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Pulmonary Atresia With Intact Septum Survival, Reintervention, and Days in Hospital Through Childhood: Contemporary

Dan-Mihai Dorobantu1,2, Qi Huang3, Ferran Espuny-Pujol3,4

  • 1Children's Health and Exercise Research Centre (CHERC), Medical School, University of Exeter, United Kingdom (D.-M.D.).

Circulation. Cardiovascular Interventions
|August 14, 2026
PubMed

Insights

Transcatheter right ventricular decompression is the preferred initial treatment for pulmonary atresia with intact ventricular septum, showing improved outcomes. Biventricular repair offers better long-term survival but requires more reinterventions than single-ventricle palliation.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Surgical Outcomes

Background:

  • Pulmonary atresia with intact ventricular septum (PA/IVS) management has evolved.
  • Increased use of transcatheter right ventricular decompression (RVAD) is noted.
  • Contemporary outcomes following RVAD in PA/IVS patients are largely unknown.

Purpose of the Study:

  • To evaluate contemporary national outcomes in PA/IVS patients aged 0-18 years.
  • To assess the impact of different treatment pathways on mortality, reintervention, and hospital stay.

Main Methods:

  • Retrospective analysis of PA/IVS patients (2000-2022) in England and Wales.
  • Linking national data sets for comprehensive outcome assessment.
  • Stratification by initial intervention: RVAD, stage 1 palliation, or pre-intervention death/loss.

Main Results:

  • 58.8% underwent RVAD (84.8% transcatheter); 32.4% received stage 1 palliation.
  • 54.5% achieved biventricular (BiV) repair; 26.7% underwent single-ventricle palliation.
  • 1-year mortality: 7.7% for RVAD vs. 37.2% for stage 1 palliation. 10-year reintervention: 62.6% post-BiV repair vs. 32.8% post-stage 3 palliation.

Conclusions:

  • Transcatheter RVAD is the predominant initial intervention for PA/IVS, with lower in-hospital mortality than historical series.
  • BiV repair is achievable in over half of patients, offering better long-term survival but higher reintervention rates.
  • Early mortality remains high in severe cases; single-ventricle palliation has lower reintervention but more hospital days.
Abstract

Keywords:
cardiac surgical proceduresheart defects, congenitallength of staypulmonary atresiarisk factors

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