Familial amyloidotic polyneuropathy (FAP) = cardiac and circulatory function during anesthesia for liver transplantation

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Abstract
Familial amyloidotic polyneuropathy is a new indication for liver transplantation. Severe cardiovascular disturbances including severe bradyarrhythmias are well known complications to FAP, during anesthesia and operation. Twenty-seven patients with FAP type I (TTR Met30) were followed during and after transplantation. Mean duration of disease was 5 years and mean modified body mass index (mBMI) 690 (normal >700). At pretransplant evaluation, six patients had A V-conduction disturbances, 13 echocardiographic signs of amyloid deposits, five had orthostatic reactions and two had permanent pacemakers. In 14 patients. hemodynamic instability with low systolic systemic blood pressure was found during the preanhepatic phase, unrelated to surgical procedure. Volume loading or vasoactive drugs had only modest effects. Preoperative cardiac findings did not correlate with hemodynamic instability. A temporary pacemaker was introduced in 19 patients, but in only one of these patients did the pacemaker actively pace the heart. in four of five patients dying, cardiovascular complications contributed significantly. The actuarial one and two year survival was 78.5%. In conclusion, liver transplantation for FAP should be performed at an early stage of the disease. Only patients with A V-conduction or other cardiac problems need a temporary or permanent pacemaker before anesthesia in other FAP patients, a central venous line, through which a pacing electrode can easily be introduced, is sufficient. Hemodynamic instability was common, including intra- and postoperative death. However, the preoperative cardiovascular evaluation could not identify patients at risk.