Datasheet1_Giant right atrium in a child with dilated cardiomyopathy: A case report.docx

Dilated cardiomyopathy (DCM) is one of the leading causes of heart failure in children with diverse clinical characteristics. To date, DCM with a giant atrium as the first manifestation is rare and has not been reported in previous literature. We report a case of a male infant born with a significantly enlarged right atrium. Due to worsened clinical symptoms and the risk of arrhythmias and thrombosis, we performed the surgical reduction of the right atrium. Unfortunately, DCM and a progressive re-enlargement of the right atrium appeared during midterm follow-up. The mother's echocardiogram also suggested DCM, and the patient was eventually considered for a diagnosis of familial DCM. This case may expand the clinical spectrum of DCM and reminds us of the importance of good follow-up of children with idiopathic dilatation of the right atrium.

扩张型心肌病（Dilated cardiomyopathy，DCM）是儿童心力衰竭的主要病因之一，临床特征具有多样性。迄今为止，以巨大心房为首发表现的DCM较为罕见，既往文献尚未见相关报道。本文报告1例出生时即伴右心房显著扩大的男性婴儿。由于临床症状恶化，并存在心律失常及血栓形成风险，我们为其实施了右心房缩减手术。遗憾的是，在中期随访期间，患儿出现了DCM及右心房进行性再扩大。其母亲的超声心动图检查也提示存在DCM，最终该患者被诊断为家族性扩张型心肌病。本病例可拓展DCM的临床谱，并提醒我们重视对特发性右心房扩张患儿的良好随访。