What is cardiac sarcoidosis?

Updated: Nov 28, 2018
  • Author: Vinh Q Nguyen, MD; Chief Editor: Gyanendra K Sharma, MD, FACC, FASE  more...
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Answer

Cardiac sarcoidosis, a noncaseating granulomatous disease, can involve a number of organs. Cardiac involvement affects the atrioventricular (AV) node, causing heart block, as well as the basal septum, papillary muscles, and focal regions in the free wall. [44]

Echocardiographic findings of cardiac sarcoidosis include wall thickening from granulomatous infiltration, with subsequent scarring/thinning that are seen as wall motion abnormalities, LV dilatation, and/or aneurysm. The wall motion abnormalities do not typically correspond to regions subtended by a specific coronary artery. Mitral regurgitation may be seen with papillary muscle involvement. Pulmonary involvement is also common and signs of pulmonary hypertension or RV dysfunction may be present. LGE is patchy and involves the basal and lateral LV walls. [55]

The diagnosis can be made via EMB, although its sensitivity is less than 20%. [56] This is due to the patchy nature of the myocardial involvement.

The use of corticosteroids is the hallmark of treatment and should be started in patients with a high suspicion of cardiac sarcoidosis, even in the presence of a negative biopsy, as early treatment is more effective than later treatment. Unfortunately, therapy does not appear to improve LV volume or function in those with an LVEF below 30%. [57] The presence of concurrent pulmonary sarcoidosis or a depressed LVEF carries a worse prognosis. [58, 59]  Immunosuppressants such as methotrexate, azathioprine, and cyclophosphamide may be used in steroid-refractory or steroid-contraindicated cases. [58, 59]


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