Clinical manifestations and diagnosis of cardiac sarcoidosis
- Ron Blankstein, MD
Ron Blankstein, MD
- Associate Professor of Medicine and Radiology
- Harvard Medical School
- Garrick C. Stewart, MD MPH
Garrick C. Stewart, MD MPH
- Associate Physician, Division of Cardiovascular Medicine
- Brigham and Women’s Hospital
- Section Editors
- Hugh Calkins, MD
Hugh Calkins, MD
- Section Editor — Cardiac Arrhythmias
- Professor of Medicine
- Johns Hopkins Medical Institutions
- William J McKenna, MD
William J McKenna, MD
- Section Editor — Myopericardial Disease
- Professor of Cardiology
- University College, London
The clinical presentation of cardiac sarcoidosis (CS) ranges from an incidentally discovered condition to heart failure and sudden death.
The diagnosis of CS is difficult to establish, and as a result, CS is often under-recognized in clinical practice . CS most often occurs as a manifestation of systemic sarcoidosis, although isolated CS can occur in patients who do not have evidence of sarcoidosis in other organs . Yet, commonly used clinical criteria require the diagnosis of extracardiac disease in order to establish the diagnosis of CS in the absence of having a positive endomyocardial biopsy. While endomyocardial biopsy provides a high specificity for diagnosing CS, this invasive test has a limited sensitivity. Furthermore, there is limited understanding of disease progression and a lack of consensus on the optimal methods for disease detection.
This topic will discuss the clinical manifestations of CS and provide an overview of how to evaluate patients with suspected CS. Management and prognosis of CS and diagnosis and management of systemic sarcoidosis are discussed separately. (See "Management and prognosis of cardiac sarcoidosis" and "Clinical manifestations and diagnosis of pulmonary sarcoidosis" and "Extrapulmonary manifestations of sarcoidosis" and "Pathology and pathogenesis of sarcoidosis" and "Treatment of pulmonary sarcoidosis: Initial therapy with glucocorticoids" and "Treatment of pulmonary sarcoidosis: Disease refractory to glucocorticoid therapy".)
The true prevalence of CS remains unknown and is potentially underestimated since many individuals with CS may have nonspecific symptoms or subclinical disease. CS can affect patients of all racial backgrounds and ages, with an average age at presentation of approximately 50 years old .
The prevalence of CS among patients with systemic sarcoidosis has been reported to be 20 to 27 percent in the United States and as high as 58 percent in Japan [4-6]. Yet, autopsy studies indicate that subclinical cardiac involvement may be present in up to 70 percent of cases. In the United States, 13 to 25 percent of deaths from sarcoidosis have been attributed to CS, while in Japan, 47 to 85 percent of deaths from sarcoidosis have been attributed to cardiac involvement .To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- CLINICAL MANIFESTATIONS
- Symptoms and signs
- Key manifestations
- - Arrhythmias
- Conduction system disease
- - Sudden cardiac death
- - Cardiomyopathy and heart failure
- - Coronary artery disease
- Test findings
- When to consider cardiac sarcoidosis
- Approach to diagnosis
- Initial evaluation and testing
- Criteria for advanced cardiac imaging
- - With extracardiac sarcoidosis
- - Without extracardiac sarcoidosis
- Patients without criteria for advanced cardiac imaging
- Selection of advanced imaging
- Role of biopsy
- How to diagnose cardiac sarcoidosis
- - Definite cardiac sarcoidosis
- - Uncertain diagnosis
- Clinical findings suggesting cardiac sarcoidosis
- Probable cardiac sarcoidosis
- - With histologic confirmation of extracardiac sarcoidosis
- - Without histologic confirmation of extracardiac disease
- Unresolved cases
- Comparison of major society guidelines
- Key diagnostic tests
- - Holter monitoring
- - Echocardiography
- Echocardiographic findings
- Limited sensitivity of echocardiography
- - Cardiovascular magnetic resonance
- CMR findings
- Diagnostic accuracy
- Limitations of CMR
- - FDG-PET
- - Comparison of CMR with FDG-PET
- - Endomyocardial biopsy
- Additional tests
- - Coronary angiography
- - Electrophysiologic study
- - Other tests
- DIFFERENTIAL DIAGNOSIS
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS