两名患有组织细胞样心肌病和左心室心肌致密化不全的婴儿接受原位心脏移植。

Orthotopic heart transplantation in two infants with histiocytoid cardiomyopathy and left ventricular non-compaction.

作者信息

Siehr Stephanie L, Bernstein Daniel, Yeh Justin, Berry Gerald J, Rosenthal David N, Hollander Seth A

机构信息

Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA.

出版信息

Pediatr Transplant. 2013 Nov;17(7):E165-7. doi: 10.1111/petr.12141.

DOI:10.1111/petr.12141

PMID:24099092

Abstract

HC is a rare cause of congestive heart failure that typically presents with malignant ventricular arrhythmias in infants, often requiring urgent intervention. Successful heart transplantation in a patient with HC has only been reported once (J Heart Lung Transplant 2004: 23: 902). The combination of HC with concurrent LVNC has only been described three times (Int J Legal Med 2009: 123: 47; Hum Pathol 2005: 36: 403; Pediatr Dev Pathol 2012: 15: 397). We report two rare cases of HC with LVNC in two infants presenting with cardiogenic shock, one requiring ECMO support who was successfully bridged to orthotopic heart transplantation with a Berlin Heart LVAD.

摘要

致心律失常性右室心肌病（HC）是导致充血性心力衰竭的罕见病因，在婴儿中通常表现为恶性室性心律失常，常需紧急干预。仅有1例HC患者成功进行心脏移植的报道（《心肺移植杂志》2004年；23卷：902页）。HC合并左室心肌致密化不全（LVNC）仅被描述过3次（《国际法医病理学杂志》2009年；123卷：47页；《人类病理学》2005年；36卷：403页；《儿科发育病理学》2012年；15卷：397页）。我们报告了2例婴儿患HC合并LVNC的罕见病例，均表现为心源性休克，其中1例需要体外膜肺氧合（ECMO）支持，并通过柏林心脏左心室辅助装置（LVAD）成功过渡到原位心脏移植。