Pediatric Cardiology, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee 37232, USA.
J Heart Lung Transplant. 2009 Dec;28(12):1261-6. doi: 10.1016/j.healun.2009.06.019. Epub 2009 Sep 26.
In critically ill children presenting with dilated cardiomyopathy (DCM), the presence of myocarditis predicts an improved chance of myocardial recovery. Noninvasive differentiation of myocarditis from other causes of DCM is difficult. However, sensitivity of endomyocardial biopsy has been questioned.
We reviewed clinical, echocardiographic, catheterization, and pathology data from all children admitted to the intensive care unit with DCM undergoing orthotopic heart transplantation since the inception of our transplant program in 1987 and all patients with definitively diagnosed myocarditis presenting since 1996.
Thirty-six patients with DCM underwent orthotopic heart transplantation. Cellular infiltrate was present in 3 of 36 (8.3%) explanted specimens. Pre-transplant biopsy was performed in 81%. No explanted heart demonstrated infiltrates after a negative biopsy. One biopsy was positive with negative explant histology after transplant 6 months later. No patient with biopsy-proven myocarditis died while listed for transplantation. Eleven additional patients with myocarditis did not undergo transplant. Ten have survived and experienced complete (n = 9) or near complete (n = 1) recovery of myocardial function. One patient died shortly after presentation from fulminant myocarditis. The 10 transplant-free survivors could not be easily distinguished from our transplant cohort by clinical features at presentation.
The incidence of cellular infiltrate in explanted hearts was significantly lower than that previously reported. Potentially, our aggressive myocarditis diagnostic protocol was useful in therapeutic stratification as a cohort of myocarditis patients avoided transplant and experienced complete recovery of myocardial function despite being difficult to distinguish clinically from our DCM transplant cohort at presentation.
在患有扩张型心肌病（DCM）的危重症儿童中，心肌炎的存在预示着心肌恢复的机会增加。非侵入性方法难以区分心肌炎和 DCM 的其他病因。然而，心内膜心肌活检的敏感性受到质疑。
我们回顾了自 1987 年我们的移植项目开始以来，所有因 DCM 入住重症监护病房并接受原位心脏移植的儿童以及自 1996 年以来所有确诊为心肌炎的患者的临床、超声心动图、心导管检查和病理学数据。
36 例 DCM 患者接受了原位心脏移植。36 例（8.3%）移植标本中存在细胞浸润。81%的患者进行了移植前活检。在阴性活检后，没有移植心脏显示浸润。一例活检阳性，而移植后 6 个月的移植心脏组织学阴性。没有活检证实为心肌炎的患者在移植名单上死亡。另外 11 例心肌炎患者未进行移植。10 例存活患者经历了心肌功能的完全（n=9）或近乎完全（n=1）恢复。1 例患者在发病后不久因暴发性心肌炎死亡。在发病时，未接受移植的 10 例存活患者在临床表现上与我们的移植队列难以区分。
移植心脏中细胞浸润的发生率明显低于先前的报告。可能是我们积极的心肌炎诊断方案在治疗分层中有用，因为一组心肌炎患者避免了移植，并经历了心肌功能的完全恢复，尽管在发病时与我们的 DCM 移植队列在临床表现上难以区分。
