A Review of the Management of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries

• Published in: The Annals of thoracic surgery Vol. 108; no. 2; pp. 601 - 612
• Main Authors: Soquet, Jerome, Barron, David J., d’Udekem, Yves
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Inc 01-08-2019
• Subjects: pPA; MAPCA; PA; RV; pRV; PA/VSD; TOF; pLV; NPA; tetralogy of Fallot; pulmonary arteries; CHD
• ISSN: 0003-4975; 1552-6259
• DOI: 10.1016/j.athoracsur.2019.01.046

The management of pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) has significantly changed over the past 20 years. Unifocalization and rehabilitation have been described as diametrically opposed strategies. An updated review focused on the management of this complex and rare condition is needed.
Articles related to PA/VSD/MAPCAs issued until December 2017 were screened. Twelve main studies published in the modern era (since 2000) were selected and analyzed.
Unifocalization and rehabilitation respectively focus on the mobilization of collateral arteries and the growth of native pulmonary vessels. A third strategy, called “combined strategy,” was distinguished from the review of the literature. Surgical cohorts and methods of data reporting were found to be heterogenous. Outcomes, regardless of the strategy, have transformed the natural history of the condition, with a complete repair rate of approximately 80% and low rates of early and late mortality. Patients with the most unfavorable anatomy (absent central pulmonary arteries and hypoplastic MAPCAs) remain a challenge and are still left palliated.
Variable surgical strategies are used in the management of PA/VSD/MAPCAs. Most teams report a repair rate of 70% to 80% and a mortality rate lower than 10%. Standardization in data reporting is necessary to better compare the existing strategies.
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