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Cardiac Amyloidosis Screening and Management in Patients With Heart Failure With Preserved Ejection Fraction: An
Anastasia Shchendrygina1, Nathan Mewton2, David Niederseer3
1Department of Hospital Therapy 2, I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation.
Insights
Cardiac amyloidosis (CA) is underdiagnosed in heart failure with preserved ejection fraction (HFpEF). Global physician survey reveals varied screening, diagnostic, and treatment approaches, highlighting a need for improved education and access to therapies.
Area of Science:
- Cardiology
- Internal Medicine
- Medical Diagnostics
Background:
- Cardiac amyloidosis (CA) is an underdiagnosed cause of heart failure (HF).
- Early recognition and treatment of CA in HF with preserved ejection fraction (HFpEF) improve patient outcomes.
- Current screening and management practices for CA in HFpEF patients are not standardized globally.
Purpose of the Study:
- To assess worldwide physician approaches to screening and managing CA in patients with HFpEF.
- To identify current diagnostic tools and treatment strategies employed by physicians for CA in HFpEF.
- To understand physician perspectives on the availability and administration of disease-modifying therapies (DMTs) for CA.
Main Methods:
- An international, web-based survey was conducted among physicians from May to July 2023.
- The survey collected data from 1,460 physicians across 95 countries on CA screening, diagnosis, and management of HFpEF patients.
- Data analysis focused on screening criteria, first-line diagnostic tools, and treatment preferences.
Main Results:
- Only 10% of physicians systematically screened all HFpEF patients for CA; 24% did not screen at all.
- Left ventricular hypertrophy was the most common screening criterion (39%).
- Serum protein electrophoresis with immunofixation and urine protein electrophoresis or cardiac MRI were common first-line diagnostics; CA DMT was available to 48% of physicians.
Conclusions:
- Significant global uncertainty exists regarding the necessity of CA screening in HFpEF patients.
- Management of CA, particularly screening and diagnostics, is highly heterogeneous.
- There is a critical need for enhanced education within the HF community and improved access to CA-specific DMTs.
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.
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