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Pulmonary atresia (PA), ventricular septal defect (VSD), with major aortopulmonary collateral arteries (MAPCAs) is an extreme variant of tetralogy of Fallot characterized by a lack of luminal continuity between the right ventricle and pulmonary artery tree, with pulmonary blood flow sourced through isolated MAPCAs, or a combination of MAPCAs and a unilateral ductus arteriosus, including absent development of central hilar pulmonary arteries. Anomalies in pulmonary artery architecture and arborization are common. This heterogeneity underlies the nuances in peri- and intraoperative decision-making.

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Complications Associated with Pulmonary Atresia with Major Aortopulmonary Collateral Arteries

  • 章节

  • 发表日期：

    2025年

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  • Arvind Bishnoi,
  • Michael Ma

摘要

Pulmonary atresia (PA), ventricular septal defect (VSD), with major aortopulmonary collateral arteries (MAPCAs) is an extreme variant of tetralogy of Fallot characterized by a lack of luminal continuity between the right ventricle and pulmonary artery tree, with pulmonary blood flow sourced through isolated MAPCAs, or a combination of MAPCAs and a unilateral ductus arteriosus, including absent development of central hilar pulmonary arteries. Anomalies in pulmonary artery architecture and arborization are common. This heterogeneity underlies the nuances in peri- and intraoperative decision-making.