SUR9 Heart-lung-liver transplant for cystic fibrosis. (Surgery).SUR Sur, Lebanon: see Tyre. 9 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 The Vanderbilt University Medical Center (VUMC) is a collection of several hospitals and clinics associated with Vanderbilt University in Nashville, Tennessee. It comprises the following units:
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 UNOS United Network for Organ Sharing Transplant surgery A database dedicated to optimizing the use of transplantable organs; according to UNOS statistics–1995, ± 20,000 major organs and tissues are transplanted/yr; since successful survival of 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 Red blood cells
Cells that carry hemoglobin (the molecule that transports oxygen) and help remove wastes from tissues throughout the body.
Mentioned in: Bone Marrow Transplantation
red blood cells , 10 units cryoprecipitate cryoprecipitate /cryo·pre·cip·i·tate/ (-pre-sip´i-tat) any precipitate that results from cooling, sometimes specifically the one rich in coagulation factor VIII obtained from cooling of blood plasma. , 6 units fresh frozen plasma fresh frozen plasma
n. Abbr. FFP
Blood plasma frozen within 6 hours of collection.
fresh frozen plasma , and 18 units platelets intra-operatively. Immunosuppression included standard peri-operative steroids, cyclosporine, azathioprine azathioprine: see metabolite. , 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.