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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2023, Journal of Heart and Lung TransplantationCitation 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