Macedo A J, Henrickson I, Kaku S, Cabral A, Pinto E, Lima M
Serviço de Cardiologia Pediátrica, do Hospital de Santa Marta, Lisboa.
Rev Port Cardiol. 1995 May;14(5):401-8, 361.
Four children, three males and one female, aged 1.5 months to 11 years, with restrictive cardiomyopathy are described. One had patent ductus arteriosus in association. Hepatomegaly, abnormal second heart sound and signs of pulmonary venous congestion in chest X-ray were the most consistent clinical findings. Diagnosis was based on echocardiographic findings, cardiac catheterization data and results of pathology. The most frequent echocardiographic sign was the enlargement of both atria but with the ventricles within normal size and normal systolic function. Abnormalities in second phase of the ventricular filling were recorded in 2D-echo Doppler and cardiac catheterization, where a dip and plateau morphology of ventricular tracings was recorded. Metabolic studies performed in two children were normal, and any child had hypereosinophilia. Two children died, one was lost for follow-up and another is asymptomatic. Remarks on medical and surgical management are made.
本文描述了4例患有限制性心肌病的儿童，其中3例男性，1例女性，年龄在1.5个月至11岁之间。1例合并动脉导管未闭。肝肿大、第二心音异常以及胸部X线显示肺静脉充血征象是最常见的临床表现。诊断基于超声心动图检查结果、心导管检查数据以及病理结果。最常见的超声心动图表现是双房增大，但心室大小正常且收缩功能正常。二维超声心动图多普勒检查和心导管检查均记录到心室充盈第二阶段异常，表现为心室曲线呈低垂和平台形态。对2例患儿进行的代谢研究结果正常，所有患儿均无嗜酸性粒细胞增多。2例患儿死亡，1例失访，另1例无症状。文中还对药物和手术治疗进行了讨论。
