Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy.

BACKGROUND: Management of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is complicated by the incomplete information on the natural history of the disease and by the lack of risk stratification for cardiovascular death. The aim of the study was the identification of risk factors related to long-term prognosis.

METHODS AND RESULTS: Data were collected from 130 patients (100 men; age at onset of symptoms, 31.8+/-14.4 years) from a tertiary center between 1977 and 2000 who fulfilled the international standardized diagnostic criteria for ARVD/C. Risk factors for cardiovascular death were determined by a logistic regression model. After a mean follow-up of 8.1+/-7.8 years, 24 deaths were recorded, with a mean age at death of 54+/-19 years (annual mortality rate, 2.3%). There were 21 deaths with a cardiovascular origin (progressive heart failure for 14 patients and sudden death for the remaining 7 patients). All patients who died had a history of ventricular tachycardia. Multivariate analysis showed that after adjustment for sex, history of syncope, chest pain, inaugural ventricular tachycardia, recurrence of ventricular tachycardia, and QRS dispersion, clinical signs of right ventricular failure and left ventricular dysfunction both remained independently associated with cardiovascular mortality. The combined presence of one of these risk factors and ventricular tachycardia identifies high-risk subjects for cardiovascular mortality, whereas patients without ventricular tachycardia displayed the best prognosis.

CONCLUSIONS: The information on the natural history of patients with ARVD allowed us to identify risks factors for cardiovascular mortality. An analysis of a large international registry is needed to refine these results.