Panza J A, Maron B J
Echocardiography Laboratory, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892.
Am J Cardiol. 1991 Feb 15;67(5):404-10. doi: 10.1016/0002-9149(91)90050-u.
This report describes the simultaneous occurrence of mitral valve prolapse (MVP) and systolic anterior motion (SAM) in hypertrophic cardiomyopathy (HC). In 25 patients (aged 7 to 62 years, mean 29), 15 (60%) of whom were male, distal portions of the anterior or posterior mitral leaflets approached or made midsystolic contact with the ventricular septum, whereas the proximal portion of the mitral leaflets showed marked cephalad excursion into the left atrium, 5 to 15 mm beyond the mitral annular plane. Three mitral valves that were available for gross visual inspection were not morphologically typical of patients with primary MVP. Clinical features and natural history (1 to 14 years [mean 6] of follow-up), cardiac dimensions, and distribution of left ventricular hypertrophy defined in the study patients did not appear to differ distinctly from those in the overall referral population of patients with HC evaluated at our institution. Hence, patients with HC may show a striking pattern of mitral valvular motion involving SAM into the left ventricular outflow tract, as well as MVP; this prolapse motion is probably due to anatomic disproportion between the mitral valve and the small left ventricular cavity rather than to the coexistence of 2 separate disease entities. Such patients further define the great diversity evident within the broad clinical spectrum of HC.
本报告描述了肥厚型心肌病(HC)中二尖瓣脱垂(MVP)和收缩期前向运动(SAM)的同时出现。在25例患者(年龄7至62岁,平均29岁)中,15例(60%)为男性,二尖瓣前叶或后叶的远端接近或在收缩中期与室间隔接触,而二尖瓣叶的近端则明显向头侧移位进入左心房,超出二尖瓣环平面5至15毫米。可进行大体视觉检查的三个二尖瓣在形态上并非原发性MVP患者的典型表现。研究患者的临床特征和自然病史(随访1至14年[平均6年])、心脏大小以及左心室肥厚的分布,与在我们机构评估的HC总体转诊患者人群相比,似乎没有明显差异。因此,HC患者可能表现出一种显著的二尖瓣运动模式,包括SAM进入左心室流出道以及MVP;这种脱垂运动可能是由于二尖瓣与小左心室腔之间的解剖比例失调,而非两种独立疾病实体的共存。此类患者进一步明确了HC广泛临床谱内明显的巨大多样性。