IDIOPATHIC LEFT-VENTRICULAR ANEURYSM - A CLINICAL AND PATHOLOGICAL-STUDY OF A NEW ENTITY IN THE SPECTRUM OF CARDIOMYOPATHIES

In a prospective study on 340 cases with primary myocardial disease, e ight patients (six mates, two females, mean age 36 years, range 24-47) with an idiopathic left ventricular aneurysm were observed. All patie nts had normal coronary arteries, no angina or history of myocardial i nfarction; all but one had no risk factors for ischaemic heart disease ; all had normal right ventricles; one patient had a history of famili al dilated cardiomyopathy, two of 'flu-like' syndrome at the time of f irst symptom and two of alcohol abuse. All patients had ventricular ta chycardia (VT), five sustained (of right bundle branch block morpholog y in three, and of different morphologies in two), three non-sustained . Patients with sustained VT had inducible VT (resembling the clinical one) on electrophysiological study. Electrocardiogram (ECG) showed an infarction pattern in three cases. Aneurysms were of limited size (2. 1 +/- 1/11 segments on echocardiography) and were located in the septu m, apex or posterior wall. Left ventricular ejection fraction (LVEF) w as reduced (<0.50) in six patients and was not correlated with the ane urysm size. The duration of illness was inversely correlated with LVEF (P<0.05). Endomyocardial biopsy showed evidence of diffuse pathologic al changes in all cases (cell hypertrophy, myofibrillar lysis, mitocho ndriosis). During follow-up (64+/-32 months), patients were successful ly treated with anti-arrhythmic drugs: no patients required surgical t reatment to control ventricular arrhythmia. Considering the clinical a nd pathological features of idiopathic left ventricular aneurysm, this primary myocardial disease could be classified as a novel peculiar fo rm of cardiomyopathy.