Bachmann M, Gander M P
Schweiz Med Wochenschr. 1980 Nov 8;110(45):1689-92.
Pressure gradients between atrium and left ventricle were measured in 5 of 17 patients with the diagnosis of hypertrophic cardiomyopathy. The diagnosis was confirmed by echocardiography and angiography. In 2 patients the hypertrophic cardiomyopathy was obstructive for the left ventricular outflow tract. Mitral stenosis was suspected in 3 out of the 5 patients who presented atrial fibrillation or frequent premature atrial beats and atrial overload phenomena of a third heart sound (which was misinterpreted as mitral opening snap) together with radiological enlargement of left atrium. Cardiac catheterization revealed middle pressure gradients between 7 and 14 mm Hg over mitral valve in 3 patients. In 2 patients significant early and middiastolic pressure gradients were recorded only after left ventricular angiography (volume load) was performed. Based on the data from cardiac catheterization, mitral stenosis was diagnosed in one patient, surgery revealed normal mitral leaflets. Echocardiography showed normally mobile mitral leaflets as well as signs of interventricular septal hypertrophy in all 5 patients. (In the patient who underwent surgery echocardiography was performed after the operation.) There were no echocardiographic signs of left ventricular inflow obstruction. The pathophysiologic mechanism of myocardial hypertrophy mimicking mitral stenosis is unknown. It may be suspected that third heart sound, diastolic murmur and the left ventricular inflow obstruction apparent from the pressure gradient are due to diminished myocardial compliance, direct mechanical effects of septal hypertrophy and decreased mobility of anterior mitral leaflet. Mimicking of mitral stenosis exists. Hypertrophic cardiomyopathy should therefore be considered in the differential diagnosis of mitral stenosis. Echocardiography may be helpful in evaluation.
在17例诊断为肥厚型心肌病的患者中,对其中5例测量了心房与左心室之间的压力梯度。诊断经超声心动图和血管造影证实。2例患者的肥厚型心肌病导致左心室流出道梗阻。在出现心房颤动或频发房性早搏以及第三心音(被误诊为二尖瓣开瓣音)的心房负荷现象并伴有左心房放射学增大的5例患者中,有3例怀疑存在二尖瓣狭窄。心导管检查显示3例患者二尖瓣上的平均压力梯度在7至14 mmHg之间。2例患者仅在进行左心室血管造影(容量负荷)后才记录到明显的舒张早期和中期压力梯度。根据心导管检查数据,1例患者被诊断为二尖瓣狭窄,手术显示二尖瓣叶正常。超声心动图显示所有5例患者的二尖瓣叶活动正常,以及室间隔肥厚的征象。(接受手术的患者术后进行了超声心动图检查。)没有左心室流入道梗阻的超声心动图征象。模仿二尖瓣狭窄的心肌肥厚的病理生理机制尚不清楚。可能怀疑第三心音、舒张期杂音以及从压力梯度明显看出的左心室流入道梗阻是由于心肌顺应性降低、室间隔肥厚的直接机械作用以及二尖瓣前叶活动度降低所致。二尖瓣狭窄的模仿现象存在。因此,在二尖瓣狭窄的鉴别诊断中应考虑肥厚型心肌病。超声心动图可能有助于评估。