Effect of Combined Systolic and Diastolic Functional Parameter Assessment for Differentiation of Cardiac Amyloidosis From Other Causes of Concentric Left Ventricular Hypertrophy

Abstract

Background-Differentiation of cardiac amyloidosis (CA) from other causes of concentric left ventricular hypertrophy remains a clinical challenge, especially in patients with preserved ejection fraction at the early disease stages. Methods and Results-Consecutive hypertrophic patients with CA, isolated arterial hypertension, Fabry disease, and Friedreich ataxia (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 2-dimensional speckle tracking imaging. Indices of left ventricular hypertrophy and ejection fraction were similar among all patient groups. Deceleration time of early filling was significantly lower in patients with CA (147±46 milliseconds) compared with those with isolated arterial hypertension, Fabry disease, or control subjects (all P<0.0125). Septal basal LSsys (−6±2%) was significantly lower in patients with CA compared with those with isolated arterial hypertension (−14±6%), Fabry disease (−12±5%), Friedreich ataxia (−16±2%), or control subjects (−17±3%; all P<0.001), whereas septal apical LSsys was similar among all patient groups and control subjects (all P>0.05). A data-driven cutoff value for the ratio of septal apical to basal LSsys ratio >2.1 differentiated CA from other causes of left ventricular hypertrophy (sensitivity, 88%; specificity, 85%; positive predictive value, 67%; negative predictive value, 96%). The prevalence of septal apical to basal LSsys ratio >2.1 plus deceleration time of early filling <200 milliseconds was 88% in CA but 0% in all other groups. Conclusions-A systolic septal longitudinal base-to-apex strain gradient (septal apical to basal LSsys ratio >2.1), combined with a shortened diastolic deceleration time of early filling (deceleration time of early filling <200 milliseconds), aids in differentiating CA from other causes of concentric left ventricular hypertrophy. (Circ Cardiovasc Imaging. 2013;6:1066-1072.)

Visual Assessment of Relative Apical Sparing Pattern Is More Useful Than Quantitative Assessment for Diagnosing Cardiac Amyloidosis in Borderline or Mildly Increased Left Ventricular Wall Thickness

“…LV short-axis function in CA is known to be preserved even when LV long-axis function is significantly Tables 1,2. with atrial fibrillation should be performed in the near future. 7, 8 Third, patients with other causes of LVH were heterogeneous. Less advanced myocardial morphology on echocardiography, however, has a wide range of etiology, and thus we believe that this study reflects real-world practice.…”

“…19 LV hypertrophy (LVH) by voltage criteria was defined as the sum of S wave in V1 and R wave in V5 or V6 ≥35 mm. 8 Pseudoinfarct pattern was defined as a QS wave in 2 consecutive anterior leads in the absence of old myocardial infarction. 8 CMR CMR was conducted using 1.5-T systems (Magnetom Avanto ® and Sonata Magnetom ® , Siemens Medical Systems, Erlangen, Germany) gated with ECG.…”

Echo Parameters for Differential Diagnosis in Cardiac Amyloidosis

“…On the basis of the previous literature, 8,9 the conventional parameters EF, ECC IND, CONC HYP, left atrial volume index, DT, and E/E′ were used for comparison with deformation-based indices.…”

Echocardiographic Assessment of the Cardiac Amyloidoses

“…64 A systolic septal longitudinal base-to-apex strain gradient (septal apical/basal longitudinal systolic ratio >2.1), combined with a shortened diastolic deceleration time of early filling (deceleration time of early filling <200 ms), aids in differentiating cardiac amyloidosis from other causes of concentric LV hypertrophy. 65 Using parametric polar maps of regional longitudinal strain, regional variations in strain are easily recognizable, and represent an accurate and reproducible means of differentiating hypertrophic cardiomyopathy or cardiac amyloidosis from hypertensive heart disease. 66 …”