Review Article
Cardiac sarcoidosis

https://doi.org/10.1016/j.ahj.2008.09.009Get rights and content

Cardiac sarcoidosis (CS) is a rare but potentially fatal condition that may present with a wide range of clinical manifestations including congestive heart failure, conduction abnormalities, and most notably, sudden death. Recent advances in imaging technology allow easier detection of CS, but the diagnostic guidelines with inclusion of these techniques have yet to be written. It has become clear that minimally symptomatic or asymptomatic cardiac involvement is far more prevalent than previously thought. Because of the potential life-threatening complications and potential benefit of treatment, all patients diagnosed with sarcoidosis should be screened for cardiac involvement. Patients with CS and symptoms such as syncope need an aggressive workup for a potentially life-threatening etiology, and often require implantable cardioverter-defibrillator therapy. CS patients without arrhythmic symptoms are still at risk for sudden death and may warrant an implantable cardioverter-defibrillator for primary prevention reasons. Athough corticosteroids are regarded as the first-line drug of choice, therapy for CS is not yet standardized, and it is unclear at this point whether asymptomatic patients require therapy. Randomized clinical trials are clearly warranted to answer these very important patient care questions, and are endorsed fully by the authors.

Section snippets

Clinical expression

Patients with CS may present with a wide variety of signs and symptoms, ranging from asymptomatic electrocardiographic (ECG) abnormalities to sudden death. Initial presenting symptoms depend on the underlying pathology, and may include dyspnea, palpitations, syncope, dizziness, chest pain, orthopnea, or peripheral edema. The clinical manifestations of CS are related to the location and extent of granulomatous involvement, which likely depend on several factors including ethnicity, sex, and

Diagnosis

Because of the potential life-threatening complications and potential benefit of treatment, all patients diagnosed with sarcoidosis should be screened for cardiac involvement (Figure 1). Initial screening usually includes a detailed medical history and physical exam, a surface ECG and a transthoracic echocardiogram. A positive history or significant ECG abnormality should prompt further evaluation, usually with Holter monitoring and additional cardiac imaging (discussed below).31 Patients with

Imaging in CS

Assessment of various imaging modalities for detection of CS remains challenging for several reasons. Most reports involve a small number of patients or are based on case reports. In addition, there appear to be differences in the incidence of CS in certain populations (eg, patients in Japan vs the United States) that may affect the reported accuracy of imaging studies. Finally, and perhaps most importantly, although endomyocardial biopsy is the gold standard for the presence of CS, the test is

Advances in echocardiography

Hyodo et al65 reported a significant improvement in sensitivity (over 2-dimensional echocardiography) for detecting CS using a technique of ultrasonic tissue characterization with integrated backscatter in a series of 22 patients with extracardiac sarcoidosis and normal echocardiograms (8 of whom were diagnosed with CS by nuclear studies). Two patients with arrhythmias were excluded from the study. Further investigation will be necessary to confirm the usefulness of this technique. Other

Prognosis and management

The data on prognosis in CS are quite variable, with 5-year survival ranging from 60% to 90% in patients with treatment and preserved systolic function, to 10% in autopsy subjects.27, 32 Important predictors of mortality include New York Heart Association functional class, left ventricular end-diastolic diameter, and sustained ventricular tachycardia.32 The question of whether prognosis is improved with treatment is discussed below.

Conclusions and future directions

Cardiac sarcoidosis is a rare but potentially fatal condition that can mimic more common conditions. It is therefore important to search for its presence in all patients diagnosed with sarcoidosis. With recent advances in imaging technology, CS can be detected more easily. It has become clear that minimally symptomatic or asymptomatic cardiac involvement is far more prevalent than previously thought. It remains unclear whether asymptomatic patients who only have disease detected by these

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