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Surgical treatment of idiopathic hypertrophic subaortic stenosis in children
Insights
Pediatric idiopathic hypertrophic subaortic stenosis (IHSS) surgery is safe and effective. Early surgical intervention for IHSS in children may improve outcomes, especially with significant pressure gradients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Medical Genetics
Background:
- Idiopathic hypertrophic subaortic stenosis (IHSS) is a progressive left ventricular outflow tract obstruction.
- Surgical intervention in childhood for IHSS is considered, but its impact on natural history is under investigation.
Purpose of the Study:
- To evaluate the safety and efficacy of surgical interventions for pediatric IHSS.
- To determine optimal timing for surgical consideration in children with IHSS.
Main Methods:
- Retrospective analysis of five pediatric patients with IHSS who underwent surgery.
- Procedures included transaortic left ventricular myotomy/myectomy and right ventricular myectomy.
- Patient ages ranged from 4 years 5 months to 14 years.
Main Results:
- All five pediatric patients survived the surgical procedures.
- Transaortic left ventricular myotomy/myectomy demonstrated safety and effectiveness.
- Surgical outcomes were favorable for localized muscular subaortic stenosis.
Conclusions:
- Transaortic left ventricular myotomy or myectomy is a safe and effective surgical option for pediatric IHSS.
- Early surgical intervention in childhood should be considered for progressive IHSS with excessive pressure gradients.
- Surgery is recommended for children with significant increases in left ventricular outflow pressure gradient upon provocation, even if resting gradients are below 50 mmHg.
Abstract:
Five children with idiopathic hypertrophic subaortic stenosis (IHSS) were operated upon during the past 7 years and all survived. Their ages ranged from 4 years and 5 months to 14 years (mean 8 years and 5 months). The operations performed were 1 right ventricular myectomy and 4 transaortic left ventricular myotomies. Whether surgery performed in childhood would change the natural history or not is still unknown, however, according to our results and many available reports, we have so far reached the following conclusions; 1. Transaortic left ventrlcular myotomy or myectomy is a safe and effective operation for localized muscular subaortic stenosis in children. 2. Because the disease is progressive, operation in childhood may be worth to be considered, when the resting or provocative pressure gradient is excessive. 3. For those who have a marked increase in left ventricular outflow pressure gradient by the provocation, operation would be considered even though the gradient at rest is less than 50 mmHg.