Clinical lung and heart/lung transplantation
The US Experience with Lung Transplantation for Pulmonary Lymphangioleiomyomatosis

Presented in part at the 84th Annual Meeting of the American Association for Thoracic Surgery, April 24–28, 2004, Toronto, Canada.
https://doi.org/10.1016/j.healun.2004.09.013Get rights and content

Purpose

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.

Methods

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).

Results

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.

Conclusions

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.

Section snippets

Materials and methods

We retrospectively analyzed the pooled data on 79 patients who underwent primary lung transplantation for end-stage pulmonary LAM at 31 US centers between October 1987 and December 2002 and were reported to UNOS. Follow-up data ranged from 0 to 128 months (mean, 37 months). The variables analyzed were donor and recipient demographics, cytomegalovirus (CMV) status, type of operation (single or double lung), allograft ischemia time (for double lungs, the ischemia time was considered to be that of

Results

Twelve centers reported performing 1 lung transplant each, 15 centers reported 2 to 4 transplants each, and 4 centers reported 5 or more transplants each. All recipients and 56% of the donors were women. Table 1 shows the donor and recipient age, the average time on the waiting list, the allograft cold ischemia time, and the duration of follow up. Forty-five (57%) of the 79 transplantations performed were double-lung and 34 (43%) were single-lung (Figure 1). Double-lung transplants constituted

Discussion

Pulmonary transplantation has been the treatment modality of choice for patients with end-stage lung disease due to isolated lung conditions. The last few years have witnessed an increase in lung transplantation for the treatment of respiratory failure in patients with LAM.1, 2 However, lung transplantation for patients with systemic diseases continues to generate controversy because of the shortage of donor organs and a mortality rate over 20% for patients awaiting a donor organ. The lack of

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