Abstrait

Catheterization and surgical collaboration in the treatment of complex congenital heart disease with discontinuous pulmonary arteries.

Ishita Datta, John P. Breinholt

A term, male neonate presents with pulmonary atresia, discontinuous branch pulmonary arteries (PA) arising from independent ducti arteriosus, and dextrocardia presents a challenging treatment plan. The right pulmonary artery (RPA) arose from the ascending aorta while the left pulmonary artery (LPA) arose from the left subclavian artery, with the proximal segments of each spaced about 1 cm apart, making primary unifocalization suboptimal. The cardiac anatomy included: dextrocardia with situs inversus, double outlet right ventricle (DORV) with malposed great arteries and pulmonary atresia, left sided inferior and superior venae cava, and a right aortic arch with mirror image branching. The neonate underwent cardiac catheterization on day of life 12 wherein the bilateral patent ducti arteriosus (PDA) were stented as an alternative to high-risk surgery. In addition, the pulsatile flow via the stents provided a stimulus for PA growth, facilitating transcatheter rehabilitation of distal PA hypoplasia, and optimizing later PA reconstruction. The patient underwent surgery at 13 months of age, with autologous tube creation to unifocalize the Pas and enable the Glenn procedure. Postoperative autograft stenosis was later managed via cardiac catheterization. This report describes the clinical management and utility of staged percutaneous intervention for complex anatomy.