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.
Background Left ventricular (LV) wall thickening is a typical echocardiographic finding in infiltrative cardiomyopathies like cardiac amyloidosis (CA) and Fabry disease (FD). The discrimination of both infiltrative diseases remains challenging by standard as well as 2D speckle tracking echocardiography (2DSTE)-based analysis of longitudinal LV strain patterns. Over the recent years, a constant development in image quality and data processing provided better possibilities to analyse layer specific myocardial deformation indices. With regards to FD, specific layer LV strain patterns may be useful to rule-in FD in patients with suspected infiltrative cardiomyopathy. Purpose The aim of the present study was to investigate differences and the diagnostic value of layer specific 2D STE-based radial LV strain indices in CA and FD. Methods Next to standard parameters of a comprehensive echocardiographic assessment (Vivid E9 or E95, GE Vingmed, Horton, Norway, with an M5S 1.5–4.5MHz transducer), we retrospectively analysed the transmural radial LV strain (GSradial), the subendocardial radial LV strain (GSendo), the subepicardial radial LV strain (GSepi), and the strain gradient (GSendo − GSepi) (EchoPAC software, GE) in FD patients and CA patient from the amyloidosis registry at our study site. A Receiver operating curve (ROC) analysis was used to assess the diagnostic value of the respective LV strain values and the layer-specific strain gradient to discriminate FD and CA. Results A total of 38 FD and 40 CA patients were included in our analyses. In patients with FD, GSepi showed a marked impairment. LV radial and layer strain were significantly reduced in CA compared to FD patients [GSradial −12.0 (−16.2 to −9.9) in CA vs. −17.7 (−20.5 to −14.6) in FD; p<0.001); GSendo (−20.6 (−27.0 to −15.7) in CA vs. −30.0 (−32.0 to −25.6) in FD; p<0.001); and GSepi (−7.4 (−8.9 to −4.8) in CA vs. −9.1 (−11.86 to −7.6) in FD; p<0.001)]. The gradient of GSendo and GSepi was significantly lower in patients with CA compared to FD (−14.0±5.6 in CA vs. −19.4±4.3 in FD respectively; p<0.001). GSendo held the highest diagnostic accuracy to discriminate CA and FD (area under the curve [AUC] 0.83, 95% confidence interval [CI] 0.73–0.92). The layer-specific strain gradient GSendo − Gsepi showed an AUC of 0.79 (CI 0.69 to 0.89). Conclusion Layer-specific strain analysis demonstrated significantly reduced strain values in CA patients compared to FD. The analysis of GSendo held a high diagnostic accuracy to discriminate FD and CA in patients. The integration of layer-specific LV strain indices into the diagnostic work-up may improve the management of patients with an unclear thick heart pathology in future. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): Alnylam Pharmaceuticals (Cambridge, MA, USA)
Background “Classic” echocardiographic signs of Fabry cardiomyopathy (FC), such as left ventricular hypertrophy (LVH) and posterolateral strain deficiency (PLSD) have a low diagnostic accuracy in clinical practice. Purpose Our aim was to evaluate the diagnostic accuracy of phasic left atrial strain impairment compared to PLSD to discriminate FC from other forms of LVH. Methods 40 patients with LVH due to bioptically and genetically confirmed FC or with LVH due to other causes, defined by exclusion of storage diseases, such as Amyloidosis or FC, by myocardial biopsy, were retrospectively analysed. Standard echocardiographic views (Vivid E9, GE, Vingmed, Horton) were used to analyse left atrial (LA) reservoir, conduit, and contraction strain using 2D speckle tracking echocardiography (2DSTE; EchoPAC software, GE) as well as the PLSD, obtained by the mean of deformation values in basal posterior and lateral segments in a 17-segment model. Receiver operating characteristic (ROC) curve analysis and a logistic regression model were performed to assess the diagnostic accuracy of LA and LV strain impairment. Results FC was confirmed in 20 patients by genetic testing and myocardial biopsy. In the LVH group, 12 patients were classified to have hypertrophic cardiomyopathy, two had hypertensive heart disease, and six expressed the pattern of LV hypertrophy combined with borderline myocarditis. LV septum thickness (15.8mm±3.4 in FC; 17.9mm±4.3 in LVH) and left atrial volume index (LAVI) (36.7ml/m2±11.3 in FC; 45.7ml/m2±16.3 in LVH) as well as LVEF (54.2%± 9.8 in FC; 52.5%±7.7 in LVH,) were not statistically different between groups. LV filling parameters such as E/A (1.2±0.5 in FC; 1.2±0.7 in LVH) and E/e' (11.0±4.9 in FC; 13.2±5.3 in LVH) showed a slightly more advanced impairment in the LVH group. Global and regional LV function was not different between groups (LVGLS −13.8±3.7% in FC and −12.8±3.7% in LVH; PLSD −10.7±5.2% in FC and −8.85±3.9% in LVH; p-value?). LA reservoir strain (LASr) and LA conduit strain (LAScd) were significantly impaired in FC compared to the LVH group (LASr 14,6±2.5% in FC and 26.3±8.5% in LVH, p<0.01; LAScd −5.9±2.6% in FC and −15.8±4.7% in LVH, p<0.01). In ROC analysis, LASr, with an area under the curve (AUC) of 0.81 (95% CI 0.64–0.97) and LAScd with an AUC of 0.85 (95% CI 0.71–0.99), respectively, showed the highest diagnostic accuracy to discriminate FC. PLSD, in contrast, held a low diagnostic accuracy with an AUC of only 0.47 (95% CI 0.27–0.68). Conclusion A substantially higher diagnostic accuracy could be shown for LASr and LAScd impairment in discriminating FD and other forms of LVH compared to PLSD. The echocardiographic assessment of phasic LA strain may help to identify FC in patients with unclear LVH. FUNDunding Acknowledgement Type of funding sources: None. ROC analysis Representative examples
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