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How do babies with genetic abnormalities tolerate heart surgery?

June 9, 2009

This abstract reports that newborns with a range of genetic abnormalities had the same rates of hospital mortality after heart surgery as those babies without genetic abnormalities. There were some overall differences in recovery stats. Should the medical community, as a whole, reassess refusal of treatment for newborns with genetic syndromes?

Do Neonates with Genetic Abnormalities Have an Increased Morbidity and Mortality following Cardiac Surgery?

Authors: Simsic, Janet M.; Coleman, Karlene1; Maher, Kevin O.2; Cuadrado, Angel2; Kirshbom, Paul M.3

Source: Congenital Heart Disease, Volume 4, Number 3, May/June 2009 , pp. 160-165(6)

Publisher: Blackwell Publishing

Abstract:

Introduction.  

Genetic 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. Methods.  

Retrospective 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). Results.  

Neonates 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. Conclusion.  

Neonates 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. Abstract:

Introduction.  

Genetic 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. Methods.  

Retrospective 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). Results.  

Neonates 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. Conclusion.  

Neonates 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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