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小儿梗阻性心肌病伴特发性肥厚性主动脉瓣下狭窄

[Obstructive cardiomyopathy with idiopathic hypertrophic subaortic stenosis in the pediatric age].

作者信息

Pilotti G, Burzio P, Favetta S, Ricci C, Pennacino G

出版信息

Minerva Med. 1979 Nov 3;70(48):3299-307.

PMID:574624
Abstract

The clinical and haemodynamic features of obstructive cardiomyopathy with hypertrophic subaortic stenosis in a 12-yr-old boy are examined. A cardiac souffle had been present for 10 yr and precordial pain, cardiopalmus and effort dyspnoea for 5-6 yr. Familial association, ischaemic and pseudonecrotic ECG changes, and signs of left ventricle hypertrophy were noted. Left catheterisation and ventriculography showed obstructive hypertrophy of the muscle, two chambers with filling at 160/10 and ejection at 120/10, and slight regurgitation into the left trium. Treatment with 0.2-0.5 mg/kg/day propranolol.

摘要

对一名12岁患有梗阻性心肌病伴主动脉瓣下狭窄的男孩的临床和血流动力学特征进行了检查。心脏杂音已存在10年,心前区疼痛、心悸和劳力性呼吸困难已有5至6年。发现有家族关联、缺血性和假性坏死性心电图改变以及左心室肥厚的体征。左心导管检查和心室造影显示肌肉梗阻性肥厚,两个腔室在160/10时充盈,在120/10时射血,并有轻微反流至左心房。用普萘洛尔0.2 - 0.5毫克/千克/天进行治疗。

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