SUR9 Heart-lung-liver transplant for cystic fibrosis. (Surgery).
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.
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|Author:||Pinson, C. Wright|
|Publication:||Southern Medical Journal|
|Article Type:||Brief Article|
|Date:||Dec 1, 2001|
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