P312Amyloid fibril composition predicts the development of cardiac dysfunction after liver transplantation for hereditary transthyretin amyloidosis

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Background: Liver transplantation (LTx) is today the only available treatment for inhibiting the amyloid deposition in transthyretin amyloidosis (TTA). However, we have recently demonstrated continuing amyloid deposition in fat tissues after LTx, along with faster turn over in patients with truncated and full length TTR (type A) compared to those with only full length TTR (type B). We therefore hypothesize that the fibril composition predicts the cardiac function after LTx.Methods: 24 patients with hereditary TTA who had undergone liver transplantation were examined by echocardiography including tissue Doppler (TDE) and speckle tracking echocardiography (STE) before and at least one year after LTx were included in the study. They were divided into two groups according to fibril composition, 10 patients (64 ± 7 years, 7 males) with type A fibrils who had LTx 4.3 ± 4.0 years after onset of disease and 14 patients (56 ± 14 years, 11 males) with type B fibrils who had LTx 3.0 ± 1.0 years after onset of disease. There was no difference in the time to the follow up echocardiography between the two groups.Results: After LTx only type A patients developed increased left atrial volume, left ventricular (LV) wall thickness, early to late diastolic ratio (E/A), reduced myocardial systolic velocity (s') and significantly reduced LVEF as well as regional and global longitudinal function (p<0.05). No changes in LV function after LTx were detected in type B patients.

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