Pediatric cardiology
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Pediatric cardiology · Jan 2013
Case ReportsEfficacy of an interleukin-1β receptor antagonist (anakinra) in idiopathic recurrent pericarditis.
Pericarditis may recur in up to 30 % of adult patients, but recurrent pericarditis is a rare disease in childhood. The etiology of the initial attack and the causes of recurrences often remain unknown. Recurrent pericarditis is accompanied by a high morbidity rate and may represent a challenge to the clinician due to problems in management. ⋯ Controlled trials have demonstrated that colchicine can reduce the recurrent rate of pericarditis, whereas early corticosteroid therapy promotes recurrences. Anakinra, a recombinant human interleukin-1β receptor antagonist, is a promising new biologic agent for the treatment of autoinflammatory diseases such as cryopyrinopathies, tumor necrosis factor receptor-associated periodic syndrome, and hyperimmunoglobulinemia D with periodic fever syndrome. This report describes an 11-year-old boy successfully treated with anakinra for a steroid-dependent recurrent pericarditis unresponsive to conventional treatment.
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Pediatric cardiology · Jan 2013
Sildenafil weaning after discharge in infants with congenital diaphragmatic hernia.
Sildenafil is used to treat pulmonary hypertension (PAH) in infants with congenital diaphragmatic hernia (CDH). However, data to guide sildenafil dosing and weaning are limited. This is concerning in light of a recent report describing increased risk associated with high-dose sildenafil regimens in non-CDH PAH. ⋯ One infant died of severe PAH. Sildenafil therapy at discharge is common in severe CDH. Variation in dosing and weaning rates highlights the need for standardized assessment and treatment of PAH after discharge to optimize the benefits and minimize the adverse effects of sildenafil.
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Pediatric cardiology · Jan 2013
Case ReportsIsolated total anomalous systemic venous drainage in an adult: case report.
Total anomalous systemic venous drainage is a rare form of congenital heart disease. All the systemic venous flow from the body (i.e., from the superior vena cava, inferior vena cava, and coronary sinus) drains abnormally into the left atrium. ⋯ This disorder may be associated with heterotaxy syndromes. This report describes a patient with unique total anomalous systemic venous drainage that was successfully corrected surgically.
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Pediatric cardiology · Jan 2013
Case ReportsMaternal diclofenac medication in pregnancy causing in utero closure of the fetal ductus arteriosus and hydrops.
This report describes a case of premature closure in utero of the ductus arteriosus (DA) diagnosed postnatally in a baby with hydrops and cardiac failure. An echocardiogram 6 h postnatally showed marked dilation of the right atrium and right ventricle with marked hypertrophy and impaired function, elevated pulmonary pressures, a small pericardial effusion, and no flow through the DA. The mother was unaware of her pregnancy until she presented in labor, and she had taken diclofenac medication in the preceding months. This case and the accompanying literature review illustrate the potential fetal and neonatal complications resulting from antenatal closure of the DA due to maternal diclofenac medication during pregnancy.
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Pediatric cardiology · Jan 2013
Clinical outcomes and resource use for infants with hypoplastic left heart syndrome during bidirectional Glenn: summary from the Joint Council for Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry.
The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry captures information on interstage management of infants with hypoplastic left heart syndrome (HLHS). The purpose of this study was to identify interstage risk factors for increased resource use and adverse outcomes during bidirectional Glenn (BDG) hospitalization. All infants in the NPC-QIC registry (31 United States hospitals) undergoing BDG surgery were included (December 2009 to August 2010). ⋯ Lower weight-for-age z-score was an independent and potentially modifiable risk factor for BDG complications. HLHS infants who met caloric recommendations before BDG had a lower duration of hospitalization at BDG. These data justify targeting nutrition in interstage strategies to improve outcomes and decrease costs for patients with HLHS.