Changing the Odds: Survival Trends in Children With Down Syndrome and Congenital Heart Defects

Changing the Odds: Survival Trends in Children With Down Syndrome and Congenital Heart Defects 1024 683 Pam Georgiana
Smiling baby with Down syndrome

About half of the children born with Down syndrome also have congenital heart defects (CHD). As a transplant cardiologist, Lydia K Wright, MD, wanted to learn if CHD was still the most significant mortality risk for these patients before age five. [1]

portrait of Dr. Lydia Wright

“Our aim was to assess whether the survival rate of kids with Down syndrome and congenital heart defects was getting closer to the survival rate of kids with Down syndrome but no heart defects,” says Dr. Wright, who was a fellow at Children’s Hospital of Atlanta at the time.

With access to the Centers for Disease Control and Prevention’s (CDC) extensive databases, she got to work. The CDC has been collecting data through the Metropolitan Atlanta Congenital Defects Program, so Dr. Wright had a rich source of patient data to review for both short- and long-term outcomes.

Her study, published in The Journal of Pediatrics, reviewed more than 1,600 children with Down syndrome, born from 1979 to 2018. About half (764) of them had associated CHD. The data revealed that the five-year survival rate for patients with Down syndrome and CHD improved consistently for children born between 1980 and 2010, from 85% to 93%. The survival rate remained steady (96% to 95%) for children with Down syndrome but without CHD during that same time. Even better, for children born after 2010, having CHD was not linked to mortality through the age of five.

Dr. Wright, who is now a pediatric cardiologist in The Heart Center at Nationwide Children’s Hospital, also categorized the data based on the type of heart defect. After accounting for other risk factors, she linked atrioventricular septal defects to early (less than one year) and late mortality (after five years). Ventricular septal defects correlated with mortality between one and five years old. She also found that atrial septal defects associated with late mortality.

Over the past 40 years, the five-year survival gap between children with Down syndrome and those with and without CHD has narrowed. In fact, the most significant determinant for early mortality in children born today with Down syndrome is not CHD at all; it’s prematurity. Dr. Wright attributes this change to better treatment options.

“These kids are improving and living longer because we have more sophisticated surgeries and post-surgery care to correct defects. If a child with Down syndrome and a heart defect can make it to their fifth birthday, the consensus is that their long-term outlook is good,” she says.

Dr. Wright hopes to study the long-term outcomes between this population and children who have CHD but do not have Down syndrome. This could help physicians better evaluate the efficacy of transplants, which are not yet universal for kids with Down syndrome. Meanwhile, this latest study shows that the prognosis is improving for children with both Down syndrome and CHD.



[1] Wright LK, Stallings EB, Cragan JD, Pabst LJ, Alverson CJ, Oster ME. Narrowing the Survival Gap: Trends in Survival of Individuals with Down Syndrome with and without Congenital Heart Defects Born 1979 to 2018. Journal of Pediatrics. 2023;260:113523.

Image credit: Adobe Stock (header); Nationwide Children’s (portrait)

About the author

Pam Georgiana is a brand marketing professional and writer located in Bexley, Ohio. She believes that words bind us together as humans and that the best stories remind us of our humanity. She specialized in telling engaging stories for healthcare, B2B services, and nonprofits using classic storytelling techniques. Pam has earned an MBA in Marketing from Capital University in Columbus, Ohio.