Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis

https://doi.org/10.1016/j.healun.2019.06.015Get rights and content

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.

Section snippets

Methods

We obtained deidentified information regarding all lung transplants performed in the United States between January 2003 and June 2017 from the Organ Procurement and Transplant Network database maintained by the United Network for Organ Sharing. As the outcomes in the US LAM patients undergoing LT between 1987 and 2002 have previously been published,15 we analyzed the data from January 2003 onwards. In May 2005, the United States began using the lung allocation score (LAS) system to determine

Results

A total of 138 patients underwent LT in the study period, accounting for 0.54% of all lung transplants performed in this duration (Table 1). Outcome data following LT was missing in 4 patients, and these patients were excluded from our analysis. From the remaining 134 patients, 87 patients were alive at the time of data analysis (65%), 43 patients had died (32%), and 4 patients were retransplanted (3%). Single LT (SLT) was performed in 25 of 134 patients (19%), and 109 patients underwent

Discussion

The first reported lung transplant for a woman with LAM was a combined heart–lung transplant performed in 1984.7 Since then, lung transplantation outcomes in LAM have been reported in multiple small studies with varying survival estimates (Table 4). The LT survival rates in our analysis are better than in some of the earlier reports8, 15, 17 and comparable to the more recent studies,13, 14 likely reflecting the effect of increased experience and improved peri-transplant care. The exact reasons

Conclusions

In patients with LAM undergoing LT, the median post-LT survival is 12 years with 1-, 5-, and 10-year survival rates of 94%, 73%, and 56%, respectively. The post-LT survival in patients with LAM is substantially better than in those with any other lung disease.

Disclosure statement

The authors have no conflicts of interest to disclose. This study was supported in part by Health Resources and Services Administration contract 234-2005-370011C. The content is the responsibility of the authors alone and does not necessarily reflect the views or policies of Department of Health and Human Services, nor does the mention of trade names of commercial products, or organizations imply endorsement by the U.S. Government.

References (31)

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