Journal article
Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis
Respiratory medicine, Vol.109(10), pp.1354-1359
10/2015
DOI: 10.1016/j.rmed.2015.08.014
PMID: 26321137
Abstract
Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that generally results in progressive decline in lung function. Despite advancement of pharmacological therapy for LAM, lung transplantation remains an important option for women with end-stage LAM.
Patients with LAM undergoing lung transplantation at the Mayo Clinic campuses in Rochester, Minnesota and Jacksonville, Florida since 1995 were retrospectively reviewed.
Overall, 12 women underwent lung transplantation. Nine of 12 (75%) underwent double lung transplant. The mean age was 42 ± 8 years at the time of transplant. One patient (8%) had a chylothorax and 7 (58%) had recurrent pneumothoraces, 4 (33%) of which required pleurodesis. All had diffuse, cystic lung disease on chest CT consistent with LAM which was confirmed in the explant of all patients. The average length of ICU and hospital stays were 5 ± 4 and 19 ± 19 days, respectively.
Mild to moderate anastomotic ischemia was evident in all patients but resolved with time. No patient was treated with sirolimus pre-transplant. Seven patients received sirolimus post-transplant; however, clinical benefit was documented in only 2 patients, 1 of which was treated for large retroperitoneal cysts with ureteral obstruction and another with persistent chylothorax and retroperitoneal lymphangioleimyomas. Five patients are deceased. The median survival by Kaplan–Meier analysis was 119 months with a median follow-up of 68 months (range 2–225 months).
Lung transplant remains a viable treatment for patients with end-stage LAM. The role of sirolimus peri-transplantation remains ill-defined.
•Our experience demonstrated a favorable outcome for LT in LAM.•There are no standard inclusion criteria for transplantation specific to LAM.•Patients with severe obstruction, compromised gas exchange and progressive respiratory failure should have LT evaluation.•LAM-related pleural complications and treatment interventions are common.•Prior pleurodesis is considered a relative contraindication to LT.Intra and postoperative morbidities should be anticipated.
Details
- Title: Subtitle
- Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis
- Creators
- Kamonpun Ussavarungsi - Pulmonary and Critical Care Medicine, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USAXiaowen Hu - Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55901, USAJ.P Scott - Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55901, USADavid B Erasmus - Transplant Medicine, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USAJorge M Mallea - Transplant Medicine, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USAFrancisco Alvarez - Transplant Medicine, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USAAugustine S Lee - Pulmonary and Critical Care Medicine, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USACesar A Keller - Transplant Medicine, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USAJay H Ryu - Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55901, USACharles D Burger - Pulmonary and Critical Care Medicine, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
- Resource Type
- Journal article
- Publication Details
- Respiratory medicine, Vol.109(10), pp.1354-1359
- Publisher
- Elsevier Ltd
- DOI
- 10.1016/j.rmed.2015.08.014
- PMID
- 26321137
- ISSN
- 0954-6111
- eISSN
- 1532-3064
- Language
- English
- Date published
- 10/2015
- Academic Unit
- Pulmonary, Critical Care, and Occupational Medicine; Internal Medicine
- Record Identifier
- 9984094405702771
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