Pediatric cardiology
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Pediatric cardiology · Mar 2002
Outcome of 121 patients with congenitally corrected transposition of the great arteries.
Congenitally corrected transposition of the great arteries (ccTGA) is a rare disorder with reduced survival that is influenced by the presence of associated anomalies, tricuspid regurgitation (TR), and right ventricular (RV) function. The double switch procedure has been proposed as an aggressive surgical approach in selected patients. We sought to review our experience with conventional repair to determine if a change in surgical strategy was warranted. ⋯ This is the largest nonselected cohort of patients with ccTGA followed at a single center. Our results confirm that significant TR and poor RV function are risk factors for poor outcome and provide convincing evidence that patients undergoing conventional biventricular repair are at higher risk for deterioration of tricuspid valve and right ventricular function compared to palliated or unoperated patients. We support a move toward an alternative surgical approach (double switch procedure) in carefully selected patients.
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Pediatric cardiology · Mar 2002
Case ReportsResolution of acquired pulmonary arteriovenous malformations in a patient with total anomalous systemic venous return.
An 11-year-old male with total anomalous systemic venous return had surgical repair except for the hepatic venous return, which drained to the left atrium. He developed progressive cyanosis and fatigue and was diagnosed with large pulmonary arteriovenous malformations (PAVMs) during cardiac catheterization with the use of bubble contrast echocardiography. ⋯ This unique case report demonstrates the development of PAVMs with exclusion of hepatic venous return through the pulmonary vascular bed while pulsatile pulmonary blood flow remains intact. It reinforces the likelihood of the absence of an as yet unidentified hepatic vasoactive substance as the source for development of PAVMs.
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Pediatric cardiology · Mar 2002
Right ventricular performance in hypotensive preterm neonates treated with dopamine.
Systemic hypotension with left ventricular dysfunction is a common complication of neonatal respiratory distress syndrome and is often treated with inotropic agents. Although pulmonary hypertension with elevated pulmonary vascular resistance is also an important pathophysiological finding in respiratory distress syndrome, the effect of inotropes on the right ventricle has not been studied. The aim of this study was to assess changes in right ventricular dimensions and function with inotropic therapy in hypotensive preterm infants. ⋯ Right ventricular ejection fraction increased significantly from 0.36 (0.29-0.46) to 0.51 (0.43-0.53) ( p < 0.01). There was a trend toward an increase in right ventricular output from 90 ml/kg/min (67-115 ml/kg/min) to 112 ml/kg/min-143 ml/kg/min) (p=0.07). Dopamine increases right ventricular ejection fraction through a reduction in right ventricular end systolic volume.