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Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy and Cardiac Sarcoidosis

2014; Lippincott Williams & Wilkins; Volume: 7; Issue: 2 Linguagem: Inglês

10.1161/circep.113.000932

1941-3149

Binu Philips, Srinivasa Madhavan, Cynthia A. James, Anneline S.J.M. te Riele, Brittney Murray, Crystal Tichnell, Aditya Bhonsale, Saman Nazarian, Daniel P. Judge, Hugh Calkins, Harikrishna Tandri, Alan Cheng,

Viral Infections and Immunology Research

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. Methods and 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.