Clinical Features and Outcomes in Adults With Childhood Repair of Partial Atrioventricular Septal Defect

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BackgroundPartial atrioventricular septal defects (pAVSDs) are mostly repaired in childhood; however, there are limited data describing these patients in adulthood. ObjectivesThe objective of this study was to describe clinical course and associations with outcomes in adults with repaired pAVSDs. MethodsA retrospective review of adults (≥18 years) with pAVSDs repaired in childhood who presented to the Adult Congenital Heart Disease Clinic at our institution was conducted. ResultsOf 121 patients, the median age was 31 years (IQR: 22-43 years) and 71.9% were female. The median number of operations at the time of presentation was 1 (IQR: 1-2). Left atrioventricular valve (LAVV) replacement had been performed in 19.8% of patients. Among those with native LAVV, 41.2% had ≥ moderate regurgitation. Atrial arrhythmias were present in 34.7% and were associated with later age at repair (P = 0.02) and a high number of prior surgeries (P = 0.005). Estimated systolic pulmonary artery pressure >40 mmHg was seen in 19.8%. Over 4 (IQR: 1-12) years of follow-up, death occurred in 13 (10.7%) patients and reoperation was required in 39.7%. One-third had a LAVV prosthesis by the end of the study. Atrial fibrillation was independently associated with death or hospitalization on multivariable analysis. ConclusionsIn this cohort of adults with pAVSDs repaired in childhood, atrial fibrillation was common at a young age and associated with worse outcomes. Thus, more studies are needed evaluating the cause of this arrhythmia burden and possible associated atrial myopathy. While many require surgery in adulthood, more information is needed regarding indications for and impacts of LAVV intervention as one-third had an LAVV prosthesis by the end of follow-up.