• Janet M Simsic, Karlene Coleman, Kevin O Maher, Angel Cuadrado, and Paul M Kirshbom.
• Children's Healthcare of Atlanta-Sibley Heart Center Cardiology, Atlanta, GA 30341, USA. simsicj@kidsheart.com
• Congenit Heart Dis. 2009 May 1;4(3):160-5.

IntroductionGenetic abnormalities occur in approximately 20% of children with congenital heart disease. The purpose of this study was to evaluate the effect of genetic abnormalities on short-term outcomes following neonatal cardiac surgery.MethodsRetrospective review of all neonates (n = 609) undergoing cardiac surgery from January 2003 to December 2006. Genetic abnormalities were identified in 93 neonates (15%). Genetic abnormalities identified were 22q11.2 deletion (23), chromosomal abnormalities including various monosomies, trisomies, deletions, duplications, and inversions (17), dysmorphic undefined syndrome without recognized chromosomal abnormality (27), Down syndrome (9), laterality sequences (9), recognixed syndromes and genetic etiology including Mendelian (i.e. Alagille, CHARGE) (8).ResultsNeonates with genetic abnormalities had lower birth weights and were older at time of surgery. There was no difference in operative variables, duration of mechanical ventilation or ICU length of stay between the two groups. There was an increase in total hospital length of stay and postoperative complications in the neonates with genetic abnormalities. Importantly, in hospital mortality was not different.ConclusionNeonates with genetic abnormalities have a higher risk of postoperative complications and a longer hospital length of stay. However, there is no increase in hospital mortality. This information may aid in patient management decisions and parental counseling. Longer-term studies are needed for understanding the total impact of genetic abnormalities on neonates with congenital heart disease.

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