In cardiac amyloidosis, SWE PSAX IVS middle >5.6 kPa predicted higher mortality (HR 2.785), while patisiran use lowered mortality risk (HR 0.071) and improved survival.
Does myocardial stiffness measured by shear wave elastography predict mortality in patients with hereditary CA-ATTR?
Higher myocardial stiffness measured by shear wave elastography is associated with increased mortality in patients with hereditary transthyretin cardiac amyloidosis.
Absolute Event Rate: 0% vs 0%
Abstract Background Cardiac amyloidosis by transthyretin (CA-ATTR) leads to increased stiffness and reduced myocardial compliance, resulting in a characteristic restrictive pattern. Shear wave elastography (SWE) allows for the non-invasive assessment of myocardial stiffness and is proposed as a prognostic predictor in patients with CA-ATTR. Purpose To evaluate myocardial elasticity using SWE as a prognostic predictor in patients with CA-ATTR. Materials ans methods A prospective, single-center study included 40 patients with hereditary CA-ATTR, divided in 20 with cardiac involvement and 20 without. Myocardial elasticity was measured at the end of diastole, with results expressed in kilopascals (kPa) in the basal, mid, and apical segments of the interventricular septum (IVS) and the free wall of the right ventricle in the parasternal long-axis (PLAX) and parasternal short-axis (PSAX). Mortality prediction data were analyzed in the entire cohort and in the affected population through Cox regression. Correlation and ROC curve analysis was conducted. Results The mean age was 55 years, with 27 men (67.5%). Five patients were on patisiran treatment (12.5%). The all-cause mortality rate was 30%. Regarding the SWE values in kPa, they were higher in the population that progressed to death. A reduction in the left ventricular ejection fraction HR 0.905 (0.825–0.993) p = 0.035, a decrease in the glomerular filtration rate (GFR) HR 0.868 (0.784–0.961) p = 0.006, and an increase in the SWE of the PSAX IVS middle HR 2.785 (1.282–6.052) p = 0.010 were identified as predictors of higher mortality. When analyzing only the affected population, a reduction in the GFR HR 0.91 (0.84–0.98) p = 0.013 and an increase in the SWE of the PSAX apical IVS HR 3.05 (1.17–7.95) p = 0.022 were associated with higher mortality. Furthermore, the use of patisiran was associated with better survival outcomes HR 0.071 (0.008–0.654) p = 0.020. In the survival analysis, patisiran use was also associated with improved survival Log-rank: 4.502 (p = 0.034). When analyzing the optimal cut-off value in kPa as a predictor in the total population, the SWE of the PSAX IVS middle showed a cut-off value 5.6 kPa, with an AUC of 0.782 (0.654–0.824); 95% CI. Regarding the cut-off value of the PSAX IVS apical in the population with cardiac involvement, a value 4.6 kPa, with an AUC of 0.641 (0.393–0.888); 95% CI, showed a reasonable specificity for stratifying a worse prognosis (75%). Conclusions The introduction of a new technique for assessing myocardial stiffness has become a reality. A higher kPa value in the short axis of the apical segment of the IVS was associated with a worse outcome. Furthermore, although the population treated with patisiran was small, its use was linked to improved survival. As anticipated, a lower functional class and a reduced filtration rate were also identified as predictors of poor prognosis.
Romero et al. (Sat,) reported a other. In cardiac amyloidosis, SWE PSAX IVS middle >5.6 kPa predicted higher mortality (HR 2.785), while patisiran use lowered mortality risk (HR 0.071) and improved survival.