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

Q: What are the key findings of this study?

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.

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).

Q: What question did this study set out to answer?

To determine whether non-invasive echocardiographic measures of right ventricular to pulmonary artery (RV-PA) uncoupling predict adverse clinical outcomes in wild-type transthyretin amyloid cardiomyopathy.

Giulio Sinigiani; Laura De Michieli; Matteo d'Addazio; Lisa Portalone; Monica De Gaspari; Alessandro Lupi; Alessandro Zorzi; Francesco Tona; Cristina Basso; Martina Perazzolo Marra; Sabino Iliceto; Domenico Corrado; Stefano Nistri; Donato Mele; Alberto Cipriani

doi:10.1007/s10554-025-03394-x

April 10, 2025The International Journal of Cardiovascular ImagingOpen Access

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

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Key result

RV-PA uncoupling assessed by TAPSE/sPAP strongly predicts mortality and HF hospitalization in wtATTR-CM.

HR 0.04
95% CI 0.01-0.24
P<0.001
n=100

Figures

Why the study?

Non-invasive right ventricular to pulmonary artery uncoupling has prognostic value in heart failure, but little is known about its application in wild-type transthyretin amyloid cardiomyopathy.

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?

Key Points

To determine whether non-invasive echocardiographic measures of right ventricular to pulmonary artery (RV-PA) uncoupling predict adverse clinical outcomes in wild-type transthyretin amyloid cardiomyopathy.
Conducted a single-centre retrospective study of consecutive patients (N=100; 91% male, median age 81 years) diagnosed with wild-type transthyretin amyloid cardiomyopathy undergoing 2D echocardiography.
Assessed RV-PA uncoupling using ratios of TAPSE, RV free wall longitudinal strain (RVFWLS), and RV four-chamber longitudinal strain (RV4CLS) to pulmonary artery systolic pressure (sPAP) against a composite endpoint of all-cause mortality and heart failure hospitalization over a 16-month median follow-up (Q1-Q3: 12-24).
The primary composite endpoint occurred in 37% of patients (37/100) over follow-up.
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) each independently predicted adverse outcomes with incremental predictive value over single metrics.
Risk prediction for clinical outcomes showed no statistically significant differences among the three uncoupling ratios (p > 0.05).

Study Design

Type

Observational (n=100)

Multicenter

No

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?

P

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.

I

Intervention

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

O

Outcome

Composite of all-cause mortality and HF hospitalisationcomposite

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

Implication

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.

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 < 0.001), 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.

Discussion

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Cite This Study

Sinigiani et al. (2025) conducted an observational in Wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM) (n=100). Right ventricular to pulmonary artery (RV-PA) uncoupling assessment vs. RV function parameters alone was evaluated on Composite of all-cause mortality and heart failure hospitalisation (HR 0.04, 95% CI 0.01-0.24, p=<0.001). 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.

synapsesocial.com/papers/6a0eb8388a6cf20890229551 https://doi.org/10.1007/s10554-025-03394-x

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

Figures

Key Points

Study Design

Structured PICO

Main Result

Limitations

Implication

Abstract

Discussion

Cite This Study

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

Figures

Key Points

Study Design

Structured PICO

Main Result

Limitations

Implication

Abstract

Discussion

Cite This Study

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