Right heart and left atrial strain to differentiate cardiac amyloidosis and Fabry disease
2024

Differentiating Cardiac Amyloidosis and Fabry Disease Using Echocardiography

Sample size: 135 publication 10 minutes Evidence: high

Author Information

Author(s): Isabel Mattig, Tilman Steudel, Karin Klingel, Gina Barzen, David Frumkin, Sebastian Spethmann, Elena Romero Dorta, Karl Stangl, Bettina Heidecker, Ulf Landmesser, Fabian Knebel, Sima Canaan-Kühl, Katrin Hahn, Anna Brand

Primary Institution: Charité – Universitätsmedizin Berlin

Hypothesis

Can right heart and left atrial strain parameters effectively differentiate between cardiac amyloidosis and Fabry disease?

Conclusion

The study found that echocardiographic parameters, particularly right heart and left atrial strain, can significantly improve the differentiation between cardiac amyloidosis and Fabry disease.

Supporting Evidence

  • Echocardiographic parameters showed significant differences between cardiac amyloidosis and Fabry disease.
  • Right ventricular strain and left atrial strain were key indicators for differentiation.
  • The best diagnostic accuracy was achieved by combining multiple echocardiographic parameters.

Takeaway

Doctors can use special heart tests to tell the difference between two heart diseases, cardiac amyloidosis and Fabry disease, which can help patients get the right treatment faster.

Methodology

The study retrospectively analyzed echocardiographic data from patients diagnosed with cardiac amyloidosis and Fabry disease to assess the diagnostic accuracy of various strain parameters.

Potential Biases

Differences in treatment received by patients at the time of echocardiography could introduce bias.

Limitations

The study's retrospective design and significant differences in baseline characteristics between the two patient groups may affect the results.

Participant Demographics

Patients included were aged 18 and older, with a significant difference in age and gender distribution between the two diseases.

Statistical Information

P-Value

p<0.001

Confidence Interval

95% CI 0.90–1.00

Statistical Significance

p<0.001

Digital Object Identifier (DOI)

10.1038/s41598-024-52890-y

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