Review ArticleCardiac sarcoidosis
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
References (128)
- et al.
Sarcoidosis of the heart: a clinicopathologic study of 35 necropsy patients and review of 78 previously described necropsy patients
Am J Med
(1977) - et al.
Sarcoidosis
Lancet
(2003) - et al.
HLA-DRB1⁎1101: a significant risk factor for sarcoidosis in blacks and whites
Am J Hum Genet
(2003) - et al.
The BTNL2 gene and sarcoidosis susceptibility in African Americans and Whites
Am J Hum Genet
(2005) Giant cell and granulomatous myocarditis
Heart Fail Clin
(2005)- et al.
Right ventricle-dominant cardiac sarcoidosis with sparing of the left ventricle
J Heart Lung Transplant
(2006) - et al.
The intricacies of cardiac sarcoidosis: a case report involving the coronary arteries and a review of the literature
Cardiovasc Pathol
(2006) - et al.
Cardiac involvement in patients with pulmonary sarcoidosis assessed at two university medical centers in the Netherlands
Chest
(2005) - et al.
Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone
Am J Cardiol
(2001) - et al.
Sarcoidosis of the aortic root
J Thorac Cardiovasc Surg
(2004)
Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis
Chest
Bi-fascicular block on EKG as the initial presenting sign of cardiac sarcoidosis
Int J Cardiol
Sustained ventricular tachycardia associated with sarcoidosis: assessment of the underlying cardiac anatomy and the prospective utility of programmed ventricular stimulation, drug therapy and an implantable antitachycardia device
J Am Coll Cardiol
Usefulness of programmed ventricular stimulation in predicting future arrhythmic events in patients with cardiac sarcoidosis
Am J Cardiol
Pericardial effusions in sarcoidosis
Chest
Sarcoidosis of the heart
Am Heart J
A proposed solution to the clinical assessment of sarcoidosis: the sarcoidosis three-dimensional assessment instrument (STAI)
Med Hypotheses
Utility of endomyocardial biopsy in the diagnosis of cardiac sarcoidosis
Chest
A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy
Am Heart J
Histologic diagnostic rate of cardiac sarcoidosis: evaluation of endomyocardial biopsies
Am Heart J
The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology
J Am Coll Cardiol
Cardiac sarcoidosis: there is no instant replay
Chest
Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing
Chest
Thallium-scan myocardial defects and echocardiographic abnormalities in patients with sarcoidosis without clinical cardiac dysfunction
Am J Med
Echocardiographic evaluation of patients with systemic sarcoidosis
Am Heart J
Two-dimensional echocardiographic findings in systemic sarcoidosis
Am J Cardiol
Subclinical cardiac dysfunction in sarcoidosis
Chest
Doppler echocardiographic detection of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis
Chest
Transesophageal echocardiographic abnormalities in a case of cardiac sarcoidosis
J Am Soc Echocardiogr
The use of 201thallium for myocardial perfusion imaging in sarcoid heart disease
Chest
Reversibility by dipyridamole of thallium-201 myocardial scan defects in patients with sarcoidosis
Am J Med
The role of iodine-123–labeled 15-(p-iodophenyl)-3R, S-methylpentadecanoic acid scintigraphy in the detection of local myocardial involvement of sarcoidosis
Int J Cardiol
Thallium-scan myocardial defects and echocardiographic abnormalities in patients with sarcoidosis without clinical cardiac dysfunction. An analysis of 44 patients
Am J Med
Diagnostic and prognostic value of myocardial scintigraphy with thallium-201 and gallium-67 in cardiac sarcoidosis
Chest
Usefulness of dual 67Ga and 99mTc-sestamibi single-photon-emission CT scanning in the diagnosis of cardiac sarcoidosis
Chest
Regional myocardial blood flow reserve impairment and metabolic changes suggesting myocardial ischemia in patients with idiopathic dilated cardiomyopathy
J Am Coll Cardiol
MR findings of myocardial sarcoidosis
Clin Radiol
Clinical significance of gadolinium-DTPA-enhanced MRI for detection of myocardial lesions in a patient with sarcoidosis
Clin Radiol
Diagnosis of cardiac sarcoidosis and evaluation of the effects of steroid therapy by gadolinium-DTPA-enhanced magnetic resonance imaging
Am J Med
Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis
J Am Coll Cardiol
Clinical significance of myocardial magnetic resonance abnormalities in patients with sarcoidosis. A 1-year follow-up study
Chest
Sarcoidosis. Epidemiology and prognosis. A 15-year European study
Am Rev Respir Dis
Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization
Am J Epidemiol
Epidemiology of sarcoidosis
Epidemiol Rev
Advances in the genetics of sarcoid
Clin Genet
HLA and sarcoidosis: new pathogenic insights
Sarcoidosis Vasc Diff Lung Dis
Increased frequency of the uncommon tumor necrosis factor -857T allele in British and Dutch patients with sarcoidosis
Am Rev Respir Crit Care Med
Association of single nucleotide polymorphisms in the IL-18 gene with sarcoidosis in a Japanese population
J Rheumatol
Sarcoidosis is associated with a truncating splice site mutation in BTNL2
Nat Genet
Racial difference in cardiac sarcoidosis incidence observed at autopsy
Sarcoidosis
Cited by (302)
The role of ICDs in patients with sarcoidosis–A comprehensive review
2024, Current Problems in CardiologyPredictors of Mortality in Sarcoidosis
2024, Clinics in Chest MedicinePrevalence of Cardiac Sarcoidosis in Middle-Aged Adults Diagnosed with High-Grade Atrioventricular Block
2024, American Journal of MedicineImpact of comorbid heart failure among hospitalized patients with sarcoidosis: A United States population-based cohort study
2023, IJC Heart and VasculatureRecurrent Cardiac Sarcoidosis and Giant Cell Myocarditis After Heart Transplant: A Case Report and Systematic Literature Review
2023, American Journal of CardiologyClinical Response to Tumor Necrosis Factor-α Inhibitor Therapy in the Management of Cardiac Sarcoidosis
2023, American Journal of Cardiology