Lung Transplantation Outcomes in Patients with Lymphangioleiomyomatosis
Emily L. Larson1, Jessica M. Ruck2, Laura B. Zeiser2, Alfred J. Casillan2, Alice L. Zhou1, Jinny S. Ha2, Christian A. Merlo2, Errol L. Bush2.
1Johns Hopkins School of Medicine, Baltimore, MD, USA, 2Johns Hopkins Hospital, Baltimore, MD, USA.
BACKGROUND: Lung transplantation is used to treat severe pulmonary lymphangioleiomyomatosis (LAM). With the recent introduction of mTOR inhibitors for LAM, which pose a risk for complications like airway dehiscence, an updated evaluation of LAM lung transplant outcomes is needed.
METHODS: We identified all adult lung transplant recipients 2005-2021 using the SRTR database; recipients were stratified by diagnosis (LAM vs. other). Descriptive statistics were performed. Kaplan-Meier survival curves were compared. Adjusted hazard ratio (aHR) for mortality by LAM diagnosis was calculated, adjusting for baseline recipient and donor characteristics by multivariable Cox regression.
RESULTS: 32,337 patients were identified, with 156 (0.5%) diagnosed with LAM. LAM patients were younger and had lower BMI and creatinine (p<0.001, Table 1). LAM patients were 100% female (p<0.001). Operative outcomes, including airway dehiscence, did not significantly differ between groups, besides prolonged chest drain use and a trend towards decreased prolonged ventilation in LAM patients (Table 1). LAM patients had higher survival at 30 days (99.4% vs. 96.8%), 1 year (94.2% vs. 86.6%), and 15 years (49.8% vs. 18.3%) post-transplant (Figure 1, p<0.001). After adjusting for donor and recipient characteristics, LAM diagnosis was associated with 45% lower mortality than other diagnoses (aHR=0.55 (1.40-2.37); p<0.001).
CONCLUSIONS: LAM diagnosis was associated with increased short- and long-term post-transplant survival compared to other diagnoses and had no increased airway complications, supporting lung transplant as an effective treatment for severe pulmonary LAM.
|Variable||LAM Diagnosis||Other Diagnosis||P|
|Length of stay *median (interquartile range)||19 (12-30.5)*||17 (12-28)*||0.11|
|Ventilator support > 48 hrs||45 (28.9%)||11512 (35.78%)||0.09|
|Drug-treated infection||41 (26.3%)||7531 (23.4%)||0.45|
|Stroke||3 (1.9%)||725 (2.3%)||0.99|
|Reoperation||23 (14.7%)||3376 (10.5%)||0.11|
|Reintubation||32 (20.5%)||5789 (18.0%)||0.48|
|Chest drain > 2 weeks||28 (17.9%)||2902 (9.0%)||0.0002|
|Airway dehiscence||1 (0.6%)||484 (1.5%)||0.58|
|Tracheostomy||3 (1.9%)||1284 (4.0%)||0.27|
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