Survival of Kids With Congenital Heart Disease Climbs, but Risks Remain

Larry Hand

December 19, 2016

GOTHENBURG, SWEDEN — Survival in children and young adults with congenital heart disease has increased "substantially" in Sweden, but mortality risk remains high compared with matched controls, according to new research[1].

"According to our national registry data in Sweden, the survivorship in children and young adults with congenital heart disease has been increased the past 40 years in Sweden," Dr Zacharias Mandalenakis (Sahlgrenska University Hospital) told heartwire from Medscape.

"Although the mortality risk has decreased by more than 100 times compared with controls, children with congenital heart disease younger than 5 years and born in the beginning of the 1990s still have a mortality risk more than 33 times higher than matched controls from the general population," he said.

Mandalenakis and colleagues analyzed data from the Swedish national patient and cause-of-death registers to identify individuals with congenital heart disease born between the beginning of 1970 and the end of 1993. They identified 21,982 patients (51.6% males, median age 4.22 years) with congenital heart disease in Sweden and matched them with 219,816 controls.

The results were published online December 19, 2016  in JAMA Internal Medicine.

They found that survival for children younger than 5 increased from 96% in children born in 1970–1979 to 98% in children born 1990–1993 and that hazard ratios (HRs) of death compared with controls decreased from 225.84 (95% CI 136.84–372.70) to 33.47 (95% CI 22.54–49.70).

Older patients had better survival (HR 24.52, 95% CI 11.72–51.26 for 5–9 years; HR 4.27, 95% CI 2.29–7.95 for 18–29 years). People with the most complex defects had the highest death risk (HR 64.07, 95% CI 53.39–76.89).

After a mean follow-up of 27 years, 1673 patients with CHD had died compared with 1077 controls after a mean follow-up of 28.5 years. Of the patients with congenital heart disease, 8352 (38%) underwent one or more cardiac surgical procedures during 42 years of follow-up, 245 (2.9%) of whom died postoperatively.

"Our study results show that the pediatric and adult care has been improved in Sweden, also as in the Western countries, the past decades," Mandalenakis told heartwire .

"Physicians must be aware that any patient presenting with a history of congenital heart disease is at increased risk of having a complication of their congenital condition. A bit of reverse thinking is applied when you discuss this with a patient with a congenital heart condition. The outlook for him/her has much improved, and the absolute risk of having a severe complication is very low," he said.

"What makes our study so special?" he asked. "It is a large, national cohort, with a large number of controls, covering 25 years of evolution and from a country with national, high-quality registers and centralized, public healthcare available for everyone."

The Swedish government, the Swedish Heart and Lung Foundation, the Swedish Research Council, and the Swedish Council for Health, Working Life, and Welfare supported this research. The authors reported no relevant financial relationships.

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