Is Down syndrome a risk factor for poor outcome after repair of congenital heart defects?

• Published in: The Journal of pediatrics Vol. 132; no. 4; pp. 738 - 741
• Main Authors: RELLER, M. D, MORRIS, C. D
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier 01-04-1998
• Subjects: Adolescent; Biological and medical sciences; Cardiac Surgical Procedures - mortality; Child; Child, Preschool; Chromosome aberrations; Down Syndrome - epidemiology; Heart Defects, Congenital - mortality; Heart Defects, Congenital - surgery; Humans; Infant; Medical genetics; Medical sciences; Oregon - epidemiology; Registries; Risk Factors; Survival Analysis; Survival Rate; Treatment Outcome; United States; North America; America; Oregon; Chromosomal aberration; Human; Short term; Congenital; Mortality; Clinical form; Aneuploidy; Cardiovascular disease; Infant; Down syndrome; Congenital disease; Long term; Association; Newborn; Heart disease; Surgery; Adolescent; Child; Comparative study
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/S0022-3476(98)70372-5

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.