Arrhythmogenic Right Ventricular Cardiomyopathy Clinical Course and Predictors of Arrhythmic Risk

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:68

Article

Mazzanti, Andrea¹  Ng, Kevin¹  Faragli, Alessandro¹  Maragna, Riccardo¹  Chiodaroli, Elena¹  Orphanou, Nicoletta¹  Monteforte, Nicola¹  Memmi, Mirella¹  Gambelli, Patrick¹  Novelli, Valeria¹  Bloise, Raffaella¹  Catalano, Oronzo¹  Moro, Guido¹  Tibollo, Valentina¹  Morini, Massimo¹  Bellazzi, Riccardo²  Napolitano, Carlo¹  Bagnardi, Vincenzo³  Priori, Silvia G.^1,4 

[1] IRCCS ICS Maugeri, Mol Cardiol, Via Maugeri 10, I-27100 Pavia, Italy

[2] Univ Pavia, Dept Elect Comp & Biomed Engn, Pavia, Italy

[3] Univ Milano Bicocca, Dept Stat & Quantitat Methods, Milan, Italy

[4] Univ Pavia, Dept Mol Med, Pavia, Italy

关键词: athletes;    genetics;    implantable cardioverter-defibrillator;    sudden cardiac death;    ventricular tachycardia;   

DOI  :  10.1016/j.jacc.2016.09.951

来源: Elsevier

【 摘 要 】

BACKGROUND Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death, but its progression over time and predictors of arrhythmias are still being defined. OBJECTIVES This study sought to describe the clinical course of ARVC and occurrence of life-threatening arrhythmic events (LAE) and cardiovascular mortality; identify risk factors associated with increased LAE risk; and define the response to therapy. METHODS We determined the clinical course of 301 consecutive patients with ARVC using the Kaplan-Meier method adjusted to avoid the bias of delayed entry. Predictors of LAE over 5.8 years of follow-up were determined with Cox multivariable analysis. Treatment efficacy was assessed comparing LAE rates during matched time intervals. RESULTS A first LAE occurred in 1.5 per 100 person-years between birth and age 20 years, in 4.0 per 100 person-years between ages 21 and 40 years, and in 2.4 per 100 person-years between ages 41 and 60 years. Cumulative probability of a first LAE at follow-up was 14% at 5 years, 23% at 10 years, and 30% at 15 years. Higher risk of LAE was predicted by atrial fibrillation (hazard ratio [HR]: 4.38; p = 0.002), syncope (HR: 3.36; p < 0.001), participation in strenuous exercise after the diagnosis (HR: 2.98; p = 0.028), hemodynamically tolerated sustained monomorphic ventricular tachycardia (HR: 2.19; p = 0.023), and male sex (HR: 2.49; p = 0.012). No difference was observed in the occurrence of LAE before and after treatment with amiodarone, beta-blockers, sotalol, or ablation. A total of 81 patients received an implantable cardioverter-defibrillator, 34 were successfully defibrillated. CONCLUSIONS The high risk of life-threatening arrhythmias in patients with ARVC spans from adolescence to advanced age, reaching its peak between ages 21 and 40 years. Atrial fibrillation, syncope, participation in strenuous exercise after the diagnosis of ARVC, hemodynamically tolerated sustained monomorphic ventricular tachycardia, and male sex predicted lethal arrhythmias at follow-up. The lack of efficacy of antiarrhythmic therapy and the life-saving role of the implantable cardioverter-defibrillator highlight the importance of risk stratification for patient management. (C) 2016 by the American College of Cardiology Foundation.

【 授权许可】

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