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Pulmonary Atresia with Intact Ventricular Septum, an Evolving Strategy in the Era of PDA Stenting: Single Center

Kamel Shibbani1,2, John Nigro3, Rohit Rao3

  • 1Department of Pediatrics, Section of Cardiology, Rady Children's Hospital, University of California-San Diego, San Diego, CA, USA. kshibbani@uiowa.edu.

Pediatric Cardiology
|January 22, 2025
PubMed

Insights

Management of pulmonary atresia with intact ventricular septum (PA/IVS) involves several strategies. This study reviews outcomes and identifies the tricuspid annulus z-score as a key metric for guiding interventions in PA/IVS patients.

Area of Science:

  • Congenital heart disease
  • Pediatric cardiology
  • Cardiac surgery

Background:

  • Pulmonary atresia with intact ventricular septum (PA/IVS) presents complex management challenges.
  • Current treatment strategies include cardiac transplant, 2-ventricle, 1.5-ventricle, or single-ventricle circulation, with ongoing debate regarding optimal approaches.

Purpose of the Study:

  • To review the management strategies and outcomes of PA/IVS patients at a single institution.
  • To identify metrics that can guide management and initial interventions for PA/IVS.

Main Methods:

  • Retrospective review of 29 PA/IVS patients.
  • Analysis of management strategies: transplant, 2-ventricle, 1.5-ventricle, and single-ventricle circulation.
  • Evaluation of survival rates and tricuspid annulus z-scores.

Main Results:

  • Overall survival was 93.1% (27/29 patients).
  • Management distribution: 3.4% transplant, 41.4% 2-ventricle, 24.1% 1.5-ventricle, 24.1% single-ventricle.
  • Significant differences in TV annulus z-scores among groups, with 1.5-ventricle having the smallest and 2-ventricle having the largest median z-scores.

Conclusions:

  • The tricuspid annulus z-score is a significant differentiator between surgical approaches in PA/IVS.
  • A post-hoc algorithm is proposed to aid in treatment strategy selection for PA/IVS patients.

Keywords:
PDA stentingPulmonary atresia intact ventricular septumRV decompressionRV dependent coronary circulation

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