Combined heart and liver transplantation for familial amyloidotic neuropathy

Familial amyloidotic polyneuropathy (FAP) is an inherited disease characterized by an abnormal systemic deposition of a mutant protein called transthyretin (TTR) with elective involvement of the peripheral nervous system, but often determining cardiac, gastrointestinal, and urinary tract dysfunction. FAP commonly affects the liver and the heart until end-organs failure. Transthyretin amyloidosis is today an accepted indication for orthotopic liver transplantation (OLT). Combined heart and liver transplantation (CHLT) may be an attractive and rational treatment option when both organs are contemporary involved by this type of amyloidotic disease. Nowadays, surgical indications and techniques are far from being consolidated because only few cases of CHLT have been previously reported in literature. From November 1999 to May 2006, we performed five orthotopic combined heart and liver transplantations for FAP at our institution. Our surgical experience and clinical outcomes are herein reported.