Cardiology in the young
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Cardiology in the young · Oct 2000
Outcomes for children with cardiomyopathy awaiting transplantation.
To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies. ⋯ Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.
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Cardiology in the young · Sep 2000
Transesophageal echocardiographic predictors for successful transcatheter closure of defects within the oval fossa using the CardioSEAL septal occlusion device.
To define the utility of transesophageal echocardiography in predicting the likelihood of a successful procedure and residual shunting in patients undergoing transcatheter closure of defects within the oval fossa using the CardioSEAL device. ⋯ Transesophageal echocardiography allows excellent assessment of the oval fossa and deficiencies of its floor in all of their dimensions. It is an important tool for guiding the deployment of the occlusion device in patients undergoing attempted transcatheter closure of defects within the fossa. Contrast echocardiography should be used for optimal detection of residual shunting.
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Cardiology in the young · May 2000
Comparative StudyGrowth of children with a functionally single ventricle following palliation at moderately increased altitude.
The bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. ⋯ Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.
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Cardiology in the young · May 2000
Comparative StudyPsychological distress and styles of coping in parents of children awaiting elective cardiac surgery.
We sought to assess the level of psychological distress, and the styles of coping of, parents of children with congenital heart disease. The study was based on questionnaires, which were completed, on average, four weeks, with a range from 0.1 to 22.1 weeks, prior to elective cardiac surgery or elective catheter intervention. ⋯ Elevated levels of psychological distress, and less adequate styles of coping, were found in the parents of patients about to undergo cardiac surgery, especially the mothers, when compared to reference groups. Future research should investigate whether these difficulties persist, and whether this will influence the emotional development of their children with congenital cardiac malformations.
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Cardiology in the young · Sep 1999
Case ReportsInterruption of the aortic arch at the isthmus with DiGeorge syndrome and 22q11.2 deletion.
A 6-day-old male with interruption of the aortic arch at the isthmus (type A) had the typical phenotype of DiGeorge syndrome. There was also a doubly committed juxta-arterial ventricular septal defect and an unobstructed left ventricular outflow tract. Hypoplasia of the thymus was confirmed during a modified Blalock-Park operation. ⋯ He was subsequently revealed by the use of fluorescence in situ hybridization analysis to have 22q11.2 deletion. Interruption of the aortic arch at the isthmus is presumed to reflect abnormal fetal hemodynamics, and is considered a distinct pathogenetic entity from interruption between the left common carotid and subclavian arteries, the latter being the variant more frequently associated with DiGeorge syndrome. In our case, the 22q11.2 deletion likely played a major role in the etiology of the interrupted aortic arch.