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 hours, aortic cross-clamp time was 62 minutes and cardiac cold ischemia time was 75 minutes. The recipient was extubated at 17 hours 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.