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大动脉转位矫正伴内脏反位和腭裂,但无其他心脏缺陷。

A corrected transposition of the great arteries with situs inversus visceralis and cleft palate, but without other cardiac defects.

作者信息

Kanemoto N, Usui K, Tagawa R, Goto Y

机构信息

Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan.

出版信息

Tokai J Exp Clin Med. 1992 Oct;17(3-4):115-9.

PMID:1300670
Abstract

A 17-year-old female admitted for cleft palate surgery was referred for further evaluation of her cardiac condition. The patient had been diagnosed as dextrocardia at birth, without any cardiac murmurs. She has led a normal life. The apical impulse was felt at the fifth intercostal space at the right mid-clavicular line. The ECG and chest x-rays were strongly suggestive of corrected transposition of the great arteries (CTGA) with situs inversus visceralis. Findings of the two-dimensional and Dopplar echocardiograms showed CTGA with a mild morphologic tricuspid regurgitation without any other complicated cardiac anomalies. In the Japanese literature, 36 CTGA patients without associated cardiac defects have been reported. Of these, four patients revealed dextrocardia. To our knowledge, our patient is the first reported adult case without any cardiac defects, only minimal tricuspid regurgitation, situs inversus totalis and cleft palate.

摘要

一名因腭裂手术入院的17岁女性被转诊以进一步评估其心脏状况。该患者出生时被诊断为右位心,无任何心脏杂音。她一直过着正常生活。心尖搏动位于右锁骨中线第五肋间。心电图和胸部X光片强烈提示矫正型大动脉转位(CTGA)伴内脏反位。二维和多普勒超声心动图检查结果显示为CTGA,伴有轻度形态学三尖瓣反流,无任何其他复杂心脏异常。在日本文献中,已报道36例无相关心脏缺陷的CTGA患者。其中,4例患者表现为右位心。据我们所知,我们的患者是首例报道的无任何心脏缺陷、仅有轻微三尖瓣反流、全内脏反位和腭裂的成年病例。

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