Survivorship trends for children and young adults with congenital heart disease

1. Based on a Swedish registry, survivorship for children and young adults with congenital heart disease (CHD) improved for children since 1970, with the largest improvements occurring in the cohort born between 1990 to 1993.

2. Despite improvement in outcomes, mortality amongst those with congenital heart disease remains significantly higher compared with the general population.

Evidence Rating Level: 2 (Good)

Study Rundown: Improvements in surgical techniques, and neonatal and perioperative care have led to substantial improvements in short term survival for children born with congenital heart disease (CHD). This prospective study using the nationwide registry for CHD in Sweden sought to describe trends in mortality for children with CHD over time and compared to matched controls from the general population.

There were 21 982 patients with CHD born between 1970 and 1993 in Sweden that were identified in the registry. Mortality for the CHD group was higher compared to the general population matched-control group, with both higher- and lower-risk congenital anomaly groups experiencing increased mortality. Mortality was highest for children aged 4-years and under. Through the different time periods, mortality for younger age groups decreased for children born in the 1990s compared to 1970s. Young adults (>18 years) demonstrated no improvement in survivorship through the different time periods. The strengths of the study included the design (large, population-wide study) and the long-term follow-up. A major limitation of the study was the reliance on diagnostic codes and databases to identify patients, which may be incomplete, especially for the older outpatient registries.

Click to read the study, published today in JAMA Internal Medicine

Relevant Reading: Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre

In-Depth [case-control study]: This study used data from the Swedish National Patient Registry and the National Hospital Outpatient Register to enroll people born between 1970 and 1993 who had a diagnosis of CHD as identified by diagnostic codes in discharges summaries, operative reports, or other records. Each CHD patient was matched by birth year, sex, and county to 9-10 control individuals without a diagnosis of CHD. CHD was categorized by hierarchy into 5 groups based on underlying anatomic pathology.

For individuals with CHD, mortality was higher compared to controls (HR 16.51, 95%CI 15.29-17.83; p < 0.001). In the youngest age group (≤4 years), HR for mortality decreased from 1970-1979 to 1990-1993 (HR 225.84, 95%CI 136.84-372.70; to 33.47, 95%CI 22.54-49.70). For young adults, the hazard ratios for mortality did not significantly decrease over time: 1970 to 1979 HR 7.79 (95%CI 6.72-8.96),1980-1989 HR4.91 (95%CI 3.99-6.02), and 1990-1993 HR 4.26 (95%CI 2.29-7.94).

Image: PD

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