Abstract
Cardiac magnetic resonance (CMR) has been helpful in adults in the diagnosis of arrhythmogenic right ventricular dysplasia. Short of direct surgical observation or autopsy, no gold standard exists. CMR diagnostic criteria include right atrial and ventricular dilation, regional right ventricular (RV) wall motion abnormalities, outflow tract ectasia, and myocardial fatty infiltration. To determine whether adult diagnostic criteria are useful in children referred for CMR for this diagnosis, the images and records of 81 patients (aged 11.5 +/- 5.5 years) over an 8-year period were reviewed. Histories included ventricular tachycardia, palpitations, dilated right ventricle, syncope, near sudden death, or family history of RV dysplasia. Four families were studied with parents who had RV dysplasia diagnosed by surgery, explanted heart, or CMR. CMR imaging included T1-weighted imaging, cine, 1-dimensional RV myocardial tagging, and phase-encoded velocity mapping, and 2 patients underwent delayed-enhancement CMR. Only 1 of the 81 patients met 5 of the criteria. None of the others met >2 of the criteria, and only 2 patients met 1 or 2 criteria. For questionable regional wall motion abnormalities, RV myocardial tagging was helpful. In conc...Continue Reading
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