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Eur J Cardiothorac Surg 2007;32:180-182. doi:10.1016/j.ejcts.2007.03.023
Copyright © 2007, European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved

Case reports

Combined heart and liver transplantation for familial amyloidotic neuropathy

Emanuele Pilato, Andrea Dell’Amore*, Luca Botta, Giorgio Arpesella

Heart and Lung Transplantation Program, S. Orsola-Malpighi Hospital, University of Bologna, Italy

Received 2 September 2006; received in revised form 7 March 2007; accepted 14 March 2007.

* Corresponding author. Address: Department of Cardiac Surgery, S. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 9, 40138 BO, Italy. Tel.: +39 03356223366; fax: +39 051345990. (Email: dellamore76{at}libero.it).

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.

Key Words: Transplantation • Heart failure • Amyloidosis • Cardiomyopathy






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Copyright © 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier. All rights reserved.