Abstract
Objectives: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is complex and diverse that has led to a variety of treatment strategies. Experience has been largely obtained in the advanced countries. The clinical diversity is greater in China. We evaluated our surgical approaches and outcomes of these patients.Methods: We reviewed 127 patients undergoing varied surgeries in our center in 2010–2019.Results: Thirty patients underwent single-stage complete repair by unifocalizing MAPCAs and VSD closure (aged 3.9–131.4 months, median 22) with 3 (10%) early deaths. Ninety-seven underwent the first-stage rehabilitation strategy including systemic-to-pulmonary shunt in 29 (aged 0.5–144 month, median 8), and palliative RV-PA conduit in 68 (aged 2.2–209.6 months, median 14) with 5 (5.2%) early deaths. Eight-one patients (63.8%) eventually achieved complete repair with a median right/left ventricular (RV/LV) pressure ratio of 0.7 (ranged 0.4–1.0). Fourteen patients (11.0%) accepted palliation as final destination. Survival for the entire cohort was 89.5, 85.2, and 76.1% at 1, 5, and 10 years, respectively. Survival for those undergoing complete repair was 88.2 and 76.6% at 1 and 5 year, respectively. RV/LV pressure ratio ≥0.8 was risk factor for mortality (HR10.3, p = 0.003).Conclusions: Our cohort, the largest from China, had distinctive clinical features with substantially wider age range and higher RV/LV pressure ratio. Using the combined approaches tailored to individual patients, complete repair was achieved in 64% of patients. The early and intermediate outcomes are acceptable compared to many of the previous reports.
Highlights
Surgical strategies for pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) remain controversial
The 1- and 5-year survival rates after complete repair were 88.2%, and 76.6%, respectively (Figure 2B)
Univariate regression analysis showed postoperative Right ventricle (RV)/Left ventricle (LV) pressure ≥0.8 (HR: 10.35, 95% CI: 2.19–48.93, P = 0.003), single-stage complete repair (HR: 3.49, 95% CI: 1.06–11.49, P = 0.039) and the era of complete repair surgery before 2016
Summary
Surgical strategies for pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) remain controversial. While some reported remarkable outcomes using the early complete unifocalization strategy, others recommended the rehabilitation strategy to promote development of native pulmonary arteries [1, 2]. Both are used in many centers with favorable results [3,4,5]. In China and other developing countries, there is substantial heterogeneity in the age of patients at the first visit and consequent questionable potential for hypoplastic pulmonary arteries to develop and morphologic and structural variabilities in MAPCAs. Selection of surgical strategies for such patients continues to pose challenges.
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
