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Is Down syndrome a risk factor for poor outcome after repair of congenital heart defects?
1Department of Pediatrics (Cardiology), Oregon Health Sciences University, Portland, USA.
Insights
Children with Down syndrome (DS) have higher mortality after heart surgery, particularly for complete atrioventricular septal defects. Other congenital heart defect repairs showed similar outcomes regardless of DS presence.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Down syndrome is frequently linked to congenital heart disease (CHD), increasing risks for perioperative and long-term mortality.
- Pulmonary hypertension can develop early in children with Down syndrome, further complicating cardiac conditions.
Purpose of the Study:
- To investigate the impact of Down syndrome on the outcomes of surgically repaired congenital heart disease.
- To analyze mortality risks associated with specific congenital cardiac malformations in patients with Down syndrome.
Main Methods:
- Utilized a population-based registry of Oregon residents undergoing reparative surgery for 14 types of CHD before age 18 (1958-present).
- Analyzed data from 3965 patients, including 289 (7%) with Down syndrome, comparing cardiac mortality rates.
Main Results:
- Only complete atrioventricular septal defects showed significantly higher perioperative mortality (13% vs 5%) and 20-year late cardiac mortality (20% vs 5%) in Down syndrome patients.
- Survival outcomes for other repaired cardiac malformations were comparable between children with and without Down syndrome.
Conclusions:
- Down syndrome significantly increases mortality risk only for complete atrioventricular septal defect repairs.
- Most other common congenital heart defect repairs have similar outcomes for children with and without Down syndrome.
Abstract:
Down syndrome is commonly associated with significant congenital heart disease with the potential for early development of pulmonary hypertension. As such, children with Down syndrome may be at increased risk for both perioperative and long-term mortality. The purpose of this study, using data collected from a population-based outcomes study, is to analyze the potential role that Down syndrome plays in the outcome of surgically "corrected" congenital heart disease. Data were collected from a registry of all Oregon residents who, in the period 1958 to the present, had a reparative operation for one of 14 congenital cardiac malformations when younger than 18 years (N = 3965 patients). Down syndrome was present in 289 (7%) of the total registry patients. In evaluating the cardiac mortality associated with Down syndrome for each of the repaired cardiac malformations, only complete atrioventricular septal defect was associated with significantly higher perioperative (13% vs 5%) as well as higher overall late cardiac mortality through 20 years after the operation (20% vs 5%; p = 0.04). The survival outcomes for each of the other cardiac malformations were similar for children with and without Down syndrome.