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Impact of combined systolic and diastolic functional parameter assessment for differentiation of cardiac amyloidosis from other causes of concentric left ventricular hypertrophy.

Purpose: Differentiation of cardiac amyloidosis (CA) from other causes of concentric left ventricular (LV) hypertrophy remains a clinical challenge, especially in the early stages with preserved ejection fraction. Methods and Results: Consecutive hypertrophic patients with CA, isolated arterial hypertension (HP), Fabry disease (FD), and Friedreich’s ataxia (FA) (n=25 per group) were investigated ; 25 healthy volunteers served as a control group. Standard echocardiography was performed and segmental longitudinal peak systolic strain (LSsys) in the septum was assessed by two- dimensional speckle tracking imaging. Indices of LV hypertrophy and ejection fraction were similar among all patient groups. Deceleration time of early filling (DT) was significantly lower in CA (147±46 ms) compared with HP, FD or controls (all P<0.05). Septal basal LSsys (-6±2%) was significantly lower in CA than in HP (-14±6%), FD (-12±5%), FA (-16±2%), or controls (-17±3% ; all P<0.001), whereas septal apical LSsys was similar amongst all patient groups and controls (all P>0.05). A data-driven cut-off value for the ratio of septal apical to basal LSsys ratio (LSsysapi/bas) >2.1 differentiated CA from other causes of LV hypertrophy (sensitivity 88.0%, specificity 85.3%, positive predictive value 66.7%, negative predictive value 95.5%). The prevalence of LSsysapi/bas >2.1 plus DT <200 ms was 88% in CA, but 0% in all other groups. Conclusions: A systolic septal longitudinal base- to-apex strain gradient (LSsysapi/bas >2.1) in combination with a shortened diastolic deceleration time of early filling (DT <200 ms) aids in differentiating CA from other causes of concentric LV hypertrophy.

Amyloidosis; hypertrophic cardiomyopathies; concentric LV hypertrophy; speckle tracking imaging.

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