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OBJECTIVE To characterize the frequency and nature of cardiovascular defects in patients with CblC-type methylmalonic aciduria and homocystinuria (cblC), an inborn error of cobalamin (vitamin B12) metabolism resulting in accumulation of methylmalonic acid and homocysteine. STUDY DESIGN A retrospective observational study was conducted investigating 10(More)
OBJECTIVES To determine if a relationship exists between regional oxyhemoglobin saturation (rSO(2)) measured at various body locations by near-infrared spectroscopy (NIRS) and blood lactate level in children after cardiac surgery. DESIGN A prospective, observational study. SETTING A pediatric cardiac intensive care unit in a university hospital. (More)
Cardiac magnetic resonance (CMR) assesses myocardial involvement in myocarditis (MYO). Current techniques are qualitative, subjective, and prone to interpretation error. Feature tracking (FT) analyzes myocardial strain using CMR and has not been examined in MYO. We hypothesize that regional left ventricular (LV) strain is abnormal in MYO. Regional strain by(More)
We describe a 3-year-old Hispanic male with cblC-type methylmalonic aciduria and homocystinuria who presented to the emergency department with progressive tachypnea, vomiting, and edema secondary to pulmonary embolism and cor pulmonale. With aggressive medical management, there was complete resolution of right heart failure and pulmonary hypertension after(More)
BACKGROUND The Pediatric Heart Network is conducting a large international randomized trial to compare aortic root growth and other cardiovascular outcomes in 608 subjects with Marfan syndrome randomized to receive atenolol or losartan for 3 years. The authors report here the echocardiographic methods and baseline echocardiographic characteristics of the(More)
Our goal was to construct three-dimensional (3D) virtual models to allow simultaneous visualization of the ventricles, ventricular septal defect (VSD) and great arteries in patients with complex intracardiac anatomy to aid in surgical planning. We also sought to correlate measurements from the source cardiac magnetic resonance (CMR) image dataset and the 3D(More)
Left-ventricular noncompaction (LVNC) is characterized by prominent myocardial trabeculae with deep intratrabecular recesses. Associated with ventricular dysfunction, LVNC can present in isolation or in conjunction with structural heart defects (i.e., “nonisolated” LVNC). Although it was once considered to be difficult and unreliable, successful detection(More)
In this study, we sought to determine whether the risk of failure of coil occlusion of patent ductus arteriosus (PDA) could be predicted on the basis of echocardiographic variables. The echocardiographic characteristics of patients in whom PDA coil occlusion failed were compared with those in whom the procedure was successful. A total of 5 variables were(More)
OBJECTIVES This study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a "best practice" protocol for imaging and(More)