State of the Art: Complex Peripheral Pulmonary Artery Reconstruction Techniques

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.