RV-PA uncoupling is associated with increased mortality in transthyretin amyloid cardiomyopathy treated with tafamidis.

Background: The 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.

Objectives: While RV-PAc is a validated prognostic parameter in pulmonary arterial hypertension (PAH), its prognostic relevance in ATTR-CM remains unknown.