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

Abstract

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