Zusammenfassung
Einleitung
Sowohl akutes als auch chronisches Herzversagen führt bei einigen Kindern trotz intensiver medikamentöser Therapie in einen nicht beherrschbaren kardiogenen Schock mit myokardialem Versagen und Tod. Durch mechanischen Kreislaufersatz können diese Kinder Wochen und Monate überleben, bis eine Erholung des eigenen Herzens eintritt oder eine Herztransplantation erfolgt. Wir berichten über 7 Kinder, deren Myokardfunktion sich erholt hat und die vom pneumatisch pulsatilen Assist Device (Excor, Berlin Heart) entwöhnt werden konnten.
Patienten
Zwischen 1990 und September 2003 wurden in unserem Herzzentrum 57 Kinder, mittleres Alter 7 Jahre, (Range 2 Tage–17 Jahre) mit pulsatil pneumatischem Assist Device (Excor, Berlin Heart AG, Berlin, Deutschland) behandelt. Das Körpergewicht lag zwischen 4 und 64 kg (mittleres Gewicht 17 kg). Die mittlere Unterstützungszeit lag bei 27 Tagen (Range 1–420 Tage). Acht Kinder litten an der fulminanten Form einer Myokarditis, 28 an einer Kardiomyopathie und 21 an postoperativem Myokardversagen oder einer terminalen Form eines angeborenen Herzfehlers.
Ergebnisse
Bei 24 Kindern erholte sich die Herzfunktion nicht ausreichend, sie wurden erfolgreich herztransplantiert. Bei 7 Kindern zeigte sich jedoch eine deutliche Verbesserung der Funktion, so dass eine Entwöhnung vom System stattfand. Darunter waren vier Kinder mit fulminanter Myokarditis. Sie zeigten eine rasche und vollständige Erholung des Herzens, so dass eine Explantation nach 11 bis 21 Tagen möglich wurde und von einer dauerhaften Heilung bei einer Nachbeobachtungszeit von bis zu 9 Jahren, gefolgt war. Ein Kind mit Kardiomyopathie konnte ebenfalls langfristig entwöhnt werden. Ferner wurde bei einem Kind mit Ebsteinanomalie nach der mechanischen Kreislaufunterstützung eine Palliativoperation vorgenommen, die eine langfristige Entwöhnung möglich machte. Bei einem Säugling lag nach Koronarverletzung ein ausgeprägter Infarkt vor. Die Erholung unter mechanischer Kreislaufunterstützung mit dem Berlin Heart war nur partiell, so dass 2 Monate nach Entwöhnung doch eine Herztransplantation erfolgen musste.
Schlussfolgerung
Unter Kreislaufentlastung kann sich das kindliche Myokard erholen, so dass es statt zur Transplantation zu einer Entwöhnung von dem System kommen kann. Insbesondere bei fulminanter Myokarditis tritt nach Wochen der Entlastung in der Regel die vollständige Erholung des eigenen Herzens ein.
Summary
Introduction
Chronic or acute heart failure in children can result in cardiac shock and death. When medical treatment fails, mechanical circulatory support can be lifesaving and can keep these children alive for several months until recovery or heart transplantation. We report on seven children with improvement of cardiac function while under support with the pneumatic pulsatile Assist Device ( Berlin Heart) in whom final weaning from the system was successful.
Patients
In 57 children, aged 2 days–17 years (median 7 years), heart function was artificially replaced by a VAD (Berlin Heart) implanted for longterm support (range 1–420, mean 27 days) in our institution between 1990 and September 2003. Eight children were suffering from fulminant myocarditis, 28 from cardiomyopathy and 21 from a congenital heart defect with either chronic failure or acute failure after weaning from cardiopulmonary bypass after surgery failed.
Results
Twenty–four children showed no recovery of myocardial function and underwent heart transplantation. Seven children showed great improvement and were weaned from the device. Four of these were children with fulminant myocarditis showed rapid and total recovery of the heart so that explantation after 11–21 days was possible; follow-up up to 9 years shows excellent cardiac function. One boy with dilated cardiomyopathy was successfully weaned from the device, and one child with Ebstein anomaly and long–term resuscitation underwent weaning after corrective surgery. One last infant suffered an infarction after cardiac surgery and underwent first ECMO and later Berlin Heart support. She was weaned, but left ventricular function again decreased, so that heart transplantation became necessary 2 months after the weaning.
Conclusion
Prolonged circulatory support with the Berlin Heart is an effective method even in small infants. Under mechanical circulatory support and ventricular unloading the myocardial function of children can improve until weaning from the assist device is successful. Especially in cases of fulminant myocarditis, recovery of the native ventricular function can be anticipated after weeks.
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Stiller, B., Weng, Y., Hübler, M. et al. Entwöhnung nach Herzunterstützung mit pneumatisch pulsatilem Assist Device (Berlin Heart) im Kindesalter. Z Herz-, Thorax-, Gefäßchir 18, 168– 175 (2004). https://doi.org/10.1007/s00398-004-0447-z
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DOI: https://doi.org/10.1007/s00398-004-0447-z