Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association

Area of Science:

• Cardiology
• Immunology
• Internal Medicine

Background:

• Cardiac sarcoidosis is an infiltrative cardiomyopathy caused by myocardial granulomatous inflammation.
• It presents with conduction disease, arrhythmias, and ventricular dysfunction, with increasing prevalence.
• Diagnostic uncertainty is common due to lack of definitive histological confirmation.

Purpose of the Study:

• To provide a framework for the diagnosis and management of cardiac sarcoidosis.
• To guide clinical practice and promote management conformity.
• To address the challenges posed by diagnostic uncertainty and lack of randomized trials.

Main Methods:

• Review of existing cohort studies and consensus opinions.
• Development of a diagnostic continuum for likelihood assessment.
• Proposal of a tiered treatment approach, starting with corticosteroids.

Main Results:

• Cardiac sarcoidosis diagnosis should be viewed as a continuum of probability, not binary.
• Treatment initiation is recommended for clinical manifestations with active inflammation.
• Corticosteroids are the first-line treatment, with management varying across centers.

Conclusions:

• A standardized framework is needed for diagnosing and managing cardiac sarcoidosis.
• Clinical judgment and a probabilistic approach are essential for diagnosis.
• Tiered, evidence-based management, starting with corticosteroids, is recommended.