Clinical lung and heart/lung transplantation
Lung Transplantation for Lymphangioleiomyomatosis: The European Experience

https://doi.org/10.1016/j.healun.2008.09.014Get rights and content

Background

Lung transplantation has been accepted widely as therapy for end-stage pulmonary lymphangioleiomyomatosis (LAM); however, single-center and national experience is limited due to the rarity of LAM.

Methods

We report the recent European experience of lung transplantation for LAM. A self-administered questionnaire was distributed to 30 European lung transplant centers to evaluate patients who underwent primary lung transplantation for LAM (1997 to 2007).

Results

Seventy percent of centers responded to the questionnaire. A total of 61 lung transplants were undertaken in women only, with mean age at transplant 41.3 years (SD 5.1). Centers performed a median of 2 (0 to 9) transplant operations. Severe pleural adhesions were the most common intra-operative complication. Early deaths (N = 6) were due to primary graft or multiple-organ failure or sepsis. Twelve recipients were diagnosed with bronchiolitis obliterans syndrome at a median of 20 months (range 10 to 86 months) post-transplant. LAM-related complications included renal angiomyolipoma and pneumothorax in the native lung. Recurrence of LAM occurred in 4 recipients. As of December 2007, actuarial Kaplan–Meier survival was 79% at 1 year and 73% at 3 years post-transplant.

Conclusions

Post-transplant outcome for pulmonary LAM in the recent era appears to have improved compared with the previous era. LAM-related complications remain common, but recurrence of LAM in the allograft is rare.

Section snippets

Study Population

In September 2007, a self-administered questionnaire was distributed to 30 adult lung transplant centers across Europe. Patients who underwent primary lung transplantation for end-stage pulmonary LAM between 1997 and 2007 were included. Information provided included the patient's family history, leading pre-transplant symptoms and clinical findings, establishment of diagnosis of LAM, medical and surgical treatment of LAM pre-transplant, and exercise and lung function test results.

Participating Centers

Seventy percent of centers across 11 European countries surveyed provided responses. Participating centers are listed in the Appendix. Centers performed a median of 2 (range 0 to 9) primary lung transplant operations.

Study Population

Sixty-one lung transplant recipients with LAM were included, all of whom were women. All except 2 (1 Asian, 1 Afro-Caribbean) were of Caucasian origin. Seventeen women (27%) reported a smoking history, with a mean of 15 pack-years (SD 7.9).

Clinical and Radiologic Findings Pre-transplant

The vast majority of patients presented

Discussion

To our knowledge, this is the largest European study reporting a multi-center experience of lung transplantation for pulmonary LAM.

This European survey has demonstrated that lung transplantation is a valuable treatment option for end-stage pulmonary LAM. Furthermore, post-transplant outcome is comparable to that for patients undergoing lung transplantation for other forms of end-stage lung disease. Moreover, LAM-related complications and morbidity are common, although not life-threatening. The

References (22)

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    Citation Excerpt :

    Recurrence of LAM was first reported in 1994 in a woman who had received a bilateral lung transplant.131 Numerous subsequent studies have shown that recurrence of LAM in the allograft is not an uncommon complication.65,69,71,72,74,75,80 This may be an occult process, discovered only on autopsy, but it can also present as a simple radiographic abnormality, bronchial strictures, worsening of graft function, or pneumothorax.131-133

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