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). Post-operative 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. Lymphangioleiomyomatosis recurrence in the allograft does not pose a substantial clinical problem.
Pechet
Taine T
TT
Division of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
Meyers
Bryan F
BF
Guthrie
Tracey J
TJ
Battafarano
Richard J
RJ
Trulock
Elbert P
EP
Cooper
Joel D
JD
Patterson
G Alexander
GA
D000328:Adult; D001989:Bronchiolitis Obliterans / Q000453:epidemiology; D016022:Case-Control Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects; D008175:Lung Neoplasms / Q000601:surgery*; D016040:Lung Transplantation; D018192:Lymphangioleiomyomatosis / Q000601:surgery*; D009017:Morbidity; D011183:Postoperative Complications / Q000453:epidemiology; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D016019:Survival Analysis; D013997:Time Factors