Aims: We sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) as information concerning the natural history and risk stratification of ARVC is still incomplete.
Methods and results: A cohort of 96 ARVC patients (68% males, 35 ± 15 years) was enrolled and underwent structured diagnostic protocol and follow-up. Primary study endpoints were death and heart transplantation (HTx). Clinical and echo-Doppler data were assessed as prognostic indicators. Sixty-five per cent of patients had right ventricular (RV) systolic dysfunction (RV fractional area change < 33%) and 24% had left ventricular (LV) systolic dysfunction (LV ejection fraction <50%). During a mean follow-up of 128 ± 92 months, 20 patients (21%) experienced cardiac death or underwent HTx. At multivariate analysis (Model 1), RV dysfunction [hazard ratio (HR): 4.12; 95% confidence interval (CI): 1.01-18.0; P = 0.05], significant tricuspid regurgitation (HR: 7.6; 95% CI: 2.6-22.0; P < 0.001), and amiodarone treatment (HR: 3.4; 95% CI: 1.3-8.8; P = 0.01) resulted as predictors of death/HTx. When inserting in the model, the 'ordinal dysfunction' (Model 2), which considers the presence of both RV and LV dysfunctions, this variable emerged as an independent prognostic predictor (HR: 6.3; 95% CI: 2.17-17.45; P < 0.001). At the receiver operating characteristic analysis, Model 2 was significantly more accurate in predicting long-term outcome compared with Model 1 (area under the curve 0.84 vs. 0.78, respectively; P = 0.04).
Conclusion: In our tertiary referral centre ARVC population, the presence of LV dysfunction at diagnosis has an incremental power in predicting adverse outcome compared with RV dysfunction alone.