Table of ContentsView AllTable of ContentsSarcoidosisCardiac SarcoidosisSymptomsDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
Sarcoidosis
Cardiac Sarcoidosis
Symptoms
Diagnosis
Treatment
Sarcoidosisis an autoimmune disease that producesgranulomas—clumps of abnormal cells similar to scar tissue—in various organs, including the heart. When granulomas develop in the heart, they are known as cardiac sarcoidosis.
While cardiac sarcoidosis may be a relatively benign condition, sometimes it can cause severe clinical problems, includingheart failureor sudden death. Diagnosing cardiac sarcoidosis and instituting appropriate treatment is critically important in these cases.
This article explores the symptoms, causes, and treatment of cardiac sarcoidosis and its impact on life expectancy.
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What Is Sarcoidosis?
Researchers believe that sarcoidosis develops when the body’s immune system responds abnormally to some unknown substance (probably something that is inhaled), resulting in the formation of granulomas. Women are affected more frequently than men and Black people are much more likely to develop sarcoidosis than white people. A propensity for sarcoidosis can also run in families.
The symptoms of sarcoidosis are quite varied. Some people develop a flu-like condition with fatigue, fever, and weight loss. Others will have predominantly pulmonary symptoms such as cough,dyspnea(shortness of breath), or wheezing. Eye problems include redness, pain, blurred vision, and light sensitivity. Skin lesions may include a painful rash, sores on the face, changes in skin coloration, and nodules.
Any combination of these symptoms may occur. Some people with sarcoidosis develop no symptoms whatsoever, and the condition is diagnosed incidentally when a chest X-ray is done for some other reason.
What Is Cardiac Sarcoidosis?
Cardiac sarcoidosis occurs when sarcoid granulomas develop in the heart. As with other types of sarcoidosis, the manifestations of cardiac sarcoidosis vary from person to person. In some people, cardiac sarcoidosis causes no problems whatsoever; in others, it can be fatal.
The severity of cardiac sarcoidosis depends on the extent and the location of granuloma formation within the heart. About 5% of people with sarcoidosis are diagnosed with cardiac involvement during their lifetimes.
However, in autopsy studies, as many as 70% of people with sarcoidosis turn out to have at least some cardiac granulomas. This suggests that cardiac sarcoidosis usually does not produce identifiable clinical problems.
However, When sarcoidosis produces cardiac problems, those problems can be severe.
Cardiac Sarcoidosis Symptoms
Diagnosing Cardiac Sarcoidosis
Making a diagnosis of cardiac sarcoidosis is often tricky. The most definitive test is a positive cardiac biopsy.However, not only is a biopsy an invasive procedure but also (because sarcoid granulomas are randomly distributed), a biopsy may come back “normal” even when cardiac sarcoidosis is present.
First, doctors need to know when they should look for cardiac sarcoidosis. Looking for cardiac sarcoidosis is essential for people known to have sarcoidosis, which affects other body organs. Also, cardiac sarcoidosis should be thought of in any young person (under age 55) who develops heart block, ventricular arrhythmias, or heart failure for unexplained reasons.
However, acardiac MRI scanis much more likely to show evidence of sarcoid granulomas within the heart and is typically the noninvasive test of choice.PET scansof the heart are also quite sensitive for detecting cardiac sarcoid granulomas, but PET scans are less readily available than MRI scans.
In most cases, based on a combination of clinical signs and symptoms, noninvasive and invasive testing, determining the presence or absence of cardiac sarcoidosis can be accomplished with a high degree of confidence.
Cardiac Sarcoidosis Treatment
If cardiac sarcoidosis is present, treatment aims to slow or stop the progression of the sarcoidosis itself and to prevent or treat the direst cardiac consequences of this disease.
Preventing Granulomas
Therapy aimed at the sarcoidosis itself attempts to suppress the immune reaction that causes the formation of granulomas. In sarcoidosis, this is usually done with glucocorticoid (steroid) therapy, most commonly with prednisone. This often starts at about 30–40 milligrams per day. Over the first year, this dose might be gradually reduced.
In people who do not appear to respond to steroid therapy, other immunosuppressants may be necessary, such as methotrexate, mycophenolate, azathioprine, or leflunomide.
Treating Cardiac Sarcoidosis
Implantable cardioverter-defibrillators (ICDs) may be used to prevent sudden death from ventricular arrhythmias. Most experts recommend them for people with sarcoidosis who have significantly reduced left ventricular ejection fractions or who have survived episodes of sustained ventricular tachycardia or ventricular fibrillation.
In addition, performing an electrophysiology study can help in determining which people with cardiac sarcoidosis are at exceptionally high risk for sudden death from ventricular arrhythmias and, thus, who ought to have an ICD.
If heart failure occurs, people with cardiac sarcoidosis should receive the same aggressive treatment for heart failure that is used for anyone with this condition. If their heart failure becomes severe and does not respond any longer to medical therapy, they should receive the same consideration for heart transplantation given to anyone else with heart failure. Their outcomes after cardiac transplantation appear to be better than the outcomes reported for people having transplantation for other underlying causes.
Summary
Cardiac sarcoidosis is a potentially serious but also sometimes benign condition where granulomas form on heart tissue.
As is the case with sarcoidosis in general, sarcoidosis affecting the heart can have a range of manifestations, from entirely benign to lethal. If there is a reason to suspect cardiac sarcoidosis, it is essential to do the testing necessary to make or rule out the diagnosis. Early and aggressive therapy (both for the sarcoidosis and its cardiac manifestations) can significantly improve the outcome for a person with cardiac sarcoidosis.
8 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Jaiswal R, Vaisyambath L, Khayyat A, et al.Cardiac sarcoidosis diagnostic challenges and management: a case report and literature review.Cureus. 2022;14(5):e24850. doi:10.7759/cureus.24850Cheung E, Ahmad S, Aitken M, et al.Combined simultaneous FDG-PET/MRI with T1 and T2 mapping as an imaging biomarker for the diagnosis and prognosis of suspected cardiac sarcoidosis.Eur J Hybrid Imaging. 2021;5(1):24. doi:10.1186/s41824-021-00119-wLehtonen J, Uusitalo V, Pöyhönen P, Mäyränpää MI, Kupari M.Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis.Eur Heart J. 2023;44(17):1495-1510. doi:10.1093/eurheartj/ehad067Roberts WC, Becker TM, Hall SA.Usefulness of total 12-lead QRS voltage as a clue to diagnosis of patients with cardiac sarcoidosis severe enough to warrant orthotopic heart transplant.JAMA Cardiol. 2018;3(1):64-68. doi:10.1001/jamacardio.2017.4172Ekström K, Lehtonen J, Nordenswan HK, et al.Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries.Eur Heart J. 2019;40(37):3121-3128. doi:10.1093/eurheartj/ehz428Lemay S, Massot M, Philippon F, et al.Ten questions cardiologists should be able to answer about cardiac sarcoidosis: case-based approach and contemporary review.CJC Open. 2020;3(4):532-548. doi:10.1016/j.cjco.2020.11.022Cheng RK, Kittleson MM, Beavers CJ, et al.Diagnosis and management of cardiac sarcoidosis: a scientific statement from the American Heart Association.Circulation. Published online April 18, 2024. doi:10.1161/CIR.0000000000001240Nordenswan HK, Lehtonen J, Ekström K, et al.Outcome of cardiac sarcoidosis presenting with high-grade atrioventricular block.Circ Arrhythm Electrophysiol. 2018;11(8):e006145. doi:10.1161/CIRCEP.117.006145
8 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Jaiswal R, Vaisyambath L, Khayyat A, et al.Cardiac sarcoidosis diagnostic challenges and management: a case report and literature review.Cureus. 2022;14(5):e24850. doi:10.7759/cureus.24850Cheung E, Ahmad S, Aitken M, et al.Combined simultaneous FDG-PET/MRI with T1 and T2 mapping as an imaging biomarker for the diagnosis and prognosis of suspected cardiac sarcoidosis.Eur J Hybrid Imaging. 2021;5(1):24. doi:10.1186/s41824-021-00119-wLehtonen J, Uusitalo V, Pöyhönen P, Mäyränpää MI, Kupari M.Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis.Eur Heart J. 2023;44(17):1495-1510. doi:10.1093/eurheartj/ehad067Roberts WC, Becker TM, Hall SA.Usefulness of total 12-lead QRS voltage as a clue to diagnosis of patients with cardiac sarcoidosis severe enough to warrant orthotopic heart transplant.JAMA Cardiol. 2018;3(1):64-68. doi:10.1001/jamacardio.2017.4172Ekström K, Lehtonen J, Nordenswan HK, et al.Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries.Eur Heart J. 2019;40(37):3121-3128. doi:10.1093/eurheartj/ehz428Lemay S, Massot M, Philippon F, et al.Ten questions cardiologists should be able to answer about cardiac sarcoidosis: case-based approach and contemporary review.CJC Open. 2020;3(4):532-548. doi:10.1016/j.cjco.2020.11.022Cheng RK, Kittleson MM, Beavers CJ, et al.Diagnosis and management of cardiac sarcoidosis: a scientific statement from the American Heart Association.Circulation. Published online April 18, 2024. doi:10.1161/CIR.0000000000001240Nordenswan HK, Lehtonen J, Ekström K, et al.Outcome of cardiac sarcoidosis presenting with high-grade atrioventricular block.Circ Arrhythm Electrophysiol. 2018;11(8):e006145. doi:10.1161/CIRCEP.117.006145
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Jaiswal R, Vaisyambath L, Khayyat A, et al.Cardiac sarcoidosis diagnostic challenges and management: a case report and literature review.Cureus. 2022;14(5):e24850. doi:10.7759/cureus.24850Cheung E, Ahmad S, Aitken M, et al.Combined simultaneous FDG-PET/MRI with T1 and T2 mapping as an imaging biomarker for the diagnosis and prognosis of suspected cardiac sarcoidosis.Eur J Hybrid Imaging. 2021;5(1):24. doi:10.1186/s41824-021-00119-wLehtonen J, Uusitalo V, Pöyhönen P, Mäyränpää MI, Kupari M.Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis.Eur Heart J. 2023;44(17):1495-1510. doi:10.1093/eurheartj/ehad067Roberts WC, Becker TM, Hall SA.Usefulness of total 12-lead QRS voltage as a clue to diagnosis of patients with cardiac sarcoidosis severe enough to warrant orthotopic heart transplant.JAMA Cardiol. 2018;3(1):64-68. doi:10.1001/jamacardio.2017.4172Ekström K, Lehtonen J, Nordenswan HK, et al.Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries.Eur Heart J. 2019;40(37):3121-3128. doi:10.1093/eurheartj/ehz428Lemay S, Massot M, Philippon F, et al.Ten questions cardiologists should be able to answer about cardiac sarcoidosis: case-based approach and contemporary review.CJC Open. 2020;3(4):532-548. doi:10.1016/j.cjco.2020.11.022Cheng RK, Kittleson MM, Beavers CJ, et al.Diagnosis and management of cardiac sarcoidosis: a scientific statement from the American Heart Association.Circulation. Published online April 18, 2024. doi:10.1161/CIR.0000000000001240Nordenswan HK, Lehtonen J, Ekström K, et al.Outcome of cardiac sarcoidosis presenting with high-grade atrioventricular block.Circ Arrhythm Electrophysiol. 2018;11(8):e006145. doi:10.1161/CIRCEP.117.006145
Jaiswal R, Vaisyambath L, Khayyat A, et al.Cardiac sarcoidosis diagnostic challenges and management: a case report and literature review.Cureus. 2022;14(5):e24850. doi:10.7759/cureus.24850
Cheung E, Ahmad S, Aitken M, et al.Combined simultaneous FDG-PET/MRI with T1 and T2 mapping as an imaging biomarker for the diagnosis and prognosis of suspected cardiac sarcoidosis.Eur J Hybrid Imaging. 2021;5(1):24. doi:10.1186/s41824-021-00119-w
Lehtonen J, Uusitalo V, Pöyhönen P, Mäyränpää MI, Kupari M.Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis.Eur Heart J. 2023;44(17):1495-1510. doi:10.1093/eurheartj/ehad067
Roberts WC, Becker TM, Hall SA.Usefulness of total 12-lead QRS voltage as a clue to diagnosis of patients with cardiac sarcoidosis severe enough to warrant orthotopic heart transplant.JAMA Cardiol. 2018;3(1):64-68. doi:10.1001/jamacardio.2017.4172
Ekström K, Lehtonen J, Nordenswan HK, et al.Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries.Eur Heart J. 2019;40(37):3121-3128. doi:10.1093/eurheartj/ehz428
Lemay S, Massot M, Philippon F, et al.Ten questions cardiologists should be able to answer about cardiac sarcoidosis: case-based approach and contemporary review.CJC Open. 2020;3(4):532-548. doi:10.1016/j.cjco.2020.11.022
Cheng RK, Kittleson MM, Beavers CJ, et al.Diagnosis and management of cardiac sarcoidosis: a scientific statement from the American Heart Association.Circulation. Published online April 18, 2024. doi:10.1161/CIR.0000000000001240
Nordenswan HK, Lehtonen J, Ekström K, et al.Outcome of cardiac sarcoidosis presenting with high-grade atrioventricular block.Circ Arrhythm Electrophysiol. 2018;11(8):e006145. doi:10.1161/CIRCEP.117.006145
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