Abstract

Introduction: Sinus venosus atrial septal defect (SVASD) is a rare congenital cardiac anomaly comprising 5-10% of all atrial septal defects. Although surgical closure is the standard treatment for SVASD, data on outcomes have been confined to small cohorts. Thus, we conducted a systematic review and meta-analysis of outcomes of SVASD repair. Methods: A comprehensive search of PROSPERO, MEDLINE, EMBASE, and Cochrane Library databases was conducted. The primary outcome was mortality. Secondary outcomes encompassed atrial fibrillation, sinus node dysfunction, pacemaker insertion, cerebrovascular accident (CVA), reoperation, residual septal defect, superior vena cava (SVC) obstruction, and reimplanted pulmonary vein obstruction. Pooled incidences of outcomes were calculated using a random effects model. Results: Forty studies involving 1,292 patients who underwent SVASD repair were included. The majority were male (59.4%), with 92.9% presenting with associated pulmonary anomalous venous connection. The weighted mean ages were 18.6 ± 12.5 years, and the overall weighted mean follow-up period was 8.6 ± 10.4 years. In-hospital mortality rate was 0.24%, with a 30-day mortality of 0.56% reported in 720 patients. Incidences of atrial fibrillation, sinus node dysfunction, pacemaker insertion, and CVA over the long-term follow-up were 2% (1-3%), 4% (2-6%), 2% (1-2%), respectively. Reoperation, residual septal defect, SVC obstruction, and reimplanted pulmonary vein obstruction occurred in 1% (0-2%), 2% (2-2%), 1% (1-1%), and 2% (2-2%) surgeries, respectively. Conclusions: This is the first comprehensive analysis of outcomes following surgical repair of SVASD. The findings affirm the safety and effectiveness of surgical intervention, establishing a reference point for evaluating emerging transcatheter therapies. Comparable safety and efficacy profiles to surgical repair are essential for widespread adoption of transcatheter treatments.

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