Piotrowska J, Bilińska Z T, Michalak E, Ruzyłło W, Rydlewska-Sadowska W
Kliniki Kardiologii Ogólnej, Instytutu Kardiologii w Warszawie.
Kardiol Pol. 1991;34(4):207-17.
Hypertrophic cardiomyopathy is characterized by a diverse clinical and morphological spectrum. In this report we describe five patients with atypical hypertrophic cardiomyopathy and significantly dilated atria. Diagnostic difficulties are stressed. All patients underwent clinical examination, noninvasive studies including 2-D echocardiogram, cardiac catheterization. In three patients endomyocardial biopsy was taken and examined by light microscopy. All but one patient were men, ranging in age from 28 years to 40 years at initial examination. In all patients disproportion between mildly elevated or, in two cases normal filling pressures and degree of dilatation of both atria was found. Two patients C.G. and D.T. had auscultatory and roentgenographic findings of mitral stenosis. Following echocardiographic and angiocardiographic studies an increase in left and right ventricular thickness associated with dilatation of both atria was found. Endomyocardial biopsy in both cases did not show endocardial thickening or infiltrative changes. In family S, two patients W.S. and Je.S had marginal left ventricular hypertrophy. The presence of left ventricular hypertrophy in patient Ja. S and finding, after family studies, hypertrophic, obstructive cardiomyopathy in patient A.S. enabled establishing the correct diagnosis. Beside varying degree of hypertrophy in family S, mild dilation of the right ventricle and incomplete right bundle branch block were found. In two patients of family S. restrictive cardiomyopathy was found, in patient Ja. S. during cardiac catheterization at initial presentation, in patient W.S. on doppler transmitral flow velocity examination at late follow-up. Paroxysmal atrial fibrillation was the first symptom in four patients, thromboembolic event in one patient. In four patients pacemaker requirement was found. During long-term follow-up (mean 4.8 years) slowly progressive heart failure associated with further dilatation of atria is observed. Mild doses of diuretics are effective in controlling congestive symptoms.
in hypertrophic cardiomyopathy significantly dilated atria and clinical signs of mitral stenosis can be present. The presence of myocardial hypertrophy is not necessary to diagnose hypertrophic cardiomyopathy. Familial studies can be helpful in establishing the correct diagnosis.
肥厚型心肌病具有多样的临床和形态学表现。在本报告中，我们描述了5例非典型肥厚型心肌病且伴有明显心房扩大的患者。强调了诊断上的困难。所有患者均接受了临床检查、包括二维超声心动图在内的无创检查以及心导管检查。3例患者进行了心内膜心肌活检并进行了光学显微镜检查。除1例患者外，其余均为男性，初诊时年龄在28岁至40岁之间。所有患者均发现轻度升高或2例患者正常的充盈压与双侧心房扩大程度之间不成比例。2例患者C.G.和D.T.有二尖瓣狭窄的听诊和X线表现。经超声心动图和心血管造影研究发现左、右心室厚度增加并伴有双侧心房扩大。这2例患者的心内膜心肌活检均未显示心内膜增厚或浸润性改变。在S家族中，2例患者W.S.和Je.S有轻度左心室肥厚。患者Ja.S存在左心室肥厚，且经家族研究后发现患者A.S患有肥厚型梗阻性心肌病，从而得以确立正确诊断。除了S家族中不同程度的肥厚外，还发现右心室轻度扩张和不完全性右束支传导阻滞。在S家族的2例患者中发现了限制性心肌病，1例是患者Ja.S在初次就诊时的心导管检查中发现，另1例是患者W.S.在随访后期的多普勒二尖瓣血流速度检查中发现。阵发性心房颤动是4例患者的首发症状，1例患者发生了血栓栓塞事件。4例患者需要起搏器治疗。在长期随访（平均4.8年）中，观察到与心房进一步扩大相关的缓慢进展性心力衰竭。小剂量利尿剂可有效控制充血症状。
肥厚型心肌病可能存在明显的心房扩大和二尖瓣狭窄的临床体征。诊断肥厚型心肌病不一定需要存在心肌肥厚。家族研究有助于确立正确诊断。
