2025 American Association for Thoracic Surgery Congenital Cardiac Surgery Working Group- Expert consensus document on the management of patients with pulmonary atresia with intact ventricular septum.

BACKGROUND

Pulmonary atresia with intact ventricular septum (PAIVS) presents significant morphologic and pathophysiologic variation. Outcomes depend on individual patient characteristics and the determination of appropriate triage for neonatal catheter-based and surgical therapy. Therapeutic interventions and practice patterns vary significantly. There is currently no consensus on many diagnostic and therapeutic options in clinical care, and sizeable gaps in evidence exist.

METHODS

The American Association for Thoracic Surgery Congenital Clinical Practice Standards Committee identified PAIVS as a complex lesion for which practitioners could benefit from an expert consensus document to provide management guidelines. This document focuses on the diagnosis, triage, identification and timing of optimal surgical or interventional strategies, and postinterventional care of patients with PAIVS.

RESULTS/

CONCLUSIONS

In this document, we identify gaps in knowledge in the care for PAIVS and provide evidence-based and expert-derived consensus statements with available strength of class of recommendation and level of evidence with supporting data and discussion. Section 1 addresses the current state of fetal diagnosis and management and the potential indications for fetal interventions. Section 2 discusses postnatal diagnosis and anatomic characterization, including right ventricle, tricuspid valve, and coronary circulation, as well as the appropriate triage of patients for initial management strategies. Section 3 concentrates on the relative efficacy of procedures to establish pulmonary blood flow and decompress the right ventricle when indicated. We also examine the appropriate use of mechanical circulatory support and indications for heart transplantation in patients with the most severe form of PAIVS. Finally, Section 4 focuses on long-term outcomes, especially with single-ventricle palliation and ongoing risk related to possible ischemia in patients with right ventricle-dependent coronary circulation. Much remains to be learned concerning the care of children and adults with PAIVS. Future research and careful review of outcomes will continue to guide clinical decision-making.