Pediatric cardiology
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Pediatric cardiology · Jan 2013
Multicenter StudyEffects of race, ethnicity, and gender on surgical mortality in hypoplastic left heart syndrome.
Information is limited regarding the effect of race, ethnicity, and gender on the outcomes of the three palliative procedures for hypoplastic left heart syndrome (HLHS). This study examined the effects of race, ethnicity, gender, type of admission, and surgical volume on in-hospital mortality associated with palliative procedures for HLHS between 1998 and 2007 using data from the University HealthSystem Consortium. According to the data, 1,949 patients underwent stage 1 palliation (S1P) with a mortality rate of 29 %, 1,279 patients underwent stage 2 palliations (S2P) with a mortality rate of 5.4 %, and 1,084 patients underwent stage 3 palliation (S3P) with a mortality rate of 4.1 %. ⋯ No racial differences with S3P were observed. The risk factors for increased mortality at S1P were black and "other" race, smaller surgical volume, and early surgical era. The risk factors for increased in-hospital mortality with S2P were black race and Hispanic ethnicity.
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Pediatric cardiology · Jan 2013
Comparative StudyInterventions after Norwood procedure: comparison of Sano and modified Blalock-Taussig shunt.
Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock-Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. ⋯ Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.
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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
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 ReportsNoonan syndrome and different morphologic expressions of hypertrophic cardiomyopathy.
Hypertrophic cardiomyopathy is the disease with the largest heterogeneity in clinical cardiology. An underrecognized and incompletely described subgroup within this spectrum comprises patients with ventricular diverticulum, coronary myocardial bridging, and left ventricular crypts and crevices. This report presents the case of a Noonan syndrome patient with these four hypertrophic cardiomyopathy characteristics.