Lung transplantation for lymphangioleiomyomatosis: a Brazilian centre experience

Lymphangioleiomyomatosis (LAM) is a rare low-grade neoplasm with lung cysts formation. Some patients with progressive disease need lung transplantation (LT). Herein we describe clinical characteristics and post operative outcomes of LT in LAM patients at a Brazilian reference centre. Our retrospective study included 11 LAM patients submitted to LT at University of Sao Paulo from 2003 to 2016 (91% double-lung). Mean follow-up after LT was 44 months. Mean age at LT was 43 ± 7 years. Preoperatively, patients presented dyspnea (100%), supplemental oxygen use (91%), pneumothorax (36%) and pulmonary hypertension (36%). One patient underwent pleurodesis and none presented chylothorax. Sirolimus was used by 36% of patients before LT. FEV1 was 28 ± 14% and DLCO 30 ± 18% of predicted. There were no intraoperative complications, whereas one patient required cardiopulmonary bypass due persistent hypoxemia. Post operatively, 18% of patients had chylothorax; 82% presented at least one episode of acute rejection, and chronic lung allograft syndrome occurred in 45%. At least one pulmonary infection occurred in 55% of patients, mainly due to Aspergillus sp. After LT, we found no LAM recurrence, episode of malignant neoplasm or retransplantation. There were 3 deaths (27%) and the survival rate at 5 years after LT was 77% (fig 1). Based on our data, LT is a feasible therapeutic option for patients with end-stage LAM, with favorable prognosis.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Diffuse Parenchymal Lung Disease Source Type: research