Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis. Issue 10 (October 2015)

• Record Type: Journal Article
• 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.
• Is Part Of: Respiratory medicine. Volume 109:Issue 10(2015)
• Page Start: 1354
• Page End: 1359
• Publication Date: 2015-10
• Subjects: Lymphangioleiomyomatosis -- Lung transplantation -- Sirolimus -- Outcome; 6MWT 6-min walking test -- DLT double lung transplant -- ECHO echocardiogram -- FEV1 forced expiratory volume in one second -- LAM lymphangioleiomyomatosis -- LT lung transplantation -- MILES Multicenter International LAM Efficacy of Sirolimus -- mTOR mammalian target of rapamycin -- PFT pulmonary function testing -- RHC right heart catheterization -- SLT single lung transplant -- SRTR Scientific Registry of Transplant Recipients -- UNOS United Network for Organ Sharing; Chest -- Diseases -- Periodicals; Chest -- Diseases -- Great Britain -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Appareil respiratoire -- Maladies -- Périodiques; Thorax -- Maladies -- Périodiques; Appareil respiratoire -- Maladies -- Traitement -- Périodiques; Electronic journals; 616.2
• DOI: 10.1016/j.rmed.2015.08.014
• Languages: English
• ISSNs: 0954-6111

Abstract

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. Highlights: 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.