In ATTRwt, reduced LVEF (<50%) doubled mortality risk (aHR 2.04), and elevated RAVi/TAPSE ratio ≥2.2 increased mortality risk by 92% (aHR 1.92, p=0.015).
Do echocardiographic parameters, specifically LVEF and the novel RAVi/TAPSE ratio, predict mortality in patients with wild-type transthyretin cardiac amyloidosis?
In patients with wild-type transthyretin cardiac amyloidosis, LVEF <50% and a novel echocardiographic marker of right ventricular failure (RAVi/TAPSE ratio ≥2.2) are strong independent predictors of mortality.
Absolute Event Rate: 0% vs 0%
Abstract Introduction Wild-type transthyretin cardiac amyloidosis (ATTRwt) is a common cause of heart failure in the elderly. It was historically viewed as a primarily diastolic dysfunction. However, recent advancements revealed that both left ventricular (LV) systolic and diastolic function may be affected, yet detailed assessments remain sparse. Furthermore, data on right-sided cardiac function are critically lacking, highlighting a substantial knowledge gap in this condition. Aims This study describes the distribution and prognostic implications of the left and right ventricular systolic and diastolic echocardiographic parameters in a contemporary ATTRwt population. Furthermore, a novel parameter that combines systolic and diastolic right ventricular function was introduced, the right atrial volume index (RAVi) to Tricuspid Annular Plane Systolic Excursion (TAPSE) ratio (RAVi/TAPSE). Methods This single-center cohort study included consecutive ATTRwt patients from Jan 1st, 2016 -Dec 31st, 2023. All patients were followed from diagnosis until death, 5 years of follow-up, or until the censoring date on Nov 1st, 2024. Echocardiographic parameters were measured according to the current guidelines and were obtained around the time of ATTRwt diagnosis. Results The study cohort comprised 257 ATTRwt patients, predominantly male (89%) with a mean (±SD) age at diagnosis of 81 ±5.9 years. At the time of diagnosis, 43% had preserved left ventricular ejection fraction (pEF, EF≥50%), 35% had mid-range EF (mrEF, EF 40-49%), and 22% had reduced EF (rEF, EF40%). Global longitudinal strain (GLS), stroke volume index (SVi) and cardiac index (CI) were reduced with GLS (absolute number) at 12±3.3 , SVi 30 ±8.9 mL/m², and CI 2.1±0.64 L/min/m². The median IQR left atrial volume index was 45 37-57 ml/m², and E/e’ was 12 9.3-16. Right-sided parameters were as follows: mean TAPSE 18±5.1 mm, median RAVi 38 28-51 ml/m², and median RAVi/TAPSE ratio 2.2 1.4-3.5. The median follow-up time was 33 months 20-49 and the median survival time estimated by Kaplan Meier analysis was 58 months (95% CI: 47-75). LVEF at the time of diagnosis was significantly associated with survival with an adjusted hazard ratio (aHR) of 0.97 (95% CI: 0.95-0.99, p=0.01). LVEF 50% had an aHR of 2.04 (95% CI:1.28 - 3.22, p=0.002) compared to LVEF≥50%. Comparing rEF to mrEF showed no significant difference in mortality (aHR 0.99, 95% CI:0.60-1.65, p =0.98). The RAVi/TAPSE ratio, as a continuous variable, had an aHR of 1.24 (95% CI: 1.14 - 1.36, p0.001). The RAVi/TAPSE ratio ≥2.2 (median) was associated with an aHR of 1.92, (95% CI: 1.14 - 3.23, p=0.015). Conclusions ATTRwt exhibits diverse phenotypes with only 43% of ATTRwt patients presenting with pEF. Despite the demonstrated prognostic value of LVEF, no mortality difference was observed between the mrEF and rEF groups. The novel RAVi/TAPSE ratio, a marker of RV failure, was strongly associated with mortality.Distribution of RAVi/TAPSE by EF groups KM estimates for RAVi/TAPSE (by median)
Mejren et al. (Sat,) reported a other. In ATTRwt, reduced LVEF (<50%) doubled mortality risk (aHR 2.04), and elevated RAVi/TAPSE ratio ≥2.2 increased mortality risk by 92% (aHR 1.92, p=0.015).
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