Clinical Classification of Arrhythmogenic Right Ventricular Cardiomyopathy

Yulia Lutokhina; Olga Blagova; Alexander Nedostup; Svetlana Alexandrova; Anna Shestak; Elena Zaklyazminskaya

doi:10.1159/000505652

Clinical Classification of Arrhythmogenic Right Ventricular Cardiomyopathy

Pulse (Basel). 2020 Aug;8(1-2):21-30. doi: 10.1159/000505652. Epub 2020 Feb 11.

Authors

Yulia Lutokhina¹, Olga Blagova¹, Alexander Nedostup¹, Svetlana Alexandrova², Anna Shestak³, Elena Zaklyazminskaya³

Affiliations

¹ Department of Cardiology of the V.N. Vinogradov Faculty Therapeutic Clinic, I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation.
² Department of Computer and Magnetic Resonance Tomography, A.N. Bakoulev Center for Cardiovascular Surgery RAMS, Moscow, Russian Federation.
³ Laboratory of Medical Genetics, B.V. Petrovsky Russian Research Center of Surgery, Moscow, Russian Federation.

Abstract

Introduction: Commonly accepted clinical classification of arrhythmogenic right ventricular cardiomyopathy (ARVC) is still not developed.

Objective: To study the clinical forms of ARVC.

Methods: Fifty-four patients (38.7 ± 14.1 years, 42.6% men) with ARVC. Follow-up period: 21 (6-60) months. All patients underwent electrocardiography, 24 h-Holter monitoring, echocardiography, and DNA diagnostic. Magnetic resonance imaging was performed in 49 patients.

Results: According to the features of clinical course of ARVC, 4 clinical forms were identified. (I) Latent arrhythmic form (n = 27) - frequent premature ventricular contractions and/or nonsustained ventricular tachycardia (VT) in the absence of sustained VT and syncope; characterized by absence of fatal arrhythmic events. (II) Manifested arrhythmic form (n = 11) - sustained VT/ventricular fibrillation; the high incidence of appropriate implantation of cardioverter-defibrillator (ICD) interventions (75%) registered. (III) ARVC with progressive chronic heart failure (CHF, n = 8) as the main manifestation of the disease; incidence of appropriate ICD interventions was 50%, mortality rate due to CHF was 25%. (IV) Combination of ARVC with left ventricular noncompaction (n = 8); characterized by mutations in desmosomal or sarcomere genes, aggressive ventricular arrhythmias, appropriate ICD interventions in 100% patients. Described 4 clinical forms are stable in time, do not transform into each other, and they are genetically determined.

Conclusions: The described clinical forms of ARVC are determined by a combination of genetic and environmental factors and do not transform into each other. The proposed classification could be used in clinical practice to determine the range of diagnostic and therapeutic measures and to assess the prognosis of the disease in a particular patient.

Keywords: Arrhythmogenic right ventricular dysplasia/cardiomyopathy; Chronic heart failure; Left ventricular noncompaction; Premature ventricular beats; Ventricular tachycardia.