SUR9 Heart-lung-liver transplant for cystic fibrosis. (Surgery).

SUR9 HEART-LUNG-LIVER TRANSPLANT FOR CYSTIC FIBROSIS. Paul E. Wise, MD, J. Kelly Wright, MD, William C. Chapman, MD, Davis C. Drinkwater, Jr., MD, Robin N. Pierson, MD, and C. Wright Pinson, MD, MBA. Vanderbilt University Medical Center, Nashville, Tenn.

Approximately 5% of all patients with cystic fibrosis (CF), the most common fatal genetic disorder in the United States, will progress to clinically significant cirrhosis. Based on a review of the UNOS Scientific Registry as of January 6, 2001, there have been three heart-lung-liver (triple) transplants in the U.S. We present the report of the third U.S. triple transplant, an 18-year-old male diagnosed with CF at age 3 months and with portal hypertension at age 14 years. His pulmonary function decreased to an FEVI of 22% and an FVC of 36% in addition to a continuous oxygen requirement. His hepatic disease progressed to cirrhosis and portal hypertension requiring transjugular intrahepatic porto-systemic shunt (TIPS) placement. Given the extent of his pulmonary and hepatic disease, evaluations for lung and liver transplants were performed. Triple transplant, rather than double lung/liver transplant, was the operation of choice for relative technical ease and shorter ischemic times. An appropriate donor was iden tified, and heart/lung transplant was performed with cardiopulmonary bypass at 29[degrees]C while the donor liver was prepared. The midline sternotomy was closed to prevent thoracic contamination and the orthotopic liver transplant performed. Cold ischemic times were 67 minutes for the heart/lung bloc and <6 hours for the liver. He required 8 units packed red blood cells, 10 units cryoprecipitate, 6 units fresh frozen plasma, and 18 units platelets intra-operatively. Immunosuppression included standard peri-operative steroids, cyclosporine, azathioprine, and thymoglobulin. He was extubated on postoperative day 2 and discharged from the hospital on postoperative day 10. He is alive and functional 14 months posttransplant. His pulmonary function has improved to an FEV1 of 67% and an FVC of 71%, and he is no longer oxygen dependent. In conclusion, simultaneous heart-lung-liver transplant can be a successful option for severe pulmonary dysfunction and cirrhosis in cystic fibrosis.