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[合并心脏缺陷:经超声心动图诊断的法洛四联症、共同心房和单心房心室瓣]

[Combined heart defects: tetralogy of Fallot, common atrium and a single atrioventricular valve diagnosed by echocardiography].

作者信息

Zach M, Dowgird M, Górny J, Shafie D

机构信息

Oddziału Chorób Wewnetrznych i Kardiologii Wojewódzkiego Szpitala Zespolonego w Olsztynie.

出版信息

Kardiol Pol. 1992 Apr;36(4):227-9.

PMID:1640669
Abstract

We present a case of the rare coincidence of three mechanisms leading to development of congenital heart disease in intrauterine life: intrinsic defect of the development of the cardiac loop (dextrocardia), failure of normal expansion of the subpulmonary infundibulum (Fallot syndrome) and endocardial cushion defect (common atrium and common atrioventricular valve ). It was associated with partial viscera inversion. A 31-year old man with congenital cyanotic heart disease, and Blalock-Taussig anastomosis was admitted to the hospital due to symptoms of severe cardiac failure. On physical examination: systolic murmur, hepatomegaly, ascites, leg's edema and cyanosis were found. In ECG--atrial fibrillation with 3-d degree a-v block. Standard echocardiography revealed: dextrocardia, a large single atrium with ostia of pulmonary and systemic veins, single atrio-ventricular valve , large ventricular, Fallot-like septal defect. The papillary muscles were not visible in the left ventricle. Aorta and pulmonary trunk arose from morphological right ventricle. The patient died on the 3-rd day of hospitalization in the course of cardiac and respiratory insufficiency. Postmorten examination confirmed the diagnosis.

摘要

我们报告一例在子宫内生活时导致先天性心脏病发生的三种机制罕见巧合的病例

心脏环发育的内在缺陷(右位心)、肺动脉漏斗部正常扩张失败(法洛四联症)和心内膜垫缺损(共同心房和共同房室瓣)。它与部分内脏反位有关。一名患有先天性青紫型心脏病且行布-塔分流术的31岁男性因严重心力衰竭症状入院。体格检查发现:收缩期杂音、肝肿大、腹水、腿部水肿和发绀。心电图显示:房颤伴三度房室传导阻滞。标准超声心动图显示:右位心、一个带有肺静脉和体静脉开口的大单一心房、单一房室瓣、大心室、法洛四联症样室间隔缺损。左心室内未见乳头肌。主动脉和肺动脉干起源于形态学上的右心室。患者在住院第3天死于心功能和呼吸功能不全。尸检证实了诊断。

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