Predictive Value of Assessing Diastolic Strain Rate on Survival in Cardiac Amyloidosis Patients with Preserved Ejection Fraction

Abstract

ObjectivesSince diastolic abnormalities are typical findings of cardiac amyloidosis (CA), we hypothesized that speckle-tracking-imaging (STI) derived longitudinal early diastolic strain rate (LSRdias) could predict outcome in CA patients with preserved left ventricular ejection fraction (LVEF >50%).BackgroundDiastolic abnormalities including altered early filling are typical findings and are related to outcome in CA patients. Reduced longitudinal systolic strain (LSsys) assessed by STI predicts increased mortality in CA patients. It remains unknown if LSRdias also related to outcome in these patients.MethodsConventional echocardiography and STI were performed in 41 CA patients with preserved LVEF (25 male; mean age 65±9 years). Global and segmental LSsys and LSRdias were obtained in six LV segments from apical 4-chamber views.ResultsNineteen (46%) out of 41 CA patients died during a median of 16 months (quartiles 5–35 months) follow-up. Baseline mitral annular plane systolic excursion (MAPSE, 6±2 vs. 8±3 mm), global LSRdias and basal-septal LSRdias were significantly lower in non-survivors than in survivors (all p<0.05). NYHA class, number of non-cardiac organs involved, MAPSE, mid-septal LSsys, global LSRdias, basal-septal LSRdias and E/LSRdias were the univariable predictors of all-cause death. Multivariable analysis showed that number of non-cardiac organs involved (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.17–3.26, P = 0.010), global LSRdias (HR = 7.30, 95% CI 2.08–25.65, P = 0.002), and E/LSRdias (HR = 2.98, 95% CI 1.54–5.79, P = 0.001) remained independently predictive of increased mortality risk. The prognostic performance of global LSRdias was optimal at a cutoff value of 0.85 S−1 (sensitivity 68%, specificity 67%). Global LSRdias <0.85 S−1 predicted a 4-fold increased mortality in CA patients with preserved LVEF.ConclusionsSTI-derived early diastolic strain rate is a powerful independent predictor of survival in CA patients with preserved LVEF.

Regional myocardial microvascular dysfunction in cardiac amyloid light-chain amyloidosis: assessment with 3T cardiovascular magnetic resonance

“…In addition, CA patients has smaller cavity sizes (reduced LVEDV) due to increased wall thickness could result in a comparative decrease in EF compared with normal controls, EF may be >50 % in patients with small cavity size result from concentric “hypertrophy”, thus may not accurate to evaluated the systolic function and may consequently mis-classifying some patients with truly impaired systolic function into the normal systolic function group. Dan Liu et al previously reported that longitudinal dysfunction was associated with poor outcome in CA patients with preserved LVEF [ 42 ]. Therefore, the association between first-pass data and more sensitive indices reflecting the LV systolic function, including strain and pulsed tissue Doppler echocardiography, which can assist in the early identification of systolic dysfunction, will be discussed in our future study.…”

Role of cardiovascular imaging for the diagnosis and prognosis of cardiac amyloidosis

“…Compared with the conventional diastolic parameters like E′ and E/E′, speckle tracking imaging-derived diastolic deformation, longitudinal early diastolic strain rate (LSR dias ) was superior in predicting outcomes in patients with CA with preserved LVEF (>50%). 23 LSR dias <0.85 s −1 alone was associated with fourfold increased mortality in these patients. A low MAPSE (mitral annular plane systolic excursion) value, which indicates the longitudinal ventricular function, is also associated with poor outcomes in patients with heart failure with preserved LVEF.…”

Association and diagnostic utility of diastolic dysfunction and myocardial fibrosis in patients with Fabry disease

“…A standard echocardiographic examination was performed on a Vivid 7 or 9e echo machine (GE Vingmed, Horten, Norway). Measurements were performed offline by a single investigator (DL) according to recommended guidelines as previously described 20. Pulsed-wave Doppler and tissue Doppler were recorded and diastolic parameters including mitral inflow peak early (E-wave) and late (A-wave) velocities, E/A ratio, deceleration time (DT) of E velocity and isovolumetric relaxation time (IVRT) were measured 21.…”

“…STI analysis was performed using EchoPAC software (GE, Horten, Norway) as described previously 20. Longitudinal peak systolic strain rate (LSr_sys) and LS_sys, longitudinal early (LSr_E) and late (LSr_A) diastolic peak strain rates of each segment were measured.…”