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.
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.
Authors: Roger D Yusen; Leah B Edwards; Anna Y Kucheryavaya; Christian Benden; Anne I Dipchand; Samuel B Goldfarb; Bronwyn J Levvey; Lars H Lund; Bruno Meiser; Joseph W Rossano; Josef Stehlik Journal: J Heart Lung Transplant Date: 2015-09-03 Impact factor: 10.247
Authors: Christian Benden; Federico Rea; Jürgen Behr; Paul A Corris; Martine Reynaud-Gaubert; Marc Stern; Rudolf Speich; Annette Boehler Journal: J Heart Lung Transplant Date: 2009-01 Impact factor: 10.247
Authors: Carolina S G Freitas; Bruno G Baldi; Carlos Jardim; Mariana S Araujo; Juliana Barbosa Sobral; Gláucia I Heiden; Ronaldo A Kairalla; Rogério Souza; Carlos R R Carvalho Journal: Orphanet J Rare Dis Date: 2017-04-20 Impact factor: 4.123
Authors: Lorriana E Leard; Are M Holm; Maryam Valapour; Allan R Glanville; Sandeep Attawar; Meghan Aversa; Silvia V Campos; Lillian M Christon; Marcelo Cypel; Göran Dellgren; Matthew G Hartwig; Siddhartha G Kapnadak; Nicholas A Kolaitis; Robert M Kotloff; Caroline M Patterson; Oksana A Shlobin; Patrick J Smith; Amparo Solé; Melinda Solomon; David Weill; Marlies S Wijsenbeek; Brigitte W M Willemse; Selim M Arcasoy; Kathleen J Ramos Journal: J Heart Lung Transplant Date: 2021-07-24 Impact factor: 13.569