Clinical Features, Genetic Findings, and Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy: Data From a Brazilian Cohort

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Olivetti, Natalia Sangiorgi ; Sacilotto, Luciana ; Wulkan, Fanny ; Pessente, Gabrielle D'Arezzo ; de Carvalho, Mariana Lombardi Peres ; Moleta, Danilo ; Hachul, Denise Tessariol ; Veronese, Pedro ; Hardy, Carina ; Pisani, Cristiano ; Wu, Tan Chen ; Vieira, Marcelo Luiz Campos ; de Franca, Lucas Arraes ; Freitas, Matheus de Souza ; Rochitte, Carlos Eduardo ; Bueno, Savia Christina ; Lovisi, Vitor Bastos ; Krieger, Jose Eduardo ; Scanavacca, Mauricio ; Pereira, Alexandre da Costa ; Darrieux, Francisco da Costa

• Document type: Journal article
• Source: CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY; v. 16, n. 2, p. 10-pg., 2023-02-01.

Abstract

Background:Arrhythmogenic right ventricular cardiomyopathy (ARVC), a rare inherited disease, causes ventricular tachycardia, sudden cardiac death, and heart failure (HF). We investigated ARVC clinical features, genetic findings, natural history, and the occurrence of life-threatening arrhythmic events (LTAEs), HF death, or heart transplantation (HF-death/HTx) to identify risk factors. Methods:The clinical course of 111 consecutive patients with definite ARVC, predictors of LTAE, HF-death/HTx, and combined events were analyzed in the entire cohort and in a subgroup of 40 patients without sustained ventricular arrhythmia before diagnosis. Results:The 5-year cumulative probability of LTAE was 30% and HF-death/HTx was 10%. Predictors of HF-death/HTx were reduced right ventricle ejection fraction (HR: 0.93; P=0.010), HF symptoms (HR: 4.37; P=0.010), epsilon wave (HR: 4.99; P=0.015), and number of leads with low QRS voltage (HR: 1.28; P=0.001). Each additional lead with low QRS voltage increased the risk of HF-death/HTx by 28%. Predictors of LTAE were prior syncope (HR: 1.81; P=0.040), number of leads with T wave inversion (HR: 1.17; P=0.039), low QRS voltage (HR: 1.12; P=0.021), younger age (HR: 0.97; P=0.006), and prior ventricular arrhythmia/ventricular fibrillation (HR: 2.45; P=0.012). Each additional lead with low QRS voltage increased the risk of LTAE by 17%. In patients without ventricular arrhythmia before clinical diagnosis of ARVC, the number of leads with low QRS voltage (HR: 1.68; P=0.023) was independently associated with HF-death/HTx. Conclusions:Our study demonstrated the characteristics of a specific cohort with a high prevalence of arrhythmic burden at presentation, male predominance, younger age and HF severe outcomes. Our main results suggest that the presence and extension of low QRS voltage can be a risk predictor for HF-death/HTx in ARVC patients, regardless of the arrhythmic risk. This study can contribute to the global ARVC risk stratification, adding new insights to the international current scientific knowledge. (AU)

• FAPESP's process: 16/15223-3 - Brazilian national center for molecular diagnosis in inherited cardiac arrhythmias
• Grantee: Alexandre da Costa Pereira
• Support Opportunities: Research Grants - Research in Public Policies for the National Health Care System (PP-SUS)