| Title |
Cardiac transthyretin amyloidosis treatment improves outcomes after aortic valve replacement for severe stenosis |
| Authors |
Nitsche, Christian ; Dobner, Stephan ; Rosenblum, Hannah R ; Patel, Kush P ; Longhi, Simone ; Yilmaz, Ali ; Merlo, Marco ; Papathanasiou, Maria ; Griffin, Jan ; Oerlemans, Marish I. F. J ; Gama, Francisco ; Hamdan, Ashraf ; Kelion, Andrew D ; Schuster, Andreas ; Glaveckaitė, Sigita ; Akyol, Nuriye ; Porcari, Aldostefano ; Schlender, Lara ; Capovilla, Teresa ; Autherith, Maximilian ; Hauptmann, Laurenz ; Halavina, Kseniya ; Cavalcante, Joao L ; Fontana, Marianna ; Scully, Paul R ; Moon, James C ; Mascherbauer, Julia ; Ristl, Robin ; Biagini, Elena ; Stortecky, Stefan ; Maurer, Matthew S ; Treibel, Thomas A |
| DOI |
10.1093/eurheartj/ehaf362 |
| Full Text |
|
| Is Part of |
European heart journal.. Oxford : Oxford University Press. 2025, Early Access, art. no. ehaf362, p. [1-12].. ISSN 0195-668X. eISSN 1522-9645 |
| Keywords [eng] |
TAVR ; SAVR ; amyloid ; tafamidis |
| Abstract [eng] |
Background and Aims Concomitant aortic stenosis (AS) and transthyretin-associated cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of structural heart failure. Aortic valve replacement (AVR) improves prognosis in this population, but the efficacy of ATTR-specific medication remains unclear. This study aimed to investigate the prognostic implications of ATTR-specific medication in patients with dual AS-CA. Methods This is a multicenter, international, transatlantic registry of patients with a concomitant pathology of significant AS (moderate/severe) and ATTR-CA (ClinicalTrials.gov identifier: NCT06129331). AS severity was diagnosed by transthoracic echocardiography and ATTR-CA by myocardial uptake on bone scintigraphy and/or positive endomyocardial biopsy in the absence of monoclonal proteins. Mortality [all-cause and cardiovascular (CV)] and hospitalisation for heart failure (HHF) served as clinical endpoints. Outcomes were compared with a control cohort of confirmed lone AS receiving AVR matched for EuroSCORE II. Results Of 226 patients with dual pathology (85 ± 6 years, 80.4% male) identified in 16 centres across 10 countries, AS was severe in 196 (86.7%), and moderate in 30 (13.3%). Valve treatment strategies were transcatheter/surgical AVR in 71.7%/3.5%, balloon angioplasty in 1.3%, and conservative management in 23.5%. Seventy-three patients (32.3%) were prescribed, and 69 patients (30.5%) eventually received ATTR-specific medication (99% tafamidis) and were younger, with lower EuroSCORE II, a higher portion of moderate AS, but higher interventricular septum thickness and more severely impaired left ventricular function compared with patients without ATTR medication. After 3.6 ± 1.7 years, 112 (49.6%) had died [CV death: 89 (79.5%)] and 58 (25.7%) experienced HHF. ATTR-specific medication was independently associated with lower all-cause [weighted hazard ratio (HR) 0.40, 95% confidence interval (CI) 0.24–0.68] and CV mortality (weighted HR 0.47, 95% CI 0.27–0.83) but not HHF. AVR improved survival in the overall (HR 0.60, 95% CI 0.39–0.93) and severe AS cohort (HR 0.42, 95% CI 0.26–0.70). Patients who received both ATTR-specific medication and AVR had the most favourable prognosis, comparable to a control cohort with lone AS undergoing AVR. Conclusions ATTR-specific treatment and AVR both result in significant survival benefit in dual pathology AS and ATTR-CA. Results should be interpreted in the context of the non-randomized study setting and differences in patient characteristics. |
| Published |
Oxford : Oxford University Press |
| Type |
Journal article |
| Language |
English |
| Publication date |
2025 |
| CC license |
|
