Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: Part II-Midline Unifocalization and Unifocalization Revision

摘要

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare and heterogeneous form of congenital heart disease. Despite improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. Part II of this paper will focus on the surgical results of unifocalization and the need and techniques used for unifocalization revision at Stanford University. We have an experience with more than 300 patients undergoing primary surgical treatment of PA/VSD/MAPCAs. Out of this entire cohort, 80% were selected for a midline unifocalization approach. For patients undergoing a primary unifocalization, 85% underwent a single-stage complete repair, while 15% had a unifocalization and shunt. Ninety-five percent of these patients ultimately achieve complete repair status with a mean right ventricle to pulmonary artery pressure ratio of 0.36 ± 0.09. Of all the patients who undergo a primary unifocalization, 19% will subsequently require unifocalization revision. This was unequally divided into patients who had an initial single-stage complete repair (where 15% required revision) compared with those who had a unifocalization/shunt (46% required revision). The majority of patients (74%) who underwent unifocalization revision had a satisfactory outcome. The surgical treatment of PA/VSD/MAPCAs is complicated due to heterogeneity of MAPCA and pulmonary artery anatomy. The preponderance (80%) of patients treated at Stanford is selected for midline unifocalization. The results of midline unifocalization demonstrate that a high percentage of patients achieve full repair with satisfactory right ventricular pressure ratios.