Literature DB >> 31303421

Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis.

Muhammad Umair Khawar1, Dina Yazdani2, Zheng Zhu3, Roman Jandarov3, Daniel F Dilling4, Nishant Gupta5.   

Abstract

BACKGROUND: The primary aim of our study was to derive disease-specific outcomes following lung transplantation (LT) in patients with lymphangioleiomyomatosis (LAM).
METHODS: We queried the Organ Procurement and Transplant Network database to identify LAM patients that have undergone LT in the United States. The overall survival was analyzed with Kaplan-Meier curves. Survival estimates between subgroups of interest were compared using the log-rank method. Cox proportional hazard models were employed to determine the pre-transplant variables that impact post-LT survival.
RESULTS: One hundred and thirty-eight women with LAM underwent LT at 31 centers between January 2003 and June 2017. The median age at listing and transplant was 44 (IQR: 36-51) and 45 (IQR: 38-52) years, respectively. The median time spent on the LT waitlist was 257 (IQR: 85-616) days. The majority of the patients (109/134, 81%) received bilateral sequential LT. The median ischemic time was 4.9 (IQR: 4.1-6.1) hours. The actuarial Kaplan-Meier survival following LT for LAM patients at 1-, 5-, and 10 years was 94%, 73% and 56%, respectively. The post-LT survival was significantly better in LAM than in other lung diseases (10-year survival 56% vs. 32%, p < 0.01), and this advantage persisted after age- and gender-matched analysis (10-year survival 54% vs. 37%, p < 0.01). Pre-transplant parameters, such as the presence of pulmonary hypertension, six-minute walk distance, age at transplant, ischemic time during transplant, or type of transplant (single vs bilateral sequential LT), did not affect post-transplant survival.
CONCLUSIONS: The median survival after LT in LAM is 12 years and is substantially better than in other lung diseases. Published by Elsevier Inc.

Entities:  

Keywords:  LAM; bronchiolitis obliterans; mortality; sirolimus; tuberous sclerosis complex

Mesh:

Year:  2019        PMID: 31303421     DOI: 10.1016/j.healun.2019.06.015

Source DB:  PubMed          Journal:  J Heart Lung Transplant        ISSN: 1053-2498            Impact factor:   10.247


  7 in total

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4.  A Retrospective Study of Lung Transplantation in Patients With Lymphangioleiomyomatosis: Challenges and Outcomes.

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