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Echocardiography has become the primary imaging tool in the diagnosis and assessment of congenital and acquired heart disease in infants, children, and adolescents. Transthoracic echocardiography (TTE) is an ideal tool for cardiac assessment, as it is noninvasive, portable, and efficacious in providing detailed anatomic, hemodynamic, and physiologic(More)
BACKGROUND Disease of the aortic valve in children and young adults is a complex entity whose management is the subject of controversy. The Ross and the Ross-Konno procedures have become the primary choices for aortic valve replacement in children because of growth potential, optimal hemodynamic performance, and lack of the need for anticoagulation.(More)
BACKGROUND Although primary repair of tetralogy of Fallot is increasingly undertaken in infancy, complete repair is generally performed in only selected symptomatic neonates. METHODS From July 1992 through March 1995, 30 consecutive neonates and young infants with tetralogy of Fallot underwent routine primary repair. Group I (n = 10) consisted of patients(More)
We sought to evaluate the relation of a prenatal diagnosis (preDx) with morbidity and mortality during the initial hospitalization in a contemporary cohort of patients with hypoplastic left heart syndrome (HLHS). A retrospective study of patients with HLHS presenting from 1999 to 2010 was performed. Patients with genetic disorders or a gestational age <34(More)
BACKGROUND Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome. METHODS AND RESULTS We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental(More)
A major advantage of echocardiography over other advanced imaging modalities (magnetic resonance imaging, computed tomographic angiography) is that echocardiography is mobile and real time. Echocardiograms can be recorded at the bedside, in the cardiac catheterization laboratory, in the cardiovascular intensive care unit, in the emergency room-indeed, any(More)
UNLABELLED The role of inhaled nitric oxide in the immediate post-bypass period after surgical repair of congenital heart disease is uncertain. In a controlled, randomized, double-blind study, we tested the hypothesis that inhaled nitric oxide (NO) would reduce pulmonary hypertension immediately after surgical repair of congenital heart disease in 40(More)
BACKGROUND Pulmonary hypertension results in increased morbidity and mortality in children after surgical repair of congenital heart defects. Various vasodilators have been unsuccessful in providing preferential pulmonary vasodilation in these patients. Identification of a more preferential pulmonary vasodilator would improve the assessment, management, and(More)
OBJECTIVES The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair. BACKGROUND Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart(More)
A child with epidermolysis bullosa dystrophica, recessive type (EBDR) developed significant anemia at 9 years of age and was treated with long-term transfusion therapy. At age 17 he had symptoms of congestive heart failure secondary to dilated cardiomyopathy. Treatment with digoxin and vasodilators for the past year has failed to improve his cardiomyopathy(More)