Cardiomyopathy
Utility of Cardiac Magnetic Resonance Imaging to Differentiate Cardiac Sarcoidosis from Arrhythmogenic Right Ventricular Cardiomyopathy

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Some patients diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) are eventually found to have cardiac sarcoidosis (CS). Accurate differentiation between these 2 conditions has implications for immunosuppressive therapy and familial screening. We sought to determine whether cardiac magnetic resonance imaging (MRI) could be used to identify the characteristic findings to accurately differentiate between CS and ARVC. Consecutive patients with a diagnostic MRI scan indicating CS and/or ARVC constituted the cohort. All patients diagnosed with CS had histologic confirmation of sarcoidosis, and all patients with ARVC met the diagnostic task force criteria. The cardiac MRI data were retrospectively analyzed to identify possible differentiating characteristics. Of the patients, 40 had CS and 21 had ARVC. Those with CS were older and had more left ventricular scar. The presence of mediastinal lymphadenopathy or left ventricular septal involvement was seen exclusively in the patients with CS (p <0.001). A family history of sudden cardiac death was seen only in the ARVC group (p = 0.012). The right ventricular ejection fraction and ventricular volumes were also significantly different between the 2 groups. In conclusion, patients with CS have significantly different cardiac MRI characteristics than patients with ARVC. The cardiac volume, in addition to the degree and location of cardiac involvement, can be used to distinguish between these 2 disease entities. The presence of mediastinal lymphadenopathy and left ventricular septal scar favors a diagnosis of CS and not ARVC. Consideration of CS should be given if these MRI findings are observed during the evaluation for possible ARVC.

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Methods

The study cohort consisted of 61 consecutive patients referred to our institutions for evaluation of the presence of CS or ARVC. Of the patients, 38% of those with ARVC and all of those with CS underwent workup at the University of Colorado. From March 2004 to December 2009, an active screening program was underway for CS in selected patients with biopsy-proven pulmonary sarcoidosis at the University of Colorado Hospital. Patients with extracardiac sarcoidosis and signs or symptoms that could

Results

The clinical characteristics of the 40 patients with CS and 21 with ARVC are listed in Table 1. Approximately 2/3 of both cohorts were men. The patients in the CS group were older and more likely to have been diagnosed with hypertension. A family history of sudden cardiac death alone (p = 0.012) and a family history of sudden cardiac death or ARVC (p <0.001) strongly distinguished these 2 cohorts. No patient in the CS group reported a history of sudden cardiac death. Most MRI characteristics

Discussion

ARVC was first described in 1977 by Fontaine et al.17 The hallmark of this genetic cardiomyopathy is fibrofatty infiltration of the right ventricle with RV dysfunction. Although classic ARVC presents with isolated RV disease, there appear to be 2 other phenotypic presentations: a biventricular form with simultaneous involvement of both ventricles and a left-dominant form with early and severe LV dysfunction and minimal RV dysfunction.1 Several of these presentations can mimic that of CS, making

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