[Arrhythmologic study of 50 patients with arrhythmogenic disease of the right ventricle: prognostic implications]

Arrhythmogenic right ventricular disease is a syndrome which involves a wide spectrum of anatomo-clinical features. It is characterised by different levels of right ventricle anomaly, and by life-threatening "right" hyperkinetic ventricular arrhythmias. Fifty consecutive pts were retrospectively examined at the Arrhythmological Centre in Trento between 1977 and 1988. The results of rigorous arrhythmological, echocardiographic and angiographic criteria showed that all pts were affected by arrhythmogenic right ventricular disease.

39/50 (78%) males, 11/50 (22%) females; age 30.6 years (11-78) at the time of the first study.

clinical history in 50/50 pts, electrocardiogram in 50/50 pts, Holter monitoring in 50/50 pts, ergometric test in 49/50 pts, non-invasive analysis using signal-averaging QRS in 17/50 pts, 2D echocardiogram in 50/50 pts, angiography in 38/50 pts, electrophysiological endocavitary study in 35/50 pts.

familial 2/50 (4%); 1/50 (2%) was in class II NYHA; first arrhythmia at 24.6 years (8-60); most severe arrhythmia at 27.7 years (9-74). Forty-three out of fifty patients (86%) were symptomatic for arrhythmias: 28/50 (56%) as a result of stress; 20/50 (40%) had life-threatening symptoms; 6/50 (12%) had aborted sudden death. Arrhythmogenic right ventricular disease was "localized" in 42/50 (84%) and "diffused" in 8/50 (16%) and was associated with anomalies of the left ventricle in 30/50 (60%). Electrocardiogram showed: right bundle branch block in 10/50 (20%), negative T wave on the right precordial leads in 19/50 (38%), delayed ventricular potentials in 4/17 (23.5%). Using the electrocardiogram, Holter monitoring and electrophysiological endocavitary study the following were documented: a) clinical ventricular tachycardia in 40/50 (80%): non-sustained ventricular tachycardia in 10/50 (20%), sustained ventricular tachycardia in 30/50 (60%); b) electrically induced ventricular tachycardia in 26/35 (74.2%): non-sustained ventricular tachycardia in 8/35 (22.8%), sustained ventricular tachycardia in 18/35 (51.4%) (clinical sustained ventricular tachycardia in 18/18); c) multiform ventricular tachycardia in 12/50 (24%) (diffused arrhythmogenic right ventricular disease in 3/12 and associated anomalies of the left ventricle in 11/12); d) pleomorphic sustained ventricular tachycardia in 9/30 (30%) (diffused arrhythmogenic right ventricular disease in 2/9, and associated anomalies of the left ventricle in 8/9). Forty-two out of fifty patients (84%) underwent antiarrhythmic treatment. When the study was carried out 6.6 years (1 month-22 years) had passed since the first symptom; follow-up was 2.1 years (1 month-11 years) while the interval between the first symptom and the last check-up was 8.4 years (1-30 years); 2/50 dropped out and 2/50 died suddenly.(ABSTRACT TRUNCATED AT 400 WORDS)