Right ventricular to pulmonary artery uncoupling is an early predictor of poor outcome in wild-type transthyretin amyloid cardiomyopathy.

Non-invasive right ventricular to pulmonary artery (RV-PA) uncoupling assessment has prognostic value in patients with heart failure (HF). Little is known about its application in patients with wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM). This single-centre retrospective study included consecutive patients with wtATTR-CM diagnosis undergoing 2D echocardiogram. RV-PA uncoupling was evaluated with the ratios between tricuspid annular plane systolic excursion (TAPSE), RV free wall longitudinal strain (RVFWLS) or RV four-chamber longitudinal strain (RV4CLS) and pulmonary artery systolic pressure (sPAP). Primary endpoint was the composite of all-cause mortality and HF hospitalisation. Overall, 100 patients (91% males, median age 81 years, 85% in National Amyloid Centre (NAC) stage ≤ 2, 18% in NAC stage Ia and 82% in New York Heart Association class ≤ II) were enrolled. Over a 16-months follow up (Q1-Q3:12-24), the primary endpoint occurred in 37 patients (37%). TAPSE/sPAP (HR 0.04, 95% CI 0.01-0.24, p < 0.001), RVFWLS/sPAP (HR 0.07, 95% CI 0.01-0.41, p = 0.003) and RV4CLS/sPAP (HR 0.06, 95% CI 0.01-0.53, p = 0.011) emerged as independent predictors of the primary endpoint and showed incremental risk prediction compared with TAPSE, RVFWLS and RV4CLS, considered as separate parameters. No differences in outcome risk prediction were observed among TAPSE/sPAP, RVFWLS/sPAP and RV4CLS/sPAP (p > 0.05). RV-PA uncoupling, as assessed by different echocardiography modalities, is an early predictor of poor outcome in patients with wtATTR-CM.