
Abstract: Congenital heart disease (CHD) occurs in 0.4-0.9% of newborn infants. In Germany approximately 6500 infants per year are born with a CHD and approximately 5500 children can expect to live into adulthood. Therefore, in the near future an increasing number of adults with CHD will be treated by anaesthesiologists for cardiac and noncardiac surgeries. Echocardiography is the mainstay in diagnosis of most patients with CHD and useful for monitoring cardiac function. During surgery, transoesophageal echocardiography can provide real-time assessment of ventricular function, intracardiac shunting, preload and valve function. After a brief review of the epidemiology and pathophysiology of different CHD defects, this article focuses on the role of echocardiography for cardiac monitoring of adult patients with CHD. The different uses of transthoracic and transoesophageal echocardiography are also discussed.
BibTeX:

@article{Kehl_Review_Intensivmed2006,

  author = {H. G. Kehl and Ch. Schmidt and T.D.T. Tjan and H.H. Scheld and H.K. van Aken and P.K. Zahn},

  title = {Kongenitale Herzvitien im Erwachsenenalter. Perioperative Echokardiographie für notfallmäßige und elektive nicht-kardiochirurgische Operationen},

  journal = {Intensivmedizin und Notfallmedizin},

  year = {2006},

  volume = {43},

  pages = {310--330},

  url = {http://www.springerlink.com/content/034x314856344n11/},

  doi = {10.1007/s00390-006-0717-y}

}

2005 Hoffmeier A, Etz C, Schmid C, Debus V, Kehl HG, Ozgun M, Maintz D, Spieker T, Franzius C, Drees G, Rothenburger M, Tjan TDT, Löher A and Scheld HH, "Images in cardiovascular medicine. Cardiac transplantation for giant sarcoma of the left ventricle.", Circulation., 2005, Vol. 112(14) : e247-e249.
Ranking: 11,632
Abstract: An 8-year-old boy with a growing left ventricular mass underwent cardiac surgery for tumor resection. Radical resection seemed impossible, and thus, only a large biopsy sample was taken with a concomitant patch closure of the left ventricular defect. Histological examination revealed a rare liposarcoma. The patient was discharged without further therapy. Five months later, the patient was transferred to our hospital because his clinical condition had worsened to New York Heart Association class IV, and a recurrent left ventricular tumor became evident. MRI (Figures 1, 2, and 3) and echocardiography (Figure 4) showed an inoperable tumor almost totally obstructing the left ventricular cavum, with excessive infiltration of the myocardium. According to T1- and T2-weighted images with fat saturation, there were only small streaky areas of fatty tissue within the tumor. Left ventricular function was significantly impaired (Movie I and Movie II).
BibTeX:

@article{Hoffmeier_Cardiac-sarcoma_Circ2005,

  author = {A. Hoffmeier and C. Etz and C. Schmid and V. Debus and H. G. Kehl and M. Ozgun and D. Maintz and T. Spieker and C. Franzius and G. Drees and M. Rothenburger and T. D T Tjan and A. Löher and H. H. Scheld},

  title = {Images in cardiovascular medicine. Cardiac transplantation for giant sarcoma of the left ventricle.},

  journal = {Circulation},

  year = {2005},

  volume = {112},

  number = {14},

  pages = {e247--e249},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/16203918},

  doi = {10.1161/CIRCULATIONAHA.104.500264}

}

Kehl HG, Debus V, Stege D, Tjan TDT, Vogt J and Schulze-Bahr E, "Vorhofflattern in der Frühphase nach orthotoper Herztransplantation", Herzschrittmacherther Elektrophysiol., 2005, Vol. 16(4) : 270-273.
Abstract: A few weeks after orthotopic heart transplantation, a male adolescent developed atrial arrhythmias of the donor heart due to an atypical recipient atrial flutter with a recipient-to-donor transatrial conduction resulting in an absolute arrhythmia. Under medication with propafenone, the atrial flutter of the donor heart could be terminated with cardioversion.
BibTeX:

@article{Kehl_Vorhofflattern_HTX_Herzschrittm2005,

  author = {H. G. Kehl and V. Debus and D. Stege and T. D T Tjan and J. Vogt and E. Schulze-Bahr},

  title = {Vorhofflattern in der Frühphase nach orthotoper Herztransplantation},

  journal = {Herzschrittmacherther Elektrophysiol},

  year = {2005},

  volume = {16},

  number = {4},

  pages = {270--273},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/16362733},

  doi = {10.1007/s00399-005-0494-5}

}

Krasemann T, Fenge H, Kehl HG, Rukosujew A, Schmid C, Scheld HH, Tjan TDT and Vogt J, "A decade of staged Norwood palliation in hypoplastic left heart syndrome in a midsized cardiosurgical center.", Pediatr Cardiol., 2005, Vol. 26(6) : 751-755.
Ranking: 0,986
Abstract: Hypoplastic left heart syndrome (HLHS) is a challenge for the pediatric cardiologist and the surgeon. It is generally assumed that the postoperative outcome after surgery for congenital heart disease is influenced by the institutional size. We present the results of 43 patients with true HLHS (situs solitus and atrioventricular and ventriculoarterial concordance) referred for operation between 1992 and 2002 in our center. Two children had atrioseptostomy: one died soon after the operation, and the other one was transplanted successfully but died at the age of 6 months following acute rejection. The remaining 41 underwent Norwood I palliation, 21 stage II palliation, and 10 stage III palliation. Early mortality was 29% after stage I operation, 4.7% after stage II palliation, and 0% after stage III operation. Overall mortality was 39% after stage I, 9.5% after stage II, and 10% after stage III operation. Low birth weight was associated with a higher mortality (p < 0.05). Mortality declined with increasing experience, comparable to the results of very large cardiosurgical centers with many more patients. The quality of surgery and perioperative management in smaller pediatric cardiosurgical centers can reach the level of very large centers.
BibTeX:

@article{Krasemann_Norwood-MS_Pediatr_Cardiol2005a,

  author = {T. Krasemann and H. Fenge and H. G. Kehl and A. Rukosujew and C. Schmid and H. H. Scheld and T. D T Tjan and J. Vogt},

  title = {A decade of staged Norwood palliation in hypoplastic left heart syndrome in a midsized cardiosurgical center.},

  journal = {Pediatr Cardiol},

  year = {2005},

  volume = {26},

  number = {6},

  pages = {751--755},

  url = {http://www.springerlink.com/content/j8ww035190014633/},

  doi = {10.1007/s00246-005-0908-5}

}

Krasemann T, Kotthoff S, Kehl HG, Debus V, Tjan TDT, Schmid C, Vogt J and Scheld HH, "Cardiac transplantation in neonatal Marfan syndrome -- a life-saving approach.", Thorac Cardiovasc Surg., 2005, Vol. 53 Suppl 2 : S146-S148.
Ranking: 0,935
Abstract: Marfan syndrome is a connective tissue disease with typical clinical signs and cardiac involvement. Its appearance in the neonatal period has a bad prognosis due to incompetence of all cardiac valves with subsequent congestive heart failure. Conservative management usually fails, the children die during their first year of life. We report on a girl with neonatal Marfan syndrome who suffered from regurgitance of all cardiac valves, enlarged ventricles, and dilated great arteries. She was NYHA class IV. At the age of six months she underwent heart transplantation. To prevent aneurysm formation and dissection of the great vessels, the whole aortic arch and pulmonary trunk were replaced as well.
BibTeX:

@article{Krasemann-Marfan2005,

  author = {T. Krasemann and S. Kotthoff and H. G. Kehl and V. Debus and T. D T Tjan and C. Schmid and J. Vogt and H. H. Scheld},

  title = {Cardiac transplantation in neonatal Marfan syndrome -- a life-saving approach.},

  journal = {Thorac Cardiovasc Surg},

  year = {2005},

  volume = {53 Suppl 2},

  pages = {S146--S148},

  url = {http://www.thieme-connect.de/ejournals/abstract/thoracic/doi/10.1055/s-2004-830455},

  doi = {10.1055/s-2004-830455}

}

Rellensmann G, Krasemann T and Kehl HG, "Should all stage-one Norwood patients receive a prolonged period of postoperative mechanical circulatory support?", Ann Thorac Surg., 2005, Vol. 79(3) : 1098-1099.
Ranking: 2,229
Abstract: Ungerleider and colleagues [1] presented promising data on their concept of routinely placing neonates with hypoplastic left heart syndrome (HLHS) on a ventricular assist device (VAD) postoperatively after a stage-one Norwood procedure. The authors suggested that prophylactic use rather than rescue use of a VAD or extracorporeal membrane oxygenation (ECMO) might result in a better outcome after this procedure. To prove this hypothesis, two vital questions need to be answered: What is the expected early postoperative morbidity and mortality using conventional therapy? What are the additional risks of an extended period of mechanical circulatory support in neonates, in particular concerning the neurologic outcome? The authors could answer the first question by comparing their results in patients with HLHS before use of the VAD with published results from major centers [2] and [3]. Taking into account a risk stratification for HLHS as reported in the February 2004 issue of The Annals by Checchia and co-workers [4] would facilitate comparison of results between centers. As for the second question, on the basis of our experience, we share the concern of Dr Austin [1] that proper anticoagulation is still an unsolved issue in neonates and young infants. It is noteworthy that in the UK Collaborative ECMO Trial [5], 29% of patients in the ECMO arm showed abnormalities on cranial ultrasound after trial entry. Other groups [6] and [7] found a 15% incidence of intracranial hemorrhage or infarction after neonatal ECMO. In 2002, the overall mortality rate for rescue ECMO in patients with congenital heart disease was 62%; it was 73% in the stage-one Norwood subgroup [8]. The number of reports on neonatal VAD use is small, but hemostasis-related complications are again a major concern [9] and [10]. Hence, serious side effects of prolonged mechanical circulatory support must be anticipated. The report of Ungerleider and associates lacked detailed data on the morphological and functional status of the patients. Normal perioperative head ultrasound examinations do not exclude infarctions and later atrophy of the brain. Eight of 13 patients in their study received neurologic follow-up, which corresponds to a drop-out rate of 38% in that subgroup, which was not explained. There was no control group. In light of this scenario, it seems premature to routinely place all patients who undergo a stage-one Norwood operation on a prolonged period of VAD use, as only a subgroup will need this expensive and potentially harmful therapy. It is unclear whether the expected benefit for that subgroup justifies the additional risk to infants in hemodynamically stable condition who could be expected to have a reasonably good outcome without a VAD [2] and [3]. Small numbers, the apparent lack of background details, and the design of the study lead us to strongly endorse the suggestion of Ungerleider and associates to collect and report data “in a randomized, comparative manner.” We congratulate Ungerleider and colleagues on their excellent results and believe absolutely that this promising approach merits cautiously conducted controlled studies with sufficient numbers of Norwood procedures. In the meantime, it would be good practice not to use treatment strategies that have not yet been validated.
BibTeX:

@article{Rellensmann_AnnThoracSurg_2005,

  author = {Georg Rellensmann and Thomas Krasemann and Hans Gerd Kehl},

  title = {Should all stage-one Norwood patients receive a prolonged period of postoperative mechanical circulatory support?},

  journal = {Ann Thorac Surg},

  year = {2005},

  volume = {79},

  number = {3},

  pages = {1098--1099},

  url = {http://www.sciencedirect.com/science/article/B6T11-4FJCW90-3P/2/25864139d95e8891a719b8e9518c1aac},

  doi = {10.1016/j.athoracsur.2004.02.149}

}

Schmidt C, Theilmeier G, Aken HV, Flottmann C, Wirtz SP, Kehl HG, Hoffmeier A and Berendes E, "Effective systolic orifice area of the aortic valve: implications for Doppler echocardiographic cardiac output determinations.", Acta Anaesthesiol Scand., 2005, Vol. 49(8) : 1135-1141.
Ranking: 1,835
Abstract: BACKGROUND: Substantial research using echocardiography has established that stroke volume (SV) or cardiac output (CO) can be measured non-invasively at the level of the aortic valve (AV) with high accuracy. Stroke volume is the product of the velocity time integral occurring at the sampling site and the effective systolic AV orifice area (AVOAeff). Nevertheless, a generally accepted method for the determination of AVOAeff is still lacking. METHODS: Aortic valve OAeff was measured in 228 consecutive patients scheduled for coronary artery surgery. Two widely adopted methods were applied to approximate the constantly changing orifice area of the AV: (1) the circular orifice model (AVOA-CM), and (2) the triangular orifice model (AVOA-TM). Aortic valve OA-CM assumes the shape of a circle as an appropriately time averaged geometrical model, and AVOA-TM takes the shape of an equilateral triangle for granted. RESULTS: The AV was easily imaged by echocardiography in both short- and long-axis views in all patients. Relying on AVOA-CM, AVOAeff was 3.49+/-0.77 cm2. AVOA-TM estimates were 2.80+/-0.55 cm2 (mean+/-SD). The results did not agree (bias analysis). CONCLUSIONS: The echocardiographic measurement of SV or CO at the level of the AV has to be reconsidered.
BibTeX:

@article{Schmidt_CO_Acta_Anest_Scand2005,

  author = {C. Schmidt and G. Theilmeier and H. Van Aken and C. Flottmann and S. P. Wirtz and H. G. Kehl and A. Hoffmeier and E. Berendes},

  title = {Effective systolic orifice area of the aortic valve: implications for Doppler echocardiographic cardiac output determinations.},

  journal = {Acta Anaesthesiol Scand},

  year = {2005},

  volume = {49},

  number = {8},

  pages = {1135--1141},

  url = {http://dx.doi.org/10.1111/j.1399-6576.2005.00763.x},

  doi = {10.1111/j.1399-6576.2005.00763.x}

}

2004 Kehl HG, Haverkamp W, Rellensmann G, Yelbuz TM, Krasemann T, Vogt J and Schulze-Bahr E, "Images in cardiovascular medicine. Life-threatening neonatal arrhythmia: successful treatment and confirmation of clinically suspected extreme long QT-syndrome-3.", Circulation., 2004, Vol. 109(18) : e205-e206.
Ranking: 12,563
Abstract: Here, we demonstrate the electrophysiological findings for a preterm baby who was referred to our center for therapy of persistent complex arrhythmia with heart rate (HR) varying between 60 and 300 bpm. The ECG showed polymorphic ventricular tachycardia (HR 280 bpm), including short runs of torsade de pointes alternating within a few seconds with bradycardia due to third-grade atrioventricular (AV) block (HR 78 bpm, atrial rate 135 bpm), together with broad QRS complexes of 90 ms1/2 (Figure 1 and Data Supplement). During bradycardia, an extreme prolongation of the normalized QT interval (QTc 760 ms1/2) was present (Figure 2). To control tachycardia, treatment with propranolol was given intravenously, resulting in a predominant 2:1 AV block (HR 68 bpm) and fewer episodes of nonsustained ventricular tachycardia (Figure 3 and Data Supplement), although QTc remained unchanged (740 ms1/2). Because of the persisting unstable hemodynamic condition, the extreme QTc prolongation combined with AV block, and the QRS and T-wave morphology, a sporadic defect in the Na+Ch-encoding SCN5A gene was suspected. Thus, an additional treatment with the Na+Ch blocker mexiletine was initiated. With this medication, sinus rhythm occurred within 90 minutes. Furthermore, within 3 hours, a significant improvement of QTc (760 to 480 ms1/2) and of the intraventricular conduction delay (QRS 90 to 50 ms1/2) occurred (Figure 4). Genetic examination of the child confirmed the suspected long-QT syndrome-3 with proof of a de novo SCN5A gene defect (P1332L) close to the central opening of the Na+ channel. Our ECG tracings and the child’s clinical course (further details are given in the Data Supplement) confirmed the concept that the Na+Ch blocker mexiletine is capable of shortening the prolonged ventricular refractoriness below the P-P interval in extreme long-QT syndrome-3 with life-threatening arrhythmia.
BibTeX:

@article{Kehl_LQTS_Circ2004,

  author = {Hans Gerd Kehl and Wilhelm Haverkamp and Georg Rellensmann and T. Mesud Yelbuz and Thomas Krasemann and Johannes Vogt and Eric Schulze-Bahr},

  title = {Images in cardiovascular medicine. Life-threatening neonatal arrhythmia: successful treatment and confirmation of clinically suspected extreme long QT-syndrome-3.},

  journal = {Circulation},

  year = {2004},

  volume = {109},

  number = {18},

  pages = {e205--e206},

  url = {http://dx.doi.org/10.1161/01.CIR.0000128874.43908.CA},

  doi = {10.1161/01.CIR.0000128874.43908.CA}

}

Schulze-Bahr E, Fenge H, Etzrodt D, Haverkamp W, Mönnig G, Wedekind H, Breithardt G and Kehl HG, "Long QT syndrome and life threatening arrhythmia in a newborn: molecular diagnosis and treatment response.", Heart., 2004, Vol. 90(1) : 13-16.
Ranking: 3,271
Abstract: Intrauterine and neonatal manifestations of congenital long QT syndrome are associated with a high cardiac risk, particularly when atrioventricular block and excessive QT prolongation (> 600 ms(1/2)) are present. In a female newborn with these features, treatment with propranolol and mexiletine led to complete reduction of arrhythmia that was maintained 1.5 years later. High throughput genetic analysis found a sodium channel gene (LQT3) mutation. Disappearance of the 2:1 atrioventricular block and QTc shortening (from 740 ms(1/2) to 480 ms(1/2)), however, was achieved when mexiletine was added to propranolol. This effect was considered to be possibly genotype related. Early onset forms of long QT syndrome may benefit from advanced genotyping.
BibTeX:

@article{Schulze-Bahr_Heart-2004,

  author = {Schulze-Bahr, E. and H. Fenge and D. Etzrodt and W. Haverkamp and G. Mönnig and H. Wedekind and G. Breithardt and H. G. Kehl},

  title = {Long QT syndrome and life threatening arrhythmia in a newborn: molecular diagnosis and treatment response.},

  journal = {Heart},

  year = {2004},

  volume = {90},

  number = {1},

  pages = {13--16},

  url = {http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1768001/?tool=pubmed},

  doi = {10.1136/heart.90.1.13}

}

2003 Gehrmann J, Sohlbach K, Linnebank M, Böhles H-J, Buderus S, Kehl HG, Vogt J, Harms E and Marquardt T, "Cardiomyopathy in congenital disorders of glycosylation.", Cardiol Young., 2003, Vol. 13(4) : 345-351.
Ranking: 0,595
Abstract: Congenital disorders of glycosylation are a group of inherited metabolic multisystem disorders characterized by defects in the glycosylation of proteins and lipids. In most cases, neuromuscular disease is present. The purpose of this study was to characterize the cardiological aspects in this disorder. From the literature, we identified six children with congenital disorders of glycosylation associated with cardiac disease. We then screened for cardiovascular manifestations 20 patients diagnosed with congenital disorders of glycosylation at our own institution. Of the 6 patients identified in the literature, 4 had hypertrophic cardiomyopathy, while in the other 2 the cardiac diagnosis was unclear. The mean age at cardiac diagnosis was 5 months, with a range from 34 weeks to 24 months. Of the patients, five had died at a mean age of 3.5 months, with a range from 1.5 to 6 months, with one documented cardiac death. Three of our 20 patients (15 had coexistent cardiomyopathy, and in three additional patients presenting with cardiomyopathy we made the diagnosis of a congenital disorder of glycosylation. In our cohort, dilated cardiomyopathy was found in two-thirds of the patients, with hypertrophic cardiomyopathy in the other third. The mean age at cardiac diagnosis was 19 months, with a range from 0.5 to 84 months. Of these patients, two died in infancy at a mean age of 4 months, specifically at 1.5 and 7 months, due to cardiac disease, with one dying suddenly. The remaining four patients are alive with minor to severe cardiac dysfunction. We conclude that congenital disorders of glycosylation have to be considered in the differential diagnosis of children presenting with cardiomyopathy, and that all patients with congenital disorders of glycosylation should be screened for an associated cardiomyopathy. Cardiac involvement contributes significantly to morbidity and mortality, and probably to sudden cardiac death in this disorder.
BibTeX:

@article{Gehrmann_CDG,

  author = {Josef Gehrmann and Kristina Sohlbach and Michael Linnebank and Hans-Josef Böhles and Stephan Buderus and Hans Gerd Kehl and Johannes Vogt and Erik Harms and Thorsten Marquardt},

  title = {Cardiomyopathy in congenital disorders of glycosylation.},

  journal = {Cardiol Young},

  year = {2003},

  volume = {13},

  number = {4},

  pages = {345--351},

  url = {http://dx.doi.org/10.1017/S1047951103000702},

  doi = {10.1017/S1047951103000702}

}

Krasemann T, Kehl HG, Hammel D and Asfour B, "Congenital aortic regurgitation due to absent aortic cusps and high-degree mitral stenosis.", Pediatr Cardiol., 2003, Vol. 24(3) : 304-306.
Ranking: 0,581
Abstract: Congenital absence of aortic cusps leads to severe aortic regurgitation. We present a newborn with this rare entity with extreme mitral stenosis. Hemodynamic features were those of hypoplastic left heart syndrome. Surgical management consisted of initial modified Norwood procedure followed by orthotopic heart transplantation.
BibTeX:

@article{Krasemann_missing_VCS_Pediatr_Cardiol2002,

  author = {T. Krasemann and H. G. Kehl and D. Hammel and B. Asfour},

  title = {Congenital aortic regurgitation due to absent aortic cusps and high-degree mitral stenosis.},

  journal = {Pediatr Cardiol},

  year = {2003},

  volume = {24},

  number = {3},

  pages = {304--306},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/12522653},

  doi = {10.1007/s00246-002-0322-1}

}

Krasemann T, Kehl G, Vogt J and Asfour B, "Unusual systemic venous return with complete absence of the superior caval veins.", Pediatr Cardiol., 2003, Vol. 24(4) : 397-399.
Ranking: 0,581
Abstract: The absence of the right superior vena cava (SVC) is usually associated with the presence of a left SVC. This is the first report of a case of systemic venous return without the presence of either upper caval veins. The blood of the upper half of the body was collected into a vessel located on the left side of the spine behind the heart. This vessel crossed over to the right side at the level of the kidneys, draining directly into the inferior vena cava. During correction of tetralogy of Fallot, these findings were confirmed. The terminology and the bearing on interventional planning are discussed.
BibTeX:

@article{Krasemann-unusualVCS_PedCard2003,

  author = {T. Krasemann and G. Kehl and J. Vogt and B. Asfour},

  title = {Unusual systemic venous return with complete absence of the superior caval veins.},

  journal = {Pediatr Cardiol},

  year = {2003},

  volume = {24},

  number = {4},

  pages = {397--399},

  url = {http://dx.doi.org/10.1007/s00246-002-0326-x},

  doi = {10.1007/s00246-002-0326-x}

}

Musante L, Kehl HG, Majewski F, Meinecke P, Schweiger S, Gillessen-Kaesbach G, Wieczorek D, Hinkel GK, Tinschert S, Hoeltzenbein M, Ropers H-H and Kalscheuer VM, "Spectrum of mutations in PTPN11 and genotype-phenotype correlation in 96 patients with Noonan syndrome and five patients with cardio-facio-cutaneous syndrome.", Eur J Hum Genet., 2003, Vol. 11(2) : 201-206.
Ranking: 3,669
Abstract: Noonan syndrome (NS) is a relatively common, but genetically heterogeneous autosomal dominant malformation syndrome. Characteristic features are proportionate short stature, dysmorphic face, and congenital heart defects. Only recently, a gene involved in NS could be identified. It encodes the non-receptor protein tyrosine phosphatase SHP-2, which is an important molecule in several intracellular signal transduction pathways that control diverse developmental processes, most importantly cardiac semilunar valvulogenesis. We have screened this gene for mutations in 96 familial and sporadic, well-characterised NS patients and identified 15 different missense mutations in a total of 32 patients (33, including 23 index patients. Most changes clustered in one exon which encodes parts of the N-SH2 domain. Five of the mutations were recurrent. Interestingly, no mutations in the PTPN11 gene were detected in five additional patients with cardio-facio-cutaneous (CFC) syndrome, which shows clinical similarities to NS.
BibTeX:

@article{Musante_Noonan_EJHG,

  author = {Luciana Musante and Hans G Kehl and Frank Majewski and Peter Meinecke and Susann Schweiger and Gabriele Gillessen-Kaesbach and Dagmar Wieczorek and Georg K Hinkel and Sigrid Tinschert and Maria Hoeltzenbein and Hans-Hilger Ropers and Vera M Kalscheuer},

  title = {Spectrum of mutations in PTPN11 and genotype-phenotype correlation in 96 patients with Noonan syndrome and five patients with cardio-facio-cutaneous syndrome.},

  journal = {Eur J Hum Genet},

  year = {2003},

  volume = {11},

  number = {2},

  pages = {201--206},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/12634870},

  doi = {10.1038/sj.ejhg.5200935}

}

Weigel S, Kloska S, Kehl HG and Freund M, "Lethal subarachnoid bleeding under immunosuppressive therapy due to mycotic arteritis.", Eur Radiol., 2003, Vol. 13 Suppl 6 : L215-L218.
Ranking: 1,969
Abstract: A subarachnoid haemorrhage (SAH) occurred 67 days after cardiac transplantation in a 10-year-old girl with consecutive immunocompromising therapy. Neither digital subtraction angiography (DSA) nor computed tomographic angiography showed signs of intracranial vascular malformations. One month before the lethal SAH occurred, she had developed arterial hypertension and attacks of severe headache with cerebrospinal fluid (CSF) pleocytosis while CT scans showed an infarct of the left thalamus. Pathologic findings established the rare diagnosis of SAH due to aspergillosis-related mycotic arteritis. Imaging characteristics are presented.
BibTeX:

@article{Weigel_mycotic-arteritis-HTX_EurRadiol2003,

  author = {Stefanie Weigel and Stephan Kloska and Hans Gerd Kehl and Michael Freund},

  title = {Lethal subarachnoid bleeding under immunosuppressive therapy due to mycotic arteritis.},

  journal = {Eur Radiol},

  year = {2003},

  volume = {13 Suppl 6},

  pages = {L215--L218},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/16440238},

  doi = {10.1007/s00330-002-1741-3}

}

2002 Debus V, Krasemann T, Kehl HG, Vogt J, Drees G and Scheld HH, "Zusammenhang zwischen kulturellen und biografischen Erfahrungen und dem Transplantationserfolg", Zeitschrift für Herz-,Thorax- und Gefäßchirurgie., 2002, Vol. 16 Suppl.1(2) : I/81-I/85.
Abstract: Die orthotope Herztransplantation ist eine akzeptierte Therapie für Kinder und Jugendliche mit angeborenem komplexen Herzfehler im Endstadium der Herzerkrankung. Wir berichten über ein knapp 8-jähriges Mädchen mit einer Pulmonalatresie Typ II nach Somerville [9] mit Ventrikelseptumdefekt, persistierendem Ductus arteriosus Botalli und Transpositionsstellung der großen Arterien, die nach 2-maliger Korrekturoperation (Rastelli) einschließlich Schrittmacherimplantation im Kleinkindalter eine zunehmende dilatative Kardiomyopathie entwickelte. Ausgeprägte Verständigungsprobleme der aus Sri Lanka stammenden Familie erschwerten die Entscheidung für eine Herztransplantation, die wir dann aber nach intensiver interner Beratung doch im Alter von 6 Jahren erfolgreich durchführten. Auch knapp 2 Jahre nach Transplantation zeigt die Patientin eine gute kardiale Funktion und hat die erste Grundschulklasse erfolgreich abgeschlossen.
BibTeX:

@article{Debus_Z-HerzThoraxGefchir02,

  author = {Debus, V. and Krasemann, T. and Kehl, H. G. and Vogt, J. and Drees, G. and Scheld, H. H.},

  title = {Zusammenhang zwischen kulturellen und biografischen Erfahrungen und dem Transplantationserfolg},

  journal = {Zeitschrift für Herz-,Thorax- und Gefäßchirurgie},

  year = {2002},

  volume = {16 Suppl.1},

  number = {2},

  pages = {I/81--I/85},

  url = {http://www.springerlink.com/content/43qvmhxq13erdxaj/},

  doi = {10.1007/s00398-002-1116-8}

}

Marquardt T, Hülskamp G, Gehrmann J, Debus V, Harms E and Kehl HG, "Severe transient myocardial ischaemia caused by hypertrophic cardiomyopathy in a patient with congenital disorder of glycosylation type Ia.", Eur J Pediatr., 2002, Vol. 161(10) : 524-527.
Ranking: 1,223
Abstract: Severely affected children with congenital disorder of glycosylation type Ia (CDG-Ia; MIM 212065) may develop hypertrophic cardiomyopathy. In this report we describe the near-death of a 10-month-old girl with CDG-Ia due to acute left-ventricular outlet obstruction caused by hypertrophic cardiomyopathy and acute dehydration. The girl had multi-organ failure and signs of severe myocardial damage mimicking myocardial infarction. CONCLUSION: hypertrophic cardiomyopathy contributes to the high mortality of young children with congenital disorder of glycosylation type Ia. Even if cardiomyopathy in this disease is non-obstructive, acute fluid-loss might cause left ventricular outflow tract obstruction and life-threatening myocardial ischaemia. Patients with congenital disorder of glycosylation type Ia are at risk for cardiac complications and should be monitored regularly by echocardiography.
BibTeX:

@article{Marquardt_EurJPed_02,

  author = {Thorsten Marquardt and Georg Hülskamp and Josef Gehrmann and Volker Debus and Erik Harms and Hans Gerd Kehl},

  title = {Severe transient myocardial ischaemia caused by hypertrophic cardiomyopathy in a patient with congenital disorder of glycosylation type Ia.},

  journal = {Eur J Pediatr},

  year = {2002},

  volume = {161},

  number = {10},

  pages = {524--527},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/12297897},

  doi = {10.1007/s00431-002-1029-2}

}

Musante L, Kehl HG, Majewski F, Meinecke P, Tinschert S, Hinkel GK, Schweiger S, Gillessen-Kaesbach G, Wieczorek D, Hoeltzenbein M, Ropers HH and Kalscheuer VM, "Spectrum of mutations found in 86 cases with Noonan syndrome (NS)", European Journal of Human Genetics., 2002, Vol. 10(Suppl.1) : 224-5.
BibTeX:

@article{Musante2002,

  author = {Musante, L. and Kehl, H. G. and Majewski, F. and Meinecke, P. and Tinschert, S. and Hinkel, G. K. and Schweiger, S. and Gillessen-Kaesbach, G. and Wieczorek, D. and Hoeltzenbein, M. and Ropers, H. H. and Kalscheuer, V. M.},

  title = {Spectrum of mutations found in 86 cases with Noonan syndrome (NS)},

  journal = {European Journal of Human Genetics},

  year = {2002},

  volume = {10},

  number = {Suppl.1},

  pages = {224-5}

}

Rickert CH, Greiner C, Rellensmann G, Kehl HG, Scheld HH, Paulus W and Fechner G, "Mycotic cerebral vasculitis in a paediatric cardiac transplant patient excludes misadventure.", Int J Legal Med., 2002, Vol. 116(4) : 233-237.
Ranking: 1,918
Abstract: We present the case of a 10-year-old girl with cardiomyopathy who received a heart transplant. Due to organ rejection, the dosage of immunosuppressive agents was increased postoperatively. The patient complained of intermittent headaches in the following days and developed a haemorrhagic necrosis of the left thalamus. A week later, an oral dose of cyclosporin A was accidentally given intravenously, and 2 weeks later a recurrent subarachnoid haemorrhage of unknown origin was diagnosed. The clinical course was then characterised by progressive deterioration resulting in coma, fluctuating brain stem symptoms and the development of a massive cerebral oedema with subsequent brain death. A coroner's autopsy was instigated to investigate a claim of medical misadventure. Neuropathological investigations found a focal infiltration of fungal hyphae in the left posterior cerebral artery resulting in necrosis of the vascular wall and thus explaining the source of the recurrent subarachnoid haemorrhage which eventually resulted in the girl's death. Medical misadventure due to the administration of cyclosporin was not directly responsible for the death of this patient. This case illustrates that it is of paramount importance to copiously sample and investigate the basal cerebral arteries in cases of subarachnoid haemorrhage of unknown origin, in particular in a medico-legal context.
BibTeX:

@article{Rickert_HTX_Pilzaneurysma,

  author = {C. H. Rickert and C. Greiner and G. Rellensmann and H. G. Kehl and H. H. Scheld and W. Paulus and G. Fechner},

  title = {Mycotic cerebral vasculitis in a paediatric cardiac transplant patient excludes misadventure.},

  journal = {Int J Legal Med},

  year = {2002},

  volume = {116},

  number = {4},

  pages = {233--237},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/12420703},

  doi = {10.1007/s00414-002-0298-7}

}

2001 Gehrmann J, Krasemann T, Kehl HG and Vogt J, "Hypoplastic left-heart syndrome: the first description of the pathophysiology in 1851; translation of a publication by Dr. Bardeleben from Giessen, Germany.", Chest., 2001, Vol. 120(4) : 1368-1371.
Ranking: 2,480
Abstract: The term hypoplastic left-heart syndrome was first proposed by Noonan and Nadas in 1958 and comprises a continuum of congenital cardiac anomalies characterized by underdevelopment of the aorta, aortic valve, left ventricle, mitral valve, and left atrium. The first published cases of aortic atresia were reported by Romberg in 1846 and Canton in 1849, and are more or less restricted to brief pathologic and anatomic descriptions. Neither pathophysiologic nor pathogenetic aspects were discussed. The account of Dr. Bardeleben, written in 1851, was probably the first complete description of the clinical features, the pathologic-anatomic characteristics, and the pathophysiology of the hypoplastic left-heart syndrome. It includes an illustration of this malformation, a hypothesis on the etiology, and reflections on the symptoms. This historical publication documents the correct understanding of the circulation in this disorder, realizing that survival depends on a patent ductus arteriosus. Also, a differentiated theory on the development of cyanosis is provided. Pathogenetically, Dr. Bardeleben attributed aortic atresia not to a simple arrest of normal development, but to a pathologic process developing during intrauterine life. The precision and the clarity of the description and figures is striking. This account given by Dr. Bardeleben remains entirely valid even today. The following literal translation of the original manuscript gives the reader a sense of the original and gives credit to this early, superb, as yet unknown description (in the international literature) of a congenital heart disease.
BibTeX:

@article{Gehrmann_HLHS,

  author = {J. Gehrmann and T. Krasemann and H. G. Kehl and J. Vogt},

  title = {Hypoplastic left-heart syndrome: the first description of the pathophysiology in 1851; translation of a publication by Dr. Bardeleben from Giessen, Germany.},

  journal = {Chest},

  year = {2001},

  volume = {120},

  number = {4},

  pages = {1368--1371},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/11591582},

  doi = {10.1378/chest.120.4.1368}

}

Gehrmann J, Kehl HG, Diallo R, Debus V and Vogt J, "Cardiac leiomyosarcoma of the right atrium in a teenager: unusual manifestation with a lifetime history of atrial ectopic tachycardia.", Pacing Clin Electrophysiol., 2001, Vol. 24(7) : 1161-1164.
Ranking: 1,197
Abstract: A 16-year-old girl presented with atrial fibrillation. Transesophageal echocardiography revealed a right atrial leiomyosarcoma. Her past medical history was remarkable for incessant atrial ectopic tachycardia (AET) beginning in early infancy and continuing throughout childhood and adolescence that was refractive to medical and nonpharmacological treatment. After combined surgical and medical therapy, normal sinus rhythm was restored and the patient is currently in complete remission with no recurrent symptoms or atrial arrhythmias at 31 months after surgery and 23 months after the discontinuation of chemotherapy. Atrial tachycardia may be the first, and for prolonged periods, the only manifestation of a cardiac tumor and should prompt thorough investigation of its underlying morphological substrate.
BibTeX:

@article{Gehrmann_Leiomyosarkom,

  author = {J. Gehrmann and H. G. Kehl and R. Diallo and V. Debus and J. Vogt},

  title = {Cardiac leiomyosarcoma of the right atrium in a teenager: unusual manifestation with a lifetime history of atrial ectopic tachycardia.},

  journal = {Pacing Clin Electrophysiol},

  year = {2001},

  volume = {24},

  number = {7},

  pages = {1161--1164},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/11475835},

  doi = {10.1046/j.1460-9592.2001.01161.x}

}

Nowak-Göttl U, Kotthoff S, Hagemeyer E, Junker R, Kehl HG, Vielhaber H and Kececioglu D, "Interaction of fibrinolysis and prothrombotic risk factors in neonates, infants and children with and without thromboembolism and underlying cardiac disease. a prospective study.", Thromb Res., 2001, Vol. 103(2) : 93-101.
Ranking: 1,446
Abstract: To evaluate the role of plasminogen activator inhibitor-1 (PAI-1) and tissue-type plasminogen activator (t-PA) in children with an estimated risk of vascular occlusion reported to range from 7% to 16 we conducted a prospective study in infants and children with underlying cardiac disease. One hundred and twenty-five children (neonate - 16 years) were investigated. In 9 infants out of the 125 children vascular occlusion occurred, closely related to cardiac catheterisation and arterial or venous lines during major cardiac surgery. Six of the nine neonates and infants with (n=6) and without (n=3) prothrombotic risk factors showed evidence of a basically impaired fibrinolytic system. Five of the nine infants showed increased PAI-1 clearly correlated to the 4G/4G genotype of the plasminogen activator-1 promoter polymorphism along with elevated t-PA concentration before the first diagnostic cardiac catheterisation was performed. One infant presented with increased t-PA concentration only. Five of the six children with reduced fibrinolytic capacity had further prothrombotic risk factors. Conclusion: Data of this study indicate that neonates and infants with underlying cardiac disease and basically increased PAI-1 due to the 4G/4G variant of the PAI-1 promoter polymorphism along with elevated t-PA levels in combination with further prothrombotic risk factors are at high risk of developing early thromboembolism during cardiac catheterisation.
BibTeX:

@article{NowakGottl_ThrombRes_2001,

  author = {Nowak-Göttl, U. and S. Kotthoff and E. Hagemeyer and R. Junker and H. G. Kehl and H. Vielhaber and D. Kececioglu},

  title = {Interaction of fibrinolysis and prothrombotic risk factors in neonates, infants and children with and without thromboembolism and underlying cardiac disease. a prospective study.},

  journal = {Thromb Res},

  year = {2001},

  volume = {103},

  number = {2},

  pages = {93--101},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/11457466},

  doi = {10.1016/S0049-3848(01)00281-X}

}

2000 Kehl HG, Jäger Jü, Papazis N, Dimitrelos D, Gehrmann J, Kassenböhmer R, Vogt J and Sakas G, "3D heart modelling from biplane, rotational angiocardiographic X-ray sequences", Computers & Graphics-Uk., 2000, Vol. 24(5) : 731-739.
Ranking: 0,483
Abstract: The 3DHeartView project produced an advanced software application for time-variant 3D cardiac modelling based on back projection of rotational X-ray angiographic sequences. Due to standard DICOM 3.0 interface, the software can be used as an add-on to any modern digital angiographer. The system accuracy has been measured and the approach has been validated with static and dynamic phantom object studies. The prototype has been tested in a clinical routine environment as well. In this paper we present the main features of the system, the methodology of 3D angiographic modelling, and certain testing and operation results. We also describe the application of high-performance computing technology in the modelling process.
BibTeX:

@article{Kehl_3dhv_ComputGraph_00,

  author = {Hans Gerd Kehl and Jürgen Jäger and Nikos Papazis and Dimitris Dimitrelos and Josef Gehrmann and Rainer Kassenböhmer and Johannes Vogt and Georgios Sakas},

  title = {3D heart modelling from biplane, rotational angiocardiographic X-ray sequences},

  journal = {Computers & Graphics-Uk},

  year = {2000},

  volume = {24},

  number = {5},

  pages = {731--739},

  note = {http://wos4.isiknowledge.com/CIW.cgi?&Func=Links&PointOfEntry=FullRecord&UT=000165255200008&CustomersIP=128.176.29.137&ServiceUser=Links&ServiceName=TransferToWos&PublisherID=ResearchSoft&CustomersID=ResearchSoft&SID=P7yPUArg-GkAAE97E98},

  url = {http://dx.doi.org/10.1016/S0097-8493(00)00075-3},

  doi = {10.1016/S0097-8493(00)00075-3}

}

Kehl HG, Papazis N, Jäger J, Sandbote C, Dimitrelos D, Kassenböhmer R, Rellensmann G, Gehrmann J, Sakas G and Vogt J, "X-ray angiocardiography in three and four dimensions", In Image Processing and Parallel Computing in Medical Applications., 2000, : 7-18. HPCN TTN Network, Sector Group Medical Applications.
Abstract: The 3D Heart View project produced an advanced software application for 3D cardiac modelling based on usual X-ray driven angiographic sequences. Due to standard DICOM 3.0 interface the software can be used as an add-on to any modern digital angiographer. The system’s accuracy has been measured and the approach has been validated with static and dynamic phantom object studies. The prototype has been tested in a clinical routine environment too. In this paper we present the main features of the system, the methodology of 3D angiographical modelling, and certain testing and operation results. We also describe the application of high performance computing technology in the modelling process.
BibTeX:

@incollection{Kehl_Starke-2000_HPCN,

  author = {Kehl, H. G. and Papazis, N. and Jäger, J. and Sandbote, C. and Dimitrelos, D. and Kassenböhmer, R. and Rellensmann, G. and Gehrmann, J. and Sakas, G. and Vogt, J.},

  editor = {Starke, D.},

  title = {X-ray angiocardiography in three and four dimensions},

  booktitle = {Image Processing and Parallel Computing in Medical Applications},

  publisher = {HPCN TTN Network, Sector Group Medical Applications},

  year = {2000},

  pages = {7--18},

  note = {Esprit Program Nr. 24484: ftp://ftp.cordis.europa.eu/pub/esprit/docs/projhpcn.pdf},

  doi = {http://www.springerlink.com/content/uxl231760673k500/}

}

1999 Dimitrelos D, Tassakos L, Papazis N, Sakas G, Jäger J, Kehl HG, Kyriakides Z, Kolettis T, Karaiskou K, Marsh A, Delibasis K and Michael C, "3DHeartView: Introducing 3-dimensional angiographical modelling", Lecture Notes in Computer Science., In High-Performance Computing and Networking, Proceedings., 1999, Vol. 1593 : 931-940.
Ranking: 0,872
Abstract: The 3DHeartView project produced an advanced prototype of a system capable of modelling 3-dimensional cardiac structures based on a single sequence of clinical angiographic X-rays. That can be used as an add-on to any modern digital angiographer. The system's accuracy has been measured and our approach validated with static and dynamic phantom object studies. The prototype has been used in many real-patient cases in a clinical routine environment. In this paper we present the main features of the system, the methodology of 3D angiographical modelling, and certain testing and operation results. We also describe the application of HPC technology in the modelling process.
BibTeX:

@article{Dimitrelos-3DHV-HPCN99_modelling,

  author = {Dimitrelos, D. and Tassakos, L. and Papazis, N. and Sakas, G. and Jäger, J. and Kehl, H. G. and Kyriakides, Z. and Kolettis, T. and Karaiskou, K. and Marsh, A. and Delibasis, K. and Michael, C.},

  title = {3DHeartView: Introducing 3-dimensional angiographical modelling},

  booktitle = {High-Performance Computing and Networking, Proceedings},

  journal = {Lecture Notes in Computer Science},

  year = {1999},

  volume = {1593},

  pages = {931-940},

  note = {ISI:000088252100094},

  url = {http://dl.acm.org/citation.cfm?id=660476},

  doi = {10.1007/BFb0100653}

}

Gehrmann J, Mueller F, Hellmich M, Kececioglu D, Kehl HG, Fetsch T and Vogt J, "Signal-averaged electrocardiography in healthy children: Influence of age, gender and anthropometric data", European Heart Journal., 1999, Vol. 20 (Abstract Supplement) : S 642.
BibTeX:

@article{Gehrmann1999,

  author = {Gehrmann, J. and Mueller, F. and Hellmich, M. and Kececioglu, D. and Kehl, H. G. and Fetsch, T. and Vogt, J.},

  title = {Signal-averaged electrocardiography in healthy children: Influence of age, gender and anthropometric data},

  journal = {European Heart Journal},

  year = {1999},

  volume = {20 (Abstract Supplement)},

  pages = {S 642},

  url = {http://eurheartj.oxfordjournals.org/content/20/Abstract_Supplement/1.full.pdf+html},

  doi = {10.1093/eurheartj/20.Abstract_Supplement.1}

}

Suedkamp M, Horst M, Mehlhorn U, Kehl HG and Dapunt O, "Pulmonary artery sling and complex cardiac malformation - Report on a two step repair", Cor Europaeum - European Journal of Cardiac Interventions., 1999, Vol. 7(4) : 190-191.
Abstract: Pulmonary artery sling is a rare malformation and in most cases fatal without surgical repair. Pulmonary artery sling can occur as an isolated phenomenon or be associated with malformation of the trachea and the heart. We report on a case of pulmonary artery sling in combination with severe cardiac malformation and tibia aplasia. Due to severe hypoxic attacks the clinical symptoms of the sling were masked, and thus, the sling was diagnosed secondarily. Consequently, surgical repair was performed in two steps. First, a modified Blalock-Taussig-shunt was done at the age of six months and, thereafter, both pulmonary artery sling and cardiac malformation were corrected at the age of 5.5 years in a second step.
BibTeX:

@article{Suedkamp_Cor1999,

  author = {Suedkamp, M. and Horst, M. and Mehlhorn, U. and Kehl, H. G. and Dapunt O.},

  title = {Pulmonary artery sling and complex cardiac malformation - Report on a two step repair},

  journal = {Cor Europaeum - European Journal of Cardiac Interventions},

  year = {1999},

  volume = {7},

  number = {4},

  pages = {190--191},

  url = {http://www.scopus.com/inward/record.url?eid=2-s2.0-0032847616&partnerID=40}

}

Tjan TD, Schmid C, Deng MC, Schmidt C, Kerber S, Kehl G and Scheld HH, "Evolving short-term and long-term mechanical assist for cardiac-failure -- a decade of experience in Münster.", Thorac Cardiovasc Surg., 1999, Vol. 47 Suppl 2 : 294-297.
Ranking: 0,766
Abstract: Technological advances and growing expertise has lead to referral of much sicker patients with a greater incidence of heart failure prior to and after cardiac surgical procedures. The diversity of the heart failure patient cohort mandates a differentiated protocol for mechanical support adapted to the clinical requirements. It is desirable to have appropriate mechanical support available for different circumstances of heart failure. In this paper, we review the first decade of the Muenster University Hospital experience with the use of intra-aortic ballon pump, extracorporal membrane oxygenators, short term uni- and biventricular assist systems such as Thoratec and Medos devices, as well as long term left ventricular assist systems such as the TCI Heartmate and the Novacor system. The patient profiles, indications, contraindications, and future trends are reviewed within the framework of a contemporary university hospital Servive.
BibTeX:

@article{Tjan_LVAD_ThorcCardiovascSurg_1999,

  author = {T. D. Tjan and C. Schmid and M. C. Deng and C. Schmidt and S. Kerber and G. Kehl and H. H. Scheld},

  title = {Evolving short-term and long-term mechanical assist for cardiac-failure -- a decade of experience in Münster.},

  journal = {Thorac Cardiovasc Surg},

  year = {1999},

  volume = {47 Suppl 2},

  pages = {294--297},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/10218603},

  doi = {10.1055/s-2007-1012051}

}

1998 Dimitrelos D, Tassakos L, Sakas G, Jaeger J, Papazis N, Marsh A, Delibasis K, Michael C, Kehl HG, Kyriakides Z and Kolettis T, "The 3D HeartView Project", Lecture Notes in Computer Science., In High-Performance Computing and Networking., 1998, Vol. 1401 : 1021-1023.
Abstract: Heart diseases are the most severe illness in industrial countries. For the diagnosis and therapy of several severe heart diseases the accurate visualisation of the 3D heart geometry (spatial heart shape and volume of the ventricles, shape and structure of the vessel tree, heart walls' movement) can play a crucial role. Attributes like the shape of the ventricles, the curvature of the vessels, or the shape of a stenosis could play an important role in operation planning (find optimal angle for next injection, decide stent type etc.). The objective of the 3D HeartView project is to demonstrate the benefits of HPCN technology in improving the diagnostic and clinical procedures of heart diseases treatment. It enables fast 3D modeling of the heart based on 2D X-ray angiographic pictures.
BibTeX:

@article{Dimitrelos_3DHV-HPCN98,

  author = {Dimitrelos, D. and Tassakos, L. and Sakas, G. and Jaeger, J. and Papazis, N. and Marsh, A. and Delibasis, K. and Michael, C. and Kehl, H. G. and Kyriakides, Z. and Kolettis, T.},

  title = {The 3D HeartView Project},

  booktitle = {High-Performance Computing and Networking},

  journal = {Lecture Notes in Computer Science},

  year = {1998},

  volume = {1401},

  pages = {1021--1023},

  url = {http://www.informatik.uni-trier.de/ ley/db/indices/a-tree/k/Kehl:Hans.html},

  doi = {10.1007/BFb0037263}

}

Hagemeyer E, Vielhaber H, Kececioglu D, Kehl HG and Nowak-Göttl U, "Genetic risks for thrombophilia in catheter-related thrombosis in childhood - a prospective study", In 27. Hämophilie Symposium Hamburg 1996. Berlin,Heidelberg,, 1998, Springer Verlag, ISBN 978-3540629153.
Abstract: To find out to what extent diagnostic cardiac percutaneous catheterisation irritates vascular endothelium in 140 children before, immediately at the end of, and 24 hours after catheterisation, t-PA, u-PA and PAI 1 were investigated. Compared to starting values, t-PA, u-PA and PAI 1 concentrations showed a significant increase at the end of catheterisation. u-PA and PAI 1 returned to pretreatment values 24 h later, t-PA remained elevated. Four out of seven children with thrombosis showed increased t-PA antigen and/or PAI 1 antigen activities before cardiac catheterisation was performed. Data of this study indicate that increased t-PA, u-PA and PAI 1 antigen levels are signs of endothelial damage. Whether increased values of t-PA or PAI 1 may predict future vascular occlusion in infants undergoing cardiac catheterisation requires a more extensive study.
BibTeX:

@incollection{Hagemeyer_27-Haemoohilie-Symposion,

  author = {Hagemeyer, E. and Vielhaber, H. and Kececioglu, D. and Kehl, H. G. and Nowak-Göttl, U.},

  editor = {Scharrer, I. and Schramm, W.},

  title = {Genetic risks for thrombophilia in catheter-related thrombosis in childhood - a prospective study},

  booktitle = {27. Hämophilie Symposium Hamburg 1996},

  publisher = {Springer Verlag, ISBN 978-3540629153},

  year = {1998},

  url = {http://amazon.de/o/ASIN/3540629157/}

}

Kehl HG, Jäger J, Kececioglu D, Sakas G, Gehrmann J, Nekarda T, Rellensmann G and Vogt J, "Pediatric angiocardiography in three and four dimensions: Evolution of methods, validation, and first clinical results", In Proceedings of the Second World Congress of Pediatric Cardiology and Cardiac Surgery., 1998, : 465-468. Futura Press, New York Armonk, Tokyo,ISBN 978-0879936990.
Abstract: Objectives: Although angiocardiography is a standard diagnostic tool in pediatric cardiology, valid volumetric data are not available for differently shaped heart defects. The aim of our study was to provide threedimensional (3D) cardiac imaging and 3D volume measurement.

Methods: Biplane rotational angiograms with two orthogonal X-ray collimators were used for image acquisition. A motorized rotation of the gantry (Fig. 1A) was performed with 2 x 8 degree per second, film rate was 2 x 25 frames per second, and the recording time lasted 3 to 4 seconds. For image transport video sequences were used first, currently we are using DICOM-3 formated CD-ROMs. Preprocessing procedures consisted of size scaling (Fig. 1B), labeling with rotation degree and heart phase (Fig. 1C), and gamma compensation in heart niveau = isocenter for all frames according to geometric properties of the X-ray system. Pictures from same heart phases were arranged like a holography. 3D reconstruction technique is based on backprojection for a pyramid beam perspective. An enhanced algorithm was developed tracing all X-rays back to its ray source and computing the absorption distribution of the shone through tissue (Fig. 2A). The recognition of contours of the heart chambers was achieved by filters and ray casting operations (Fig 2B). Volume
measurements were performed by triangulation of chamber surfaces with marching tetrahedrons (Fig. 3A).
Results: Phantoms and heart chambers filled with contrast agent could be extracted from the acquired sequences. Recognized objects could be visualized by 3D rendering (Fig. 2C). Additionally virtual views into the objects were created (Fig. 2D). 3D volume measurements of phantoms (Figure 3 B) showed reliable and valid results compared to true volume in a range from 14 mL to 52 mL (Fig. 3C). First clinical examples of 3D heart reconstruction with 3D volume measurements are available on the Internet (http://www.igd.fhg.de/www/igd-a7/Projects/ventrikel/proj_ventrikl.html).

Comments: 3D reconstructions of heart chambers by rotational angiography are feasible, can improve qualitative cardiac analysis, and provide better functional data of heart performance. Therefore 3D cine-angiocardiography is a promising new tool for cardiac imaging and volume measurements.

BibTeX:

@incollection{Kehl_Hawai,

  author = {Kehl, H. G. and Jäger, J. and Kececioglu, D. and Sakas, G. and Gehrmann, J. and Nekarda, T. and Rellensmann, G. and Vogt, J.},

  editor = {Imai, Y. and Momma, K.},

  title = {Pediatric angiocardiography in three and four dimensions: Evolution of methods, validation, and first clinical results},

  booktitle = {Proceedings of the Second World Congress of Pediatric Cardiology and Cardiac Surgery},

  publisher = {Futura Press, New York Armonk, Tokyo,ISBN 978-0879936990},

  year = {1998},

  pages = {465--468},

  url = {http://amazon.de/o/ASIN/0879936991/}

}

Loick H, Kehl G and Schmidt C, "Transösophageale Echokardiographie (TEE) in der Anästhesie und Intensivmedizin - Grundlagen und Indikationen", Anästhesiologie & Intensivmedizin., 1998, Vol. 39(6) : 288-298.
Abstract: Transesophageal echocardiography (TEE) has an increasing impact on diagnostic tools in anaesthesia and intensive care medicine. However, practice of TEE is based on the knowledge of basic echocardiographic principles and their limitations. The present overview aims to provide basics and indications of TEE in anaesthesia and intensive care.
BibTeX:

@article{Loick_Anaesthesiol1998,

  author = {Loick, H.M. and Kehl, G. and Schmidt, C.},

  title = {Transösophageale Echokardiographie (TEE) in der Anästhesie und Intensivmedizin - Grundlagen und Indikationen},

  journal = {Anästhesiologie & Intensivmedizin},

  year = {1998},

  volume = {39},

  number = {6},

  pages = {288--298},

  url = {http://www.scopus.com/inward/record.url?eid=2-s2.0-0031835731&partnerID=40}

}

Nowak-Göttl U, Dübbers A, Kehl HG, Runde J and Vielhaber H, "Genetic risks for thrombophilia in catheter-related thrombosis in childhood - a prospective study", In 27. Hämophilie Symposium Hamburg 1996. Berlin, Heidelberg, 1998, Springer Verlag, ISBN 978-3540629153.
Abstract: To evaluate the incidence of genetic risk factors of familial thrombophilia in catheter-related thrombosis, 163 consecutively admitted infants and children were prospectively investigated and compared with an age-matched healthy control group undergoing elective surgery. Whereas no differente was found in the total prevalence of genetic risk factors of thrombophilia in the population tested (catheter 10.4%, elective surgery 11.6%), the incidence of symptomatic thromboembolism in the catheter groups was significantly higher. Data of this study indicate that genetic risk factors of familial thrombophilia play an important role in the manifestation of catheter-related thrombosis.
BibTeX:

@incollection{Nowak-Gottl_27-HamburgerHaemophilie,

  author = {Nowak-Göttl, U. and Dübbers, A. and Kehl, H. G. and Runde, J. and Vielhaber, H.},

  editor = {Scharrer, I. and Schramm, W.},

  title = {Genetic risks for thrombophilia in catheter-related thrombosis in childhood - a prospective study},

  booktitle = {27. Hämophilie Symposium Hamburg 1996},

  publisher = {Springer Verlag, ISBN 978-3540629153},

  year = {1998},

  url = {http://amazon.de/o/ASIN/3540629157/}

}

Rellensmann G, Kehl HG, Thies WR, Crespo-Martinez E and Meyer H, "A newborn with multiple congenital anomalies and complex tachyarrhythmia.", Eur J Pediatr., 1998, Vol. 157(2) : 163-164.
Ranking: 1,050
BibTeX:

@article{Rellensmann_Costello_EJP98,

  author = {G. Rellensmann and H. G. Kehl and W. R. Thies and E. Crespo-Martinez and H. Meyer},

  title = {A newborn with multiple congenital anomalies and complex tachyarrhythmia.},

  journal = {Eur J Pediatr},

  year = {1998},

  volume = {157},

  number = {2},

  pages = {163--164},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/9504794},

  doi = {10.1007/s004310050792}

}

Weyand M, Kececioglu D, Kehl HG, Schmid C, Loick HM, Vogt J and Scheld HH, "Neonatal mechanical bridging to total orthotopic heart transplantation.", Ann Thorac Surg., 1998, Vol. 66(2) : 519-522.
Ranking: 2,190
Abstract: BACKGROUND: Until recently, newborns with medically intractable cardiac failure caused by congenital malformations were mostly doomed to death because of the severity of the disease, which precludes a palliative operation, or because of fatal deterioration before availability of a suitable donor heart. METHODS: The recently developed paracorporeal pneumatically driven Medos HIA ventricular assist device offers a therapeutic option for these small infants because it is manufactured in various sizes and is even suitable for cardiac assistance in neonates with a body surface area less than 0.3 m2. RESULTS: We report our initial experience with this device, which we used for univentricular bridging to total orthotopic cardiac transplantation in 3 infants. The device was inserted to support the left ventricle in two instances and to support the right heart in one. Successful bridging to transplantation was achieved in 2 infants for periods of 2 and 7 weeks. CONCLUSIONS: Our experience demonstrates the feasibility of univentricular mechanical support followed by successful cardiac transplantation in infants and newborns.
BibTeX:

@article{Weyand_Medos,

  author = {M. Weyand and D. Kececioglu and H. G. Kehl and C. Schmid and H. M. Loick and J. Vogt and H. H. Scheld},

  title = {Neonatal mechanical bridging to total orthotopic heart transplantation.},

  journal = {Ann Thorac Surg},

  year = {1998},

  volume = {66},

  number = {2},

  pages = {519--522},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/9725395},

  doi = {10.1016/S0003-4975(98)00442-1}

}

1997 Asfour B, Weyand M, Kececioglu D, Kehl HG, Hammel D, Loick M, Vogt G and Scheld HH, "A novel paracorporeal mechanical assist device for newborns and infants allows bridging to transplantation.", Transplant Proc., 1997, Vol. 29(8) : 3330-3332.
Ranking: 0,698
BibTeX:

@article{Asfour_Medos_TransplProc_1997,

  author = {B. Asfour and M. Weyand and D. Kececioglu and H. G. Kehl and D. Hammel and M. Loick and G. Vogt and H. H. Scheld},

  title = {A novel paracorporeal mechanical assist device for newborns and infants allows bridging to transplantation.},

  journal = {Transplant Proc},

  year = {1997},

  volume = {29},

  number = {8},

  pages = {3330--3332},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/9414737},

  doi = {10.1016/S0041-1345(97)00933-0}

}

Kececioglu D, Deng MC, Schmid C, Kehl HG, Baba HA, Yelbuz M, Scheld HH and Vogt J, "Anomalous origin of the left coronary artery from the pulmonary artery with large anterior myocardial infarction and ischemia: successful tunnel repair and concomitant heterotopic heart transplantation as biological bridge to recovery.", Transpl Int., 1997, Vol. 10(2) : 161-163.
Ranking: 1,211
BibTeX:

@article{Kececioglu_TransplInt97,

  author = {D. Kececioglu and M. C. Deng and C. Schmid and H. G. Kehl and H. A. Baba and M. Yelbuz and H. H. Scheld and J. Vogt},

  title = {Anomalous origin of the left coronary artery from the pulmonary artery with large anterior myocardial infarction and ischemia: successful tunnel repair and concomitant heterotopic heart transplantation as biological bridge to recovery.},

  journal = {Transpl Int},

  year = {1997},

  volume = {10},

  number = {2},

  pages = {161--163},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/9090006},

  doi = {10.1007/s001470050033}

}

Kececioglu D, Kohlhase B, Kehl HG, Kassenböhmer R, Scheld HH and Vogt J, "Transoesophageal Echocardiography during Repair of Congenital Heart Defects. Chapter 3 in: Loick HM, Scheld HH, Van Aken H: Impact of Perioperative Transoesophageal Echocardiography on Cardiac Surgery", Thorac Cardiovasc Surgeon., 1997, Vol. 45 : 321-25.
Ranking: 0,527
Abstract: Severe postoperative mitral regurgitation renders information on the underlying mechanism before reoperation very important, as a potential for mitral valve reconstruction may facilitate the decision whether to reoperate, especially in the very young. This study compares the efficacy of transthoracic echo-cardiography (TTE) and left-ventricular angiography with that of transesophageal echocardiography (TEE) for detection of the mechanism underlying mitral regurgitation and its quantitative assessment in children after repair of common atrioventricular septal defect. Five children aged 1.5 to 16 years were evaluated by TTE, TEE, and angiography for postoperative mitral regurgitation 1 to 21 months after initial repair. TEE showed septal detachment of the mitral leaflet in four patients and reopening of the mitral cleft in one patient as the cause of mitral regurgitation whereas TTE failed in four and angiography in all patients. TEE allows definite identification of morphologic characteristics of mitral regurgitation and reliable assessment of its severity. Thus redo surgery may be safely performed on the bases of TEE findings alone without confirmation by cardiac catheterization.
BibTeX:

@article{Keci_inLoick_TEE_ThoracCardiovascSurg1997,

  author = {Kececioglu, D. and Kohlhase, B. and Kehl, H. G. and Kassenböhmer, R. and Scheld, H. H. and Vogt, J.},

  title = {Transoesophageal Echocardiography during Repair of Congenital Heart Defects. Chapter 3 in: Loick HM, Scheld HH, Van Aken H: Impact of Perioperative Transoesophageal Echocardiography on Cardiac Surgery},

  journal = {Thorac Cardiovasc Surgeon},

  year = {1997},

  volume = {45},

  pages = {321--25},

  url = {http://www.thieme-connect.de/ejournals/abstract/thoracic/doi/10.1055/s-2007-1013760},

  doi = {10.1055/s-2007-1013760}

}

Kececioglu D, Kehl HG, Schmid C, Deng MC, Gehrmann J, Weyand M, Scheld HH and Vogt J, "Morphologic characterization and assessment of mitral regurgitation after repair of atrioventricular defects in children.", Thorac Cardiovasc Surg., 1997, Vol. 45(2) : 70-74.
Ranking: 0,527
Abstract: Severe postoperative mitral regurgitation renders information on the underlying mechanism before reoperation very important, as a potential for mitral valve reconstruction may facilitate the decision whether to reoperate, especially in the very young. This study compares the efficacy of transthoracic echo-cardiography (TTE) and left-ventricular angiography with that of transesophageal echocardiography (TEE) for detection of the mechanism underlying mitral regurgitation and its quantitative assessment in children after repair of common atrioventricular septal defect. Five children aged 1.5 to 16 years were evaluated by TTE, TEE, and angiography for postoperative mitral regurgitation 1 to 21 months after initial repair. TEE showed septal detachment of the mitral leaflet in four patients and reopening of the mitral cleft in one patient as the cause of mitral regurgitation whereas TTE failed in four and angiography in all patients. TEE allows definite identification of morphologic characteristics of mitral regurgitation and reliable assessment of its severity. Thus redo surgery may be safety performed on the bases of TEE findings alone without confirmation by cardiac catheterization.
BibTeX:

@article{Keci_TEE_ThorSurg_1997,

  author = {D. Kececioglu and H. G. Kehl and C. Schmid and M. C. Deng and J. Gehrmann and M. Weyand and H. H. Scheld and J. Vogt},

  title = {Morphologic characterization and assessment of mitral regurgitation after repair of atrioventricular defects in children.},

  journal = {Thorac Cardiovasc Surg},

  year = {1997},

  volume = {45},

  number = {2},

  pages = {70--74},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/9175222},

  doi = {10.1055/s-2007-1013690}

}

Kehl HG, Kececioglu D, Nekarda T, Vielhaber H, Gehrmann J, Vogt J and Nowak-Göttl U, "Zeichen der aktivierten Gerinnung und Fibrinolyse nach kardio- pulmonalem Bypass mit Aprotinin", In 26. Hämophilie Symposium Hamburg 1995. Berlin, Heidelberg, 1997, : 236-240. Springer Verlag, ISBN 978-3540611011.
Abstract: Hintergrund:Herzoperationen mit kardiopulmonalem Bypass (CPB) sind häufi g assoziiert mit einem erhöhten Blutverlust durch eine gesteigerte Fibr inolyse [1, 2,4 - 8]. Mehrere Studien zu diesem Problem konnten zeigen, daß Aprotinin, ein potenter Inhibitor von Plasmin und Kallikrein, den Bedarf an postoperativen Transfusionen signifi kant senkt [4, 5]. Das Ziel der vorliegenden Studie war, das Ausmaß der Gerin nungsaktivierung bei Säuglingen und Kindern nach kardiopulmonalen Bypass operationen mit Aprotinin prospektiv zu untersuchen.
Methoden: 32 konsekutive Kinder mit Palliativ- oder Korrekturoperationen kongenitaler Herzfehler gingen in diese prospektiv angelegte Studie ein. Die Untersuchungen erfolgten jeweils vor Narkoseeinleitung, 2 h nach Beendigung der Operation sowie an den postoperativen Tagen 1, 2, 3, 4 bis 6 und 7 bis 9. Hämatokritwerte und Thrombozytenzahlen wurden mit dem Cell counter K looo Fa. Sysmex gemessen. Plasma für die Gerinnungsanalysen wurde mit markierten Probenröhrchen (Citrat 3,8 %/Blut: I + 9; Fa. Saarstedt) gewonnen, die bei 4’C mit 3000 g für 20 min gekühlt zentrifugiert wurden. Fibrinogen (Fib, Clauss Methode, Testkit Behringwerke; Marburg) und Anti thrombin III (AT; enzymatische Messung; Chromogenix; Mölndal,
Schweden) wurden sofort bestimmt, das übrige Plasma in Aliquots bei - 8o °C in Plastikröhrchen gelagert. Prothrombinfragmente F I + 2 (F 1 + 2; EnzygnostR F I + 2, Behringwerke), D Dimere (D-D, EnzygnostR D-Dimer micro, Behringwerke), Antithrombinserinesterasekomplexe (ATM,ATM, Stago; Asnieres, France), Plasminogen-Aktivator Inhibitor- i-Antigen (PAI-I) (PAI-i, Chromogenix), Complement-I-Inhibitor (C I-I) (Ci-I Partigen, Behringwerke) und gewebeständiger Plasminogenaktivator (t PA) (Tint Elize t PA, Biopool; Schweden) wurden seriell in Doppelbestimmun gen innerhalb von 4 Wochen untersucht. Kontrollbestimmungen erfolgten mit Poolplasma gesunder Kinder, Kallibrationsplasma sowie Normalkontrollplasma. Die statistischen Berechnungen erfolgten mit einem Apple Macintosh Computer mit dem Stat-view-4.02-Programm, zum Einsatz kamen nichtparametrische Verfahren sowie Wilcoxon-Test und Spearman-Korrelationen.
Patienten
Anzahl: 32 Patienten, Geschlecht: 19 männlich, 13 weiblich; Alter bei Operation: 1,10 ± 3 Jahre (Range o,1-15); Gewicht bei Operation: 9 ± ii kg (Range 1,7 -6 o); azyanotisch: 21 Patienten; zyanotisch: 11 Patienten; Dauer des CPB: 65 ± 28 min; Aprotinindosis: 2 - 17 000 IU/kg KG.
Ergebnisse: Abnorme Blutungen oder Thrombembolien traten in unserer Studie nicht auf. Die Hämotokritwerte zeigten keine signifi kante Differenzen [%]: 39 ± 2 präope rativ gegenüber 38,8 ± 3,2 postoperativ und 40 ± 5, 40,7 ± 4,7, 40 ± 4, 36 ± 2,42 ± 3,8 an den Tagen 1, 2, 3, 4 bis 6 und 7 bis 9. Die Thrombozyten waren signifikant vermindert ( 109/1): 338 ± 68 präoperativ gegenüber 130 ± 64 postoperativ und 173 ± 59,124 ± 18,117 ± 38,135 ± 40,121 ± 41 an den Tagen 1, 2, 3, 4 bis 6 sowie 7 bis 9. Abbildung 1a-h zeigt die Gerinnungsparameter im perioperativem Verlauf.
Acht Kinder entwickelten ein Gefäßlecksyndrom [3]. Drei von diesen Kindern mit klinisch schwerem Verlauf und den niedrigsten Werten von C 1-1 (=,098 g/1; 0,14 g/1; 0,167 g/1) wurden mit C 1-Inhibitorkonzentrat (Berinert HS, Behring werke; Marburg) substitutiert; 2 mit 2 - 500 LU.; ein Patient mit 2 - l000 I.U. Der klinische Befund und der Katecholaminbedarf besserten sich danach innerhalb von 48 h. Im weiteren Verlauf verstarben dennoch 2 dieser Patienten an Leberver sagen bei Low cardiac output (ein Kind mit hypoplastischem Linksherzsyndrom 4o Tage nach Norwood-II-Operation; ein Kind mit Double inlet left ventricle 24 Tage nach Fontan-Operation).

Schlußfolgerung: Obwohl Aprotinin beim kardiopulmonalem Bypass verwendet wurde, finden sich bei Säuglingen und Kindern postoperativ eine gesteigerte Gerinnung mit erhöh ten F 1 + 2, D-Dimeren, ATM und PAI-1 sowie ein Verbrauch von Gerinnungsfak toren wie AT III, Fibrinogen und C 1-Inhibitor im Sinne einer disseminierten intravasalen Gerinnung.

BibTeX:

@incollection{Kehl_Scharrer_Hamburg1997,

  author = {Kehl, H. G. and Kececioglu, D. and Nekarda, T. and Vielhaber, H. and Gehrmann, J. and Vogt, J. and Nowak-Göttl, U.},

  editor = {Scharrer, I. and Schramm, W.},

  title = {Zeichen der aktivierten Gerinnung und Fibrinolyse nach kardio- pulmonalem Bypass mit Aprotinin},

  booktitle = {26. Hämophilie Symposium Hamburg 1995},

  publisher = {Springer Verlag, ISBN 978-3540611011},

  year = {1997},

  pages = {236--240},

  url = {http://amazon.de/o/ASIN/3540611010/}

}

Nekarda T, Kececioglu D, Kehl HG, Gehrmann J, Miny P and Vogt J, "Eine seltene Kombination einer partiellen Trisomie 9 mit einer Pulmonalatresie.", Klin Padiatr., 1997, Vol. 209(3) : 127-129.
Ranking: 0,307
Abstract: We report of an previously unpublished combination of partial trisomy 9 and a membranous pulmonary atresia with a large conotruncal ventricular septal defect. The dystrophic female, term newborn presented after delivery with microcephaly, prominent nose and several other facial and skeletal deformities. The echocardiography and angiography showed a membranous pulmonary atresia with ventricular septal defect. Chromosomal analysis revealed a partial trisomy of the short arm with parts of the long arm of chromosome 9 and a small part of the long arm of chromosome 4. A surgical repair of the heart defect was not performed by the known high risk of severe mental retardation of partial trisomy 9. The child died at the age of six months.
BibTeX:

@article{Nekarda_KlinPad1997,

  author = {T. Nekarda and D. Kececioglu and H. G. Kehl and J. Gehrmann and P. Miny and J. Vogt},

  title = {Eine seltene Kombination einer partiellen Trisomie 9 mit einer Pulmonalatresie.},

  journal = {Klin Padiatr},

  year = {1997},

  volume = {209},

  number = {3},

  pages = {127--129},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/9244820},

  doi = {10.1055/s-2008-1043941}

}

Vielhaber H, Kohlhase. B, Kehl HG, Fliedner M, Kececioglu D, Veltmann H, Vogt J and Nowak-Göttl U, "Flush heparin infusion during cardiac catheterization in childhood prevents coagulation and fibrinolytic activation", In 26. Hämophilie Symposium Hamburg 1995. Berlin, Heidelberg, 1997, : 254-258. Springer Verlag, ISBN 978-3540611011.
BibTeX:

@incollection{Vielhaber_Hamburg1997,

  author = {Vielhaber, H. and Kohlhase. B. and Kehl, H. G. and Fliedner, M. and Kececioglu, D. and Veltmann, H. and Vogt, J. and Nowak-Göttl, U.},

  editor = {Scharrer, I. and Schramm, W.},

  title = {Flush heparin infusion during cardiac catheterization in childhood prevents coagulation and fibrinolytic activation},

  booktitle = {26. Hämophilie Symposium Hamburg 1995},

  publisher = {Springer Verlag, ISBN 978-3540611011},

  year = {1997},

  pages = {254--258},

  url = {http://amazon.de/o/ASIN/3540611010/}

}

Weyand M, Kececioglu D, Schmid C, Kehl HG, Tandler R, Loick HM and Scheld HH, "Successful bridging to cardiac transplantation in a dystrophic infant with the use of a new paracorporeal pneumatic pump.", J Thorac Cardiovasc Surg., 1997, Vol. 114(3) : 505-507.
Ranking: 3,068
BibTeX:

@article{Weyand_Medos_J_Thoracic_CardioVascSurg1997,

  author = {M. Weyand and D. Kececioglu and C. Schmid and H. G. Kehl and R. Tandler and H. M. Loick and H. H. Scheld},

  title = {Successful bridging to cardiac transplantation in a dystrophic infant with the use of a new paracorporeal pneumatic pump.},

  journal = {J Thorac Cardiovasc Surg},

  year = {1997},

  volume = {114},

  number = {3},

  pages = {505--507},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/9305212},

  doi = {10.1016/S0022-5223(97)70206-X}

}

1996 Asfour B, Bruker B, Kehl HG, Fründ S and Scheld HH, "Renal insufficiency in neonates after cardiac surgery.", Clin Nephrol., 1996, Vol. 46(1) : 59-63.
Ranking: 1,643
Abstract: Renal failure after cardiac surgery using cardiopulmonary bypass (CPB) is well understood for infants, children and adults. The perioperative risk factors after CPB for immature kidneys in newborns are not well known. This retrospective study investigates perioperative risk factors for renal insufficiency in neonates. I) Preoperative: Age; weight, performed angiography, amount of dye used in angiography, renal disease and creatinine. II) Intraoperative: Duration of operation, duration of MAP < 40 mmHg, use of deep hypothermia, in-out fluid balance, duration of CPB, duration of circulatory arrest and cross-clamp time. III) Postoperative: Creatinine, use of catecholamines, use of nitroglycerine (NG) or phosphodiesterase inhibitors (PDI) and additional antibiotics. From Jan. 1990 to Dec. 1994 50 neonates underwent cardiac surgery using CPB (n = 23 transposition of the great arteries; n = 4 pulmonary atresia; n = 6 critical pulmonary stenosis; n = 5 hypoplastic left heart syndrome; n = 3 Ebstein's anomaly; n = 2 interrupted arch with hypoplastic left ventricle; n = 2 single ventricle; n = 1 each: double outlet right ventricle, tricuspid atresia, critical aortic stenosis, rhabdo-myosarkoma, corrected transposition of the great arteries.) Thirty-one patients entered the study. Depending on the postoperative creatinine level two groups (group I: creatinine <1 mg/dl and group II: >1 mg/dl) were created. The diureses between the two groups did not differ. Comparing the patients of group I vs. group II, patients of group I were younger (mean age: 7.7 d. vs. 11.4 d), lighter (mean weight: 3260 g vs. 3430 g), less had angiography (44% vs. 77, received more dye (mean amount: 14 ml vs. 7 ml), the duration of MAP < 40 mmHg while on CPB was longer (mean duration 3 min vs. 21 min), more patients were operated on using deep hypothermia (55% vs. 27, the postoperative in-out-fluid balance was more positive (mean balance +413 ml vs. +221 ml), received postop. more frequently high doses of catocholamines and less common NG or PDI, but more often additional antibiotics. The duration of circulatory arrest (mean time: 60 min vs. 55 min) and cross clamp time (mean time: 68 min vs. 65 min) seems not to be a risk factor and vasodilators given simultaneously with catecholamines may have preventive effects on postoperative renal insufficiency. Immature kidneys may play an outstanding role in the susceptibility of damaging factors. Further investigation with a larger number of patients allowing to obtain statistical significant risk factors are required.
BibTeX:

@article{Asfour_ClinicalNephrology,

  author = {B. Asfour and B. Bruker and H. G. Kehl and S. Fründ and H. H. Scheld},

  title = {Renal insufficiency in neonates after cardiac surgery.},

  journal = {Clin Nephrol},

  year = {1996},

  volume = {46},

  number = {1},

  pages = {59--63},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/8832154},

  doi = {http://www.scopus.com/inward/record.url?eid=2-s2.0-0029931254\&partnerID=40}

}

Kececioglu D, Gehrmann J, Kehl HG, Kohl T, Vielhaber H, Weyand M and Vogt J, "Pädiatrische transösophageale Echocardiographie", Herz Kreislauf., 1996, Vol. 28(2) : 43-47.
Ranking: 0,146
Abstract: 291 transesophageal echocardiographic studies (TEE) were performed in 247 children: 31 in the intensive care unit, 35 in the cardiac catheterization laboratory and 225 intraoperatively (190 pre-, 217 postoperatively).In the cardiac catheterization laboratory we obtained new diagnostic information in 29 of 35 TEE (83%). 7 of 31 TEE (22%) revealed significant findings with therapeutic consequences. The results of surgery could be assessed completely by postoperative TEE (217) in simple (120) in 86% and complex congenital heart defects (97) in 83% of the cases. 5 re-operations (2.3%) were performed based on postoperative TEE. TEE should be used routinely for the assessment of surgical repair of congenital heart defects, for evaluation of postoperative complications and for monitoring of interventions in the catheterization laboratory.
BibTeX:

@article{Keci_TEE_HerzKreisl1996,

  author = {Kececioglu, D. and Gehrmann, J. and Kehl, H. G. and Kohl, T. and Vielhaber, H. and Weyand, M. and Vogt, J.},

  title = {Pädiatrische transösophageale Echocardiographie},

  journal = {Herz Kreislauf},

  year = {1996},

  volume = {28},

  number = {2},

  pages = {43--47},

  url = {http://www.scopus.com/inward/record.url?eid=2-s2.0-0030071065&partnerID=40}

}

Kececioglu D, Kehl HG, Weyand M, Gehrmann J, Scheld H and Vogt J, "Vorteile und Risiken der intraoperativen transösophageale Echocardiographie im Kindesalter", Zeitschrift für Herz-,Thorax- und Gefäßchirurgie., 1996, Vol. 10(2) : 296-302.
Abstract: Zur Bestimmung der Sicherheit und des Stellenwertes einer routinemäßigen intraoperativen transösophagealen Echokardiographie (TEE) wurden 500 Untersuchungen an Kindern mit angeborenen Herzfehlern im Alter von 2 Tagen bis 17 Jahren (im Mittel 4,9 Jahre) analysiert. Ernsthafte Komplikationen wurden bei zwei Kindern (0,4%) festgestellt; unter Einschluß leichter Probleme betrug die totale Komplikationsrate 1,4%. Aufgrund eines mit der TEE diagnostizierten Restdefektes wurde bei 27 von 500 Kindern (5,4%) eine erneute Korrektur durchgeführt. Von diesen Kindern waren 10 (37%) entweder vom kardiopulmonalen Bypass nicht zu entwöhnen oder mußten wegen Kreislaufversagens erneut an den Bypass angeschlossen werden. Bei allen reoperierten Kindern wurde die TEE-Diagnose durch den Chirurgen bestätigt. In drei Fallen wurde mit der TEE ein bedeutsamer Restdefekt übersehen. Die Sensitivität der TEE hinsichtlich des Nachweises von reoperationspftichtigen Restdefekten betrug 90%, die Spezifizität 100%. Diese Ergebnisse belegen, daß mit der TEE als eine komplikationsarme und diagnostisch zuverlässige Methode bedeutsame Restdefekte bereits intraoperativ erkannt und dadurch spätere Re-Operationen vermieden werden können. Daher sollte die TEE routinemäßig während Korrekturoperationen kongenitaler Herzfehler eingesetzt werden.
BibTeX:

@article{Keci_TEE_ZeitschrTHG_1996,

  author = {Kececioglu, D. and Kehl, H. G. and Weyand, M. and Gehrmann, J. and Scheld, H.H. and Vogt, J.},

  title = {Vorteile und Risiken der intraoperativen transösophageale Echocardiographie im Kindesalter},

  journal = {Zeitschrift für Herz-,Thorax- und Gefäßchirurgie},

  year = {1996},

  volume = {10},

  number = {2},

  pages = {296--302}

}

Kehl HG, Vielhaber H and Nowak-Göttl U, "Thrombolytische Therapieformen im Kindesalter. Eine Übersicht", Häaemostaseologie., 1996, Vol. 16 : 212-216.
Abstract: Thrombembolism in childhood is a serious event. Beside primary or secondary defects of clotting inhibitors, the increased use of central lines in pediatric intensive care, catheter interventions in pediatric cardiology, and modern trials in pediatric oncology were followed by thrombosis. Experience in thrombolytic therapy in infants and children is limited. Thrombolysis of acute arterial occlusion after retrograde cardiac catheterisation is the best proven indication: lysis was successful in 147 of 164 (90%) patients from ten studies, local bleeding occurred in 41 of 164 patients (25%). Experience with treatment of thrombosis of renal veins, right atrium, pulmonary arteries, or left atrium and left ventricle is available only in small series or case reports.
BibTeX:

@article{Kehl_Haemostasiol1996,

  author = {Kehl, H. G. and Vielhaber, H. and Nowak-Göttl, U.},

  title = {Thrombolytische Therapieformen im Kindesalter. Eine Übersicht},

  journal = {Häaemostaseologie},

  year = {1996},

  volume = {16},

  pages = {212--216},

  url = {http://www.scopus.com/inward/record.url?eid=2-s2.0-0030443471&partnerID=40}

}

Kehl HG, Kececioglu D, Vielhaber H, Kotthoff S, Weyand M, Jorch G, Vogt J and Nowak-Göttl U, "Left atrial thrombus in a 10-month-old boy--successful thrombolysis with recombinant tissue-type plasminogen activator after open-heart surgery: review of the literature.", Intensive Care Med., 1996, Vol. 22(9) : 968-971.
Ranking: 2,323
Abstract: A 10-month-old boy with major left atrial thrombus following cardiac surgery was treated with intravenously administered recombinant tissue-type plasminogen activator (rt-PA; Actilyse, Thomae-Behring, Germany). The left atrial thrombus was diagnosed by Doppler echocardiography 8 days after complete correction of a ventricular septal defect. rt-PA therapy was administered over a 10-day period. Significant hemopericardium occurred 50 h after the start of thrombolytic therapy. rt-PA infusion was discontinued for 20 h to insert a pericardial drainage. The initial rt-PA dose was 0.1 mg/kg over 10 min followed by a continuous daily infusion of 1.7 mg/kg together with low-dose heparin. Thrombolytic therapy was restarted 20 h after pericardial drainage was inserted. The daily rt-PA dose was gradually raised to 3 mg/kg (total dose: 18 mg/kg). On day 7 and 8 a clear decrease in P-plasminogen and P-antithrombin occurred, requiring additional fresh frozen plasma and P-antithrombin concentrate substitution. One day later, without further side effects, complete thrombolysis occurred. Although hemopericardium demanded discontinuation of thrombolytic therapy, rt-PA administration, closely monitored by Doppler echocardiography, was continued, leading to complete thrombolysis of the left atrial thrombus in the early postoperative period. We consider the literature dealing with rt-PA thrombolysis in infancy we discuss this case report.
BibTeX:

@article{Kehl_IntCareMed1996,

  author = {H. G. Kehl and D. Kececioglu and H. Vielhaber and S. Kotthoff and M. Weyand and G. Jorch and J. Vogt and U. Nowak-Göttl},

  title = {Left atrial thrombus in a 10-month-old boy--successful thrombolysis with recombinant tissue-type plasminogen activator after open-heart surgery: review of the literature.},

  journal = {Intensive Care Med},

  year = {1996},

  volume = {22},

  number = {9},

  pages = {968--971},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/8905435},

  doi = {10.1007/BF02044125}

}

Kohlhase B, Vielhaber H, Kehl HG, Kececioglu D, Koch HG and Nowak-Göttl U, "Thromboembolism and resistance to activated protein C in children with underlying cardiac disease.", J Pediatr., 1996, Vol. 129(5) : 677-679.
Ranking: 3,011
Abstract: OBJECTIVES: In the majority of cases, resistance to activated protein C is caused by the point mutation Arg506 to Gln in the factor V gene and has emerged as the most important hereditary cause of thromboembolism. METHODS: To determine to what extent resistance to activated protein C was present in children with thromboembolism and underlying cardiac disease, its occurrence was retrospectively investigated. By using a method based on activated partial thromboplastin time, with DNA technique derived from the polymerase chain reaction, we investigated nine children with underlying cardiac disease in whom thromboembolism had previously occurred. RESULTS: Heterozygous Arg506-to-Gln mutation in the factor V gene was diagnosed in five of the nine children investigated. In addition, protein C type I deficiency w as found in three patients, and two of the nine children showed increased lipoprotein (a) plasma values. Risk factors were present in all children with symptoms. CONCLUSIONS: These data indicate that deficiencies in the protein C anticoagulant pathway are likely to play an important role in the early manifestation of thromboembolism in children with underlying cardiac disease.
BibTeX:

@article{Kohlhase_J_Pediatr1996,

  author = {B. Kohlhase and H. Vielhaber and H. G. Kehl and D. Kececioglu and H. G. Koch and U. Nowak-Göttl},

  title = {Thromboembolism and resistance to activated protein C in children with underlying cardiac disease.},

  journal = {J Pediatr},

  year = {1996},

  volume = {129},

  number = {5},

  pages = {677--679},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/8917233},

  doi = {10.1016/S0022-3476(96)70149-X}

}

Nowak-Göttl U, Koch HG, Aschka I, Kohlhase B, Vielhaber H, Kurlemann G, Oleszcuk-Raschke K, Kehl HG, Jürgens H and Schneppenheim R, "Resistance to activated protein C (APCR) in children with venous or arterial thromboembolism.", Br J Haematol., 1996, Vol. 92(4) : 992-998.
Ranking: 3,065
Abstract: Resistance to activated protein C (APCR), in the majority of cases due to the point mutation Arg 506 Gln of the factor V gene, has emerged as the most important hereditary cause of venous thromboembolism. Using an activated thromboplastin time (aPTT) based method in the presence of APC together with a DNA technique based on the polymerase chain reaction, we investigated 37 children with venous (V: n=19) or arterial (A: n=18) thromboembolism and 196 age-matched healthy controls for the presence of this mutation. In the control group 10 children were detected to be heterozygous for the factor V Leiden mutation, indicating a prevalence of 5.1 10/19 children (52 with venous thrombosis and 7/18 (38 patients with arterial thromboembolism showed the common factor V gene mutation. Additional inherited coagulation disorders were found in 1/10 (V:10 and 2/7 (A:28 APC-resistant patients. Inherited coagulation disorders without APCR were diagnosed in 3/9 (V: 33 and 2/11 (A:18 children. Furthermore, we diagnosed exogenous risk factors in 6/10 (V: 60 and 2/7 (A: 28 children with thrombosis and APCR. These data are evidence that APCR combined with exogenous reasons may play an important role in the early manifestation of thromboembolism during infancy and childhood.
BibTeX:

@article{NowakGottl_BrJHaematol1996,

  author = {Nowak-Göttl, U. and H. G. Koch and I. Aschka and B. Kohlhase and H. Vielhaber and G. Kurlemann and K. Oleszcuk-Raschke and H. G. Kehl and H. Jürgens and R. Schneppenheim},

  title = {Resistance to activated protein C (APCR) in children with venous or arterial thromboembolism.},

  journal = {Br J Haematol},

  year = {1996},

  volume = {92},

  number = {4},

  pages = {992--998},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/8616099},

  doi = {10.1046/j.1365-2141.1996.424957.x}

}

Nowak-Göttl U, Kehl HG and Vielhaber H, "Laborkontrollen vor, während und nach einer Thrombolysetherapie im Kindesalter", Häaemostaseologie., 1996, Vol. 16 : 217-219.
Abstract: Childhood thromboembolism is serious enough to require effective and safe intervention. Thrombolytic therapy administered to neonates and children should be accompanied by compulsory laboratory monitoring. To evaluate possible bleeding disorders, haemoglobin (hb), platelet count (PC), PT, aPTT, fibrinogen, antithrombin, plasminogen and D-Dimer should be measured before thrombolytic therapy. To control thrombolysis and anticoagulation administered Hb, PC, aPTT, antithrombin, plasminogen and D-Dimer should be assayed during therapy. APTT, antithrombin along with D-Dimer formation should be analysed to prevent reocclusion when thrombolysis was stopped.
BibTeX:

@article{NowakGottl_Haemostasioll1996,

  author = {Nowak-Göttl, U. and Kehl, H. G. and Vielhaber, H.},

  title = {Laborkontrollen vor, während und nach einer Thrombolysetherapie im Kindesalter},

  journal = {Häaemostaseologie},

  year = {1996},

  volume = {16},

  pages = {217--219},

  url = {http://www.scopus.com/inward/record.url?eid=2-s2.0-0030443055&partnerID=40}

}

Vielhaber H, Kehl HG, Kececioglu D, Vogt J and Nowak-Göttl U, "Enhanced soluble thrombomodulin, t-PA and u-PA concentrations caused by short-term endothelial damage during percutaneous cardiac catheterisation", Fibrinolysis., 1996, Vol. 10 Suppl. 2 : 47-49.
Abstract: Objective: circulating plasma thrombomodulin (TM), tissue-type plasminogen activator (t-PA) and urokinase-type plasminogen activator (u-PA) are endothelial cell markers which may reflect endothelial injury. To find out to what extent diagnostic cardiac percutaneous catheterisation irritates vascular endothelium we conducted a prospective study in 114 children. Results: compared to starting values TM, t-PA and u-PA concentrations show a clearly significant increase at the end of cardiac catheterisation. TM and u-PA returned to pretreatment values 24 hours later, t-PA remained elevated. Four of 114 children developed vascular occlusion near the puncture sites within 48 hours of cardiac catheterisation. Two patients < 1 year of age showed resistance to APC, one child with recurrent thromboembolism showed familial thrombophilia with elevated lipoprotein (a) levels. Conclusions: data of this study indicate that increased TM, t-PA and u-PA concentrations after percutaneous cardiac catheterisation in children are signs of endothelial damage.
BibTeX:

@article{Vielhaber_Fibrinolysis,

  author = {Vielhaber, H. and Kehl, H. G. and Kececioglu, D. and Vogt, J. and Nowak-Göttl, U.},

  title = {Enhanced soluble thrombomodulin, t-PA and u-PA concentrations caused by short-term endothelial damage during percutaneous cardiac catheterisation},

  journal = {Fibrinolysis},

  year = {1996},

  volume = {10 Suppl. 2},

  pages = {47--49},

  doi = {10.1016/S0268-9499(96)80046-4}

}

Vielhaber H, Kohlhase B, Kehl HG, Kececioglu D, Koch H and Nowak-Göttl U, "Apcr and thromboembolism in children with underlying cardiac disease", Fibrinolysis., 1996, Vol. 10 Suppl. 3(SUPPL. 3) : 134.
Abstract: Objectives: Resistance to activated protein C (APCR), in the majority of cases due to the point mutation Arg 506 Gin of the factor V gene, has emerged as the most important hereditary cause of venous thromboembolism. Study design: To determine to what extent APCR was present in children with underlying cardiac disease nine out of 130 children in whom thromboembolism had occurred (ASD n=l, VSD n=2, TrA and TGA n=l, TGA n=l, TOP n=l, DORV n=l) during an 15months period were investigated. One out of eight patients admitted showed a positive history of well documented unexplained familial thrombophilia. Using an aPTT-based method in the presence of APC together with DNA technique based on polymerase chain reaction, we investigated these patients with venous (n=3) or arterial (n=6) thromboembolism for the presence of this mutation. In addition, antithrombin, protein C, protein S and factor V were analysed in these patients. Results: One child with venous thrombosis and four out of six children with arterial thromboembolism showed APCR. Furthermore, one child with stroke and one patient with venous thrombosis showed heterozygous protein C deficiency. Conclusion: These data are evidence that APCR or inherited thrombophilia may play an important role in the early manifestation of thromboembolism in children with underlying cardiac disease. In addition, the occurrence of APCR in four out of six patients with arterial insults challenge the view that APCR is associated with venous thrombosis but not with arterial vascular occlusion.
BibTeX:

@article{Vielhaber_Fibrinolysis_1996a,

  author = {Vielhaber, H. and Kohlhase, B. and Kehl, H. G. and Kececioglu, D. and Koch, H.G. and Nowak-Göttl, U.},

  title = {Apcr and thromboembolism in children with underlying cardiac disease},

  journal = {Fibrinolysis},

  year = {1996},

  volume = {10 Suppl. 3},

  number = {SUPPL. 3},

  pages = {134},

  url = {http://www.scopus.com/inward/record.url?eid=2-s2.0-33846681416&partnerID=40}

}

Vielhaber H, Kehl HG, Kececioglu D and Nowak-Göttl U, "Plasma thrombomodulin concentrations in infants and children undergoing cardiac catheterization.", Haematologica., 1996, Vol. 81(5) : 457-459.
Ranking: 1,403
Abstract: Circulating plasma thrombomodulin (TM) is an endothelial cell marker which may reflect endothelial injury. To find out to what extent diagnostic cardiac catheterization irritates vascular endothelium we conducted a prospective study in 91 children. Soluble TM concentrations, along with thrombin generation, were measured before, at the end of and 24 hours after cardiac catheterization. Compared to starting values, TM concentrations showed a clearly significant increase at the end of cardiac catheterization and returned to pretreatment values 24 hours later. Thrombin generation followed a similar pattern. Five out of the 91 children demonstrated resistance to activated protein C (APCR). With respect to the remaining 86 children, all five APCR cases showed increased thrombomodulin concentrations along with enhanced thrombin generation. Data from this study indicate that increased TM concentrations after cardiac catheterization in children are a sign of short-term endothelial damage. Furthermore, together with enhanced thrombin generation, elevated plasma concentration of soluble TM may reflect this receptor's possible anticoagulant properties.
BibTeX:

@article{Vielhaber_Haematologica_96,

  author = {H. Vielhaber and H. G. Kehl and D. Kececioglu and U. Nowak-Göttl},

  title = {Plasma thrombomodulin concentrations in infants and children undergoing cardiac catheterization.},

  journal = {Haematologica},

  year = {1996},

  volume = {81},

  number = {5},

  pages = {457--459},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/8952160},

  doi = {http://www.haematologica.org/cgi/reprint/81/5/457}

}

Vielhaber H, Kohlhase B, Koch HG, Kehl HG, Fliedner M, Kececioglu D, Kehrel B, Veltmann H, Vogt J and Nowak-Göttl U, "Flush heparin during cardiac catheterisation prevents long-term coagulation activation in children without APC-resistance-preliminary results.", Thromb Res., 1996, Vol. 81(6) : 651-656.
Ranking: 1,420
Abstract: This study was designed to prospectively evaluate haemostatic activation in 75 children undergoing cardiac catheterisation with intermittent flush heparin (10 IU/ml saline) and to relate these data to clinical findings and inherited risk factors for thrombophilia. In addition to flush heparin in infants < 6 months of age in whom additional arterial catheterisation was performed (n = 5) or patients with thrombophilia, heparin (300-400 IU/kg/d) was administered for a further 24 h. APTT was prolonged and anti Xa activity was significantly increased at the end of catheterisation and returned to normal 24 hours later. Whereas thrombin generation (F1 + 2) showed a significant coagulation activation at the end of catheterisation, no concomitant fibrinolytic activation (D-Dimer) was observed. Four children showed resistance to APC: one of them in whom stroke had occurred before and one additional child heterozygous for APCR received further prophylactic heparin. Two neonates with APCR and flush heparin only suffered from thrombosis after catheterisation. No further thrombotic events occurred. This study indicates that low-dose flush heparin during catheterisation may prevent long-term haemostatic activation in children without thrombophilia. Whether further heparin after cardiac catheterisation in children with APCR prevents vascular insults requires a more intensive study.
BibTeX:

@article{Vielhaber_ThrombRes1996,

  author = {H. Vielhaber and B. Kohlhase and H. G. Koch and H. G. Kehl and M. Fliedner and D. Kececioglu and B. Kehrel and H. Veltmann and J. Vogt and U. Nowak-Göttl},

  title = {Flush heparin during cardiac catheterisation prevents long-term coagulation activation in children without APC-resistance-preliminary results.},

  journal = {Thromb Res},

  year = {1996},

  volume = {81},

  number = {6},

  pages = {651--656},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/8868515},

  doi = {10.1016/0049-3848(96)00041-2}

}

1995 Kehl HG, Kececioglu D, Gehrmann J, Nekarda T and Nowak-Göttl U, "Increased F1+2, D-Dimer, ATM and PAI-1 with Evidence of DIC after Cardiopulmonary - Bypass Operation with Aprotinin in Infancy - a Prospective-Study", Thrombosis and Haemostasis., 1995, Vol. 73(6) : 1296-1296.
BibTeX:

@article{Kehl1995,

  author = {Kehl, H. G. and Kececioglu, D. and Gehrmann, J. and Nekarda, T. and Nowak-Göttl, U.},

  title = {Increased F1+2, D-Dimer, ATM and PAI-1 with Evidence of DIC after Cardiopulmonary - Bypass Operation with Aprotinin in Infancy - a Prospective-Study},

  journal = {Thrombosis and Haemostasis},

  year = {1995},

  volume = {73},

  number = {6},

  pages = {1296--1296},

  doi = {ISI:A1995RP38501517}

}

Kehl HG, Hentschel R, Gehrmann J, Rossi R, Hulskamp G, Kececioglu D and Jorch G, "Surfactant treatment in ARDS", Biology of the Neonate., 1995, Vol. 67(4) : 306-306.
BibTeX:

@article{Kehl1995a,

  author = {Kehl, H. G. and Hentschel, R. and Gehrmann, J. and Rossi, R. and Hulskamp, G. and Kececioglu, D. and Jorch, G.},

  title = {Surfactant treatment in ARDS},

  journal = {Biology of the Neonate},

  year = {1995},

  volume = {67},

  number = {4},

  pages = {306--306},

  url = {https://www.karger.com/Article/Abstract/244178},

  doi = {10.1159/000244178; 10.1159/000244179}

}

Vielhaber H, Kehl G, Kotthoff S, Jorch G and Nowak-Goettl U, "Thrombolysis with rt-PA in a ten-month old boy suffering from a left atrial thrombus after open heart surgery", Annals of Hematology., 1995, Vol. 70(SUPPL. 1) : A17-A17.
BibTeX:

@article{Vielhaber1995,

  author = {Vielhaber, H. and Kehl, G. and Kotthoff, S. and Jorch, G. and Nowak-Goettl, U.},

  title = {Thrombolysis with rt-PA in a ten-month old boy suffering from a left atrial thrombus after open heart surgery},

  journal = {Annals of Hematology},

  year = {1995},

  volume = {70},

  number = {SUPPL. 1},

  pages = {A17--A17},

  url = {http://link.springer.com/content/pdf/10.1007%2FBF01725155.pdf}

}

1994 Kececioglu D, Vogt J, Kotthoff S, Kehl G and Scheld HH, "Perioperative changes of cardiac areas after repair of congenital lesions: Quantitation by intra-operative transoesophageal echocardiography", European Heart Journal., 1994, Vol. 15(Suppl 3.) : 376-376.
Abstract: The aim of this study was to assess the ability of TEE demonstrate irrtracardiac volume changes after repair of lesions wtth Intrecardlac shunting by changing cardiac areas on echocardiography.
25 patients with atrial septal defect (ASD), 16 pat with ventricular septal defect (VSD) and 12 pat with tetralogy of Fallot (TOF) were studied perioperatively before stemotomy (pr). at sternal closure (sc) and at the Intensive car unit (in) by TEE: Manually traced TEE Image3 of the cardiac areas were used to determine perioperativa volume changes.Repair of ASD resulted in a significant decrease of the right atria I area (pr: 11.9 cm2 , sc:6.6 cm2 , in:6.9 cm2) and right ventricular area (pr: 10.7 cm2, sc:6.0 cm2, in:6.5cm2).
Repair of VSD resulted m decreasing areas of the left atrium (pr: 4.7 cm2, sc:3.7 cm2, in:3.0 cm2), of the right ventricle in diastole (pr 6.4 cm2, sc:4.9 cm2, in:4.4 cm2) and of the left ventricle in systole (pr: 4.7 cm2, sc:4.4 cm2, in:3.5cm2). After repair of TOF TEE showed a moderate reduction of the right ventricle (pr 5.8 cm2, sc:4.9 cm2) and left ventricle (pr 5.7 cm2, sc:5.0 cm2).
We conclude that penoperatrve TEE may detect early changes of cardiac filling during repair of congenital lesions. These volume changes after repair of shunt lesions should be considered, when the postoperative cardiac filling status is monitored by TEE.
BibTeX:

@article{Kececioglu1994,

  author = {Kececioglu, Deniz and Vogt, Johannes and Kotthoff, Stefan and Kehl, Gerhard and Scheld, Hans H.},

  title = {Perioperative changes of cardiac areas after repair of congenital lesions: Quantitation by intra-operative transoesophageal echocardiography},

  journal = {European Heart Journal},

  year = {1994},

  volume = {15},

  number = {Suppl 3.},

  pages = {376--376},

  doi = {10.1093/eurheartj/15.suppl_3.373}

}

Moskopp D, Kehl G, Horch C, Puskás Z, Wassmann H, Schuierer G and Fingerhut D, "Increased intracranial pressure and cardiac arrest after heart transplantation. What about the Cushing response in a denervated heart? Case report.", Neurosurg Rev., 1994, Vol. 17(2) : 151-156.
Ranking: 0,256
Abstract: A 13-year-old boy suffered cerebrovascular complications after heart transplantation (ischemic mass effect in the posterior cranial fossa). He had to be resuscitated from cardiac arrest with coma. After a modified cerebellar hemispherectomy the course was favorable.--The most conclusive explanation for the acute event is that a Cushing response was preserved even in the presumably denervated heart.
BibTeX:

@article{Moskopp_Neurosurg1994,

  author = {D. Moskopp and G. Kehl and C. Horch and Z. Puskás and H. Wassmann and G. Schuierer and D. Fingerhut},

  title = {Increased intracranial pressure and cardiac arrest after heart transplantation. What about the Cushing response in a denervated heart? Case report.},

  journal = {Neurosurg Rev},

  year = {1994},

  volume = {17},

  number = {2},

  pages = {151--156},

  url = {http://www.ncbi.nlm.nih.gov/pubmed/7970021},

  doi = {10.1007/BF00698772}

}

1993 Egbring R, Kailing A, Kehl HG, Seitz R, Maasberg M, Holst F, Fuchs G, Wallin R and Saldeen T, "Chapter: Disorders with severe aquired factor XIII deficiency: Lack of synthesis or increased consumption (DIC). Efficacy of factor XIII replacement in bleeding complications. S. 216-240 In: Mc Donagh J, Seitz R, Egbring R (Eds.) Factor XIII: Second International Conference Marburg, July 9-10, 1991" John Wiley & Sons Inc, ISBN 9780471307860, 1993, : 216-240.
BibTeX:

@book{McDonaghSeitzEgbring199305,

  author = {Egbring, R. and Kailing, A. and Kehl, H. G. and Seitz, R. and Maasberg, M. and Holst, F. and Fuchs, G. and Wallin, R. and Saldeen, T.},

  title = {Chapter: Disorders with severe aquired factor XIII deficiency: Lack of synthesis or increased consumption (DIC). Efficacy of factor XIII replacement in bleeding complications. S. 216-240 In: Mc Donagh J, Seitz R, Egbring R (Eds.) Factor XIII: Second International Conference Marburg, July 9-10, 1991},

  year = {1993},

  pages = {216-240},

  url = {http://amazon.de/o/ASIN/0471307866/}

}

1992 Kececioglu D, Kotthoff S, Kehl HG and Vogt J, "Williams-Beuren's Syndrome with Supravalvular Aortic Stenosis Natural Course and Postoperative Long-term Follow-up", European Heart Journal., 1992, Vol. 13(Suppl.) : 411-411.
Abstract: Reports of 103 pts.(53 male, 50 female) were reviewed. Overall mortality was 8 %. 2 opera- tive deaths; 3 sudden deaths, 1 by car acci- dent, 1 by renal failure, 1 during balloon an- gioplasty of peripheral pulmonary stenosis. 39 pts. were reexomined (age: 16-40y; follow-up: 5-27y) including ECG, exercise testing, 2-d- echo, Doppler and evaluation of the psycho- social status. During 1. cath. RV pressure was elevated in 54/98 pts.(mean pRV 45 mm Hg; range 25-140) and decreased in recath. in 15/ 98 pts. by 23 mm Hg. Mild progression by 10 mm Hg was observed in 4/98 pts. only. Mean aortic gradient in 54/84 cath. pts. was 41 mm Hg (in 30/84 pts. no gradients). In 10/18 recath. pts gradients increased by 32 mm Hg (range 10-75 mm Hg). 25/103 pts. with a mean aortic gra- dient of 72 mm Hg (35-110) were treated sur- gically by insertion of a patch. In 15/25 ope- rated pts. aortic gradient was reduced by 61 mm Hg (30-90). 1 pat. had to be reoperated 8 y p.o. No late deaths occured.
Conclusions:
(1) highly elevated RV pressures may decrease by regression of peripheral pulmonary stenosis
(2) in children even primary low aortic gra- dients may increase considerably.
(3) early and late operative results are good. (4) adult pts. can live a normal life without restrictions.
BibTeX:

@article{Kececioglu1992,

  author = {Kececioglu, D and Kotthoff, S and Kehl, H G and Vogt, J},

  title = {Williams-Beuren's Syndrome with Supravalvular Aortic Stenosis Natural Course and Postoperative Long-term Follow-up},

  journal = {European Heart Journal},

  year = {1992},

  volume = {13},

  number = {Suppl.},

  pages = {411--411},

  url = {http://eurheartj.oxfordjournals.org/content/13/supplement/373.full.pdf+html},

  doi = {10.1093/eurheartj/13.suppl.373}

}

1990 Egbring R, Keiling A, Kehl HG, Seitz R, Wallin R and Saldeen T, "Factor XIII Deficiency In Inflammatory Bowel Systemic Hematological Disorders Morbus Schoenlein Henoch Msh and Bacterial Infections Treatment of Bleeding Complications By Factor XIII Concentrates", Blut., 1990, Vol. 60(2) : 128-128.
BibTeX:

@article{Egbring1990,

  author = {Egbring, R and Keiling, A and Kehl, H G and Seitz, R and Wallin, R and Saldeen, T},

  title = {Factor XIII Deficiency In Inflammatory Bowel Systemic Hematological Disorders Morbus Schoenlein Henoch Msh and Bacterial Infections Treatment of Bleeding Complications By Factor XIII Concentrates},

  journal = {Blut},

  year = {1990},

  volume = {60},

  number = {2},

  pages = {128--128},

  url = {http://link.springer.com/article/10.1007/BF01720517}

}

Kehl HG, "Morbus Crohn und Colitis ulcerosa - Angaben zur Klinik und experimentelle Untersuchungen zur Hämostase". Dissertation, Philipps-Universität Marburg, Signaturen der Deutschen Nationalbibliothek, Katalog Frankfurt: H 90/9315, Katalog Leipzig: Di 1991 A 686., 1990,
Abstract: Morbus Crohn und Colitis ulcerosa sind ätiologisch ungeklärte chronisch-entzündliche Darmerkrankungen (CED) mit einem breiten Spektrum von Begleitmanifestationen.
In vorliegender Arbeit wurden die klinischen und laborchemischen Daten von 49 Patienten mit Morbus Crohn, 31 Patienten mit Colitis ulcerosa und 5 Patienten, die noch nicht in Morbus Crohn oder Colitis ulcerosa differenziert werden konnten, mit den Angaben in der Literatur verglichen. Es zeigte sich eine gute Übereinstimmung in der Alters- und Geschlechtsverteilung, der Erkrankungsdauer, der Ausdehnung des Darmbefalls, der intestinalen und extraintestinalen Komplikationen, der Therapie und der Routinelaborparameter.
Da Morbus Crohn- und Colitis ulcerosa-Patienten ein erhöhtes Thromboserisko haben und oft intestinale Blutungen aufweisen, die gelegentlich sogar massive intestinale Blutungskomplikationen bedingen, wurden in vorliegender Arbeit neben der Messung einer Reihe von Gerinnungsfaktoren und –inhibitoren neue Parameter miterfasst, die Aussagen über Gerinnungs-, Fibrinolyse- und/oder Leukoyzten-Aktivierung zulassen. Bei den neuen Parametern handelt es sich um
1.Intermediärprodukte der Gerinnung in Form von Proteinasen-Inhibitor-Komplexen sowie Prothrombinfragmenten, die Aussagen über eine intravasale Thrombinaktivierung zulassen;
2.Plasmin-Alpha2-Antiplasmin(PAP)-Komplexe, die zusammen mit anderen Parametern Aussagen über eine Fibrinolyseaktivierung erlauben;
3.Elastase-Alpha1-Antitrypsin (ELP-Alpha1-AT)-Komplexe und die Inhibitoren der Elastase (Alpha1-AT, Alpha2-M), weil PMNL bei CED im Bereich der entzündeten Darmwand aktiviert und ihre lysosomalen Enzyme wie die ELP freigesetzt werden und so Gerinnungsfaktoren abbauen können.
Da Morbus Crohn und Colitis ulcerosa verschieden aktiv verlaufen, wurden die Ergebnisse der Gerinnungsuntersuchung denen eines speziellen Aktivierungsindexes gegenübergestellt.
In den untersuchten Patientengruppen traten während des Untersuchungszeitraumes keine Thrombosen oder profusen rektalen Blutverluste auf. Dennoch konnten deutliche Veränderungen der Hämostase nachgewiesen werden.
Eine gesteigerte Gerinnung, erkennbar an deutlich erhöhten Prothrombinfragmenten F1+2 und erhöhten Thrombin-AntithrombinIII-Komplexen, fand sich in mehr als einem Drittel der Morbus Crohn- und einem Viertel der Colitis ulcerosa-Patienten, vorwiegend wenn die Erkrankung nach dem Index aktiv war. Morbus Crohn-Patienten hatten im Mittel wesentlich stärker erhöhte Prothrombinfragmente F1+2 sowie TAT-Komplexe. Die hierdurch erfasste gesteigerte Prothrombinumwandlung, die bei den Patienten gefundene Erhöhung des Fibrinogens und der Thrombozyten sowie die reduzierte spezifische Aktivität des AntithrombinIII sind eindeutig mit einem Thromboserisiko in Einklang zu bringen.
Eine sekundäre Hyperfibrinolyse, erkennbar an erhöhten Plasmin-Alpha2-Antiplasmin(PAP)-Komplexen, bestand bei einem Sechstel der Morbus Crohn– und einem Zehntel der Colitis ulcerosa-Patienten. Die gefundene verminderte Aktivität des Alpha2-AP würde dagegen Blutungskomplikationen durch verminderte Fibrinolyseinhibition – wie dies bei angeborenem heterozygoten Mangel von Alpha2-AP belegt ist – begünstigen. Auch ein Mangel an F.XIII, wie er in 27% festzustellen war, soll bei CED verstärkte Blutungen bedingen; dies und auch die therapeutische Wirksamkeit von FaktorXIII-Konzentrat-Gaben wurde von mehreren Autoren berichtet [23, 171, 191].
Obwohl die ELP-Alpha1-AT-Komplexe bei Patienten mit aktiver CED erhöht sind und die spezifische Aktivität von Alpha1-AT und Alpha2-M erniedrigt nachgewiesen wurde, benötigt man zusätzliche Parameter, um eine ELP-induzierte Proteolyse von Gerinnungsfaktoren wahrscheinlich oder beweisend machen zu können. Hierzu gehören beispielsweise ELP-induzierte Fibrinopeptide wie Fp Bß 30-43. Diese können bei Patienten mit CED in der Blutzirkulation entstehen, aber auch im entzündlichen Gewebe und dann in die Zirkulation abgegeben werden. Das Fibrinolyse-Blutungsrisiko besteht besonders bei Patienten mit aktiv entzündlichen Darmerkrankungen; Patienten mit inaktivem Morbus Crohn oder inaktiver Colitis ulcerosa haben im Durchschnitt nur geringe Veränderungen der Hämostaseparameter.
BibTeX:

@mastersthesis{Kehl_Promotion,

  author = {Kehl, Hans Gerd},

  title = {Morbus Crohn und Colitis ulcerosa - Angaben zur Klinik und experimentelle Untersuchungen zur Hämostase},

  school = {Dissertation, Philipps-Universität Marburg, Signaturen der Deutschen Nationalbibliothek, Katalog Frankfurt: H 90/9315, Katalog Leipzig: Di 1991 A 686},

  year = {1990},

  note = {Dissertation Marburg, Univ., Diss.; Signatur: BAY <19> : 0700/UMB 36280, Signatur: BAY <355> : 23/X12653, IDN: 0004238339},

  url = {http://d-nb.info/910155399}

}