Characteristics and prognostic implications of tricuspid regurgitation in patients with arrhythmogenic cardiomyopathy

Aims: Arrhythmogenic cardiomyopathy (AC) is characterized by right ventricular (RV) dilatation and dysfunction and is often seen in combination with tricuspid regurgitation (TR). The aim of this study was to investigate the characteristics and prognostic implications of TR in patients with AC. Methods and results: Clinical, echocardiographic, and cardiac magnetic resonance data of 52 patients with AC fulfilling 2010 Task Force criteria in a single centre were retrospectively evaluated. TR in AC was classified as no/mild, moderate, or severe on the basis of the current guidelines. Significant TR was defined as at least moderate TR. The primary endpoint was a composite of death, heart transplantation, and tricuspid valve surgery. There were seven patients (13.4%) with moderate TR and 13 patients (25.0%) with severe TR at initial diagnosis. Patients with severe TR showed a higher prevalence of atrial fibrillation and a higher mean NT-pro-BNP than other groups (68%, P = 0.013; 2423 ± 1578 pg/mL, P < 0.001, respectively). Patients with significant TR revealed a higher incidence of heart failure at initial presentation than did those without significant TR (30.0 vs. 3.1%, P = 0.022). Patients with severe TR showed significantly larger RV and lower RV and left ventricular functional parameters. During a mean follow-up of 4.2 years, three groups classified by TR severity considerably discriminated clinical outcomes (log rank P = 0.019). Patients with significant TR had a poorer prognosis than those with no or mild TR (42.9 vs. 3.1%, log rank P = 0.005). Cox regression analysis showed significant TR as an independent prognostic factor (hazard ratio 11.41, 95% confidential interval 1.30-99.92, P = 0.028). Conclusions: Significant TR at initial diagnosis in patients with AC is a poor prognostic factor.ope