Main pulmonary artery swing back for univentricular heart

Abstract

Left pulmonary artery (LPA) surgical arterioplasty done for juxtaductal pulmonary artery coarctation is a challenging technique that has often produced unsatisfactory outcomes because of poor outcome later and incomplete understanding of the evolution of structural changes in these vessels. We report a unique technique for LPA plasty involving main pulmonary artery (MPA) swing back, in a toddler who presented with history of increased work of breathing, cyanosis and failure to thrive since birth, where the MPA was detached and was swung back cranially to be anastomosed to LPA. This technique can be done, especially in univentricular heart (future Fontan candidate), since MPA needs to be interrupted. The patient had heterotaxy syndrome, left atrial isomerism, hypoplastic pulmonary valve with severe sub-valvar stenosis, duct dependent pulmonary circulation, good sized confluent branch pulmonary arteries with LPA narrowing at ductal insertion site, interrupted IVC with hemiazygous continuation and left SVC draining into coronary sinus. Initially underwent right modified Blalock taussig (BT) shunt, atrial septectomy, LPA plasty and PDA ligation at 6 months of age. Upon follow up after a year, diagnostic angiogram confirmed good flow in BT shunt and LPA re-stenosis with malpositioned great arteries. Redo sternotomy and Kawashima procedure was performed. BT shunt and MPA stump were divided. To conclude, the detached MPA segment was swung cranially and anstomosed to LPA (end to end) inferiorly and left SVC-LPA anstomosed superiorly onto this by end to side fashion. The early outcome was satisfactory, however long-term outcome needs to be followed up