Single lung transplantation for idiopathic pulmonary alveolar proteinosis with intraoperative veno-venous extracorporeal membrane oxygenation support.

Pulmonary alveolar proteinosis (PAP) is a rarely progressive disease. This disease is characterized by the accumulation of a large amount of pulmonary surfactant in the alveolar cavity and terminal bronchiole, which is caused by the obstruction of clearance due to the weakened function of alveolar macrophages in vivo. Idiopathic PAP(IPAP) is the most common type of PAP, accounting for about 90%, and its pathogenesis remains unclear. The treatments of PAP include whole lung lavage, inhaled/subcutaneous GM-CSF, rituximab, plasmapheresis and lung transplantation. We describe a patient with IPAP who is in good condition five years after undergoing a single lung transplantation(SLT). This is the first report of IPAP treated with SLT. Accourding to the previous report and the follow-up result, lung transplantation may be an effective long-term treatment for both secondary PAP and IPAP.