JACC. Clinical electrophysiology · 2025
Clinical Presentation and Long-Term Outcomes of Late-Onset Symptomatic ARVC.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Uetake Shunsuke, Alahwany Salah H, Togashi Daisuke, Katsume Yumi, Davogustto Giovanni E, Yoneda Zachary T, Richardson Travis D, Kanagasundram Arvindh N, Stevenson William G, Tandri Harikrishna
Аннотация
BACKGROUND: Diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) is often made after arrhythmias are detected in the second or third decade but can also present later.
OBJECTIVES: The authors sought to compare the phenotypes and the long-term outcome between patients with early- vs late-onset ARVC.
METHODS: Patients with a definite ARVC diagnosis fulfilling the 2010 Task Force criteria and symptomatic arrhythmias at initial presentation were candidates. Based on age of onset of symptoms, patients were divided into 2 groups: early ARVC: ≤40 years (E-ARVC) and late ARVC: >40 years (L-ARVC). Clinical characteristics and outcomes were compared between the groups.
RESULTS: Between 2004 and 2024, 76 probands were included. Fifty patients were in the E-ARVC group (median 27.5 years) and 26 in L-ARVC (45.5 years). Gender, genotype, family history, and RV function did not show any statistical differences. Patients with L-ARVC were significantly less likely to present with cardiac arrest or syncope than E-ARVC patients (7.6% vs 40.0%; P = 0.003). During follow-up, patients with L-ARVC had a slower VT cycle length (321.5 ms vs 259.5 ms; P < 0.001), lower incidence of electrical storm (19.2% vs 54.0%; P = 0.004), and fewer ICD therapies (42.3% vs 80.0%; P < 0.001) than patients with E-ARVC. During a mean follow-up of 11.1 years, there was a lower incidence of heart failure in L-ARVC patients (46.0% vs 19.2%; P = 0.028). VT ablation was performed with similar success rates.
CONCLUSIONS: Clinical onset of ARVC after age 40 years is usually less severe than early-onset disease, despite similar structural phenotype, and is associated with slower VTs and less VT storm, and appears to have a lower risk of progression to clinical heart failure.
OBJECTIVES: The authors sought to compare the phenotypes and the long-term outcome between patients with early- vs late-onset ARVC.
METHODS: Patients with a definite ARVC diagnosis fulfilling the 2010 Task Force criteria and symptomatic arrhythmias at initial presentation were candidates. Based on age of onset of symptoms, patients were divided into 2 groups: early ARVC: ≤40 years (E-ARVC) and late ARVC: >40 years (L-ARVC). Clinical characteristics and outcomes were compared between the groups.
RESULTS: Between 2004 and 2024, 76 probands were included. Fifty patients were in the E-ARVC group (median 27.5 years) and 26 in L-ARVC (45.5 years). Gender, genotype, family history, and RV function did not show any statistical differences. Patients with L-ARVC were significantly less likely to present with cardiac arrest or syncope than E-ARVC patients (7.6% vs 40.0%; P = 0.003). During follow-up, patients with L-ARVC had a slower VT cycle length (321.5 ms vs 259.5 ms; P < 0.001), lower incidence of electrical storm (19.2% vs 54.0%; P = 0.004), and fewer ICD therapies (42.3% vs 80.0%; P < 0.001) than patients with E-ARVC. During a mean follow-up of 11.1 years, there was a lower incidence of heart failure in L-ARVC patients (46.0% vs 19.2%; P = 0.028). VT ablation was performed with similar success rates.
CONCLUSIONS: Clinical onset of ARVC after age 40 years is usually less severe than early-onset disease, despite similar structural phenotype, and is associated with slower VTs and less VT storm, and appears to have a lower risk of progression to clinical heart failure.