Mitha A S, Matisonn R E, Chesler E
S Afr Med J. 1975 Feb 15;49(7):229-32.
Two young Black female patients with pulmonary valve stenosis and intact ventricular septa are presented in protracted congestive cardiac failure with severe tricuspid insufficiency and, in one, atrial fibrillation. Right ventricular systolic dysfunction was manifested by peak systolic pressures below systemic level, raised end-diastolic pressures and low cardiac output, but without right-to-left shunt. These findings are in strong contrast to those found in most patients with pulmonary stenosis of long standing, where persistent impairment of right ventricular function is diastolic with a high end-diastolic pressure and reversal of an interatrial shunt which result from poor right ventricular compliance. Evidence of left ventricular dysfunction was also present in both cases. Protracted heart failure in these patients is believed to have been the result of coincidental cardiomyopathy in a racial group highly predisposed to this disorder. A diagnostic appreciation of this phenomenon is important in the evaluation of heart disease in the Black, since cardiomyopathy may modify or even mask the features of the underlying disorder.
本文报告了两名患有肺动脉瓣狭窄且室间隔完整的年轻黑人女性患者，她们长期患有充血性心力衰竭，伴有严重的三尖瓣关闭不全，其中一名患者还患有房颤。右心室收缩功能障碍表现为收缩压峰值低于体循环水平、舒张末期压力升高和心输出量降低，但无右向左分流。这些发现与大多数长期患有肺动脉狭窄的患者形成强烈对比，在这些患者中，右心室功能的持续损害是舒张期的，舒张末期压力高，且由于右心室顺应性差导致心房分流逆转。两例患者均存在左心室功能障碍的证据。这些患者的长期心力衰竭被认为是在一个极易患这种疾病的种族群体中巧合出现心肌病的结果。对这种现象的诊断认识在评估黑人心脏病时很重要，因为心肌病可能会改变甚至掩盖潜在疾病的特征。
