Key result
Secondary-prevention ICDs linked to ~2.5-fold more appropriate shocks than primary in Marfan syndrome.
Why the study?
Ventricular arrhythmias and long-term outcomes after implantable cardioverter-defibrillator implantation remain poorly characterized in adults with Marfan syndrome.
What are the long-term rates of appropriate ICD shocks, device-related complications, and ventricular arrhythmia ablation outcomes in adults with Marfan syndrome undergoing primary- versus secondary-prevention ICD implantation?
Cohort (n=783)
What are the long-term rates of appropriate ICD shocks, device-related complications, and ventricular arrhythmia ablation outcomes in adults with Marfan syndrome undergoing primary- versus secondary-prevention ICD implantation?
Answers are grounded in this paper and cite their sources.
Adults with Marfan syndrome who receive an ICD experience a high burden of both appropriate shocks (especially in secondary prevention, though clinically meaningful in primary prevention) and long-term device-related complications.
Background Ventricular arrhythmias and outcomes after implantable cardioverter-defibrillator (ICD) implantation remain poorly characterized in Marfan syndrome (MFS). Objective To characterize appropriate ICD shocks, longitudinal shock burden, device-related complications, and ventricular arrhythmia ablation in adults with MFS. Methods We retrospectively studied 783 adults with confirmed MFS evaluated from 2018 through 2024. Ever-documented appropriate shocks were analyzed in all 56 ICD recipients. Cumulative incidence of first appropriate shock was estimated with the Aalen-Johansen method, treating death before shock as a competing event; prevention groups were compared with Gray's test. Exploratory cause-specific Cox models evaluated prespecified clinical characteristics. Results Twenty-five recipients had a primary-prevention indication and 31 had a secondary-prevention indication. Appropriate shocks occurred in 26 patients (46.4%): 6/25 (24.0%) primary-prevention and 20/31 (64.5%) secondary-prevention recipients. The longitudinal cohort included 52 patients with 23 first-shock events. Five-year cumulative incidence was 18.6% after primary-prevention and 47.0% after secondary-prevention implantation (P<.001). No prespecified structural or clinical characteristic was associated with time to first shock after adjustment for age at implantation, sex, and prevention indication. Device-related complications occurred in 13/56 patients (23.2%). Among 11 with known timing, median time to first complication was 3.5 [1.0–7.8] years; 8 occurred after 1 year. Sixteen patients underwent ventricular arrhythmia ablation, with heterogeneous targets and 2 procedural complications. Conclusion Patients with MFS selected for ICD implantation experienced substantial long-term appropriate-shock and device-complication burdens. Shock burden was greatest after secondary-prevention implantation, while clinically meaningful therapy also occurred after primary-prevention implantation.
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Nabi et al. (2026) conducted a cohort in Marfan syndrome with implantable cardioverter-defibrillator (ICD) (n=783). Secondary-prevention ICD implantation vs. Primary-prevention ICD implantation was evaluated on 5-year cumulative incidence of first appropriate ICD shock (p=<.001). Secondary-prevention ICD implantation in Marfan syndrome had a higher 5-year cumulative incidence of appropriate shocks than primary-prevention ICDs.
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