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Lung
Published in: Lung 6/2017

01-12-2017

Experience of Lung Transplantation in Patients with Lymphangioleiomyomatosis at a Brazilian Reference Centre

Authors: Bruno Guedes Baldi, Marcos Naoyuki Samano, Silvia Vidal Campos, Martina Rodrigues de Oliveira, José Eduardo Afonso Junior, Rafael Medeiros Carraro, Ricardo Henrique Oliveira Braga Teixeira, Isabela Pasqualini Minguini, Roni Burlina, Eduardo Zinoni Silva Pato, Carlos Roberto Ribeiro Carvalho, André Nathan Costa

Published in: Lung | Issue 6/2017

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Abstract

Introduction

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.

Methods

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.

Results

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.

Conclusions

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.
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Metadata
Title
Experience of Lung Transplantation in Patients with Lymphangioleiomyomatosis at a Brazilian Reference Centre
Authors
Bruno Guedes Baldi
Marcos Naoyuki Samano
Silvia Vidal Campos
Martina Rodrigues de Oliveira
José Eduardo Afonso Junior
Rafael Medeiros Carraro
Ricardo Henrique Oliveira Braga Teixeira
Isabela Pasqualini Minguini
Roni Burlina
Eduardo Zinoni Silva Pato
Carlos Roberto Ribeiro Carvalho
André Nathan Costa
Publication date
01-12-2017
Publisher
Springer US
Published in
Lung / Issue 6/2017
Print ISSN: 0341-2040
Electronic ISSN: 1432-1750
DOI
https://doi.org/10.1007/s00408-017-0045-y

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