Elisabeth Martin, Michael Ma, Frank Hanley
Stanford University School of Medicine and Lucile Packard Children’s Hospital.
United States
Seminars in Thoracic and Cardiovascular Surgery Pediatric Cardiac Surgery Annual
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2025; 28: 101-106
DOI: 10.1053/j.pcsu.2025.03.002
Abstract
Over the last several years, we developed and improved our surgical approach to patients requiring pulmonary artery reconstruction in the setting of TOF-PA-MAPCA and other forms of peripheral pulmonary artery stenosis (PPAS). PPAS is a relatively rare form of congenital heart disease and typically associated with genetic syndromes, such as Williams or Alagille syndromes. We no longer do a staged approach and use a midline incision in all cases. We have now operated on almost 1000 patients with TOF-PA-MAPCA or extensive bilateral PPAS. Early mortality ranges from 2.1% (3/145 PPAS) to 4.9% (38/780 TOF-PA-MAPCA, total cohort). In our TOF-PA-MAPCA cohort, independent factors for early mortality were Alagille syndrome (HR 2.8, 95% CI: 1.4-5.7; P < 0.004), any preoperative respiratory support (HR 2.0, 95% CI: 1.2-3.3; P < 0.008), and previous palliative surgery at our center (HR 3.5, 95% CI: 2.3-5.4; P < 0.001) on multivariable Cox regression analysis. Complete intracardiac repair was achieved in 90% (704/780) of this cohort. This document reports our surgical approach to pulmonary artery reconstruction with emphasis on certain key concepts. Our surgical strategy is applicable to essentially every patient with TOF-PA-MAPCA or PPAS.
Category
Segmental Pulmonary Arterial Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
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