Researchers at UC Davis Children's Hospital have shown that children with Down syndrome have significantly better in-hospital survival rates after surgeries for congenital heart disease (CHD) than their genetically typical peers.
While post-surgical mortality rates for children with Down syndrome have been studied before, this is by far the most comprehensive effort to date. Using the Kids' Inpatient Database (KID), the researchers looked at results from almost 80 different procedures, performed on more than 50,000 children across the United States during 2000, 2003, 2006 and 2009.
The overall in-hospital mortality rate for children with Down children was 1.9 percent as compared to 4.3 percent for children without Down syndrome. The paper was published in the journal Circulation: Cardiovascular Quality and Outcomes.
"We've known for some time that children with Down syndrome do better after certain procedures," said lead author Jacqueline Evans, assistant professor at UC Davis Children's Hospital. "But even when you correct for surgical risk, prematurity, the presence of a major non-cardiac structural abnormality and age at the time of surgery, children with Down syndrome have lower in-hospital mortality rates across a wide spectrum of repairs."
In order to adjust for differences in patient mix, the team used the Risk Adjustment for Congenital Heart Surgery (RACHS-1) tool, which stratifies patients into six categories, based on degree of surgical risk.
Although children with Down syndrome had better survival rates across all surgeries, there were variations between individual procedures. The mechanisms behind the improved survival for children with Down children will require further study; however, there is some evidence that anatomical differences and resistance to oxidative stress may be contributing factors.
Even though these results are unlikely to affect clinical practice, they may be helpful while counseling prospective parents. Information from the KID can also be used to address other clinical issues surrounding children with CHD.
"Now that we've done the work to create this database for pediatric patients with CHD, we have a very powerful tool that can be used to ask a host of different questions," said Evans. "If we have a question generated by our experience at the bedside, we can use this database to rapidly query a much larger sample."Other researchers included Madan Dharmar, Erin Meierhenry, James P. Marcin and Gary W. Raff.