Jatene Marcelo Biscegli, Miana Leonardo Augusto, Pessoa Alexander John, Riso Arlindo, Azeka Estela, Tanamati Carla, Gimenez Solange, Lopes Antonio Augusto, Marcial Miguel Barbero, Stolf Noedir Antonio Groppo
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP - Brasil.
Arq Bras Cardiol. 2008 May;90(5):329-33. doi: 10.1590/s0066-782x2008000500010.
In children with dilated cardiomyopathy who are on the waiting list for heart transplantation, we evaluate the seriousness of their hemodynamic conditions. Some develop cardiogenic shock, and the mortality rate is high. Even with inotropic and respiratory support, heart transplantation is considered an extremely grave circumstance.
The objective of this study is to report on our experience with children in this condition, in an attempt to analyze the viability, applicability and results of heart transplantation in these children.
From March 2001 to February 2004, 22 children with dilated cardiomyopathy who were on the waiting list for heart transplantation developed cardiogenic shock, requiring transfer to pediatric intensive care unit (ICU), intubation and inotropic support. Their ages ranged from 11 months to 11 years (mean age: 4.3 years), 55% were males, 14 could be listed as clinical priority, and the remaining 8 were removed from the waiting list due to their unfavorable clinical conditions.
Eight heart transplantations were performed, and 6 children died while on the waiting list (42.9%). Two children died (25%) after transplantation and the remaining 6 were discharged from hospital in good clinical condition. The two main complications were organ rejection in 4 cases and infection in 5 cases. Two patients developed neurological complications, and one of them fully recovered.
Children with cardiomyopathy and cardiogenic shock require immediate heart transplantation; only 57.1% could be transplanted, with an early 25% mortality rate. Those who survived transplantation showed good clinical progress, similar to that of children transplanted on an elective basis.
对于等待心脏移植的扩张型心肌病患儿，我们评估其血流动力学状况的严重性。一些患儿会发展为心源性休克，死亡率很高。即使给予了强心和呼吸支持，心脏移植仍被认为是极其严峻的情况。
本研究的目的是报告我们在这类患儿中的经验，试图分析心脏移植在这些患儿中的可行性、适用性和结果。
从2001年3月至2004年2月，22例等待心脏移植的扩张型心肌病患儿发展为心源性休克，需要转入儿科重症监护病房（ICU），进行插管和强心支持。他们的年龄从11个月至11岁（平均年龄：4.3岁），55%为男性，14例可列为临床优先，其余8例因临床状况不佳被从等待名单中移除。
进行了8例心脏移植，6例患儿在等待名单上死亡（42.9%）。2例患儿在移植后死亡（25%），其余6例临床状况良好出院。两个主要并发症是4例发生器官排斥反应和5例发生感染。2例患者出现神经并发症，其中1例完全康复。
患有心肌病和心源性休克的患儿需要立即进行心脏移植；只有57.1%的患儿能够接受移植，早期死亡率为25%。那些移植后存活的患儿临床进展良好，与择期移植的患儿相似。
