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Original research article
Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum
  1. Lydia K Wright1,2,
  2. Jessica H Knight3,
  3. Amanda S Thomas1,
  4. Matthew E Oster1,2,
  5. James D St Louis4,
  6. Lazaros K Kochilas1,2
  1. 1 Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, USA
  2. 2 Sibley Heart Center, Children’s Healthcare of Atlanta, Atlanta, GA
  3. 3 Department of Epidemiology and Biostatistics, University of Georgia School of Public Health, Athens, GA
  4. 4 Department of Pediatric Surgery, University of Missouri-Kansas City School of Medicine, Kansas City, Missouri, USA
  1. Correspondence to Dr Lazaros K Kochilas, Department of Pediatrics, Emory University School of Medicine and Children’s Healthcare of Atlanta, Atlanta GA 30341, USA; lazaros.Kochilas{at}


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.

  • pulmonary atresia
  • congenital heart defect
  • congenital heart surgery
  • long-term outcomes

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  • Contributors LKW and LKK conceived and designed the study. Data acquisition was performed by LKW and AST. Statistical analyses were performed by JHK and AST. MEO and JDSL provided data interpretation and critical revision of the manuscript. LKW, LKK and JHK drafted the manuscript and all authors approved the final version. LKW and LKK are responsible for the overall content as guarantors.

  • Funding This study was supported by the National Heart, Lung, and Blood Institute (R01 HL122392).

  • Disclaimer The data reported here have been supplied by the United Network for Organ Sharing (UNOS) as the contractor for the Organ Procurement and Transplantation Network (OPTN). The interpretation and reporting of these data are the responsibility of the author(s) and in no way should be seen as an official policy of or interpretation by the OPTN or the US Government.

  • Competing interests None declared.

  • Ethics approval The study was approved by the institutional review board at Emory University, the National Death Index and the United Network for Organ Sharing that maintains the Organ Procurement and Transplantation Network.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement A database of included patients was created from the larger PCCC database and is available in spreadsheet form. Additional unpublished data include additional cardiac surgeries or catheter-based procedures undergone in a PCCC centre. This can be shared by contacting the corresponding author.

  • Patient consent for publication Not required.