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The Heart After Transplant: Long-Term Cardiovascular Outcomes in Hereditary ATTRv Amyloidosis
Session:
Sessão de Posters 15 - Prognóstico, estratificação de risco e comorbilidades na amiloidose cardíaca
Speaker:
Nuno Alexandre Dias Madruga
Congress:
CPC 2026
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.2 Myocardial Disease – Epidemiology, Prognosis, Outcome
Session Type:
Posters Eletrónicos
FP Number:
---
Authors:
Nuno Madruga; Daniel Inácio Cazeiro; Marta Vilela; João Cravo; Diogo Ferreira; João Fernandes Pedro; Sofia Esteves; Catarina Campos; Isabel Conceição; Fausto J. Pinto; Dulce Brito; João Agostinho
Abstract
<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><strong><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Introduction</span></span></strong><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">: </span></span></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Hereditary transthyretin amyloidosis (ATTRv) is an autosomal dominant disease caused by TTR gene mutations, leading to misfolded protein deposition, especially in the heart and peripheral nervous system. This multisystem involvement results in cardiomyopathy, neuropathy and significant morbidity with poor survival. As the liver is the main source of mutated TTR, orthotopic liver transplantation (LT) emerged in the 1990s as the first disease-modifying therapy, improving outcomes and survival. Despite recent advances in TTR-stabilizing and gene-silencing treatments, cardiac involvement remains a key prognostic factor.</span></span></span></span></span></p> <p style="text-align:start"> </p> <p style="text-align:start"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><strong><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Methods:</span></span></strong></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">We conducted a single-center observational study including patients with diagnosis of hereditary ATTR amyloidosis (V30M mutation) who underwent liver transplantation between 1992 and 2020. Clinical, laboratory and echocardiographic data were collected at baseline and follow-up.</span></span></span></span></span></p> <p style="text-align:start"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"> </span></span></span></p> <p style="text-align:start"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><strong><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Results: </span></span></strong></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">114 hATTR V30M patients were included (56% male; mean age at liver transplantation 38 ± 0.9 years) with a mean follow-up of 13.6 ± 0.6 years. Most received tacrolimus (74%), cyclosporine (11%) or mycophenolate (9%). During follow-up, chronotropic incompetence increased from 14% to 40% (p=0.10). Echocardiography showed progression of interventricular septal thickness (10 ± 0.2 vs 11 ± 0.3 mm, p=0.01), posterior wall thickness (9 ± 0.2 vs 10 ± 0.2 mm, p=0.01), LV mass (92 ± 2.8 vs 101 ± 3 g, p=0.04) and NT-proBNP (101 [58–218] to 193 [109–373] pg/mL, p=0.003).</span></span></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Pacemakers were implanted in 86 patients (75%), and age at liver transplantation independently predicted implantation (OR 1.043; 95% CI 1.012–1.075; p=0.006), corresponding to a 4.3% increase in risk per additional year. Cardiovascular hospitalization occurred in 6 patients (5%). Higher follow-up E/E′ was an independent predictor of cardiovascular hospitalization after adjustment for age at liver transplantation (OR 1.30; 95% CI 1.04–1.63; p=0.023), indicating a 30% increase in risk per unit rise.</span></span></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Thirty patients (26%) died during follow-up. Mortality was unrelated to pacemaker implantation or cardiovascular hospitalization. In multivariable analysis, cyclosporine use (OR 5.06; 95% CI 1.41–18.12; p=0.013) and older age at liver transplantation (OR 1.05 per year; 95% CI 1.00–1.10; p=0.036) were independent predictors of mortality. Kaplan–Meier analysis showed a trend toward lower survival among patients on cyclosporine (HR 2.04; p=0.105). </span></span></span></span></span></p> <p style="text-align:start"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"> </span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><strong><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Conclusion: </span></span></strong></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">In this long-term cohort of hATTR V30M patients after liver transplantation, older age at transplantation was associated with higher risks of pacemaker implantation and mortality, while elevated follow-up E/E′ independently predicted cardiovascular hospitalization. These results underline the ongoing cardiac vulnerability in hATTR despite liver transplantation and reinforce the need for sustained, targeted cardiac monitoring.</span></span></span></span></span><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"> </span></span></span></p>
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