The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis

Lung transplantation has been increasingly used as a treatment modality for patients with pulmonary lymphangioleiomyomatosis (LAM). In this study, we evaluated the outcome of patients with LAM who underwent lung transplantation with the aim of making some recommendations regarding patient management.

We conducted a retrospective review of 79 patients who underwent primary lung transplantation for end-stage pulmonary LAM at 31 US transplant centers between January 1987 and December 2002 and were reported to the United Network for Organ Sharing (UNOS).

All patients were women with a mean age of 41.1 years (range, 24-65 years). Thirty-four patients (43%) received single-lung transplants. Bilateral lung transplantation was performed in 45 patients (57%). The mean cold ischemia time was 4.7 hours. There were 2 intra-operative deaths. The 30-day mortality was 5% (4 patients). The causes of early death were primary graft failure in 2 patients, hyperacute rejection in 1 patient, and a cardiac event in 1 patient. Twenty late deaths (>30 days post-transplant) occurred. Of those, 5 were from multisystem organ failure, 5 from pulmonary complications, and 2 from fungal infection. Rejection and bronchiolitis obliterans accounted for 2 deaths each. The cause of death was a cardiac event in 1 patient and was not recorded in the remaining 3. Four patients were re-transplanted. Fifty-five patients (70%) were alive at a mean follow-up of 37 months (range 0-128 months). The actuarial Kaplan-Meier survival was 85.75% at 1 year, 76.35% at 3 years, and 64.91% at 5-years. Log-rank analysis showed a statistically significant difference in the survival rate of LAM patients compared with a historical group of patients who had transplantation for all lung conditions during the same period (45.12%, p = 0.0012). Transplant era, type of transplant, donor gender, ischemia time of more than 4 hours, age more than 40 years, and donor/recipient cytomegalovirus did not impact survival.

Lung transplantation is a valuable therapeutic option for patients with end-stage pulmonary LAM. Transplantation offers survival rates that are equivalent to or better than those of patients who received a lung transplant for other indications.