Left Ventricular Involvement in ARVD/C: Is It Time to Readjust Our Views?

Abstract

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an important cause of sudden death in young adults.1,2 The past decade has witnessed remarkable progress in the understanding of all aspects of this disease. This progress includes an improved understanding of (1) the natural history of ARVD/C, (2) optimal approaches for diagnosis, (3) the genetic basis of this condition, (4) the fundamental pathophysiologic mechanisms of ARVD/C, (5) the link between ARVD/C and exercise, (6) sudden death prevention, and (7) the importance of left ventricular (LV) involvement in ARVD/C. Article see p 1403 In the present issue of Circulation: Arrhythmia and Electrophysiology , Berte et al3 report the results of their investigation of 32 patients with ARVD/C (47 + 14 years, 6 women). All patients underwent a comprehensive noninvasive evaluation. All patients underwent multidetector computerized tomography. Magnetic resonance imaging (MRI) was performed in 22 patients. The recently described Bordeaux high-dose isoproterenol infusion protocol was used in all patients (45 μg/min for 3 minutes).4 Genetic testing, although not part of the standardized evaluation, was performed in 20 patients, revealing mutations in PKP2 in 9 patients and ACTN2 , TNNC1 , DSC2 , TMEM43 in 1 each. A subset of 14 patients who were referred for ventricular tachycardia (VT) ablation underwent endocardial and epicardial mapping. There are several interesting findings of this study. Right ventricular (RV) fibrosis and wall motion abnormalities were present in 91% and 95% of the 22 patients who underwent cardiac magnetic resonance imaging. Fat was found on multidetector computerized tomography on the …