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