Matsunaga S, Rikitake N, Yoshioka F, Kato H
J Cardiogr. 1985 Sep;15(3):931-9.
Two children, aged one year and nine months, and six years, both with hypertrophic cardiomyopathy, presented as severe right ventricular outflow obstruction were presented. Initially, both of them were suspected of having heart disease because of precordial systolic murmurs. Their tentative diagnosis was pulmonary stenosis. Their apex cardiograms and carotid pulse waves were not diagnostic. Asymmetrical septal hypertrophy with systolic anterior motion of the mitral valve was identified by echocardiography in both cases. Cardiac catheterization revealed left ventricular outflow tract pressure gradients of 5 mmHg and 30 mmHg, respectively. The right ventricular peak systolic gradient was 100 mmHg and 82 mmHg and left ventricular end-diastolic pressure was 26 mmHg and 18 mmHg, respectively. Selective right and left ventricular angiocardiograms demonstrated severe right ventricular outflow obstruction, and hypertrophy of the interventricular septum. One of them underwent resection of the right ventricular outflow tract muscle, which showed histological findings characteristic of hypertrophic cardiomyopathy. This patient died suddenly at the age of 10 years. Infants or young children with hypertrophic cardiomyopathy often have associated predominant right ventricular outflow tract obstruction, progressive congestive heart failure, and death. Therefore, correct diagnosis and careful treatment are mandatory for proper management.
两名儿童，年龄分别为1岁9个月和6岁，均患有肥厚型心肌病，表现为严重的右心室流出道梗阻。最初，两人均因心前区收缩期杂音而被怀疑患有心脏病。他们的初步诊断为肺动脉狭窄。他们的心尖心电图和颈动脉脉搏波均无法确诊。经超声心动图检查，两例均发现不对称性室间隔肥厚伴二尖瓣收缩期前向运动。心导管检查显示左心室流出道压力梯度分别为5 mmHg和30 mmHg。右心室收缩期峰值梯度分别为100 mmHg和82 mmHg，左心室舒张末期压力分别为26 mmHg和18 mmHg。选择性右心室和左心室心血管造影显示严重的右心室流出道梗阻和室间隔肥厚。其中一人接受了右心室流出道肌肉切除术，病理结果显示为肥厚型心肌病的特征。该患者10岁时突然死亡。患有肥厚型心肌病的婴幼儿常伴有明显的右心室流出道梗阻、进行性充血性心力衰竭和死亡。因此，正确的诊断和精心的治疗对于妥善管理至关重要。
