20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries

Doff B McElhinney; Ritu Asija; Yulin Zhang; Ayush Jaggi; Jennifer Shek; Lynn F Peng; M Gail Boltz; Michael Ma; Elisabeth Martin; Frank L Hanley

doi:10.1016/j.jacc.2023.06.041

20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries

J Am Coll Cardiol. 2023 Sep 19;82(12):1206-1222. doi: 10.1016/j.jacc.2023.06.041.

Authors

Affiliations

¹ Department of Cardiothoracic Surgery, Lucile Packard Children's Hospital Heart Center Clinical and Translational Research Program, Stanford University School of Medicine, Stanford, California, USA; Department of Pediatrics, Lucile Packard Children's Hospital Heart Center Clinical and Translational Research Program, Stanford University School of Medicine, Stanford, California, USA. Electronic address: doff@stanford.edu.
² Department of Pediatrics, Lucile Packard Children's Hospital Heart Center Clinical and Translational Research Program, Stanford University School of Medicine, Stanford, California, USA.
³ Department of Cardiothoracic Surgery, Lucile Packard Children's Hospital Heart Center Clinical and Translational Research Program, Stanford University School of Medicine, Stanford, California, USA.
⁴ Department of Anesthesia, Perioperative, and Pain Medicine, Lucile Packard Children's Hospital Heart Center Clinical and Translational Research Program, Stanford University School of Medicine, Stanford, California, USA.

PMID: 37704311
DOI: 10.1016/j.jacc.2023.06.041

Abstract

Background: We have followed a consistent, albeit evolving, strategy for the management of patients with pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) that aims to achieve complete repair with low right ventricular pressure by completely incorporating blood supply and relieving stenoses to all lung segments.

Objectives: The purpose of this study was to characterize our 20-year institutional experience managing patients with MAPCAs.

Methods: We reviewed all patients who underwent surgery for MAPCAs and biventricular heart disease from November 2001 through December 2021.

Results: During the study period, 780 unique patients underwent surgery. The number of new patients undergoing surgery annually was relatively steady during the first 15 years, then increased substantially thereafter. Surgery before referral had been performed in almost 40% of patients, more often in our recent experience than earlier. Complete repair was achieved in 704 patients (90%), 521 (67%) during the first surgery at our center, with a median right ventricular to aortic pressure ratio of 0.34 (25th, 75th percentiles: 0.28, 0.40). The cumulative incidence of mortality was 15% (95% CI: 12%-19%) at 10 years, with no difference according to era of surgery (P = 0.53). On multivariable Cox regression, Alagille syndrome (HR: 2.8; 95% CI: 1.4-5.7; P = 0.004), preoperative respiratory support (HR: 2.0; 95% CI: 1.2-3.3; P = 0.008), and palliative first surgery at our center (HR: 3.5; 95% CI: 2.3-5.4; P < 0.001) were associated with higher risk of death.

Conclusions: In a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis and MAPCAs have continued to improve.

Keywords: balloon angioplasty; pulmonary artery; pulmonary atresia; surgery; tetralogy of Fallot.

MeSH terms

Aorta
Constriction, Pathologic
Heart Defects, Congenital*
Humans
Pulmonary Artery / surgery
Pulmonary Atresia* / surgery