Abstract

Liver transplantation is a standard treatment for patients with familial amyloidotic polyneuropathy (FAP) with disease progression. Given the multiorgan involvement by amyloidosis, the heart is often involved. When poor cardiac function becomes prohibitive to liver transplantation, a combined heart-liver transplantation (CHLT) is the only realistic treatment. This article records a CHLT for a patient with FAP whose removed liver was immediately transplanted as an amyloidotic hepatic allograft (AHA) to a patient having hepatocellular carcinoma and cirrhosis in a sequential liver transplantation. In the CHLT, the heart and liver are donated by a deceased donor. The newly implanted heart did not tolerate cross clamping of the inferior vena cava (IVC), so a side-to-side anastomosis was performed to connect the IVC and that of the liver graft. Therefore, the AHA was devoid of an IVC. The infrarenal cava procured from the deceased donor was used for reconstruction of the AHA to match a whole graft used in routine deceased-donor liver transplantation. Venoplasty was performed using the graft right hepatic vein and the middle and left hepatic vein stump to form a single cuff. The reconstructed AHA was implanted to the recipient conveniently like a usual whole graft.

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