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Global Burden of Cardiac Amyloidosis in Heart Failure: A Systematic Review and Meta-Analysis
Syed Bukhari1, Mohammad Hamza, Muhammad Abdul Rehman
1Johns Hopkins University.
Insights
Cardiac amyloidosis (CA) affects 10% of heart failure (HF) patients globally, predominantly wild-type transthyretin amyloidosis (ATTRwt-CA). Underdiagnosis may occur in certain regions and among females, necessitating further research.
Area of Science:
- Cardiology
- Internal Medicine
- Medical Diagnostics
Background:
- Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy increasingly recognized as a cause of heart failure (HF).
- Accurate global prevalence data for CA is lacking, hindering effective management and screening strategies.
- Advances in noninvasive diagnostics have improved CA detection rates.
Purpose of the Study:
- To systematically review and meta-analyze existing studies to determine the global prevalence of CA in heart failure populations.
- To investigate geographic, subtype, sex-based, and study design-related variations in CA prevalence.
- To identify the predominant subtype of CA and assess its prevalence within screened populations.
Main Methods:
- Systematic review and meta-analysis of studies identified through comprehensive database searches (PubMed Central, Cochrane, EMBASE, Web of Science).
- Inclusion of 28 studies with 7,393 heart failure patients, diagnosed via technetium-labeled bone scintigraphy or biopsy.
- Random-effects proportional meta-analysis to pool prevalence data and assess heterogeneity.
Main Results:
- The pooled prevalence of CA among heart failure patients was 10% (95% CI, 7%-13%), with significant heterogeneity observed (prediction interval, 2%-41%).
- Wild-type transthyretin amyloidosis (ATTRwt-CA) was the predominant subtype, accounting for 76% of CA diagnoses.
- Prevalence varied geographically (6% in North America to 15% in Asia), and 24% of CA cases occurred in females, suggesting potential underdiagnosis.
Conclusions:
- Cardiac amyloidosis is present in approximately one in ten heart failure patients worldwide, with ATTRwt-CA being the most common form.
- Geographic variations and a notable proportion of cases in females suggest potential underdiagnosis in underrepresented regions and populations.
- Standardized, multinational studies are crucial for a precise global burden assessment and to inform equitable screening strategies for CA.
Abstract:
Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy increasingly recognized as an important contributor to heart failure (HF), particularly with advances in noninvasive diagnostics. However, the global prevalence of CA remains poorly defined, with substantial variability across regions. We conducted a systematic review and meta-analysis of studies reporting the prevalence of CA among HF populations. The protocol was registered with PROSPERO and followed the PRISMA reporting guideline. Searches of PubMed Central, Cochrane, EMBASE and Web of Science identified eligible studies from inception through 2025. CA was diagnosed using technetium-labeled bone scintigraphy or biopsy. A random-effects proportional meta-analysis was conducted to estimate the pooled prevalence and assess geographic, subtype, sex-based, and study design-related differences. Twenty-eight studies encompassing 7,393 HF patients were included, of whom 627 were diagnosed with CA. The pooled prevalence of CA among HF patients was 10% (95% CI, 7%-13%), with substantial heterogeneity (prediction interval, 2-41%). Prevalence varied by region, ranging from 6% in North America to 15% in Asia, though subgroup differences were not statistically significant. Wild-type transthyretin amyloidosis (ATTRwt-CA) accounted for the majority of cases, representing 76% (95% CI, 57%-89%) of CA diagnoses and a pooled prevalence of 11% among screened populations. Approximately 24% of CA cases occurred in females. In conclusion, CA is present in one in ten patients with HF worldwide, with ATTRwt-CA being the predominant subtype. Uneven geographic distribution may suggest underdiagnosis, particularly in underrepresented regions and in females. Standardized, multinational studies are needed to define the global burden of CA better and guide equitable screening strategies.
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