Please use this identifier to cite or link to this item: https://observatorio.fm.usp.br/handle/OPI/24227
Title: Experience of Lung Transplantation in Patients with Lymphangioleiomyomatosis at a Brazilian Reference Centre
Authors: BALDI, Bruno GuedesSAMANO, Marcos NaoyukiCAMPOS, Silvia VidalOLIVEIRA, Martina Rodrigues deAFONSO JUNIOR, Jose EduardoCARRARO, Rafael MedeirosTEIXEIRA, Ricardo Henrique Oliveira BragaMINGUINI, Isabela PasqualiniBURLINA, RoniPATO, Eduardo Zinoni SilvaCARVALHO, Carlos Roberto RibeiroCOSTA, Andre Nathan
Citation: LUNG, v.195, n.6, p.699-705, 2017
Abstract: Lung transplantation (LT) is the standard of care for patients with advanced lung diseases, including lymphangioleiomyomatosis (LAM). LAM accounts for only 1% of all LTs performed in the international registry. As a result, the global experience, including the use of mechanistic target of rapamycin (mTOR) inhibitors before and after LT in LAM, is still limited. We conducted a retrospective review of all LAM patients who underwent LT at our centre between 2003 and 2016. Pre- and post-transplant data were assessed. Eleven women with LAM underwent LT, representing 3.3% of all procedures. Ten (91%) patients underwent double-LT. The mean age at diagnosis was 39 +/- 6 years and the mean FEV1 before LT was 28 +/- 14%. Only one patient underwent pleurodesis for recurrent pneumothorax. Pulmonary hypertension was confirmed in 3 (27%) patients. Four (36%) patients received sirolimus preoperatively; three of them received it until the day of LT, and there was no occurrence of bronchial anastomotic dehiscence after the procedure. Four patients (36%) received mTOR inhibitors post-transplant. The median follow-up from LT was 44 months. There were 3 deaths (27%) during the study and survival probabilities at 1, 3, and 5 years after LT were, 90, 90, and 77%, respectively. This data reinforces the role of LT for LAM patients with end-stage disease. The use of sirolimus seems to be safe before LT and the occurrence of complications after LT, including those LAM-related, should be continuously monitored.
Appears in Collections:Artigos e Materiais de Revistas Científicas - FM/MCP
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - HC/InCor
Artigos e Materiais de Revistas Científicas - LIM/09
Artigos e Materiais de Revistas Científicas - LIM/61

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