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Objective: The management of Pulmonary Atresia and Ventricular Septal Defect (PA/VSD) without Major Aortopulmonary Collaterals (MAPCAs) is variable. This study aims to describe the practice patterns, choice of interventions, and outcomes of patients treated using various surgical strategies. Methods: Data from the Public Health Information System (PHIS) database was analyzed for patients with a diagnosis of PA/VSD without MAPCAs who followed a two-ventricle strategy between 2003 and 2023. Patients were categorized based on the initial surgical approach into four groups: single-stage early repair, ductal stent, systemic to pulmonary artery shunt, and right ventricle to pulmonary artery (RV-PA) connection. Outcomes, including in-hospital mortality, ECMO use, and 2-year cumulative mortality rates were analyzed. Results: A total of 304 patients were included in the analysis. Of these, 135 underwent single-stage early repair, 41 had a ductal stent, 97 had a systemic to pulmonary artery shunt, and 31 underwent RV-PA connection. The overall in-hospital mortality varied significantly across groups at initial procedure: 11.85% for single-stage early repair, 4.88% for stent, 2.06% for shunt (p=0.015), and 9.68% for RV-PA connection. ECMO use at initial intervention was 8.89% vs 2.44% vs 3.09% vs 19.35% with no statistical difference between the different groups. Among those staged to complete repair, 22 of the stent group, 82 of the shunt group, and 21 of the RV-PA group achieved full repair with respective in-hospital mortality of 9.09%, 0%, and 4.8% (p=0.004). ECMO use in these groups at complete repair was 4.6%, 9.8%, and 23.8% respectively. The cumulative 2-year mortality (adjusted for era) did not show any statistical significance between the different groups neither did Kaplan Meier analysis with follow up to two years show any statistical difference in survival (Figure 1). Compared to earlier eras, a greater proportion of centers were performing early single stage repair in majority of patients (Figure 2). Conclusion: Both Early and staged repair yield similar early and medium-term outcomes. The increasing utilization of early single-stage repair highlights its growing preference among the participating institutions.

Publication Date

10-2024

Disciplines

Cardiology | Pediatrics

When and Where Presented

Presented at the 51st Annual Congential Heart Surgeon's Society (CHSS) Meeting; October 27-28, 2024; Chicago, Illinois.

Early versus Staged Repair of Pulmonary Atresia with Ventricular Septal Defect without MAPCAs: A PHIS Analysis

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