[Seventeen cases of lung transplantations for lymphangioleiomyomatosis]

The number of lung transplantation has tended to increase as a treatment for patients with pulmonary lymphangioleiomyomatosis (LAM) in Japan. However, we have little evidence about the correlation between preoperative medical parameters and intraoperative complications.

A retrospective study was performed in 17 patients with LAM undergoing the lung transplantations between 2006 and 2011.

Major intraoperative complications were as follows: hypotension, pulmonary hypertension, hypoxemia and blood loss of more than 1,000 ml. According to the medical records, no patients was diagnosed pulmonary hypertension before surgery and no preoperative parameters predicted intraoperative pulmonary hypertension. Therefore, according to the medical records it seemed difficult to predict the risk of the intraoperative pulmonary hypertension and hypotension. Intraoperative hypoxemia and the requirements of percutaneous cardiopulmonary support (PCPS) are not associated with preoperative PaO2 and %FEV1.0. Pleurodesis and the use of PCPS were significantly correlated with an increased risk of intraoperative massive bleeding. The four year survival rate was 87.8%. %FEV1.0, PaO2/FI(O) and %DL(CO) after surgery improved when compared to those before surgery.

Although the intraoperative complications such as hypotension, pulmonary hypertension, hypoxemia and/or massive bleeding frequently occurred in patients with LAM during the lung transplantations, it was difficult to predict them except the massive bleeding of more than 1,000 ml before surgery. However, lung transplantation is a valuable therapy for patients with end-stage LAM.