SPECTRUM OF CLINICOPATHOLOGICAL MANIFESTATIONS OF ARRHYTHMOGENIC RIGHT-VENTRICULAR CARDIOMYOPATHY DYSPLASIA - A MULTICENTER STUDY/

Objectives. The aim of the present investigation was to redefine the c linicopathologic profile of arrhythmogenic right ventricular cardiomyo pathy/dysplasia (ARVC), with special reference to disease progression and left ventricular (LV) involvement. Background. Long-term follow-up data from clinical studies indicate that ARVC is a progressive heart muscle disease that with time may lead to more diffuse right ventricul ar (RV) involvement and LV abnormalities and culminate in heart failur e. Methods. Forty two patients (27 male, 15 female; 9 to 65 years old, mean [+/-SD] age 29.6 +/- 18) from six collaborative medical centers, with a pathologic diagnosis of ARVC at autopsy or heart transplantati on, and with the whole heart available, were studied according to a sp ecific clinicomorphologic protocol.Results. Thirty-four patients died suddenly (16 during effort); 4 underwent heart transplantation; 2 died as a result of advanced heart failure; and 2 died of other causes. Su dden death was the first sign of disease in 12 patients; the other 30 had palpitations, with syncope in 11, heart failure in 8 and stroke in 3. Twenty seven patients experienced ventricular arrhythmias (ventric ular tachycardia in 17), and 5 received a pacemaker. Ten patients had isolated RV involvement (group A); the remaining 32 (76%) also had fib rofatty LV involvement that was observed histologically only in 15 (gr oup B) and histologically and macroscopically in 17 (group C). Patient s in group C were significantly older than those in groups A and B (39 +/- 15 years vs. 20 +/- 8.8 and 25 +/- 9.7 years, respectively), had significantly longer clinical follow-up (9.3 +/- 7.3 years vs. 1.2 +/- 2.1 and 3.4 +/- 2.2 years, respectively) and developed heart failure significantly more often (47% vs. 0 and 0, respectively). Patients in groups B and C had warning symptoms (80% and 87%, respectively, vs. 30 %) and clinical ventricular arrhythmias (73% and 82%, respectively, vs . 20%) significantly more often than patients in group A. Hearts from patients in group C weighed significantly more than those from patient s in groups A and B (500 +/- 150 g vs. 328 +/- 40 and 380 +/- 95 g, re spectively),,whereas hearts from both group B and C patients had sever e RV thinning (87% and 71%, respectively, vs. 20%) and inflammatory in filtrates (73% and 88%, respectively, vs. 30%) significantly more ofte n than those from group A patients. Conclusions. LV involvement was fo und in 76% of hearts,vith ARVC, was age dependent and was associated w ith clinical arrhythmic events, more severe cardiomegaly, inflammatory infiltrates and heart failure. ARVC can no longer be regarded as an i solated disease of the right ventricle. (C) 1997 by the American Colle ge of Cardiology.