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Natural and modified history of complete atrioventricular septal defect--a 17 year study
P Frontera-Izquierdo1, G Cabezuelo-Huerta
1Department of Paediatrics, Hospital Infantil Universitario La Fe, Valencia, Spain.
Insights
Early surgical repair of isolated complete atrioventricular septal defect significantly improves survival and functional outcomes. Delayed medical management leads to poor prognosis and severe heart disease.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Isolated complete atrioventricular septal defect is a complex congenital heart defect.
- It represents a significant subset of congenital heart disease cases.
- Down's syndrome is frequently associated with this condition.
Purpose of the Study:
- To evaluate the outcomes of surgical intervention for isolated complete atrioventricular septal defect.
- To compare early versus later surgical repair strategies.
- To assess the long-term prognosis for medically managed patients.
Main Methods:
- Retrospective review of 103 cases of isolated complete atrioventricular septal defect.
- Analysis of surgical interventions including pulmonary artery banding and complete repair.
- Comparison of outcomes between two distinct surgical periods (1971-1982 and 1983-1988).
- Long-term follow-up of medically treated patients.
Main Results:
- Surgical mortality for complete correction decreased from 88.8% (1971-1982) to 43.2% (1983-1988).
- Mean age at complete correction decreased from 23 to 13 months between the periods.
- Five-year actuarial survival improved to 46.8% in the later period.
- Medically treated survivors developed severe pulmonary vascular obstructive disease and functional decline.
Conclusions:
- Early complete surgical repair is crucial for improving survival in patients with isolated complete atrioventricular septal defect.
- Timely intervention significantly enhances long-term functional capacity.
- Medical management alone results in poor outcomes and disease progression.
Abstract:
We reviewed 103 cases of isolated complete atrioventricular septal defect. These cases represented 4.4% of the cases of congenital heart disease diagnosed in our hospital by catheterisation and angiography during 1971-88. Most children (n = 76) had Down's syndrome. Banding of the pulmonary artery was performed in seven cases and complete repair in 67 cases. In the period 1971-82 the complete correction was performed at a mean age of 23 months with a surgical mortality of 88.8%. In the period 1983-8 the mean age at complete correction was 13 months, the mortality 43.2%, and the five year actuarial survival was 46.8%. The 22 patients that survived after complete correction were in functional classes I and II of the New York Heart Association classification. After a mean follow up of 10 years only eight (36%) of the 22 who were followed up and treated medically survived; all had developed pulmonary vascular obstructive disease and were in functional classes III or IV. Our findings stress the importance of early complete surgical repair.