Isolated Cardiac Sarcoidosis in a Young Caucasian Female with Progressive Decline
Grand Strand Medical Center
cardiomyopathy, cardiac sarcoidosis, CHF, congestive heart failure
Cardiology | Cardiovascular Diseases
Cardiac sarcoidosis (CS) is a rare cause of cardiomyopathy, with an estimated prevalence of 20-27% in patients who have systemic sarcoidosis. We present a case of a woman with sudden onset CHF who continued to deteriorate despite appropriate medical management. Cardiac MRI and endomyocardial biopsy were performed that confirmed the diagnosis of CS.
A 47 year old Caucasian female with a past medical history of non-ischemic heart failure with reduced ejection fraction presented with severe epigastric pain and vomiting. Echocardiogram demonstrated reduced ejection fraction (15-20%) and grade 3 diastolic dysfunction. Cardiologist recommend initiation of dobutamine, which led to drastic improvement in her symptoms. Given that the patient had failed medical therapy and required a dobutamine drip to maintain her cardiac function, discussions of heart transplant began. She was transferred to a tertiary care center where Cardiac MRI and endomyocardial biopsy were performed revealing non-caseating granulomas with occasional asteroid bodies, pathognomic features of sarcoidosis. CS was medically managed with immunosuppression and she eventually received a cardiac transplant.
CS can be diagnosed with multiple imaging modalities and histopathological evidence. Asymptomatic electrocardiogram changes such as bundle branch block are the most common manifestations of cardiac sarcoid. Echocardiogram findings of systolic or diastolic dysfunction or regional wall motion abnormalities have been reported in 14-56% of patients. However, these findings are nonspecific. Gadolinium-enhanced cardiac MRI for sarcoidosis had a sensitivity of 100% and specificity of 78%.
In patients without systemic manifestations of sarcoidosis, the diagnosis of CS is very difficult to confirm. In appropriate clinical settings, it is imperative for a physician to consider isolated CS for patients who present with idiopathic non-ischemic cardiomyopathy, even if they lack clinical features of sarcoidosis. Steroids form the mainstay of treatment for sarcoidosis. However, no guidelines are available to determine dosing and use of additional immune-suppressants.
Publisher or Conference
Journal of the American College of Cardiology
Patel D, Udani K, Esperti S, Nambudiri V. Isolated Cardiac Sarcoidosis in a Young Caucasian Female with Progressive Decline. J Am Coll Cardiol. 2020;75(11 Supplement 1):2470. doi:10.1016/S0735-1097(20)33097-7