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Although patients with systemic right ventricle (SRV) represent a significant proportion of patients with congenital heart disease (CHD) implanted with cardiac resynchronization therapy (CRT), there are limited and conflicting data in this specific patient group.We aimed to analyze outcomes of patients with SRV implanted with a CRT device.Data were analyzed from an observational, retrospective, multicenter cohort study including all patients with CHD implanted with a CRT device from 6 French centers from 2004 to 2020. Response to CRT was defined as an increase in systemic ventricular ejection fraction of ≥10% and/or an improvement in New York Heart Association functional class by at least 1 grade.A total of 85 patients with CHD were enrolled (mean age 39.8 ± 20.0 years; 55 [64.7%] males; 25 defibrillators [29.4%]), including 31 patients with SRV (36.5%) (mean age 43.9 ± 19.8 years; 16 [51.6%] males). The mean change in QRS duration after implantation was similar as compared with patients with systemic left ventricle (-46 ± 26 ms vs -35 ± 32 ms; P = .16). During a mean follow-up of 5.1 ± 3.5 years, late complications included 2 lead dysfunctions (6.5%), 3 CRT-related infections (9.7%), and 1 inappropriate implantable cardioverter-defibrillator shock (3.2%). The proportion of CRT responders at 6, 12, and 24 months were 82.6%, 80.0%, and 77.8% in patients with SRV vs 66.7%, 64.3%, and 69.6% in patients with systemic left ventricle (P = NS).In this multicenter cohort, one-third of patients with CHD implanted with a CRT device had SRV. CRT in patients with SRV was associated with a high rate of responders, comparable to that of patients with systemic left ventricle.

Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): French Federation of Cardiology Background The number of adults with atrioventricular septal defects (AVSD) is growing, with however, very few data regarding the natural history of atrial arrhythmias in this specific population. We aimed to assess the incidence, associated factors and outcomes of atrial arrhythmias among adult patients with AVSD. Methods Multicentric retrospective cohort of patients with AVSD from 3 referral centers specialized in adult congenital heart disease. Unbalanced AVSD, univentricular hearts, and Eisenmenger syndromes were excluded. Lifetime cumulative incidences of different types of atrial arrhythmia (>30 seconds) were analyzed (atrial fibrillation [AF] and intra atrial reentrant tachycardia/focal atrial tachycardia [IART/FAT]). Multiple logistic regression models were used to identify risk factors for atrial arrhythmias. Results The cohort comprised of 391 patients (61.6% of women) with a mean age of 36.3 ± 16.3 years and 17.3 ± 14.2 years of follow-up after surgical repair in operated patients, including 333 (85.1%) partial/intermediate and 58 (1.0%) complete AVSD. Overall, atrial arrhythmias were documented in 98 patients (25.1%). The lifetime risks for developing atrial arrhythmia to ages 20, 40, and 60 were 3.7%, 17.6%, and 54.8%. IART/FAT was the leading arrhythmia until the age of 45 then AF surpassed IART/FAT. Age (OR = 1.4, 95%CI = 1.2-1.6 by 5 years increment), the number of cardiac surgeries (OR = 4.1, 95%CI = 2.5-6.9), left atrial dilatation (OR = 3.1, 95%CI = 1.4-6.8), right atrial dilatation (OR = 4.1, 95%CI = 1.7-10.3), and moderate or severe left AV valve regurgitation (OR = 3.7, 95%CI = 1.2-11.7) were independently associated with a higher risk of atrial arrhythmias. Patients with atrial arrhythmias more frequently had pacemaker implantation (41.8% vs. 8.5%, p Conclusions The lifetime risk of atrial arrhythmias in patients with AVSD is considerable with more than half of patients who will develop an atrial arrhythmia by the age of 60. Atrial arrhythmias are associated with a significant morbidity in this population. Abstract Figure. Central Illustration AVSD