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努南综合征婴儿的快速进展性梗阻性心肌病。两例报告。

Rapidly progressive obstructive cardiomyopathy in infants with Noonan's syndrome. Report of two cases.

作者信息

Hirsch H D, Gelband H, Garcia O, Gottlieb S, Tamer D M

出版信息

Circulation. 1975 Dec;52(6):1161-5. doi: 10.1161/01.cir.52.6.1161.

Abstract

Two patients with hypertrophic obstructive cardiomyopathy and Noonan's syndrome are presented. Both patients were found at postmortem examination to have gross malformation of the mitral valve and obliteration of the left ventricle due to muscle hypertrophy. Each case demonstrated similar clinical, echocardiographic, and angiographic findings. The poor response to medical and surgical therapy are noted.

摘要

本文报告了两名患有肥厚性梗阻性心肌病和努南综合征的患者。尸检发现,两名患者均有二尖瓣严重畸形,且因肌肉肥大导致左心室闭塞。每个病例都表现出相似的临床、超声心动图和血管造影结果。同时也指出了药物和手术治疗效果不佳的情况。

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