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Iatrogenic transthyretin cardiac amyloidosis after sequential liver transplantation
Session:
CO 07 - Miocardiopatias Infiltrativas
Speaker:
Ricardo Costa
Congress:
CPC 2021
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.2 Myocardial Disease – Epidemiology, Prognosis, Outcome
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Ricardo Costa; Patrícia Rodrigues; Rita Félix; André Frias; Andreia Campinas; Mário Santos; Hipólito Reis; Severo Torres
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif">Background: Sequential liver transplantation (SLT) uses livers excised from patients with hereditary transthyretin-related amyloidosis during liver transplantation as grafts to other patients with severe hepatic pathologies and a reserved prognosis. The analysis of this highly selected population could help to a better understanding of the transthyretin amyloidosis’ pathophysiology.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif">Purpose: We aimed to investigate the development of cardiac manifestations consistent with iatrogenic transthyretin amyloidosis (iATTR) in patients submitted to SLT.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif">Methods: We retrospectively analyzed the medical records of 72 consecutive patients submitted to SLT between 2007 and 2010, who received livers with V30M mutation.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif">Results: Our sample had 79% males and mean age at transplantation was 55±6 years. During a median follow-up time of 80 months 44% patients died. One-year mortality rate after SLT was 7%. Clinical manifestations of iATTR occurred in 29% of individuals, on average 6 years after SLT, and amyloid was identified in 76% of those who underwent biopsy. At baseline, left ventricular hypertrophy (LVH) was identified in 58% of patients. During follow-up, there was a significant increase of the left ventricular wall thickness (11±1 to 13±3 mm; p<0.001) and 61% of patients reached the criteria of <em>de novo</em> LVH or basal LVH worsening. Age (55±5 vs 58±5, p=0.25) and incidence of hypertension (52% vs 64%, p=0.365) were similar between groups but patients with <em>de novo</em> LVH or basal LVH worsening had higher incidence of chronic kidney disease (CKD; 68% vs 29%, p=0.02). During follow-up, all-cause death was numerically higher in patients with <em>de novo</em> LVH or worsening LVH but not significantly, probably due to the sample size (23% vs 7%, p=0.221, log rank test p=0.262). Significant conduction changes were rarely seen (1 patient); however, there was a trend towards an increase in PR interval. Atrial fibrillation was reported in 8% of cases. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif">Conclusions: In our sample, probable iATTR was often seen within a decade after SLT. Development of possible infiltrative pattern was more common and conduction disorders were rarer than one would extrapolate from hereditary early onset ATTR V30M patients, suggesting a phenotype more similar to late onset ATTR V30M. Our data suggests that these patients should probably undergo periodic cardiac imaging. </span></span></p>
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