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[Congenital aortic stenosis]
1Department of Cardio-Thoracic Surgery, Kobe Children's Hospital, Kobe, Japan.
Insights
Management of pediatric congenital valvular aortic stenosis has advanced with surgical and balloon valvuloplasty techniques. Long-term outcomes for newer procedures like the Ross procedure require further study.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Aortic Valve Disease
Context:
- Congenital valvular aortic stenosis (AS) presents complex management challenges in children.
- Recent advancements offer improved survival rates for neonates with critical AS.
Purpose:
- To discuss current management strategies and controversies in pediatric congenital valvular aortic stenosis.
- To compare the efficacy and indications of surgical open valvotomy versus balloon valvuloplasty.
- To evaluate newer techniques like annulus enlargement and the Ross procedure.
Summary:
- Improved survival reported for neonates with critical AS via surgical open valvotomy and balloon valvuloplasty, though direct comparison is pending.
- Extended aortic valvuloplasty shows promise for recurrent AS/insufficiency, but requires longer follow-up.
- Annulus enlargement technique (since 1991) allows larger valve insertion without significant risks.
- The Ross procedure demonstrates favorable early outcomes (mortality <5%, survival 80-90% at 4-8 years).
Impact:
- Provides an overview of current and evolving treatment options for pediatric aortic stenosis.
- Highlights the need for extended follow-up to assess long-term efficacy and potential complications of various procedures.
- Informs clinical decision-making regarding the optimal management of congenital valvular aortic stenosis in children.
Abstract:
Recent advances in and controversies concerning the management of children with congenital valvular aortic stenosis are discussed. In neonates with critical aortic stenosis, improved survival has recently been reported after surgical open valvotomy and balloon valvuloplasty, although it is difficult at this point to compare the results of the two procedures and determine their differential indications. Good results have also been achieved after extended aortic valvuloplasty for recurrent aortic stenosis and/or insufficiency, but the length of follow-up in these patients is still short. The technique first reported in 1991 for bilateral enlargement fo a small annulus permits the insertion of an aortic valve 3-4 sizes larger than the native annulus. It entails no risk of distorting the mitral valve, damaging the conduction system or important branches of the coronary arteries, or resulting in left ventricular dysfunction. The Ross procedure is now widely applied in the West, with reports of early mortality rates of less than 5% and event-free survival rates of 80-90% during follow-up of 4-8 years. Longer follow-up and continued careful evaluation are required to resolve the issue of possible dilatation and subsequent neoaortic valve dysfunction and pulmonary stenosis due to allograft degeneration after pulmonary autograft root replacement in children.