Prevalence and clinical significance of right ventricular pulmonary arterial uncoupling in cardiac amyloidosis

• Published in: International journal of cardiology Vol. 388; p. 131147
• Main Authors: Palmiero, Giuseppe, Monda, Emanuele, Verrillo, Federica, Dongiglio, Francesca, Caiazza, Martina, Rubino, Marta, Lioncino, Michele, Diana, Gaetano, Vetrano, Erica, Fusco, Adelaide, Cirillo, Annapaola, Mauriello, Alfredo, Ciccarelli, Giovanni, Ascione, Luigi, De Rimini, Maria Luisa, D'Alto, Michele, Cerciello, Giuseppe, D'Andrea, Antonello, Golino, Paolo, Calabrò, Paolo, Bossone, Eduardo, Limongelli, Giuseppe
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.10.2023
• Subjects: Cardiac amyloidosis; Echocardiography; Outcome; Cardiac amyloidosis; Outcome; Echocardiography
• ISSN: 0167-5273; 1874-1754; 1874-1754
• DOI: 10.1016/j.ijcard.2023.131147

This study aims to evaluate the prevalence and the clinical significance of the right ventricular pulmonary arterial (RV-PA) uncoupling in patients with cardiac amyloidosis (CA). 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]). A pre-specified tricuspid anulus 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. 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 ventricular and RV 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. RV-PA uncoupling is common among patient with CA, and it is a marker of advanced disease and worse outcome. This study suggest that TAPSE/PASP ratio has the potential to improve risk stratification and guide management strategies in patients with CA of different etiology and advanced disease. Prevalence of RV-PA uncoupling (defined as TAPSE/PASP <0.31) was evident in 31% of patients in our cohort. Moreover, patients with RV-PA uncoupling exhibited worse symptoms, more pronounced LV and RV dysfunction, and reduced survival than those with RV-PA coupling. TAPSE/PASP was identified as independent predictors of CV mortality. Abbreviations: CI, confidence interval; CV, cardiovascular; GLS, global longitudinal strain; HR, hazard ratio; LVEF, left ventricular ejection fraction; NT-proBNP, N-terminal pro b-type natriuretic peptide; NYHA, New York Heart Association; TAPSE, tricuspid annular plane systolic excursion; PASP, pulmonary arterial systolic pressure; RV-PA, right ventricular pulmonary arterial. [Display omitted] •35% of patients showed RV-PA uncoupling at baseline.•Patients with RV-PA uncoupling showed worse clinical parameters.•TAPSE/PASP was an independent predictor of cardiovascular death.