From the Guidelines
There is no typical or normal profile for a person with cardiac sarcoidosis as this condition presents with significant variability. Cardiac sarcoidosis affects individuals of all ages, though it most commonly appears in adults between 30-50 years old, with a slightly higher prevalence in women and certain ethnic groups, particularly African Americans and Northern Europeans 1. Patients may be completely asymptomatic with the condition discovered incidentally, or they may present with serious manifestations like heart failure, arrhythmias (particularly heart blocks or ventricular tachycardia), sudden cardiac death, or chest pain. Common symptoms include palpitations, syncope (fainting), dyspnea (shortness of breath), and fatigue. Many patients with cardiac sarcoidosis also have extracardiac involvement, particularly in the lungs, lymph nodes, skin, or eyes.
Key Characteristics and Diagnosis
The disease course is unpredictable, ranging from benign self-limiting inflammation to progressive heart failure requiring transplantation. Diagnosis typically requires advanced cardiac imaging such as cardiac MRI or PET scans, as conventional tests may appear normal in early disease 1. Cardiac MRI with late gadolinium enhancement has emerged as a valuable imaging tool for the diagnosis of cardiac involvement in sarcoidosis, suggesting the presence of inflammation and edema, and late enhancement suggests fibrotic changes and scarring 1.
Treatment Approach
Treatment generally involves immunosuppression with corticosteroids as first-line therapy, often supplemented with steroid-sparing agents like methotrexate or mycophenolate mofetil for long-term management, along with cardiac-specific treatments for arrhythmias or heart failure when present 1. Corticosteroids are recommended to treat patients with cardiac sarcoidosis, with the goal of reducing inflammation and preventing further cardiac damage 1. The use of immunosuppressive therapies such as methotrexate, azathioprine, cyclophosphamide, pentoxifylline, and thalidomide may also be considered in patients who cannot tolerate corticosteroids or who continue to worsen clinically despite treatment with corticosteroids 1.
Prognosis and Management
The prognosis of patients with symptomatic cardiac sarcoidosis has not been well characterized, but survival has been reported to be 89% for patients with an EF ≥50% 1. Important predictors of mortality include LV end-diastolic diameter, NHYA functional class, and sustained ventricular tachycardia 1. ICD implantation is reasonable for patients with cardiac sarcoidosis, particularly those with a history of ventricular arrhythmias or significant left ventricular dysfunction 1. Electrophysiological study has been proposed as a potential tool for risk stratification of VA and SCD in patients with cardiac sarcoidosis, but its role is still being defined 1.
Summary of Recommendations
- An echocardiogram should be performed in patients with signs and symptoms of HF to assess LVEF 1.
- Cardiac MRI or positron emission tomography with fluorodeoxyglucose imaging can be useful to diagnose cardiac sarcoidosis or follow response to therapy 1.
- Corticosteroids are recommended to treat patients with cardiac sarcoidosis 1.
- Standard guideline-directed medical therapy for HF is recommended to treat HF with reduced EF in patients with cardiac sarcoidosis 1.
- ICD implantation is reasonable for patients with cardiac sarcoidosis 1.
From the FDA Drug Label
Symptomatic sarcoidosis The FDA drug label does not answer the question.
From the Research
Normal Profile for Cardiac Sarcoidosis
A normal profile for a person with cardiac sarcoidosis is not well-defined, as the disease can present with a range of symptoms and severity. However, some common characteristics of cardiac sarcoidosis include:
- Conduction abnormalities, such as atrioventricular block 2, 3, 4
- Ventricular arrhythmias, such as ventricular tachycardia 2, 3, 4
- Heart failure, with reduced left ventricular ejection fraction 2, 3, 4
- Normal cardiac function, with left ventricular ejection fraction > 50% 2
Diagnostic Criteria
Diagnosis of cardiac sarcoidosis is challenging and requires a combination of clinical manifestations, extracardiac histology, and cardiac imaging findings 3, 5. A definitive diagnosis requires myocardial biopsy and histopathology, but a sufficient diagnostic likelihood can be achieved through a combination of these factors.
Treatment and Management
Treatment of cardiac sarcoidosis typically involves corticosteroid-based immunosuppression to control myocardial inflammation, as well as medical and device-based therapy for symptomatic atrioventricular block, ventricular tachyarrhythmias, and heart failure 2, 3, 4, 5. The use of implantable cardioverter defibrillators is also recommended in most patients with clinically manifest cardiac sarcoidosis 4.
Prognosis and Outcome
The prognosis for cardiac sarcoidosis is variable, with 5-year survival rates ranging from 90% to 96% and 10-year survival rates ranging from 80% to 90% 3. Early recognition and treatment are key to reducing the risk of fatal arrhythmias and heart failure 6.