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Abstract Details
Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease
Roger Luong1, Jeffrey A Feinstein2, Michael Ma3, Noelle H Ebel2, Lisa Wise-Faberowski4, Yulin Zhang3, Lynn F Peng2, Vamsi V Yarlagadda2, Jennifer Shek3, Frank L Hanley3, Doff B McElhinney5
Author information
1Department of Pediatrics, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA. Electronic address: rluong07@stanford.edu.
2Department of Pediatrics, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA.
3Department of Cardiothoracic Surgery, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA.
4Department of Anesthesia, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA.
5Department of Pediatrics, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA; Department of Cardiothoracic Surgery, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA.
Abstract
Objective: To assess outcomes in a large cohort of patients with Alagille syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex pulmonary artery disease.
Study design: Patients with ALGS who underwent pulmonary artery reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch pulmonary artery stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs.
Results: Fifty-one patients with ALGS underwent pulmonary artery surgery at our center, including 22 with severe branch pulmonary artery stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) underwent complete repair at the first surgery, compared with 8 (89%) with TOF without MAPCAs and 19 (86%) with isolated branch pulmonary artery stenosis. At a median follow-up of 1.7 years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median pulmonary artery:aortic systolic pressure of 0.38. Nine patients (18%), including 8 with isolated branch pulmonary artery stenosis, underwent liver transplantation.
Conclusions: Most patients with ALGS and complex pulmonary artery disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcomes, with higher mortality and more frequent pulmonary artery interventions compared with patients with TOF without MAPCAs or isolated branch pulmonary artery stenosis. Complex pulmonary artery disease is not a contraindication to liver transplantation in patients with ALGS.