Long-Term Prognostic Role of the Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Noriko Kikuchi; Dai Yumino; Tsuyoshi Shiga; Atsushi Suzuki; Nobuhisa Hagiwara

doi:10.1016/j.jacep.2015.09.009

Long-Term Prognostic Role of the Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

JACC Clin Electrophysiol. 2016 Feb;2(1):107-115. doi: 10.1016/j.jacep.2015.09.009. Epub 2015 Oct 17.

Authors

Noriko Kikuchi¹, Dai Yumino¹, Tsuyoshi Shiga², Atsushi Suzuki¹, Nobuhisa Hagiwara¹

Affiliations

¹ Department of Cardiology, Tokyo Women's Medical University, Tokyo, Japan.
² Department of Cardiology, Tokyo Women's Medical University, Tokyo, Japan. Electronic address: mshiga@hij.twmu.ac.jp.

PMID: 29766843
DOI: 10.1016/j.jacep.2015.09.009

Abstract

Objectives: The aim of this study was to evaluate the long-term prognostic role of the 2010 task force criteria (TFC)-based scoring in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).

Background: Categories of the 2010 TFC include the risk factors for cardiovascular mortality and sudden cardiac death in patients with ARVC/D.

Methods: Ninety patients with ARVC/D who met the definitive diagnosis of the 2010 TFC were retrospectively studied. ARVC/D risk score was calculated as the sum of major (2 points) and minor (1 point) criteria in all 6 subdivided categories of the TFC and was divided into tertile groups of scores; group A (4 to 6 points), group B (7 to 9 points), and group C (10 to 12 points). The primary endpoints were major adverse cardiovascular events: cardiovascular death, heart failure hospitalization, and sustained ventricular tachycardia or ventricular fibrillation.

Results: During the follow-up period of 10.2 ± 7.1 years, 19 patients died because of cardiovascular causes, 28 patients were admitted because of worsened heart failure, and 47 patients experienced sustained ventricular tachycardia or ventricular fibrillation. Patients in groups B and C were at increased risk for major adverse cardiovascular events compared with those in group A (hazard ratio [HR]: 4.80; 95% confidence interval [CI]: 1.87 to 12.33; p = 0.001; and HR: 6.15; 95% CI: 2.20 to 17.21; p = 0.001, respectively). Patients in groups B and C were at increased risk for sustained ventricular tachycardia or ventricular fibrillation compared with those in group A (HR: 6.64; 95% CI: 2.00 to 22.03; p = 0.002; and HR: 9.18; 95% CI: 2.60 to 32.40; p = 0.001, respectively).

Conclusions: Our study suggests that risk scoring based on the 2010 TFC is useful to predict major adverse cardiovascular events in patients with ARVC/D.

Keywords: arrhythmia; arrhythmogenic right ventricular cardiomyopathy/dysplasia; diagnosis; heart failure; prognosis; risk stratification.