C63 PREVALENCE AND CLINICAL SIGNIFICANCE OF RIGHT VENTRICULAR PULMONARY ARTERIAL UNCOUPLING IN CARDIAC AMYLOIDOSIS

Abstract Background This study aims to evaluate the prevalence and clinical significance of right ventricular pulmonary arterial (RV–PA) uncoupling in patients with cardiac amyloidosis (CA). Methods The population consisted 92 consecutive CA (age 71.1±12.2 years, 71% males; 47% immunoglobulin light chain (AL), 53% transthyretin [ATTR]). All underwent a comprehensive evaluation. A pre–specified tricuspid annulus plane systolic excursion on pressure (TAPSE/PASP) value <0.31 mm/mmHg was used define RV–PA dichotomize population. Results Thirty–two (35%) showed at baseline evaluation (15/44 [34%] AL 17/48 [35%] ATTR). Patients both ATTR worse NYHA functional class, lower systemic blood pressure, more pronounced left dysfunction than those coupling. During median follow–up 8 months (IQR 4–13), 26 (28%) experienced cardiovascular death. survival 12 coupling (42.7% [95%CI 21.7–63.7%] vs. 87.3% 78.3–96.3%], p–value<0.001). Multivariate analysis identified high–sensitivity troponin I values (HR 1.01 1.00–1.02] per 1 pg/mL increase; p–value 0.013) TAPSE/PASP 1.07 1.03–1.11] 0.01 decrease; 0.002) as independent predictors Conclusions is common among CA, it marker advanced disease outcomes. suggest that ratio has potential improve risk stratification guide management strategies CA.