title: Right ventricular sarcoidosis: is it time for updated diagnostic criteria?
abstract: A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient's clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient's right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. In addition to discussing the patient's case, we review the relevant medical literature and discuss the need for updated differential diagnostic criteria for end-stage right ventricular failure that mimics arrhythmogenic right ventricular cardiomyopathy.
authors.affiliation: Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington 98195.
authors.forename: Kairav
authors.initials: K
authors.lastname: Vakil
authors.affiliation: Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington 98195.
authors.forename: Elina
authors.initials: E
authors.lastname: Minami
authors.affiliation: Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington 98195.
authors.forename: Daniel P
authors.initials: DP
authors.lastname: Fishbein
mesh_terms: D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis*; D001282:Atrial Flutter / Q000209:etiology; D054537:Atrioventricular Block / Q000209:etiology; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D002316:Cardiotonic Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D018450:Disease Progression; D004452:Echocardiography / Q000379:methods; D005260:Female; D006333:Heart Failure / Q000209:etiology; D016027:Heart Transplantation / Q000379:methods*; D006352:Heart Ventricles / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D049268:Positron-Emission Tomography / Q000379:methods; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D016896:Treatment Outcome; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery
chemical_list: D002316:Cardiotonic Agents
keywords: Arrhythmogenic right ventricular dysplasia/diagnosis; cardiomyopathies/complications; diagnosis, differential; diagnostic techniques, cardiovascular; sarcoidosis/diagnosis/physiopathology; ventricular dysfunction, right/diagnosis/etiology/physiopathology