Mortality after cancer diagnosis among children with congenital heart disease in Denmark and Sweden

Abstract

Background: Recent decades have witnessed tangible improvements in childhood cancer survival. However, the prognosis for children with congenital heart disease (CHD), the most prevalent birth defect, remains unclear. Due to improved survival of CHD and childhood cancer, evaluating outcomes within this intersection is important for clinical practice. We aimed to assess mortality post-cancer diagnosis among children with CHD.

Methods: We conducted a study on the population of Denmark and Sweden, born 1970-2014, with a cancer diagnosis before age 20 in the national cancer registers (end of follow-up 2015; n = 20 665). CHD diagnoses (n = 397) and recorded deaths were retrieved from national health registers. We evaluated the effect of CHD on 5-year mortality post-cancer diagnosis fitting Cox proportional hazards regression.

Results: When excluding children with Down syndrome, children with CHD had a higher 5-year mortality post-cancer diagnosis compared to children without (hazard ratio [HR] 1.48, 95% confidence interval [CI] = 1.18 to 1.86). This was particularly notable in children with lymphoma (HR = 2.17, 95% CI = 1.11 to 4.25) and neuroblastoma (HR = 2.39, 95% CI = 1.11 to 5.15). In more recent decades (post-1990), children with CHD had similar 5-year mortality as their counterparts without, except for children diagnosed with lymphoma, where mortality remained elevated (HR = 3.37, 95% CI = 1.65 to 6.89).

Conclusions: In this large, register-based cohort study, children with CHD fared worse post-cancer diagnosis-particularly lymphoma and neuroblastoma. While a more positive trend emerged in recent years, lymphoma-related mortality remained disproportionately high among children with CHD, underscoring the need for continued research and interventions to improve outcomes for this vulnerable group.

Figure 1.

Five-year overall survival from childhood cancer. Row 1 (A and B) displays survival for all cancer diagnoses across the entire study period, while Row 2 (C and D) shows survival for cancers diagnosed after 1990. (A) and (C) compare survival between children with CHD and those without CHD. (B) and (D) compare survival between children with CHD, excluding those with DS, and children without CHD or DS. Abbreviations: CHD = congenital heart disease; DS = Down syndrome.

Figure 2.

Five-year overall survival from childhood cancer among children with varying combinations of CHD and DS. (A) shows survival for all cancer diagnoses across the entire study period, while (B) focuses on cancers diagnosed after 1990. The risk table is omitted due to Statistics Denmark regulations requiring a minimum difference of 5 individuals between each year to prevent identification of individuals. Abbreviations: CHD = congenital heart disease; DS = Down syndrome.