Keren A, Billingham M E, Weintraub D, Stinson E B, Popp R L
Circulation. 1985 Aug;72(2):302-9. doi: 10.1161/01.cir.72.2.302.
Five patients with only mildly dilated ventricles but other features typical of congestive cardiomyopathy underwent cardiac transplantation for class IV NYHA heart failure. The findings of clinical studies, cardiac catheterization, endomyocardial biopsy, and pathologic examination of the removed hearts in this group with mildly dilated congestive cardiomyopathy (MDCM) were compared with similar data in four patients with idiopathic restrictive cardiomyopathy (IRCM) and in 10 patients with typical dilated congestive cardiomyopathy (DCM). In comparison with the other groups, patients with MDCM had a higher incidence of familial cardiomyopathy (p = .02) and a shorter symptomatic period than patients with IRCM (p less than .02). Patients with both MDCM and DCM had globular hearts (with predominant left ventricular dilatation), congestive hemodynamics and poor left ventricular contractility (ejection fraction 12% to 19%), and high incidence of ventricular thrombi. Patients with IRCM showed normal ventricular size, marked atrial dilatation, restrictive hemodynamics, mild-to-moderate decrease in left ventricular contractility (ejection fraction 29% to 55%), and absence of ventricular thrombi. Cardiac index, ventricular wall thickness, and light microscopic findings were similar in the three groups of patients. Electron microscopy showed no myofibrillar loss in patients with IRCM but mild (partial) or moderate-to-severe (almost total) myofibrillar loss in those with MDCM and DCM, respectively. We conclude that end-stage congestive cardiomyopathy may occur without significant ventricular dilatation and patients with MDCM have heart sizes intermediate between those found in IRCM and DCM but their clinical, hemodynamic, and pathologic findings are virtually identical to those of patients with typical DCM.
五名仅有轻度心室扩张但具有充血性心肌病其他典型特征的患者因纽约心脏协会(NYHA)心功能IV级心力衰竭接受了心脏移植。将这组轻度扩张型充血性心肌病(MDCM)患者的临床研究、心导管检查、心内膜心肌活检及切除心脏的病理检查结果,与四名特发性限制性心肌病(IRCM)患者及十名典型扩张型充血性心肌病(DCM)患者的类似数据进行了比较。与其他组相比,MDCM患者家族性心肌病的发病率更高(p = 0.02),且症状期比IRCM患者短(p < 0.02)。MDCM和DCM患者均有球形心脏(以左心室扩张为主)、充血性血流动力学改变及左心室收缩功能差(射血分数12%至19%),且心室血栓发生率高。IRCM患者心室大小正常、心房明显扩张、限制性血流动力学改变、左心室收缩功能轻度至中度降低(射血分数29%至55%)且无心室血栓。三组患者的心脏指数、心室壁厚度及光镜检查结果相似。电镜检查显示,IRCM患者无肌原纤维丢失,而MDCM和DCM患者分别有轻度(部分)或中度至重度(几乎完全)肌原纤维丢失。我们得出结论,终末期充血性心肌病可能在无明显心室扩张的情况下发生,MDCM患者的心脏大小介于IRCM和DCM患者之间,但其临床、血流动力学及病理表现实际上与典型DCM患者相同。