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Long-Term Hemodynamic Stabilization With TTR-Stabilizing Therapy and Pressure-Guided Management in ATTR
Jamschid Sedighi1, Bernhard Unsöld1, Priyanka Boettger1
1Department of Cardiology and Angiology, Justus Liebig University Giessen, Giessen, Germany.
Insights
Transthyretin amyloidosis with cardiomyopathy (ATTR-CM) treatment with stabilizing therapy and pulmonary artery pressure (PAP) monitoring improved heart function. This case suggests PAP-guided management may aid ATTR-CM patients on disease-modifying drugs.
Area of Science:
- Cardiology
- Pharmacology
- Medical Devices
Background:
- Transthyretin amyloidosis with cardiomyopathy (ATTR-CM) is often underdiagnosed in heart failure with preserved ejection fraction (HFpEF).
- Disease-modifying therapies for ATTR-CM can slow progression, but their long-term impact on hemodynamics is not fully understood.
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.
Discussion:
In this case, the combination of transthyretin-stabilizing therapy and PAP-guided follow-up was associated with sustained hemodynamic improvement and clinical stability. However, the independent contribution of pulmonary artery monitoring cannot be determined from a single uncontrolled observation.
Take-Home Message:
Remote PAP-guided management may support disease-modifying treatment in selected patients with ATTR-CM.
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