[Sequential heart and liver transplantation for familial amyloid polyneuropathy]

Abstract

Background and objective: Combined heart and liver transplantation for familial amyloid polyneuropathy (FAP) is currently the best treatment for patients with cardiomyopathy related to FAP. However, its optimal timing and the possibility of domino liver transplantation in this setting remain under discussion. Most such cases in the medical literature have been performed simultaneously, although many of them have required the use of veno-venous bypass and the majority have not used the liver as a graft for domino liver transplantation.

Patients and method: We report 3 cases of non-Val30Met mutation that underwent sequential heart and domino liver transplantation at our institution.

Results: We describe the 3 cases and the medical literature, with special attention to the reason for sequential heart and liver transplantation, the role of transient elastography in this setting, and the feasibility of domino liver transplantation.

Conclusion: In our experience, combined heart and liver transplantation is a feasible but challenging procedure for patients with FAP. Performing the procedure sequentially rather than simultaneously seems safer and easier, both technically and hemodynamically. More importantly, such an approach allows the use of livers from FAP patients as grafts for domino liver transplantation.

Keywords: Cardiopathy; Cardiopatía; Domino; Dominó; Donación; Donation; Mutación; Mutation; Piggy-back; Polineuropatía; Polyneuropathy.