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OBJECTIVES To compare a steady-state free precession cine sequence-based technique (feature tracking [FT]) to tagged harmonic phase (HARP) analysis for peak average circumferential myocardial strain (epsilon(cc)) analysis in a large and heterogeneous population of boys with Duchenne muscular dystrophy (DMD). BACKGROUND Current epsilon(cc) assessment(More)
STUDY OBJECTIVE To examine the dose-response effects of dexmedetomidine (DEX) and propofol (PROP) on airway morphology in children and adolescents with a history of obstructive sleep apnea (OSA). DESIGN Prospective, single-blinded, controlled comparative study. SETTING University-affiliated teaching hospital. PATIENTS 60 patients with a history of OSA(More)
OBJECTIVES This study sought to evaluate the natural history of occult cardiac dysfunction in Duchenne muscular dystrophy (DMD). BACKGROUND Duchenne muscular dystrophy is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that left ventricular myocardial peak circumferential strain(More)
The present study evaluated progressive cardiac dysfunction using serial circumferential strain (epsilon(cc)) measurements in patients with Duchenne muscular dystrophy (DMD). DMD is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that serial epsilon(cc) changes could be detected in(More)
OBJECTIVE Turner syndrome affects one in 2,500 girls and women and is associated with cardiovascular anomalies. Visualizing the descending thoracic aorta in adults with Turner syndrome with echocardiography is difficult. Therefore, cardiac MRI is the preferred imaging modality for surveillance. Our goals were to use cardiac MRI describe the spectrum and(More)
BACKGROUND Patients with Duchenne muscular dystrophy exhibit progressive cardiac and skeletal muscle dysfunction. Based on prior data, cardiac dysfunction in Duchenne muscular dystrophy patients may be influenced by myocardial fibrosis and steroid therapy. We examined the longitudinal relationship of myocardial fibrosis and ventricular dysfunction using(More)
OBJECTIVE The purpose of our study was to describe patterns of airway compression identified on cross-sectional imaging in infants and children with either right aortic arch and aberrant left subclavian artery or left aortic arch with aberrant right subclavian artery. MATERIALS AND METHODS Data from MR imaging and CT performed to evaluate pediatric(More)
The cardiac disease ubiquitously associated in Duchenne Muscular Dystrophy (DMD) has traditionally been considered a progressive dilated cardiomyopathy (DCM). However, left ventricular (LV) dilatation as measured with cardiac MRI has not been a consistent finding in this population, even as circumferential strain (ε(cc)) declines with advancing disease. We(More)
RATIONALE Bronchopulmonary dysplasia (BPD) is a prevalent yet poorly characterized pulmonary complication of premature birth; the current definition is based solely on oxygen dependence at 36 weeks postmenstrual age without objective measurements of structural abnormalities across disease severity. OBJECTIVES We hypothesize that magnetic resonance imaging(More)
BACKGROUND Serial surveillance of liver iron concentration (LIC) provides guidance for chelation therapy in patients with iron overload. The diagnosis of iron overload traditionally relies on core liver biopsy, which is limited by invasiveness, sampling error, cost and general poor acceptance by pediatric patients and parents. Thus noninvasive diagnostic(More)