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Sarcoidosis-Related Cardiomyopathy: Current Knowledge, Challenges, and Future Perspectives State-of-the-Art Review
Nisha A Gilotra1, Jan M Griffin2, Noelle Pavlovic3
1Advanced Heart Failure/Transplant Cardiology Section, Division of Cardiology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Insights
Cardiac sarcoidosis (CS) is an increasing cause of heart disease, presenting diagnostic challenges. Early recognition and multidisciplinary care are crucial for managing this complex condition.
Area of Science:
- Cardiology
- Immunology
- Pathology
Background:
- Sarcoidosis-related cardiomyopathy (CS) prevalence is rising, affecting cardiac function via granulomatous infiltration.
- CS can manifest as conduction disease, arrhythmia, or heart failure, mimicking other cardiomyopathies.
- Accurate diagnosis of CS is challenging, requiring awareness and differentiation from similar conditions.
Purpose of the Study:
- To highlight the diagnostic challenges of cardiac sarcoidosis.
- To underscore the importance of an interdisciplinary approach in CS patient care and research.
- To emphasize current therapeutic strategies and knowledge gaps in CS management.
Main Methods:
- Review of clinical manifestations, extracardiac involvement, histopathology, and advanced cardiac imaging for CS diagnosis.
- Discussion of current therapeutic mainstays, primarily immunosuppression.
- Consideration of advanced therapies like ventricular assist devices and heart transplantation for end-stage disease.
Main Results:
- Diagnosis of CS requires high clinical suspicion and exclusion of other cardiac conditions.
- Immunosuppression is the primary treatment, though evidence from prospective trials is lacking.
- Disease progression can lead to advanced heart failure and ventricular arrhythmias.
Conclusions:
- Cardiac sarcoidosis presents diagnostic complexities requiring careful evaluation.
- Interdisciplinary collaboration is essential for improving patient outcomes and advancing research in CS.
- Further investigation is needed to refine understanding and therapeutic strategies for CS.
Abstract:
The prevalence of sarcoidosis-related cardiomyopathy is increasing. Sarcoidosis impacts cardiac function through granulomatous infiltration of the heart, resulting in conduction disease, arrhythmia, and/or heart failure. The diagnosis of cardiac sarcoidosis (CS) can be challenging and requires clinician awareness as well as differentiation from overlapping diagnostic phenotypes, such as other forms of myocarditis and arrhythmogenic cardiomyopathy. Clinical manifestations, extracardiac involvement, histopathology, and advanced cardiac imaging can all lend support to a diagnosis of CS. The mainstay of therapy for CS is immunosuppression; however, no prospective clinical trials exist to guide management. Patients may progress to developing advanced heart failure or ventricular arrhythmia, for which ventricular assist device therapies or heart transplantation may be considered. The existing knowledge gaps in CS call for an interdisciplinary approach to both patient care and future investigation to improve mechanistic understanding and therapeutic strategies.
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