Do most patients with sarcoidosis who experience sudden death exhibit symptoms prior to the event?

From the Guidelines

Most patients with sarcoidosis who experience sudden death do not exhibit symptoms prior to the event. Sudden cardiac death in sarcoidosis patients often occurs without warning signs, which makes this complication particularly dangerous. Cardiac sarcoidosis can cause fatal arrhythmias or conduction abnormalities that lead to sudden death even in patients who appear clinically stable. According to the most recent guideline, 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 1, patients with cardiac sarcoidosis are at high risk of sudden cardiac death, and the guideline provides recommendations for the prevention of sudden cardiac death in these patients.

The silent progression of cardiac sarcoidosis occurs because granulomatous inflammation can affect the myocardium and conduction system without causing noticeable symptoms until a critical threshold is reached. The lack of preceding symptoms highlights the importance of cardiac screening in all sarcoidosis patients, even those without cardiac complaints. Screening typically includes electrocardiogram, echocardiography, and in some cases cardiac MRI or PET imaging to detect early cardiac involvement before life-threatening complications develop.

Some key points to consider in the management of cardiac sarcoidosis include:

• The use of immunosuppressive therapy to prevent progression of the cardiac involvement
• The use of antiarrhythmic medications to prevent life-threatening ventricular arrhythmias
• The use of implantable cardioverter-defibrillators (ICDs) in patients with cardiac sarcoidosis who are at high risk of sudden cardiac death, as recommended by the 2017 AHA/ACC/HRS guideline 1
• The importance of considering symptoms such as syncope, heart failure status, LV function, and spontaneous or induced ventricular arrhythmias in making management decisions regarding ICD therapy, as noted in the 2012 ACCF/AHA/HRS focused update 1.

Overall, the management of cardiac sarcoidosis requires a comprehensive approach that includes cardiac screening, immunosuppressive therapy, antiarrhythmic medications, and ICD therapy, as appropriate, to prevent sudden cardiac death and improve outcomes in patients with this condition.

From the Research

Sudden Death in Sarcoidosis

• Sudden death is a potential complication of cardiac sarcoidosis, and it may occur without prior symptoms 2, 3, 4.
• Cardiac sarcoidosis can present with a wide range of clinical manifestations, including congestive heart failure, conduction abnormalities, and sudden death 2.
• Minimally symptomatic or asymptomatic cardiac involvement is more prevalent than previously thought, and patients with cardiac sarcoidosis may be at risk for sudden death even without arrhythmic symptoms 2, 3.

Symptoms Prior to Sudden Death

• Some patients with cardiac sarcoidosis may exhibit symptoms such as syncope, ventricular arrhythmias, or conduction abnormalities before sudden death 2, 4.
• However, sudden death may be the presenting feature of cardiac sarcoidosis, and it is not always preceded by symptoms 3, 4.
• The extent of left ventricular dysfunction and the extent of myocardial late gadolinium enhancement are important predictors of prognosis among patients with clinically manifest cardiac sarcoidosis 5.

Diagnosis and Management

• Diagnosis of cardiac sarcoidosis is difficult, and it often requires a combination of imaging modalities, including radionuclide scans, 18F-fluorodeoxyglucose positron emission tomography/computed tomography, and gadolinium-enhanced magnetic resonance imaging scans 3, 6.
• Treatment of cardiac sarcoidosis typically involves immunosuppressive therapy, such as corticosteroids, and device therapy, including implantable cardioverter-defibrillators 3, 6, 5.