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Transthyretin amyloidosis with cardiomyopathy after domino liver transplantation: Results of a cross-sectional study |
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| Authors: | Antonio Grande-Trillo Carmen Baliellas Laura Lladó Carlos Casasnovas Joaquín V. Franco-Baux Laura Gracia-Sánchez Miguel Á. Gómez-Bravo Emma González-Vilatarsana Luis Caballero-Gullón Eduardo Echeverri José González-Costello |
| Affiliation: | 1. Advanced Heart Failure and Heart Transplant Unit, Virgen del Rocío University Hospital, Seville, Spain;2. Liver Transplant Unit, Department of Gastroenterology, IDIBELL, Bellvitge University Hospital, Barcelona, Spain Multidisciplinary Familial Amyloidosis Unit, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain;3. Multidisciplinary Familial Amyloidosis Unit, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain;4. Multidisciplinary Familial Amyloidosis Unit, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain Neuromuscular Unit, Neurology Department, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain;5. Department of Nuclear Medicine, Virgen del Rocío University Hospital, Seville, Spain;6. Multidisciplinary Familial Amyloidosis Unit, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain Department of Nuclear Medicine, PET Unit-IDI, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain;7. Hepatobiliary and Liver Transplant Unit, General and Digestive Surgery Department, Virgen del Rocio University Hospital, Seville, Spain;8. Multidisciplinary Familial Amyloidosis Unit, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain Liver Transplant Unit, Department of Surgery, IDIBELL, Bellvitge University Hospital, Barcelona, Spain;9. Advanced Heart Failure and Heart Transplant Unit, Heart Disease Institute, Bellvitge University Hospital, Bellvitge Biomedical Research Institute, Barcelona, Spain |
| Abstract: | Domino liver transplantation (DLT) has been used widely in patients with hereditary amyloid transthyretin (ATTR) amyloidosis. New-onset polyneuropathy in recipients of DLT has been reported, but there are few cases of cardiac involvement reported. We aimed to perform a cross-sectional study for ATTR amyloidosis with cardiomyopathy (ATTR-CM) in DLT recipients. We evaluated 23 living DLT recipients a median of 9 years since DLT at 2 referral centers with a systematic cardiac evaluation, including bone scintigraphy. Median age was 72 years, 91% had hypertension, 35% had diabetes mellitus, 67% had chronic renal failure, and 8 patients (35%) developed new-onset polyneuropathy. Only 13% had a normal electrocardiogram and a normal echocardiography, and most of them showed some conduction disturbance or increase in left ventricular wall thickness, but only 1 patient with a Glu89Lys mutation developed ATTR-CM diagnosed by bone scintigraphy and endomyocardial biopsy. None of the recipients of a DLT with Val30Met mutation showed cardiac involvement by bone scintigraphy. In conclusion, DLT from Val30Met donors seems to be safe regarding the development of ATTR-CM. Evaluation of cardiomyopathy in DLT recipients is challenging due to concomitant comorbidities and in this context, bone scintigraphy can be helpful to evaluate ATTR-CM. |
| Keywords: | clinical research/practice domino transplantation heart disease: immune/inflammatory heart transplantation/cardiology liver transplantation/hepatology |