Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry

• Published in: European heart journal 2011-05, Vol.32 (9), p.1105-1113
• Main Authors: Pinamonti, Bruno, Dragos, Andreea Mihaela, Pyxaras, Stylianos A, Merlo, Marco, Pivetta, Alberto, Barbati, Giulia, Di Lenarda, Andrea, Morgera, Tullio, Mestroni, Luisa, Sinagra, Gianfranco
• Format: Article
• Language: English
• Subjects: Adult; Amiodarone - therapeutic use; Anti-Arrhythmia Agents - therapeutic use; Arrhythmogenic Right Ventricular Dysplasia - drug therapy; Arrhythmogenic Right Ventricular Dysplasia - mortality; Arrhythmogenic Right Ventricular Dysplasia - surgery; Death, Sudden, Cardiac - epidemiology; Female; Heart Failure - mortality; Heart Transplantation - mortality; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Observer Variation; Prognosis; Registries; Ventricular Dysfunction, Left - drug therapy; Ventricular Dysfunction, Left - mortality; Ventricular Dysfunction, Left - surgery; Ventricular Dysfunction, Right - drug therapy; Ventricular Dysfunction, Right - etiology; Ventricular Dysfunction, Right - mortality; Young Adult
• ISSN: 0195-668X; 1522-9645
• DOI: 10.1093/eurheartj/ehr040

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. 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