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Updated: Dec 7, 2025

Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery ALCAPA
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Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery ALCAPA

Published on: April 24, 2017

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Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease

Roger Luong1, Jeffrey A Feinstein1, Michael Ma2

  • 1Department of Pediatrics, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA.

The Journal of Pediatrics
|September 27, 2020
PubMed

Insights

Pulmonary artery reconstruction surgery can lead to successful outcomes in Alagille syndrome (ALGS) patients with complex pulmonary artery disease. However, outcomes vary by diagnosis, with Tetralogy of Fallot and major aortopulmonary collateral arteries presenting the greatest challenges.

Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Genetics

Background:

  • Alagille syndrome (ALGS) is a genetic disorder that can cause complex pulmonary artery disease.
  • Pulmonary artery reconstruction surgery is a potential treatment for these patients.
  • Outcomes of this surgery in ALGS patients are not well-established.

Purpose of the Study:

  • To assess the outcomes of pulmonary artery reconstruction surgery in a large cohort of patients with Alagille syndrome.
  • To compare outcomes based on the primary cardiovascular diagnosis.

Main Methods:

  • Retrospective review of Alagille syndrome patients who underwent pulmonary artery reconstruction surgery.
  • Cohort analysis based on diagnosis: severe isolated branch pulmonary artery stenosis, Tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs.

Main Results:

  • Eighty percent of 51 ALGS patients achieved complete repair.
  • Patients with TOF/MAPCAs had lower complete repair rates (25%) and worse outcomes compared to other groups.
  • At 1.7 years follow-up, 76% of patients were alive with a complete repair.

Conclusions:

  • Most ALGS patients with complex pulmonary artery disease can achieve complete repair with acceptable outcomes.
  • TOF/MAPCAs are associated with higher mortality and increased need for interventions.
  • Complex pulmonary artery disease does not preclude liver transplantation in ALGS patients.
Abstract

Keywords:
cardiovascular interventioncongenital heart diseaseisolated pulmonary artery stenosisliver diseaseliver transplantationmajor aortopulmonary collateral arteriespulmonary artery reconstructiontetralogy of Fallot

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