Lin Shan-Miao, Hwang Haw-Kwei, Chen Ming-Ren
Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan.
Pediatr Neonatol. 2008 Apr;49(2):48-51. doi: 10.1016/S1875-9572(08)60012-1.
Restrictive cardiomyopathy in young children is rare and carries a poor prognosis. We report an 18-month-old girl with poor feeding and abdominal distension. Except for hepatomegaly, no other gastrointestinal abnormalities were found. She had normal-sized ventricles but biatrial enlargement. Echocardiography demonstrated normal systolic but impaired diastolic function. Cardiac catheterization revealed a characteristic dip-and-plateau configuration of the right ventricular pressure tracing. The diagnosis turned out to be typical restrictive cardiomyopathy. The patient was maintained on aspirin while awaiting cardiac transplant.
小儿限制性心肌病罕见且预后不良。我们报告一名18个月大喂养困难和腹胀的女童。除肝肿大外，未发现其他胃肠道异常。她的心室大小正常，但双房增大。超声心动图显示收缩功能正常但舒张功能受损。心导管检查显示右心室压力曲线呈典型的下陷-高原形态。诊断结果为典型的限制性心肌病。该患者在等待心脏移植期间一直服用阿司匹林。
