Unifocalization of Major Aortopulmonary Collateral Arteries and Pulmonary Arteries-Lessons Learned Over 20 years.

BACKGROUND

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a complex and heterogeneous form of congenital heart disease. The purpose of invited review is to provide a 20-year perspective on the lessons learned and related advancements in unifocalization of MAPCAs and pulmonary arteries.

METHODS

This review provides an unapologetic Stanford-centric perspective on the most salient advances in treatment of PA/VSD/MAPCAs.

RESULTS

There have been a multitude of advances in the treatment of PA/VSD/MAPCAs over the past 20 years. Some of these advances have occurred in surgical techniques for performing unifocalization and unifocalization revision. Significant advances have also occurred in the nonsurgical aspects of care. Cumulatively and incrementally, these advances have resulted in better and better results over time. Dissemination of this knowledge and information has been an important step in a more widespread understanding of these techniques and results. Finally, the improvements in results for unifocalization of MAPCAs and pulmonary arteries have further widened the gap compared with the pulmonary artery rehabilitation approach; thus, what was once a controversy, is now largely settled.

CONCLUSIONS

Unifocalization of MAPCAs and pulmonary arteries has undergone numerous refinements over the past 20 years. It is now clear that this is the procedure of choice for patients with single-supply MAPCAs.