Lung transplantation for lymphangioleiomyomatosis

Background: Lymphangioleiomyomatosis is a rare disease in women leading to respiratory failure. We describe a single-institution experience with lung transplantation for end-stage lymphangioleiomyomatosis. Methods: We retrospectively reviewed records of patients transplanted for lymphangioleiomyomatosis between 1989 and 2001. Follow-up was-complete on all patients (median 3.5 years). Results: Seven single and 7 bilateral transplants were performed on 14 recipients (mean age 41.8 years). Eleven patients suffered the following intra-operative complications: dysrhythmia (1); blood loss > 1,000 ml (7); extensive pleural adhesions (10); hypothermia (1); phrenic nerve injury (1); and graft dysfunction (2). The following post-operative complications occurred in 11 recipients: dysrhythmia (7); respiratory failure (5); sepsis (3); airway dehiscence (2); vocal cord dysfunction (1); cholecystitis (1); deep vein thrombosis (1); acute renal failure (1); and pericarditis (1). Postoperative chylous fistulas necessitated thoracic duct ligation (1); sclerosis (6); and drainage of ascites (1). There were no peri-operative deaths. Late deaths occurred due to sepsis in 2 patients and obliterative bronchiolitis in 1 patient. Survival rates were 100%, 90% and 69% at (1, 2 and 5 years, respectively.) Mean FEV1 (1.77 +/- 1.06 vs 0.60 +/- 0.91) and 6-minute walk (1,519 +/- 379 vs 826 +/- 293 feet) improved at 1 year as compared with pre-transplant evaluation. Five patients reached criteria for bronchiolitis obliterans syndrome. One recipient has had a documented recurrence of lymphangioleiomyomatosis in the transplanted lung. Conclusions: Early and late survival after lung transplant are comparable in lymphangioleiomyomatosis patients versus patients with other diseases. Morbidity is common after transplant for lymphangioleiomyomatosis and is usually due to lymphangioleiomyomatosis-related complications. Lymph angioleiomyomatosis recurrence in the allograft does not pose a substantial clinical problem.