Pulmonary Atresia with MAPCAs: Staged Unifocalization Techniques

Abstract

tricular septal defect, nonconfluent or absent central pulmonary arteries with multiple major aortopulmonary collateral arteries (MAPCAs) presents a significant surgical challenge. Complete repair requires unifocalization of all available segmental and lobar pulmonary blood supply so that a central pulmonary ventricle to pulmonary arterial reconstruction can be created. When central pulmonary arteries are not merely nonconfluent but actually absent, there are additional surgical challenges as the central main pulmonary arteries need to be created. When there are central pulmonary arteries, unilateral reconstructions are usually staged with the intention of encouraging pulmonary artery growth as well as eliminating dual blood supplies and the recruitment of segments dependent upon only collateral arteries into the central circulation prior to the final correction. Currently, neonatal unifocalization with complete repair is the approach of choice (See next chapter). Such neonatal corrections are most successful when the number of segments receiving separate multiple aortopulmonary collateral arteries are relatively few and particularly when at least one lung is limited to only one or two MAPCAs. Patients requiring complex unifocalizations sometimes do not present to the surgeon during the neonatal stage of life (as they are often neither in failure or cyanotic), and staged procedures are usually needed in the older children. We have used cryopreserved valveless pulmonary homografts for hilar reconstructions and unifocalizations as staged procedures prior to completing the central reconstruction with a valved homograft. We have also used PTFE grafts for portions of the repairs and as the conduit of choice for any systemic to pulmonary artery connections.