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Subaortic stenosis in childhood: frequency of associated anomalies and surgical options
P A Penkoske1, R L Collins-Nakai, N F Duncan
1Department of Surgery, University of Alberta, Edmonton, Canada.
Insights
Surgical treatment for subaortic stenosis in children, often with associated heart defects, can be performed with low mortality. Early resection can effectively manage this condition and reduce aortic insufficiency.
Area of Science:
- Cardiology
- Pediatric Surgery
- Congenital Heart Disease
Background:
- Subaortic stenosis is a significant cause of left ventricular outflow tract obstruction in children.
- Associated cardiac anomalies are frequent, complicating surgical management.
- This study reviews surgical outcomes for children with subaortic stenosis.
Observation:
- Twenty-one children underwent surgical treatment for subaortic stenosis between 1984 and 1988.
- The mean age at operation was 7.3 years, with a wide range.
- Sixty-two percent of patients had associated anomalies, including valve defects and septal defects.
Findings:
- Surgical approaches included transaortic resection, transventricular resection, and modified Konno procedures.
- There were no early deaths, with one late death due to an aortopulmonary window.
- One patient required reoperation for residual gradient, and three had persistent aortic insufficiency.
Implications:
- Surgical resection of subaortic stenosis in children can be achieved with low mortality and morbidity.
- Early intervention may prevent or resolve aortic insufficiency in many cases.
- The heterogeneous nature of subaortic stenosis necessitates individualized surgical planning.
Abstract:
In a 4-year period from July 1984 to July 1988, 21 children required surgical treatment for subaortic stenosis. Age at operation ranged from 12 months to 17 years (mean, 7.3 +/- 4.8 years). Associated anomalies were common and occurred in 13 patients (62%), including aortic valve insufficiency (eight), and stenosis (two), mitral valve stenosis (two) and insufficiency (one), and pulmonary valve stenosis (two) and absent pulmonary valve syndrome (one). A ventricular septal defect was present in five patients, and coarctation and patent ductus arteriosus occurred in three patients each. One patient had a complete atrioventricular septal defect, and one had an aortopulmonary window. Six patients had 15 previous procedures. The left ventricular to aortic gradient ranged from 20 to 170 mm Hg (mean, 59 +/- 43 mm Hg). The operation consisted of a transaortic resection (17), transventricular septal defect resection (two), a modified Konno procedure or septoplasty with preservation of the aortic valve (one), and a combined aortoventriculoplasty with homograft reconstruction of the ascending aorta and coronary reimplantation (one). There were no early deaths. One patient required pacemaker insertion for heart block. Follow-up ranged from 1 to 48 months (mean, 26.4 +/- 11.6 months). There was one late death 2 years after operation in the child with the aortopulmonary window who had Heath Edwards Type IV changes on a lung biopsy specimen at the original operation. One patient required reoperation for a residual gradient of 34 mm Hg 1 year after resection. Three patients still have aortic insufficiency. No cases of iatrogenic aortic valve injury occurred. Patients with subaortic stenosis represent a heterogeneous group. Early resection and additional procedures can be performed with a low mortality rate and can eliminate aortic insufficiency in many cases.