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家族性肥厚型心肌病伴右心室异常受累。

Familial hypertrophic cardiomyopathy with unusual involvement of the right ventricle.

作者信息

Casanova M, Gamallo C, Quero-Jiménez M, García-Aguado A, Burgueros M, García S, Suarez A

出版信息

Eur J Cardiol. 1979 Feb;9(2):145-59.

PMID:569583
Abstract

4 cases of familial hypertrophic cardiomyopathy are reported. In all of them, the onset of symptoms occurred before the 8th mth of life, and were characterized by severe right cardiac failure. In 3 cases the catheterization revealed significant decrease in right ventricular compliance and severe reduction of its cavity. The pathological studies were consistent with hypertrophic cardiomyopathy with predominant involvement of the right ventricle. The pedigree disclosed a Mendelian autosomal recessive type of inheritance.

摘要

报告了4例家族性肥厚型心肌病。所有病例症状均在出生后8个月内出现,以严重右心衰竭为特征。3例心导管检查显示右心室顺应性显著降低,腔室严重缩小。病理研究符合以右心室受累为主的肥厚型心肌病。家系显示为孟德尔常染色体隐性遗传类型。

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