孤立性左心室心肌致密化不全导致难治性心力衰竭

[Isolated left ventricular noncompaction causing refractory heart failure].

作者信息

Meneguz-Moreno Rafael Alexandre, Rodrigues da Costa Teixeira Felipe, Rossi Neto João Manoel, Finger Marco Aurélio, Casadei Carolina, Castillo Maria Teresa, Sanchez de Almeida Antonio Flávio

机构信息

Seção Médica de Transplantes, Ambulatório de Disfunção Ventricular e Transplante Cardíaco, Instituto Dante Pazzanese de Cardiologia, São Paulo, Brasil.

出版信息

Rev Port Cardiol. 2016 Mar;35(3):185.e1-4. doi: 10.1016/j.repc.2015.09.015. Epub 2016 Feb 28.

DOI:10.1016/j.repc.2015.09.015

PMID:26928017

Abstract

Left ventricular noncompaction is a rare congenital anomaly characterized by excessive left ventricular trabeculation, deep intertrabecular recesses and a thin compacted layer due to the arrest of compaction of myocardial fibers during embryonic development. We report the case of a young patient with isolated left ventricular noncompaction, leading to refractory heart failure that required extracorporeal membrane oxygenation followed by emergency heart transplantation.

摘要

左心室心肌致密化不全是一种罕见的先天性异常，其特征为左心室小梁增多、小梁间深陷以及由于胚胎发育过程中心肌纤维致密化停滞导致的致密层变薄。我们报告了一例孤立性左心室心肌致密化不全的年轻患者，该患者出现难治性心力衰竭，需要体外膜肺氧合支持，随后进行了紧急心脏移植。