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A3520 - Atrioventricular (AV) Block as the First Manifestation of Cardiac Sarcoidosis
Author Block: A. Kardasheva; Virginia Mason Medical Center, Seattle, WA, United States.
Introduction: Cardiac sarcoidosis remains a diagnostic challenge in patients without evidence of another organ involvement. Case Description: A 58-year-old man with history of diabetes, hypertension, hyperlipidemia and a recent third-degree heart block with a permanent pacemaker presented to an outside hospital with chest discomfort. He was found to have ventricular tachycardia. A coronary angiogram revealed critical left anterior descending artery disease. A stent was placed to stabilize the patient and he was transferred to our institution where he underwent 3-vessel bypass surgery. Intraoperatively a left atrial mass, later found to be myxoma, was identified and resected. Additionally, abnormal tissue at the superior vena cava and multiple other lesions within the pericardium were resected and subsequent surgery pathology report revealed non-necrotizing granuloma consistent with cardiac sarcoidosis. Postoperative course was complicated by cardiogenic shock requiring vasoactive medications. Steroids were not started while inpatient to allow enough time for optimal cardiac healing. Patient was discharged in stable condition to outpatient follow up with cardiology and pulmonology regarding timing of immunosuppressive therapy in the setting of newly diagnosed cardiac sarcoidosis. Discussion: Sarcoidosis is a heterogeneous, non-caseating granulomatous disorder of unknown pathogenesis that can involve any organ system. Cardiac involvement may be detected alone and may precede, follow or occur concurrently with another organ involvement. The signs and symptoms of cardiac sarcoidosis correlate to location and extent of granulomatous inflammation. Clinical manifestations include conduction abnormalities, tachyarrhythmias, cardiomyopathy, congestive cardiac failure leading to hemodynamic instabilities and sudden cardiac death. The substrate for arrhythmias may be active granuloma formation in the myocardium or myocardial fibrosis. Presentation with sustained ventricular tachycardia just like in our patient or ventricular fibrillation is an indication for a defibrillator implantation. The decision to implant a defibrillator in patient with cardiac sarcoidosis not presenting with ventricular arrhythmia is complex, and there are no controlled data to guide clinicians. Other studies have suggested an algorithmic approach to sarcoidosis and arrhythmia, with pacemaker implantation alone for patients presenting with AV block in the absence of severe left ventricular dysfunction or heart failure. Defibrillation implantation should be reserved for those with spontaneous ventricular tachycardia and a narrow QRS whereas a defibrillator plus cardiac resynchronization therapy should be offered to patients with spontaneous ventricular arrhythmias associated with impaired ventricular function and wide QRS.