Clinical Features, Genetic Findings, and Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy: Data From a Brazilian Cohort.
Circ Arrhythm Electrophysiol
; 16(2): e011391, 2023 02.
Article
em En
| MEDLINE
| ID: mdl-36720007
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.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Displasia Arritmogênica Ventricular Direita
/
Insuficiência Cardíaca
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Female
/
Humans
/
Male
País como assunto:
America do sul
/
Brasil
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article