CARDIAC SARCOID AND RISK OF SUDDEN DEATH. A. DeFilippis 1 , A. Palacio 1 , D.W. Brady 1 ; 1 Emory University, Decatur, GA (Tracking ID #76794) LEARNING OBJECTIVES: 1. Recognize the clinical presentation of cardiac sarcoid. 2. Treat cardiac sarcoid and its inherent risk for sudden cardiac death. CASE INFORMATION: A 58-year-old female with cutaneous sarcoid presents to the emergency room with a 3 month history of progressive dyspnea on exertion. She also reports a 2-week history of proximal nocturnal dyspnea, cough, 2-pillow orthopnea and lower extremity edema. She denies chest pain, fever, chills, or palpitations. Her past medical history includes hypertension, diabetes, hypercholesterolemia and obstructive sleep apnea. On presentation, she was afebrile, pulse 106, blood pressure 120/60, respiratory rate of 28 and oxygen saturation of 88% on room air. Physical exam was significant for S3 gallop, normal pulmonary exam, lower extremity edema and several silver dollar size hyperpigmented lesions on her legs. Routine laboratory testing was unremarkable, chest radiograph revealed mild cardiomegaly as compared to a normal radiography 1 month earlier. EKG revealed a new right conduction system block as compared to a normal EKG 2 years earlier. High resolution CT scan was negative for pulmonary embolus and deep venous thrombosis but revealed multiple enlarged mediastinal lymph nodes. Echocardiogram was notable for hypokinetic lateral and inferior wall motion, new from a normal dobutamine stress echocardiogram 2 years earlier. Cardiac catheterization revealed normal coronary angiography, an ejection fraction of 20% and regional wall motion abnormalities, which lead to a cardiac biopsy that revealed non-caseating granulomas consistent with active cardiac sarcoid. The patient improved with diuresis and systemic afterload reduction. She was started on a 6-month course of prednisone and equipped with an automated internal cardiac defibrillator with rescue pacing, prior to discharge from the hospital. IMPLICATIONS/DISCUSSION: Dyspnea and cough are amongst the most common clinical presentations of sarcoid but are attributed to pulmonary rather than cardiac pathology. Although less than 10% of sarcoid patients demonstrate symptoms secondary to cardiac sarcoid, cardiac involvement is found in up 76% of sarcoid patients at autopsy and accounts for 50% of the mortality in this patient population. The most common presentation of cardiac sarcoid, found in approximately 50% of patients, is arrhythmias or cardiac conduction abnormalities as a result of granuloma formation in the conduction system. Sarcoid granulomas in the ventricular myocardium may lead to ventricular arrhythmias in as many as 22% of patients with sarcoid. Granulomas in myocardium can result in diastolic or systolic dysfunction and has accounted for 25±75% of the deaths in some studies of cardiac sarcoid patients. Endomyocardial biopsy is highly specific but due to the non-homogeneous pattern of infiltration will only lead to a diagnosis of cardiac sarcoid in approximately 20% of cases. With arrhythmias or cardiac conduction abnormalities accounting for 30±65% of the deaths inpatients with cardiac sarcoid, automated internal cardiac defibrillators (AICD) with rescue pacing are standard of care in survivors of deadly arrhythmias. Although no trials have assessed the efficacy of AICDs in the absence of ventricular tachyarrhythmias, many advocate their use in all cases of known cardiac sarcoid; others reserve the use to patients with EKG abnormalities by standard of Holter monitor testing. Treatment of the underlying pathological process with immunosuppression is controversial and void of any large randomised trials to access efficacy. A small uncontrolled study demonstrated improvement and/or reversal of EKG abnormalities in greater than 50% of patients treated with corticosteroids. In one retrospective study of patients with cardiac sarcoid, 5-year survival was increased from 10% to 75% with the use of corticosteroids. Opponents to the use of corticosteroids raise concern that corticosteroids convert granulomas to scar tissue increasing the risk of aneurysms. Cardiac transplant has been successful with recurrent disease possible but rare.