Key result
Left atrial conduction strain accurately discriminates Fabry cardiomyopathy from other forms of LVH.
Why the study?
Classical echocardiographic signs of Fabry cardiomyopathy may have limited diagnostic accuracy in clinical practice, prompting evaluation of left atrial strain impairment compared to classical findings.
Does left atrial strain analysis improve diagnostic accuracy to discriminate Fabry cardiomyopathy from other forms of left ventricular hypertrophy compared to classical echocardiographic signs?
Observational (n=40)
Does left atrial strain analysis improve diagnostic accuracy to discriminate Fabry cardiomyopathy from other forms of left ventricular hypertrophy compared to classical echocardiographic signs?
Effect estimate: AUC 0.81 (95% CI 0.66-0.96)
Left atrial strain analysis, particularly left atrial conduction strain, provides superior diagnostic accuracy compared to classical echocardiographic signs for discriminating Fabry cardiomyopathy from other causes of left ventricular hypertrophy.
BACKGROUND: "Classical" echocardiographic signs of Fabry cardiomyopathy (FC), such as left ventricular hypertrophy (LVH), posterolateral strain impairment (PLSI), and papillary muscle hypertrophy may be of 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 .81 (95% confidence interval [CI] .66-.96) showed the highest diagnostic accuracy to discriminate FC. The PLSI pattern showed an AUC of .49, quantification of papillary muscle hypertrophy an AUC of .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.
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Frumkin et al. (2021) conducted an observational in Fabry cardiomyopathy and other forms of left ventricular hypertrophy (n=40). Left atrial strain analysis vs. Classical echocardiographic signs was evaluated on Diagnostic accuracy to discriminate Fabry cardiomyopathy (AUC 0.81, 95% CI 0.66-0.96). Left atrial conduction strain showed high diagnostic accuracy to discriminate Fabry cardiomyopathy from other forms of left ventricular hypertrophy (AUC 0.81; 95% CI 0.66-0.96).
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