Domino liver transplantation (DLT) from donors with hereditary transthyretin amyloidosis (hATTR) harboring the Val30Met mutation seems to be safe, despite frequent observations of cardiac abnormalities.
“Recipients of DLT have significant cardiomyopathy mostly attributable to their age, comorbidities, and immunosuppressive regimen, because we could not demonstrate cardiac amyloid deposition in recipients from Val30Met donors by bone scintigraphy, even though more than one third of patients had developed significant polyneuropathy,” the study authors said.
Only 1 patient who underwent DLT from a donor with a Glu89Lys mutation and severe cardiomyopathy developed hATTR with cardiomyopathy.
At follow-up evaluation, almost half (47%) of the patients showed some degree of polyneuropathy, but only 35% developed new-onset polyneuropathy after DLT. The other patients had pre-existing polyneuropathy associated with diabetes mellitus, and the degree of polyneuropathy did not change with DLT. Patients with new-onset polyneuropathy were treated with diflunisal, except for 2 patients who had poor renal function and 1 patient who was referred for sural nerve biopsy.
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Moreover, almost half (48%) of the patients presented with conduction system alterations on electrocardiography, and 83% had pathological findings on transthoracic echocardiography, including increased ventricular wall thickness (pre-existing condition in 8 out of 18 patients), diastolic dysfunction, and midrange left ventricular ejection fraction. The patient presenting with midrange left ventricular ejection fraction suffered from ischemic heart disease. In addition, 35% of patients were diagnosed with heart failure with preserved ejection fraction.
The study enrolled 23 patients (61% male) who underwent DLT at 2 referral centers for liver transplantation in Spain. All patients were still alive at the time of the study and had been followed up for at least 1 year. Most (65%) patients were referred for liver transplantation due to hepatitis C-related liver cirrhosis. Other indications included alcoholic liver cirrhosis (13%), primary biliary cirrhosis (13%), and hepatitis B-related liver cirrhosis (9%). All donors were diagnosed with hATTR caused by the Val30Met mutation, except for 1 who carried the Glu89Lys mutation.
Grande-Trillo A, Baliellas C, Lladó L, et al. Transthyretin amyloidosis with cardiomyopathy after domino liver transplantation: results of a cross-sectional study. Am J Transplant. 2021;21(1):372-381. doi:10.1111/ajt.16216