Anesthesia, cystic fibrosis and organ transplantation

Patients affected by cystic fibrosis (CF) may present several problems if undergone general anaesthesia, according to the surgical procedure, to the age and to the clinical status of patients. Lung or liver transplantation offer a <<chance>> for patients with respiratory or hepatic insufficiency. In our institution 13 lung transplantations and 4 liver transplantations were performed in pts affected by CF. From November 1996 to November 1997, 13 patients underwent bilateral sequential single lung transplantation (BS-SLTx) Anesthesia was induced with propofol, fentanyl, scopolamine and/or midazolam, and/or lorazepam, and maintained with isoflurane, fentanyl in O-2 100%. Vecuronium bromide was used as muscle relaxant. For intubation a single lumen tube was introduced to perform a bronchial toilette, then a left endobronchial tube (Robert Shaw) was positioned. Hemodynamic management was performed with prostaglandin E-1, norepinephrine, ephedrine, dobutamine, dopamine and inhaled nitric oxide (iNO). An adequate pharmacological support with pulmonary vasodilators, inotropic and systemic vasoactive drugs led to successful lung transplantation with cardio-pulmonary bypass. From April 1996 to May 1997, four patients affected by CF with end-stage liver disease with mild to moderate pulmonary abnormalities underwent liver transplantation. The patients received pancreatic enzyme and two had insulin-dependent diabetes mellitus. General anaesthesia was induced with fentanyl, thiopental and pancuronium, and maintained with isoflurane supplemented by fentanyl, thipental and pancuronium, and maintained with isoflurane supplemented by fentanyl in O-2 : air (FiO(2) = 0.5). After the intubation and at the end of the procedure, all patients received a bronchial toilette through fiberoptic bronchoscopy. All patients were successfully transplanted, PaO2 increased proportionally to the decreasing in Qsp/Qt during intraoperative time of the transplantation. The follow-up at 1 year showed fev(1) and FVC improved from preoperative time in all patients.