Combined heart–liver transplantation (CHLT) is an effective treatment for hereditary transthyretin amyloidosis (hATTR) with progressive cardiac involvement. The Phe53Leu variant, a rare TTR mutation, is frequently associated with early and severe cardiac involvement and may warrant consideration for CHLT.

A case of a 53-year-old male patient, presented with NYHA II heart failure and peripheral polyneuropathy. Cardiac MRI and 99mTc-MDP bone scintigraphy revealed extensive myocardial amyloid infiltration, and the diagnosis was confirmed by Congo red staining of a labial salivary gland biopsy and TTR gene sequencing (c.157T>C, p.Phe53Leu). CHLT was performed using a modified en-bloc technique that avoided division of the diaphragm, and the amyloidosis liver was used for domino transplantation in a patient with hepatocellular carcinoma. Total operative time was 8 h, aortic cross-clamp time was 62 min and cardiac cold ischemia time was 75 min. The recipient was extubated at 17 h without respiratory complications, and was discharged on postoperative day 29.

The uneventful perioperative course supports the feasibility of CHLT with the modified en-bloc technique in a centre with combined cardiac and hepatobiliary transplant expertise. To our best knowledge, this is the first reported CHLT for Phe53Leu hATTR performed in Vietnam and in Asia.