Long-Term Hemodynamic Stabilization With TTR-Stabilizing Therapy and Pressure-Guided Management in ATTR Cardiomyopathy

Abstract

Background: Transthyretin amyloidosis with cardiomyopathy (ATTR-CM) is underdiagnosed in heart failure with preserved ejection fraction. Although disease-modifying therapies slow the disease progression, their long-term hemodynamic effects remain unclear.

Case summary: An 82-year-old woman with recently confirmed wild-type ATTR-CM and NYHA functional class III heart failure with preserved ejection fraction underwent pulmonary artery pressure (PAP) sensor implantation for recurrent heart failure hospitalizations shortly after the initiation of transthyretin-stabilizing therapy in 2022. During 3.5 years of follow-up, diastolic PAP decreased from 21 to 7 mm Hg, accompanied by torasemide reduction from 60 to 10 mg/day.