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Updated: Jan 29, 2026
Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
Published on: June 27, 2025
Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum
Lydia K Wright1,2, Jessica H Knight3, Amanda S Thomas1
1Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, USA.
Insights
Long-term survival for Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) patients is poor due to early mortality. However, survival into adulthood is excellent for those who complete their intended repair strategy.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery Outcomes
Background:
- Pulmonary atresia with intact ventricular septum (PA/IVS) is a complex congenital heart defect requiring early intervention.
- Understanding long-term outcomes is crucial for guiding treatment strategies and improving patient survival.
- Previous studies have focused on early outcomes, with less data on long-term transplant-free survival across different interventions.
Purpose of the Study:
- To evaluate the long-term transplant-free survival rates in patients diagnosed with PA/IVS.
- To compare survival outcomes based on different initial surgical or catheter-based treatment strategies.
- To identify risk factors associated with mortality in the early stages of PA/IVS management.
Main Methods:
- A cohort study utilizing data from the Pediatric Cardiac Care Consortium, a multi-institutional registry.
- Inclusion criteria: patients with PA/IVS undergoing neonatal surgery or catheter-based intervention between 1982 and 2003.
- Data linkage with the National Death Index and Organ Procurement and Transplantation Network for comprehensive outcome tracking.
Main Results:
- A total of 616 patients received initial interventions (aortopulmonary shunt, right ventricular decompression, or both).
- Risk factors for early death included earlier birth era, chromosomal abnormalities, and coronary ostia atresia.
- Among 491 survivors of initial hospitalization, 20-year survival was 66%. Patients achieving definitive repair had excellent survival: 97.6% for single-ventricle, 90.9% for one-and-a-half ventricle, and 98.0% for two-ventricle repairs.
Conclusions:
- Transplant-free survival in PA/IVS is significantly impacted by infantile and interstage mortality.
- Despite early challenges, patients who successfully complete their intended repair strategy demonstrate excellent long-term survival into early adulthood.
- Treatment strategy plays a key role in long-term outcomes for PA/IVS patients, highlighting the importance of achieving definitive repair.
Objective:
Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. We aim to understand the long-term transplant-free survival of patients with PA/IVS by treatment strategy.
Methods:
Cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional registry with prospectively acquired outcome data after linkage with the National Death Index and the Organ Procurement and Transplantation Network.
Results:
Eligible patients underwent neonatal surgery or catheter-based intervention for PA/IVS between 1982 and 2003 (median follow-up of 16.7 years, IQR: 12.6-22.7). Over the study period, 616 patients with PA/IVS underwent one of three initial interventions: aortopulmonary shunt, right ventricular decompression or both. Risk factors for death at initial intervention included earlier birth era (1982-1992), chromosomal abnormality and atresia of one or both coronary ostia. Among survivors of neonatal hospitalisation (n=491), there were 99 deaths (4 post-transplant) and 10 transplants (median age of death or transplant 0.7 years, IQR: 0.3-1.8 years). Definite repair or last-stage palliation was achieved in the form of completed two-ventricle repair (n=201), one-and-a-half ventricle (n=39) or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle and 98.0% for those with complete two-ventricle repair (log-rank p=0.052).
Conclusions:
Transplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended path independent of type of repair.
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