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心脏移植治疗的一种罕见心力衰竭病因：心室心肌致密化不全。

A rare cause of heart failure treated by heart transplantation: noncompaction of the ventricular myocardium.

作者信息

Bordes Julien, Jop Bertrand, Imbert Sandrine, Hraiech Sami, Collard Frédéric, Kerbaul François

机构信息

Department of Intensive Care, Sainte Anne Hospital, 83800 Toulon, France.

出版信息

Case Rep Med. 2009;2009:725879. doi: 10.1155/2009/725879. Epub 2010 Mar 18.

DOI:10.1155/2009/725879
PMID:20339508
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2842893/
Abstract

Noncompaction of the ventricular myocardium is a rare cardiomyopathy due to an arrest of myocardial morphogenesis. The characteristic echocardiographic findings are prominent myocardial trabeculations and deep intertrabecular spaces communicating with the left ventricular cavity. The clinical manifestations include heart failure (HF) signs, ventricular arrhythmias, and cardioembolic events. We describe an illustrative case of noncompaction of the ventricular myocardium associated with bicuspid aortic valve, a 42-year-old male presenting a refractory acute heart failure successfully treated by emergency heart transplantation.

摘要

心室肌致密化不全是一种由于心肌形态发生停滞导致的罕见心肌病。其特征性超声心动图表现为显著的心肌小梁和与左心室腔相通的深小梁间隙。临床表现包括心力衰竭体征、室性心律失常和心脏栓塞事件。我们描述了一例与二叶式主动脉瓣相关的心室肌致密化不全的典型病例，一名42岁男性因难治性急性心力衰竭成功接受了紧急心脏移植治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ce6/2842893/92efb600044b/CRM2009-725879.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ce6/2842893/8ff2dc3d554d/CRM2009-725879.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ce6/2842893/92efb600044b/CRM2009-725879.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ce6/2842893/8ff2dc3d554d/CRM2009-725879.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ce6/2842893/92efb600044b/CRM2009-725879.002.jpg

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Noncompaction of the ventricular myocardium: report of two cases with bicuspid aortic valve demonstrating poor prognosis and with prominent right ventricular involvement.心室心肌致密化不全：两例合并二叶式主动脉瓣病例报告，显示预后不良且右心室受累显著
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