Cardiac sarcoidosis: the cardiomyopathy of a thousand faces-a narrative review
George Michas1, Efstathia Prappa1, Ioannis Liatakis1
1Department of Cardiology, Evangelismos General Hospital of Athens, Athens, Greece.
Insights
Cardiac sarcoidosis (CS) diagnosis is evolving, with advanced imaging now central. Multidisciplinary care and immunomodulation improve outcomes, but further research is needed for optimal management.
Area of Science:
- Cardiology
- Immunology
- Medical Imaging
Background:
- Cardiac sarcoidosis (CS) is a challenging inflammatory cardiomyopathy due to granulomatous inflammation.
- Diagnosis is complicated by variable presentations and lack of definitive tests, leading to significant morbidity and mortality.
- CS contributes to conduction abnormalities, arrhythmias, heart failure (HF), and sudden cardiac death (SCD).
Purpose of the Study:
- To synthesize current knowledge on the epidemiology, pathophysiology, diagnosis, and management of CS.
- To highlight recent advancements in diagnostic and therapeutic strategies for CS.
Main Methods:
- A non-systematic literature search was conducted across major databases (PubMed/MEDLINE, Embase, Google Scholar).
- Included English-language articles published from January 2010 to May 2025, encompassing original research, reviews, meta-analyses, and guidelines.
Main Results:
- Advanced cardiac imaging, including cardiac magnetic resonance (CMR) and FDG-PET, is now pivotal in CS diagnosis.
- Extracardiac biopsy is preferred for histological confirmation, with cardiac involvement assessed via clinical and imaging criteria.
- Immunomodulation (corticosteroids) remains primary, supplemented by steroid-sparing agents and biologics; device therapy and guideline-directed HF management are crucial.
Conclusions:
- International consensus stresses advanced imaging and multidisciplinary teams for CS diagnosis and management.
- While current therapies improve outcomes, gaps remain in optimal treatment strategies.
- Future research should focus on refining diagnostic algorithms, identifying biomarkers, and developing novel therapeutics for CS.
Introduction:
Cardiac sarcoidosis (CS) is a complex inflammatory cardiomyopathy resulting from myocardial granulomatous inflammation. Its variable clinical presentation and lack of a single definitive test make diagnosis challenging. CS causes significant morbidity and mortality through conduction abnormalities, ventricular arrhythmias, heart failure (HF), and sudden cardiac death (SCD). This review synthesizes current knowledge on CS epidemiology, pathophysiology, diagnosis, and management.
Materials And Methods:
A comprehensive, non-systematic literature search was performed using PubMed/MEDLINE, Embase, and Google Scholar for English-language articles focusing on publications from January 2010 to May 2025. Original research, systematic reviews, meta-analyses, consensus statements, and guidelines were included.
Results:
Recent evidence indicates a paradigm shift in CS diagnosis, with advanced cardiac imaging gaining central place in international algorithms. Cardiac magnetic resonance (CMR), 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), and hybrid CMR/FDG-PET have revolutionized CS diagnosis and management. Histological confirmation of sarcoidosis, when pursued, is obtained via extracardiac biopsy; cardiac involvement is then established using clinical and imaging criteria. Endomyocardial biopsy is reserved for select cases. Immunomodulation, primarily corticosteroids, remains the therapeutic cornerstone, with adjunctive steroid-sparing agents and biologics. Implantable cardioverter-defibrillators are indicated in high-risk patients to prevent ventricular arrhythmias and SCD. HF management follows guideline-directed therapy, reserving advanced therapies for CS-related advanced HF.
Conclusion:
CS is "the cardiomyopathy of a thousand faces". International consensus emphasizes advanced imaging and multidisciplinary approaches for timely diagnosis and management. While immunomodulation and device therapy have improved outcomes, knowledge gaps persist regarding optimal treatment strategies. Future research should refine diagnostic algorithms, identify CS-specific biomarkers, and develop optimal and novel therapeutics to improve CS management.
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