April 10, 2025Open Access

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

Q: What is the clinical evidence from this study?

Study design: Observational. Population: Wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM) (n=100). Intervention: Right ventricular to pulmonary artery (RV-PA) uncoupling assessment vs. RV function parameters alone. Primary outcome: Composite of all-cause mortality and heart failure hospitalisation (HR 0.04, 95% CI 0.01-0.24, p=<0.001).

Key Result

Right ventricular to pulmonary artery uncoupling, assessed by TAPSE/sPAP (HR 0.04), independently predicted the composite of all-cause mortality and heart failure hospitalization in wtATTR-CM.

Study Design

Type

Observational (n=100)

Multicenter

Structured PICO

Does right ventricular to pulmonary artery (RV-PA) uncoupling predict the composite of all-cause mortality and HF hospitalisation in patients with wild-type transthyretin amyloid cardiomyopathy?

Population

100 patients with wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM) undergoing 2D echocardiogram, median age 81 years, 91% male, 85% in NAC stage ≤ 2, 18% in NAC stage Ia, and 82% in NYHA class ≤ II.

Intervention

Non-invasive right ventricular to pulmonary artery (RV-PA) uncoupling assessment via 2D echocardiogram (TAPSE/sPAP, RVFWLS/sPAP, RV4CLS/sPAP)

Outcome

Composite of all-cause mortality and HF hospitalisationcomposite

RV-PA uncoupling assessed by echocardiography is an early independent predictor of mortality and heart failure hospitalization in patients with wild-type transthyretin amyloid cardiomyopathy.

Main Result

Effect estimate: HR 0.04 (95% CI 0.01-0.24)

p-value: p=<0.001

Limitations

Observational and single-centre design
Relatively small sample size
Evaluation of disease-modifying therapy efficacy could be affected by small sample size and different timing of prescription

Abstract

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 0.05). RV-PA uncoupling, as assessed by different echocardiography modalities, is an early predictor of poor outcome in patients with wtATTR-CM.

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