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胎儿左心室心尖部动脉瘤导致糖原贮积病性扩张型心肌病

Fetal Left Ventricular Apical Aneurysm Progressing to Dilated Cardiomyopathy Due to Glycogen Storage Disease.

机构信息

Department of Pediatric Cardiology, Children's Hospital of Mercy, Kansas City, Missouri.

Department of Pediatric Cardiology, Cleveland Clinic Children's Hospital, Cleveland, Ohio.

出版信息

Tex Heart Inst J. 2022 Jul 1;49(4). doi: 10.14503/THIJ-20-7364.

Abstract

Fetal dilated cardiomyopathy is a rare anomaly characterized by ventricular dilation and dysfunction. Its causes are diverse, and its outcomes are generally dismal. We describe a rare case of prenatally diagnosed left ventricular apical aneurysm that progressed rapidly to dilated cardiomyopathy. At age 2 months, the infant underwent heart transplantation. Pathologic examination of the explanted heart revealed that the cause of the dilated cardiomyopathy was glycogen storage disease. This case highlights the crucial roles of timely diagnosis, frequent close monitoring, and multidisciplinary care in achieving a successful postnatal outcome.

摘要

胎儿扩张型心肌病是一种罕见的异常,其特征为心室扩张和功能障碍。其病因多样,预后通常较差。我们描述了一例罕见的左心室心尖部动脉瘤的产前诊断病例,该病例迅速进展为扩张型心肌病。患儿在 2 个月龄时接受了心脏移植。移植心脏的病理检查显示,扩张型心肌病的病因是糖原贮积症。该病例强调了及时诊断、密切频繁监测和多学科治疗在实现成功的新生儿预后中的关键作用。

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