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Comparative analysis of phasic left atrial strain and left ventricular posterolateral strain pattern to discriminate Fabry cardiomyopathy from other forms of left ventricular hypertrophy
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  • David Frumkin,
  • Isabel Mattig,
  • Maamoun Al-Daas,
  • Nina-Maria Laule,
  • Sima Canaan-kühl,
  • Fabian Knebel,
  • Karl Stangl,
  • Anna Brand
David Frumkin
Charité Universitätsmedizin Berlin
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Isabel Mattig
Charité Universitätsmedizin Berlin
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Maamoun Al-Daas
Charité Universitätsmedizin Berlin
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Nina-Maria Laule
Charité Universitätsmedizin Berlin
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Sima Canaan-kühl
Charité Universitätsmedizin Berlin
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Fabian Knebel
Charité Universitätsmedizin Berlin
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Karl Stangl
Charité-Universitätsmedizin Berlin
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Anna Brand
Charite Universitätsmedizin Berlin, Campus Charite Mitte
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Abstract

Background ‘Classical’ echocardiographic signs of Fabry cardiomyopathy (FC), such as left ventricular hypertrophy (LVH), posterolateral strain deficiency (PLSD) and papillary muscle hypertrophy may have a limited diagnostic accuracy in clinical practice. Our aim was to evaluate the diagnostic value of left atrial (LA) strain impairment compared to ‘classical’ echocardiographic findings to discriminate FC. Methods In standard echocardiographic assessments, we retrospectively analyzed the diagnostic value of the “classical” red flags of FC as well as LA strain in 20 FC patients and in 20 subjects with other causes of LVH. Receiver operating characteristic (ROC) curve analysis was performed to assess the respective diagnostic accuracy. Results FC was confirmed in 20 patients by genetic testing. In the LVH group, 12 patients were classified by biopsy to have hypertrophic cardiomyopathy, two had hypertensive heart disease, and six LVH combined with borderline myocarditis. Global and regional left ventricular (LV) strain was not significantly different between groups while LA strain was significantly impaired in FC (Left atrial reservoir strain (LASr) 19.1%±8.4 in FC and 25.6%±8.9 in LVH, p=0.009; left atrial conduction strain (LAScd) -8.4%±4.9 in FC and -15.9%±8.4 in LVH, p<0.01). LAScd, with an area under the curve (AUC) of 0.81 [95% confidence interval (CI) 0.66-0.96] showed the highest diagnostic accuracy to discriminate FC. The PLSD pattern showed an AUC of 0.49, quantification of papillary muscle hypertrophy an AUC of 0.47. Conclusion Adding LA strain analysis to a comprehensive echocardiographic work-up of unclear LVH may be helpful to identify FC as a possible cause.

Peer review status:UNDER REVIEW

11 Jun 2021Submitted to Echocardiography
21 Jun 2021Submission Checks Completed
21 Jun 2021Assigned to Editor
22 Jun 2021Reviewer(s) Assigned
19 Jul 2021Review(s) Completed, Editorial Evaluation Pending
06 Aug 2021Editorial Decision: Revise Major
06 Sep 20211st Revision Received
08 Sep 2021Submission Checks Completed
08 Sep 2021Assigned to Editor
08 Sep 2021Reviewer(s) Assigned