title: Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear.
abstract: BACKGROUND
  Cardiac sarcoidosis (CS) may show overlap in the clinical presentation with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). We sought to investigate patients with CS who were misdiagnosed with ARVD/C and identify clinical features to distinguish these 2 groups.
  
  
  RESULTS
  Among patients enrolled in the Johns Hopkins ARVD/C registry, 15 patients with definite 2010 diagnostic criteria for ARVD/C were subsequently diagnosed with CS. Forty-two pathogenic desmosomal mutation carriers with definite ARVD/C based on the 2010 diagnostic criteria served as a control group. Patients with CS were older at the age of symptom onset, more likely to have comorbidities, and develop heart failure symptoms over time (P<0.05). Electrocardiographically, PR interval prolongation and high-grade atrioventricular block were exclusively associated with CS (P<0.05). HV interval prolongation and increased number of ventricular tachycardias induced were also associated with CS (P<0.05). Radiographically, significant left ventricular dysfunction, myocardial delayed enhancement of the septum, and mediastinal lymphadenopathy were more often see in those with CS (P<0.05).
  
  
  CONCLUSIONS
  The 2010 diagnostic criteria for ARVD/C have limited discrimination in distinguishing between ARVD/C and CS. Despite the overlay in clinical presentation, older age of symptom onset, presence of cardiovascular comorbidities, nonfamilial pattern of disease, PR interval prolongation, high-grade atrioventricular block, significant left ventricular dysfunction, myocardial delayed enhancement of the septum, and mediastinal lymphadenopathy should raise the suspicion for CS.
authors.affiliation: Section of Cardiac Electrophysiology, Cardiovascular Institute, Warren Alpert Medical School of Brown University, Providence, RI.
authors.forename: Binu
authors.initials: B
authors.lastname: Philips
authors.forename: Srinivasa
authors.initials: S
authors.lastname: Madhavan
authors.forename: Cynthia A
authors.initials: CA
authors.lastname: James
authors.forename: Anneline S J M
authors.initials: AS
authors.lastname: te Riele
authors.forename: Brittney
authors.initials: B
authors.lastname: Murray
authors.forename: Crystal
authors.initials: C
authors.lastname: Tichnell
authors.forename: Aditya
authors.initials: A
authors.lastname: Bhonsale
authors.forename: Saman
authors.initials: S
authors.lastname: Nazarian
authors.forename: Daniel P
authors.initials: DP
authors.lastname: Judge
authors.forename: Hugh
authors.initials: H
authors.lastname: Calkins
authors.forename: Harikrishna
authors.initials: H
authors.lastname: Tandri
authors.forename: Alan
authors.initials: A
authors.lastname: Cheng
mesh_terms: D000293:Adolescent; D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D003937:Diagnosis, Differential; D004452:Echocardiography; D004554:Electric Countershock; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D010641:Phenotype; D049268:Positron-Emission Tomography; D011379:Prognosis; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D014057:Tomography, X-Ray Computed; D055815:Young Adult
keywords: arrhythmogenic right ventricular dysplasia; diagnosis; sarcoidosis