World journal for pediatric & congenital heart surgery
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World J Pediatr Congenit Heart Surg · Apr 2015
ReviewThe role of home monitoring in interstage management of infants following the Norwood procedure.
Although outcomes for infants with complex single ventricle heart defects have steadily improved in recent decades, there is still a significant risk for mortality and morbidity during the interstage period between stage 1 Norwood hospitalization discharge and stage 2 palliation. Home monitoring programs, which involve parental surveillance of daily weight and oxygen saturations during the interstage period, have been shown to significantly improve survival rates. This article describes the potential risk factors or causes of interstage mortality and reviews the role of home monitoring in early detection and potential prevention of adverse outcomes.
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World J Pediatr Congenit Heart Surg · Apr 2015
Review Biography Historical ArticleJaroslav Stark, MD, FRCS, FACS, FACC--celebrating an icon of pediatric cardiac surgery at age 80 years.
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Heterotaxy patients' hearts may or may not be suitable for biventricular repair depending on anatomy. Even in the subset that are amenable to surgical septation, cardiac anatomy may present multiple difficulties in achieving a satisfactory repair. However, it is also well known that heterotaxy patients are not ideal candidates for univentricular repair. ⋯ Satisfactory survival outcomes can be achieved in heterotaxy patients who undergo hemodynamically acceptable biventricular repair. Borderline ventricular hypoplasia and trivial atrioventricular valve regurgitation should not be considered as discouraging factors in anatomically suitable heterotaxy patients as it is possible to adopt a two-stage repair in such patients to achieve biventricular repair at a later stage. Anticipating a higher incidence of conduction problems and gut malrotation preemptively can help reduce the morbidity.
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World J Pediatr Congenit Heart Surg · Apr 2015
Case ReportsLeft coronary artery stenosis causing left ventricular dysfunction in two children with supravalvular aortic stenosis.
Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome (WBS) and other isolated elastin gene deletions. Cardiovascular manifestations associated with WBS are characterized by obstructive arterial lesions such as SVAS and pulmonary artery stenosis in addition to bicuspid aortic valve and mitral valve prolapse. However, coronary artery ostial stenosis may be associated with SVAS, and it increases the risk of sudden death and may complicate surgical management. In this report, we present our experience with two patients having SVAS and left coronary artery ostial stenosis with associated left ventricular dysfunction.