Cardiac transplantation as resolution for Uhl's anomaly: A case report

Juan J. Bacigalupe MD , Natalia Vensentini MD , Santiago Torroba MD , Javier A. Mariani MD , Sandra Defelitto MD , Norberto Blanco MD , Fabian Paetz MD , Julieta Ximena Saenz MD , Marcelo Nahin MD , Maximiliano de Abreu MD
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Abstract

Uhl's anomaly is an extremely rare congenital cardiac defect characterized by the absence of myocardium in the right ventricle. It has a poor prognosis, typically fatal during the perinatal period, with very few patients reaching adulthood. We present the case of a 28-year-old woman with heart failure and ventricular arrhythmia associated with Uhl's anomaly, who required cardiac transplantation, with favorable postoperative outcomes. Uhl's anomaly represents an exceedingly rare cause of cardiac transplantation in adults.
心脏移植治疗Uhl异常1例报告
Uhl异常是一种极其罕见的先天性心脏缺陷,其特征是右心室没有心肌。该病预后差,通常在围产期死亡,很少有患者能活到成年。我们报告一例28岁的女性心力衰竭和室性心律失常合并Uhl异常,谁需要心脏移植,术后良好的结果。Uhl异常是一种非常罕见的成人心脏移植病因。
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