Prevalence and prognostic impact of valvular heart disease in transthyretin cardiac amyloidosis

Background Significant valvular heart disease (VHD) is associated with adverse prognosis in transthyretin amyloidosis cardiomyopathy (ATTR-CM). However, data is limited to isolated VHD. We aimed at evaluating the difference in outcome between isolated and multiple VHD, and particularly with and without aortic stenosis (AS).Methods Patients with ATTR-CM were retrospectively included in a multicentric registry. The endpoint was a composite of heart failure hospitalizations and all-cause death.Results Of the 694 patients (mean age 75 ± 13 years,74%male), 302(44%) presented with significant VHD. Of these, 98 had VHD including AS (isolated AS in 45%;multiple VHD with AS in 55%), while 204 had VHD without AS (isolated in 64%, multiple VHD in 36%). During a median follow-up of 22(8–38) months, 217(31%) patients reached the endpoint. Significant VHD was associated with worse outcomes than no-VHD (log-rank p < 0.001; HR:3.275; 95%CI (2.472–4.337), remaining significant after adjustment (HR2.006 (1.193–3.047);p = 0.007). Stratification by the presence of AS, and according to isolated or multiple VHD, revealed that isolated significant AS ((HR: 2.462; 95%CI (1.233–4.917); p = 0.011), and multiple VHD including significant AS, had the highest risk for adverse outcomes ((HR: 3.385; 95%CI (1.744–6.573);p < 0.001).Conclusion In ATTR-CM, significant VHD is strongly associated with poor prognosis, with the highest risk for adverse events in significant multiple VHD including AS.

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Journal
Amyloid
Published
2026-10-29
DOI
https://doi.org/10.1080/13506129.2026.2746736
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
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article
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article

Prevalence and prognostic impact of valvular heart disease in transthyretin cardiac amyloidosis

Philippe Debonnaire, Kensuke Hirasawa, Steven Droogmans, Erwan Donal et al.
4 links
Amyloid
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Prevalence and prognostic impact of valvular heart disease in transthyretin cardiac amyloidosis

Philippe Debonnaire, Kensuke Hirasawa, Steven Droogmans, Erwan Donal, Mathias Claeys, Ruxandra Oana Jurcut, Francesca Graziani, Nicole Sturkenboom, Jeroen Joost Bax, Maria Chiara Meucci, Camille Sarrazyn, Nina Ajmone Marsan, Takeru Nabeta, Madelien Veronique Regeer, Caroline M. Van De Heyning, Ching‐Hui Sia, Nico Van de Veire, P Bertrand, Arnold Ng, Fausto Jose Pinto, Robert Adam
article en

Abstract

Background Significant valvular heart disease (VHD) is associated with adverse prognosis in transthyretin amyloidosis cardiomyopathy (ATTR-CM). However, data is limited to isolated VHD. We aimed at evaluating the difference in outcome between isolated and multiple VHD, and particularly with and without aortic stenosis (AS).Methods Patients with ATTR-CM were retrospectively included in a multicentric registry. The endpoint was a composite of heart failure hospitalizations and all-cause death.Results Of the 694 patients (mean age 75 ± 13 years,74%male), 302(44%) presented with significant VHD. Of these, 98 had VHD including AS (isolated AS in 45%;multiple VHD with AS in 55%), while 204 had VHD without AS (isolated in 64%, multiple VHD in 36%). During a median follow-up of 22(8–38) months, 217(31%) patients reached the endpoint. Significant VHD was associated with worse outcomes than no-VHD (log-rank p < 0.001; HR:3.275; 95%CI (2.472–4.337), remaining significant after adjustment (HR2.006 (1.193–3.047);p = 0.007). Stratification by the presence of AS, and according to isolated or multiple VHD, revealed that isolated significant AS ((HR: 2.462; 95%CI (1.233–4.917); p = 0.011), and multiple VHD including significant AS, had the highest risk for adverse outcomes ((HR: 3.385; 95%CI (1.744–6.573);p < 0.001).Conclusion In ATTR-CM, significant VHD is strongly associated with poor prognosis, with the highest risk for adverse events in significant multiple VHD including AS.

Amyloid
Vrije Universiteit Brussel (BE), University of Lisbon (PT), Leiden University Medical Center (NL), AZ Groeninge (BE), Agostino Gemelli University Polyclinic (IT), Université Rennes 2 (FR), UNSW Sydney (AU), Antwerp University Hospital (BE), National University Heart Centre Singapore (SG), AZ Sint-Jan (BE), Universitair Ziekenhuis Brussel (BE), AZ Maria Middelares (BE), Ziekenhuis Oost-Limburg (BE), Centre Hospitalier Universitaire de Rennes (FR), Institute of Science Tokyo (JP), Centro Académico de Medicina de Lisboa (PT), Kitasato University (JP)
Pfizer, Philips, AstraZeneca, Alnylam Pharmaceuticals, Edwards Lifesciences, Omron Healthcare, GE Healthcare, Abbott Vascular, Pfizer Pharmaceuticals, Pharmaceuticals Bayer
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Openalex Percentile: Top 22%
Amyloidosis: Diagnosis, Treatment, Outcomes
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