Right Ventricular Function, Clinical Outcomes, and Effect of Vutrisiran in Transthyretin Amyloidosis With Cardiomyopathy: Insights from the HELIOS-B Trial

Highlights

• Right ventricular dysfunction in transthyretin amyloidosis cardiomyopathy (ATTR-CM) is highly prevalent and better identified by RV free wall strain (RVFWS) compared to conventional echocardiographic measures.
• RVFWS and the ratio of RVFWS to pulmonary artery systolic pressure (RVFWS/PASP) independently predict all-cause mortality and recurrent cardiovascular events, beyond established risk factors and left ventricular function.
• Vutrisiran, an RNA interference therapeutic, stabilizes RV function measured by RVFWS and improves RVFWS/PASP over 30 months, reflecting beneficial cardiac remodeling in ATTR-CM.
• Conventional RV functional indices such as RV S’ and RV fractional area change (FAC) underestimate RV dysfunction and lack robust prognostic value in ATTR-CM.