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Transthyretin amyloidosis with cardiomyopathy after domino liver transplantation: Results of a cross-sectional study

2020; Elsevier BV; Volume: 21; Issue: 1 Linguagem: Inglês

10.1111/ajt.16216

1600-6143

Antonio Grande‐Trillo, Carme Baliellas, Laura Lladó, Carlos Casasnovas, Joaquín Víctor Ruiz Franco-Baux, Laura Gràcia-Sánchez, Miguel Ángel Gómez Bravo, Emma González-Vilatarsana, Luis Caballero-Gullón, Eduardo Echeverri, José González‐Costello,

Pneumocystis jirovecii pneumonia detection and treatment

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.