Pediatric cardiology
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Pediatric cardiology · Feb 2013
Comparative StudyImpact of 22q11.2 deletion on the postoperative course of children after cardiac surgery.
The primary objective of this study was to describe the impact of 22q11.2 deletion (del22q11) on the clinical characteristics, postoperative course, and short-term outcomes of children undergoing surgery for congenital heart disease. The charts of all children ages 1 day-18 years who received cardiac surgery for interrupted aortic arch (IAA), tetralogy of Fallot (TOF), or truncus arteriosus (TA) repair from 1 January 2001 to 31 December 2011 were retrospectively reviewed. The patients were divided into two groups: the 22q11 group including children with del22q11 undergoing surgery for TOF, IAA, or TA and the non-22q11 or control group including children with no chromosomal or genetic abnormality undergoing surgery for TOF, IAA, or TA. ⋯ Mortality did not differ significantly between the two groups. In conclusion, children with del22q11 have a higher risk of postoperative complications after cardiac surgery, with no difference in length of mechanical ventilation, ICU LOS, hospital LOS, or mortality. However, short-term outcomes may differ in certain subgroups.
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Pediatric cardiology · Feb 2013
Comparative StudyThe role of transesophageal echocardiography during surgery for patients with tetralogy of Fallot.
Routine use of intraoperative transesophageal echocardiography (TEE) is a safe monitoring and diagnostic method during pediatric congenital cardiac surgery. However, the question of whether intraoperative TEE is accurate and cost effective for patients with tetralogy of Fallot (TOF) has not been raised. This study aimed to analyze the cost-benefit of routine TEE during the repair of TOF. ⋯ Intraoperative TEE can be used as a tool for surgeons in making decisions in the operating room. In addition, intraoperative TEE decreased the frequency of reoperations and postoperative interventions. The results of this study demonstrate that routine intraoperative TEE during repair of TOF was both clinically beneficial and cost effective.
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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.