Pediatric cardiology
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Pediatric cardiology · Nov 2005
Case ReportsIsolated left-sided scimitar vein connecting all left pulmonary veins to the right inferior vena cava.
When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary artery, as well as abnormal parenchyma of the involved lung. Such cases have been described as "scimitar syndrome." We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. Surgical intervention was successful, and our patient is still alive.
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Pediatric cardiology · Nov 2005
Case ReportsAsystole during outbursts of laughing in a child with Angelman syndrome.
A girl with Angelman syndrome had recurrent episodes of ventricular asystole and syncope caused by severe vagal hypertonia during outbursts of laughing. After intravenous administration of atropine, laughing no longer induced asystole or syncope. The vast majority of patients with Angelman syndrome have seizures. Since hypoxia associated with asystole can provoke convulsions, we suggest electrocardiographic evaluation of Angelman patients with symptomatic bradycardia, loss of consciousness, or convulsions related to laughing.
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Pediatric cardiology · Nov 2005
Case ReportsRecurrent supraventricular tachycardia in a newborn treated with amiodarone: is hyperkalemia the apparent cause?
Supraventricular tachycardia (SVT) is the most common type of arrhythmia observed in children, especially in newborns. Infants with severe SVT must be treated immediately with first-line drugs such as amiodarone. There are some minor and major side effects of amiodarone in this patient group, but no associated electrolyte disorders have been observed. This report describes a newborn whose recurrent SVT attacks during amiodarone treatment were suspected to have been caused by hyperkalemia.
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Pediatric cardiology · Nov 2005
High mortality rate after extubation failure after pediatric cardiac surgery.
The objective of this study was to evaluate the different causes of extubation failure and the consequent mortality rates in a pediatric population after cardiac surgery. We studied 184 consecutive patients with a median age of 9 months (range, 0-165). In 158 patients, extubation was successful (group A). ⋯ Group C patients had more reoperations (30% vs 4% in group A patients, p < 0.001), a lower PaO2 on admission at the intensive care unit as well as just prior to extubation, a lower base deficit before extubation, and needed more inotropic support during their stay in the intensive care unit. We conclude that extubation failure after pediatric cardiac surgery due to cardiorespiratory failure is a bad prognostic sign. Patients with high inotropic support and a low PaO2 prior to extubation are especially at risk and probably need careful evaluation before final extubation.
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Pediatric cardiology · Nov 2005
Elastin mutation screening in a group of patients affected by vascular abnormalities.
Supravalvular aortic stenosis is an uncommon but well-characterized congenital form of left ventricular outflow obstruction. The lesion involves the ascending aorta and often occurs in association with pulmonary arterial stenoses or stenoses of other arteries, especially at major branch points. It can occur sporadically, as an autosomal dominant condition, or as one component of Williams-Beuren syndrome. ⋯ We report the results obtained in a mutation screening of the elastin gene in 28 patients with supravalvular aortic stenosis and other vascular abnormalities. The aim of the screening was to characterize the molecular cause of this lesion. We have detected 11 changes, including nine polymorphisms and two novel putative missense mutations.