Background: Sarcoidosis is a rare multisystem disease characterized histologically by non-caseating granuloma formation in the affected organ. While cardiac sarcoidosis is found on autopsy in up to 25% of sarcoidosis cases, it is still underdiagnosed and is associated with a poor prognosis. Although the etiology of sarcoidosis remains unclear, an antigen triggered exaggerated immune response has been hypothesized. Early detection and prompt management of cardiac sarcoidosis remains pivotal.
Case summary: A 60-year-old female, with pulmonary sarcoidosis in remission, presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion (New York Heart Association class II) that was relieved by rest. Submaximal exercise stress test showed multifocal ventricular extrasystoles, followed by a self-limiting torsades de pointes. Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis. The magnetic resonance imaging-derived left ventricular ejection fraction was 45%. Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles, all in favor of extensive cardiac involvement of sarcoidosis. A double-chamber implantable cardiac defibrillator was implanted, and methylprednisolone (12 mg/d) and methotrexate (12.5 mg/wk) treatment was initiated. Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.
Conclusion: Along an extensive review of the literature, this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis, in order to avoid potential complications and increase survival.
Keywords: Cardiac sarcoidosis; Case report; Immunotherapy; Sarcoidosis; Torsades de pointes; Ventricular tachycardia.
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