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法洛四联症合并左心房异构

[Tetralogy of Fallot associated with left atrial isomerism].

作者信息

Ferrín L M, Atik E, Aiello V, Marcial M B, Ebaid M

机构信息

Instituto do Caração do Hospital das Clínicas-FMUSP.

出版信息

Arq Bras Cardiol. 1996 Oct;67(4):249-53.

PMID:9181723
Abstract

The association of tetralogy of Fallot with atrial isomerism has been rarely reported. Eight cases (five with left isomerism and three with right isomerism) are known. This paper reports two other cases of tetralogy of Fallot with left atrial isomerism. The syndrome's defects were disguised and without clinical expression because of the presence of the right ventricular outlet obstruction of tetralogy of Fallot. These diagnostic elements, not recognized in one of the patients previous to surgical correction of tetralogy of Fallot, were present: junctional rhythm, bronchial isomerism, partial anomalous pulmonary vein connection, agenesy of inferior vena cava and abdominal heterotaxy; their identification previous to surgical correction of tetralogy of Fallot, is necessary for an adequate surgical management.

摘要

法洛四联症与心房异构的关联鲜有报道。已知有8例(5例为左异构,3例为右异构)。本文报告另外2例法洛四联症合并左心房异构的病例。由于法洛四联症存在右心室流出道梗阻,该综合征的缺陷被掩盖且无临床表现。这些诊断要素在1例法洛四联症手术矫正前未被识别,包括交界性心律、支气管异构、部分性肺静脉异位连接、下腔静脉缺如和腹部脏器异位;在法洛四联症手术矫正前识别它们,对于进行充分的手术管理是必要的。

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