Transthyretin Cardiac Amyloidosis Evaluation and Management: 2025 ACC Concise Clinical Guidance

Area of Science:

• Cardiology
• Genetics
• Pharmacology

Background:

• Transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure, particularly in elderly individuals.
• ATTR-CM diagnosis is often delayed, but awareness is growing, especially in patients with musculoskeletal conditions like carpal tunnel syndrome or spinal stenosis.
• Noninvasive diagnosis via radionuclide scintigraphy is now accurate, even with a negative monoclonal protein screen.

Purpose of the Study:

• To provide updated clinical guidance for diagnosing and managing ATTR-CM.
• To address the expanding therapeutic landscape, including disease-modifying and heart failure therapies.
• To reinforce best practices and discuss future directions in ATTR-CM care.

Main Methods:

• This guidance synthesizes current evidence and expert opinion on ATTR-CM diagnosis and management.
• It reviews advances in diagnostic imaging, specifically radionuclide scintigraphy.
• It evaluates the efficacy and application of emerging therapies, including transthyretin stabilizers (tafamidis, acoramidis) and silencers (vutrisiran), alongside standard heart failure treatments.

Main Results:

• ATTR-CM is a significant contributor to heart failure, often presenting with subtle or confounding symptoms.
• Accurate noninvasive diagnosis is achievable, facilitating earlier intervention.
• A growing number of effective therapies, including disease-specific agents and advanced heart failure medications, are available.

Conclusions:

• Clinicians should maintain a high index of suspicion for ATTR-CM, particularly in older patients with heart failure and suggestive comorbidities.
• Early and accurate diagnosis is crucial for initiating timely and appropriate treatment.
• The evolving therapeutic options offer improved management strategies and outcomes for patients with ATTR-CM.