ArticleClinical research in cardiology : official journal of the German Cardiac Society2024
RV-PA uncoupling is associated with increased mortality in transthyretin amyloid cardiomyopathy treated with tafamidis.
Article in Clinical research in cardiology : official journal of the German Cardiac Society, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed.
- The pathophysiology of wild-type transthyretin amyloidosis: implications for exercise intolerance.American journal of physiology. Heart and circulatory physiology · 2026Review
- Left ventriculo-arterial coupling in a contemporary cohort of patients with wild-type transthyretin cardiac amyloidosis treated with tafamidis.Clinical research in cardiology : official journal of the German Cardiac Society · 2026Article
- Distribution and prognostic implications of right and left ventricular systolic dysfunction in wild-type transthyretin amyloid cardiomyopathy.The international journal of cardiovascular imaging · 2026Article
- Right Ventricular-Pulmonary Artery Coupling as a Prognostic Marker in Cardiac Amyloidosis: A Comprehensive Review.Life (Basel, Switzerland) · 2026Review
- The left ventricular mechanical dispersion as a marker of disease severity in transthyretin cardiac amyloidosis.Scientific reports · 2025Observational
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Authors and funding
11 authors.
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Abstract
backgroundThe right ventricle to pulmonary artery coupling (RV-PAc) describes the right ventricle's ability to adjust to an increased afterload. In case of transthyretin amyloid cardiomyopathy (ATTR-CM), impaired RV-PAc can result from increased left ventricular diastolic stiffness due to fibril deposition.
objectivesWhile RV-PAc is a validated prognostic parameter in pulmonary arterial hypertension (PAH), its prognostic relevance in ATTR-CM remains unknown. We sought to evaluate the prognostic implications of impaired RV-PAc on survival in ATTR-CM.
methodsIn this multicentre study, RV-PAc was investigated by the echocardiographic surrogate of ratio of tricuspid annular plane systolic excursion to estimated pulmonary arterial systolic pressure (TAPSE/PASP) in 418 ATTR-CM patients, all treated with a TTR stabilizer. The primary outcome was all-cause mortality.
resultsWithin a median time of 1.52 [IQR 0.72-2.56] years after diagnosis, 49 (11.7%) patients died. In multivariate Cox regression analysis impaired RV-PAc was a strong independent predictor of mortality (Hazard Ratio (HR) 2.16, 95% Confidence Interval (CI) 1.14-4.07, p = 0.018). RV-PAc ratio at first presentation emerged as a robust marker for risk stratification with a determined optimal cut-off of 0.382 mm/mmHg (area under the curve (AUC) 0.73, 95% CI 0.65-0.81). Patients with RV-PAc ratio ≤ 0.382 mm/mmHg exhibited significantly lower survival (HR 4.17, 95% CI 2.21-7.87, p < 0.001) within 3 years of follow up than those with RV-PAc ratio above the cut-off (Graphical Abstract).
conclusionImpaired adaptation of the RV to increased afterload is associated with worse outcome in ATTR-CM patients. RV-PAc ratio can serve as an echocardiographic predictor for all-cause mortality. Therefore, the determination of RV-PAc could improve risk stratification for ATTR-CM patients.
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