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布兰德-怀特-加兰综合征与房间隔缺损——罕见关联及诊断挑战。

Bland-White-Garland syndrome and atrial septal defect - rare association and diagnostic challenge.

作者信息

Schneider T, Rickli H, Gliech V, Maeder M

机构信息

Kantonsspital St. Gallen, Department Innere Medizin, Fachbereich Kardiologie, Rorschacherstr. 95, 9007, St. Gallen, Switzerland.

出版信息

Clin Res Cardiol. 2006 May;95(5):295-300. doi: 10.1007/s00392-006-0369-8. Epub 2006 Mar 8.

Abstract

We report on a 40-year-old woman referred for evaluation of a cardiac murmur and dyspnea on exertion. The electrocardiogram (ECG) showed incomplete right bundle branch block, and echocardiography revealed a large atrial septal defect (ASD, ostium secundum type) with dilated right-sided heart chambers. At cardiac catheterization, a large left-to-right shunt (78% of the pulmonary blood flow) was found, and surprisingly, the additional diagnosis of anomalous origin of the left coronary artery from pulmonary artery (ALCAPA) was established. After ASD closure and left coronary artery ligation with implantation of a vein graft to the left anterior descending artery, she had an uneventful 18-years follow-up. We discuss the interaction of the two associated conditions, and based on the herein reported unusual combination, we highlight typical features of non-invasive examinations including auscultation, ECG, and echocardiography in adult patients with ALCAPA.

摘要

我们报告了一名40岁女性,因心脏杂音和劳力性呼吸困难前来评估。心电图(ECG)显示不完全性右束支传导阻滞,超声心动图显示巨大房间隔缺损(ASD,继发孔型)伴右侧心腔扩大。心导管检查发现大量左向右分流(占肺血流量的78%),令人惊讶的是,还确诊了左冠状动脉起源于肺动脉(ALCAPA)。在进行ASD封堵及左冠状动脉结扎并向左前降支植入静脉移植物后,她进行了18年的顺利随访。我们讨论了这两种相关疾病的相互作用,并基于本文报道的不寻常组合,强调了成年ALCAPA患者无创检查(包括听诊、ECG和超声心动图)的典型特征。

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