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High mortality risk among children, young adults with CHD in Sweden

Although survivorship has increased significantly in children and young adults with congenital heart disease, their all-cause mortality risk is still high compared with matched controls, according to a study conducted in Sweden.

“Congenital heart disease (CHD) is one of the most common types of birth defects present in approximately 1% of children born alive,” Zacharias Mandalenakis, PhD, from the department of molecular and clinical medicine/cardiology at Sahlgrenska University Hospital, and colleagues wrote.

“However, there are limited data on survival trends in children and young adults with CHD compared with the general population,” they added.

Mandalenakis and colleagues performed a registry-based, prospective, matched-cohort study to investigate the survivorship in children and young adults with CHD relative to their matched controls. Patients born between Jan. 1, 1970 and Dec. 31, 1993 who were registered in the Swedish national patient and cause of death registries with a diagnosis of CHD were identified and enrolled in the study (n = 21,982; 48.4% female; median age at index registration, 4.22 years). The researchers collected follow-up and comorbidity data until Dec. 31, 2011. They used the Cox proportional hazards model for survival analyses and followed up with patients at a mean of 27 years. Each participant had 10 randomly selected matched controls (n = 219,816) from the general population based on birth year, sex and county.

Results indicated that among children younger than 5 years, survivorship increased from 96% among those born from 1970 to 1979, to 98% in those born in 1990 to 1993. Compared with matched controls, hazard ratios (HRs) of death declined from 225.84 (95% CI, 136.84-372.7) in those born from 1970 to 1979, to 33.47 (95% CI, 22.54-49.7) in those born from 1990 to 1993. Older participants displayed a less distinct, yet still substantial increase in absolute and relative survivorship (HRs: 24.52 [95%CI, 11.72-51.26] at 5-9 years vs. 4.27 [95%CI, 2.29-7.95] at 18-29 years).

Overall, the all-cause mortality risk in children and young adults with CHD was almost 17 times greater than their matched controls. The highest risk for death was observed in patients with the most severe complex defects, such as common arterial trunk, transposition of the great vessels, double inlet ventricle, hypoplastic left heart syndrome, tetralogy of Fallot and atrioventricular septal defect.

“Our study results show that the pediatric and adult care has been improved in Sweden, as in other western countries, over the last decades,” Mandalenakis told Healio Internal Medicine. “Young patients with congenital heart disease are still a vulnerable group of patients and due to increasing survivorship, the risk of acquired diseases is also increased. Despite that, the risk of mortality has been decreased and the relative risk in relation to people of similar age and gender is much higher. Physicians must be aware that any patient presenting with a history of CHD 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.”

“However, further research on reducing the death rate in this vulnerable group of patients is required,” he concluded. “Can meticulous care of conventional risk factors for cardiovascular disease, i.e. blood pressure, glucose metabolism, physical activity and cholesterol, decrease the risk for patients with congenital heart disease, should they be the focus of special interest and special care in order to avoid these complications, if they can indeed be avoided?” – by Alaina Tedesco

Disclosure: The authors report receiving support from the Swedish state, the Swedish Heart and Lung Foundation, the Swedish Research Council and the Swedish Council for Health, Working Life, and Welfare.