Outcomes After Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: A Tailored Approach in a Developing Setting

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.

A Novel 3D Visualized Operative Procedure in the Single-Stage Complete Repair With Unifocalization of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

“…Although this operation is routine and has achieved good results in some groups, such as the Stanford group ( 1 ), the Birmingham group ( 4 , 5 ), the Toronto group ( 7 , 21 ), and the Rome group ( 6 ), the above complexities still lead very few other groups to routinely and skillfully perform it, especially in developing countries like China. However, in developing countries, the age of the first treatment for patients with PA/VSD/MAPCAs is usually older ( 22 , 23 ); thus, the best time to conduct the rehabilitation strategy may have passed and they are more in need of the midline unifocalization strategy. In the past, since many patients had some MAPCAs originating from the descending aorta, the dissection of these MAPCAs was completed through the posterolateral thoracotomy approach for the sake of safety, and the median sternotomy was then performed to complete the dissection of other MAPCAs and the rest of the operation in our center and some other centers ( 24 ).…”

Current development of bovine jugular vein conduit for right ventricular outflow tract reconstruction

“…The Contegra has been available in a ring-supported model, which has two external polypropylene rings sutured to the adventitial layer of the conduit intended for limiting dilatation at the valvular section ( Boudjemline et al, 2003b ; Morray et al, 2017 ), although no clinically significant echocardiographic or outcome differences seemed to have been found between the ring-supported and unsupported Contegras ( Lueth et al, 2019 ). The Balance Medical BJVC also has a polyester film covering the outer wall of the vein to prevent dilatation, and the device has been preferred for older children with hypoplastic pulmonary arteries ( Zou et al, 2021 ).…”