Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis

Abstract Objectives 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. Methods Patients wi...

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Published in:Respiratory medicine 2015-10, Vol.109 (10), p.1354-1359
Main Authors: Ussavarungsi, Kamonpun, Hu, Xiaowen, Scott, J.P, Erasmus, David B, Mallea, Jorge M, Alvarez, Francisco, Lee, Augustine S, Keller, Cesar A, Ryu, Jay H, Burger, Charles D
Format: Article
Language:English
Subjects:
Adult
Echocardiography - methods
Female
Humans
Hyperinflation
Immunosuppressive Agents - therapeutic use
Lung diseases
Lung Neoplasms - diagnostic imaging
Lung Neoplasms - pathology
Lung Neoplasms - surgery
Lung transplantation
Lung Transplantation - adverse effects
Lung Transplantation - methods
Lymphangioleiomyomatosis
Lymphangioleiomyomatosis - diagnostic imaging
Lymphangioleiomyomatosis - pathology
Lymphangioleiomyomatosis - surgery
Middle Aged
Mortality
Ostomy
Outcome
Pleurodesis - methods
Pulmonary arteries
Pulmonary/Respiratory
Retrospective Studies
Severity of Illness Index
Sirolimus
Sirolimus - therapeutic use
Standard deviation
Tomography, X-Ray Computed - methods
Transplants & implants
Treatment Outcome
Ventilation
Womens health
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Summary:Abstract Objectives 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. Methods Patients with LAM undergoing lung transplantation at the Mayo Clinic campuses in Rochester, Minnesota and Jacksonville, Florida since 1995 were retrospectively reviewed. Results 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). Conclusions Lung transplant remains a viable treatment for patients with end-stage LAM. The role of sirolimus peri-transplantation remains ill-defined.
ISSN:0954-6111
1532-3064
DOI:10.1016/j.rmed.2015.08.014