Demers C, Rouleau J L, Leung T K, Tardif J C
Department of Medicine, Montréal Heart Institute, Québec.
Can J Cardiol. 1998 Nov;14(11):1397-400.
A 72-year-old woman presented to hospital with rapidly progressive dyspnea and chest pain on exertion. Physical findings included a grade 3/6 systolic murmur increased by the Valsalva manoeuvre. Transthoracic echocardiography revealed concentric left ventricular hypertrophy, systolic anterior motion of the mitral valve and critical dynamic outflow tract obstruction. The myocardium was strikingly heterogeneous with hyperdynamic left ventricular systolic function. Laboratory findings included severe hypercalcemia secondary to primary hyperparathyroidism. The patient's outcome was unfavourable with nephrogenic diabetes insipidus, pancreatitis, shock, severe acidosis and death. Postmortem examination confirmed the presence of severe concentric left ventricular hypertrophy, a narrowed left ventricular outflow tract and localized endocardial fibrosis of the left interventricular septum. Microscopic findings showed diffuse calcium deposits of the myocardium, coronary arteries, kidneys and lungs. This appears to be the first report of two-dimensional and Doppler echocardiographic findings in hypercalcemic cardiomyopathy mimicking obstructive hypertrophic cardiomyopathy.
一名72岁女性因劳力性呼吸困难迅速加重和胸痛入院。体格检查发现,瓦尔萨尔瓦动作(Valsalva maneuver)可使收缩期杂音增强至3/6级。经胸超声心动图显示左心室向心性肥厚、二尖瓣收缩期前向运动以及严重的动态流出道梗阻。心肌明显不均质,左心室收缩功能亢进。实验室检查发现继发于原发性甲状旁腺功能亢进的严重高钙血症。患者预后不佳,出现肾性尿崩症、胰腺炎、休克、严重酸中毒并死亡。尸检证实存在严重的左心室向心性肥厚、左心室流出道狭窄以及左心室间隔局部心内膜纤维化。显微镜检查发现心肌、冠状动脉、肾脏和肺部有弥漫性钙沉积。这似乎是首例关于模仿梗阻性肥厚型心肌病的高钙血症性心肌病的二维及多普勒超声心动图表现的报告。