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. 2025 Feb 1:236:42-48.
doi: 10.1016/j.amjcard.2024.10.009. Epub 2024 Oct 29.

Cardiac Amyloidosis Screening and Management in Patients With Heart Failure With Preserved Ejection Fraction: An International Survey

Anastasia Shchendrygina  1 , Nathan Mewton  2 , David Niederseer  3 , Keisuke Kida  4 , Federica Guidetti  5 , Antoine Jobbe Duval  2 , Ivan Milinkovic  6 , Marish I F J Oerlemans  7 , Marta Zaleska-Kociecka  8 , Sydney Goldfeder de Gracia  9 , Maria Isabel Palacio  9 , Ilya Giverts  10 , Irina Komarova  11 , Yasmin Rustamova  12 , Fadel Bahouth  13 , Anja Zupan Mežnar  14 , Massimo Mapelli  15 , Alexandr Suvorov  16 , Irina Dyachuk  17 , Michail Shutov  18 , Violetta Sitnikova  11 , Antoine Garnier-Crussard  19 , Anders Barasa  20 , Goran Loncar  21 , Mariya Tokmakova  22 , Hadi Skouri  23 , Frank Ruschitzka  24 , Clara Saldarriaga  9
Affiliations

Cardiac Amyloidosis Screening and Management in Patients With Heart Failure With Preserved Ejection Fraction: An International Survey

Anastasia Shchendrygina et al. Am J Cardiol. .

Abstract

Cardiac amyloidosis (CA) is still an underdiagnosed cause of heart failure (HF) and early disease recognition and timely disease-modifying therapy (DMT) administration translate to better outcomes. We aimed to assess CA screening and management approaches for patients with HF preserved ejection fraction (HFpEF) among physicians worldwide. An independent academic web-based survey was distributed worldwide between May 2023 and July 2023. Overall, 1,460 physicians (61% were men, median age was 42 [34 to 49] years) from 95 countries completed the survey. A total of 2/3 of respondents had experience diagnosing CA and reported having 10% of patients with CA in patients with HFpEF. Systematic screening for CA of all patients with HFpEF was performed by 10% of responders, whereas 24% did not consider the screening. Most responders (39%) used left ventricular hypertrophy as a screening criterion. Serum protein electrophoresis with immunofixation of free light chain and urine protein electrophoresis or cardiac magnetic resonance were selected by half of the responders as a first-line diagnostic tool. The combination of serum protein electrophoresis with immunofixation free light chain, urine protein electrophoresis, and bone scintigraphy was considered by 32% of the participants. CA DMT was available for 48% of the physicians. About 82% of responders would administrate HF to patients with HFpEF with CA, with the most preferable drugs being diuretics, sodium-glucose cotransporter-2 inhibitors, and renin-angiotensin-aldosterone system inhibitors. In conclusion, the results reveal the uncertainties among physicians worldwide regarding the need for CA screening of patients with HFpEF. CA remains a disease with very heterogeneous management, particularly, in the screening and diagnostic workup. The HF community should aim to educate on CA and improve access to DMT.

Keywords: awareness; cardiac amyloidosis; decease management survey; heart failure.

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Conflict of interest statement

Declaration of competing interest Dr. Mewton reports consulting fees from Bayer, Novartis, Amgen, and AstraZeneca and payment or honoraria for lectures from Boehringer Ingelheim. Dr. Niederseer reports consulting fees from Amgen, AstraZeneca, Bayer, Boehringer Ingelheim, Daiichi Sankyo, Gerson Lehman Group (GLG) Consulting, Novo Nordisk, Pfizer (New York, New York), and Zoll and payment or honoraria for lectures from Amgen, Astra Zeneca, Bayer, Boehringer Ingelheim (Ingelheim, Germany), Daiichi Sankyo, Novartis, Novo Nordisk, and Pfizer. Dr. Kida reports payment or honoraria for lectures from AstraZeneca K.K., Ono Pharmaceutical Co., Ltd., Nippon Boehringer Ingelheim Co., Ltd., Otsuka Pharmaceutical Co., Ltd., and Novartis Pharmaceuticals Co., Ltd.. Dr. Duval reports grants or contracts from Pfizer and Alnylam. Dr. Milinkovic reports payment or honoraria for lectures from Boehringer Ingelheim and support for attending meetings from Boehringer Ingelheim. Dr. Oerlemans reports payment or honoraria for lectures to the institution from Novartis and Pfizer. Dr. Barasa reports consulting fees, payment, or honoraria for lectures from Boehringer Ingelheim and AstraZeneca (to the institution); support for attending meetings from Novartis, Bayer, Boehringer Ingelheim (to the institution), AstraZeneca (to the institution); participation on a Data Safety Monitoring Board or Advisory Board for AstraZeneca, Bayer, and Boehringer Ingelheim (to the institution); and membership of Heart Failure Task Force under the Danish Society of Cardiology. Dr. Tokmakova reports being HFA board member. Dr. Skouri reports payment or honoraria for lectures or advisory board meetings from Boehringer Ingelheim, AstraZeneca, Novartis, Novo Nordisk, Servier, Abbott, Roche, and Vifor. Dr. Ruschitzka has not received personal payments by pharmaceutical companies or device manufacturers in the last 3 years (remuneration for the time spent in activities, e.g., participation as steering committee member of clinical trials and member of the Pfizer Research Award selection committee in Switzerland, were made directly to the University of Zurich). The Department of Cardiology (University Hospital of Zurich/University of Zurich) reports research-, educational-, and/or travel grants from Abbott, Amgen, Astra Zeneca, At the Limits Ltd., Bayer, Biotronik, Bristol-Myers Squibb, Boehringer Ingelheim, Boston Scientific, Bracco, CM MicroPort, Concept Medical, CTI, Daiichi Sankyo, Edwards Lifesciences, FomF GmbH, Hamilton Health Sciences, IHF, Innosuisse, IumiraDX, Kantar, LabPoint, MedAlliance, Medcon International, Medical Education Global Solutions, Medtronic, MicroPort, Monocle, Novartis, Novo Nordisk, OM Pharma, Pfizer, Quintiles Switzerland Sarl, RecorMedical, Roche Diagnostics (Pleasanton, California), Roche Pharma, Sahajanand IN, Sanofi, Sarstedt AG, Servier, Terumo Deutschland, Trama Solutions, V- Wave, Vifor, and ZOLL. These grants do not impact Prof. Ruschitzka's personal remuneration. Remuneration for the time spent in following consulting activities were made directly to the University of Zurich and do not impact Prof. Ruschitzka's personal remuneration: AstraZeneca (IMC), Bayer, Boehringer Ingelheim, Citi Research, Klub Class, Novo Nordisk, Radcliffe Group, Stiftung Pfizer Forschungspreis, and Vifor. Remuneration for following lectures were made directly to the University of Zurich and do not impact Prof. Ruschitzka's personal remuneration: Abbott, Amgen, AstraZeneca (A+ Science AB), Bayer (At the Limits), Boehringer Ingelheim, Boston Scientific (CCE Services), Brigham and Women's Hospital Boston, C.T.I GmbH, FomF, Hôpitaux Universitaires des Genève (GECORE), Luzerner Kantonsspital, Sanofi-Aventis (Bridgewater, New Jersey), Servier, Medcon, Medscape (WebMD), Medtronic, Medworld, Novartis, Roche, Ruwag, Swiss Heart Failure Academy, the Hong Kong Heart Failure Society, Trama Solutions SL, Inselspital Bern, Charité – Universitätsmedizin Berlin (Medical Education Global Solutions), Romanian Society of Cardiology, ÖKG Österreichische Gesellschaft für Kardiologie, and Zoll; reports support for attending meetings from AstraZeneca (IMC / A+ Science AB), Boehringer Ingelheim, Centro Hospitaler de Vila Nova de Gaia, C.T.I. GmbH (Universitätsklinikum Düsseldorf), European Society of Cardiology, Monocle, Novartis, Spektar Putovanja, Austrian Heart Failure Association, and Heart Failure Association of the ESC. Remuneration for following advisory boards were made directly to the University of Zurich and do not impact Prof. Ruschitzka's personal remuneration: Bayer: HF Expert Summit, advisory board meeting Roche: advisory board meeting IMC/AstraZeneca: advisory board meeting Amgen: advisory board meeting. Dr. Saldarriaga reports grants/contracts from Novartis, Merck, and Bayer; payment or honoraria for lectures from Servier, Novartis, AstraZeneca, Boehringer Ingelheim, Novo Nordisk, Ely Lilly, Pfizer, and Sanofi and participation in advisory board for Merck, Bayer, and Novo Nordisk. The remaining authors have no competing interests to declare.

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