Noncompaction in Duchenne muscular dystrophy:: Frustrated attempt to create a compensatory left ventricle?

被引：18

作者：

Finsterer, Josef

Stoellberger, Claudia

Feichtinger, Hans

机构：

[1] Krankenanstalt Rudolfstiftung Wien, Dept Neurol, Vienna, Austria

[2] Krankenanstalt Rudolfstiftung Wien, Dept Med 2, Vienna, Austria

[3] Krankenanstalt Rudolfstiftung Wien, Dept Pathol, Vienna, Austria

来源：

CARDIOLOGY | 2006年 / 105卷 / 04期

关键词：

myocardium; cardiomyopathy; muscular dystrophy; dystrophinopathy; heart failure;

D O I：

10.1159/000091738

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The histological workup of the myocardium of a patient with Duchenne muscular dystrophy and left ventricular hypertrabeculation/noncompaction (LVHT) revealed an extremely thin left ventricular wall and a noncompacted layer double in size compared to the compacted layer. Within the compacted layer islets of fibrous tissue predominated, surrounded by areas of myxoid appearance hardly producing Collagen, and occasionally normal or dystrophic cardiomyocytes. The noncompacted layer consisted largely of intact cardiomyocytes rarely intermingled with collagen-producing, fibrous tissue. This variant appearance of the compacted and noncompacted layer was found in all areas of noncompaction. These histopathological findings suggest that LVHT represents a compensatory attempt to overcome the failing compacted but dystrophic myocardium. Copyright (c) 2006 S. Karger AG, Basel

引用

页码：223 / 225

页数：3

共 5 条

[1]

Emery A. E. H., 2001, MUSCULAR DYSTROPHIES

[2] Left ventricular hypertrabeculation/noncompaction as a cardiac manifestation of Duchenne muscular dystrophy under non-invasive positive-pressure ventilation [J].