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Cardiac surgery in Down syndrome
A Sheehan1, O C Ward, D F Duff
1Department of Cardiology, Our Lady's Hospital for Sick Children, Dublin.
Insights
This study analyzed cardiac surgery outcomes for 42 children with Down syndrome and congenital heart disease between 1976-1987. A conservative surgical approach was adopted, resulting in a 6.6% overall mortality rate.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Down Syndrome Research
Background:
- Down syndrome is frequently associated with congenital heart defects.
- Surgical management of these complex conditions presents unique challenges.
- Previous outcomes data for this specific population were limited.
Purpose of the Study:
- To evaluate the outcomes of cardiac surgery in children with Down syndrome and congenital heart disease.
- To assess the mortality and morbidity associated with different surgical procedures.
- To inform surgical decision-making and patient management strategies.
Main Methods:
- Retrospective review of 42 pediatric patients with Down syndrome and congenital heart disease undergoing cardiac surgery between 1976 and 1987.
- Analysis of operative procedures, including repair of patent ductus arteriosus and atrioventricular canal defects.
- Calculation of operative mortality rates for specific procedures and the overall series.
Main Results:
- The most common procedure was patent ductus arteriosus repair.
- Four patients (9.5%) died post-operatively.
- Overall mortality was 6.6%, with higher rates for complex repairs like complete atrioventricular canal defects.
Conclusions:
- A conservative surgical approach was adopted due to factors including life expectancy and lesion complexity in Down syndrome.
- Cardiac surgery in this cohort demonstrated a manageable mortality rate, particularly with less complex procedures.
- Findings support individualized, risk-stratified surgical management for children with Down syndrome and congenital heart disease.
Abstract:
Between January 1976 and December 1987 42 children with Down syndrome and congenital heart disease underwent cardiac surgery. Four children had two operations. Age at the time of surgery ranged from 11 days to 14 years. The commonest operative procedure was repair of a patent ductus arteriosus. Four patients died post-operatively, two following repair of a complete atrio-ventricular canal defect (CAVD), one following correction of tetralogy of Fallot in association with a CAVD, and a fourth following closure of ventricular septal defect and atrial septal defect. The mortality for those who had open heart surgery was 13.3% and for the series as a whole the mortality was 6.6% over a period of follow-up ranging from two months to four years. A relatively conservative approach has been adopted with regard to surgery, based on the shorter natural expectation of life in Down syndrome, the complexity of many of the cardiac lesions involved and the recognition of the frequency of early intellectual deterioration in Down patients.
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