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. 2024 Apr 17;14(4):e083237.
doi: 10.1136/bmjopen-2023-083237.

Outcomes after cancer diagnosis in children and adult patients with congenital heart disease in Sweden: a registry-based cohort study

Christina Karazisi  1   2 , Mikael Dellborg  3   2   4 , Karin Mellgren  5 , Kok Wai Giang  3   2 , Kristofer Skoglund  3 , Peter Eriksson  3   2   4 , Zacharias Mandalenakis  3   2   4
Affiliations

Outcomes after cancer diagnosis in children and adult patients with congenital heart disease in Sweden: a registry-based cohort study

Christina Karazisi et al. BMJ Open. .

Abstract

Objective: Patients with congenital heart disease (CHD) have an increased cancer risk. The aim of this study was to determine cancer-related mortality in CHD patients compared with non-CHD controls, compare ages at cancer diagnosis and death, and explore the most fatal cancer diagnoses.

Design: Registry-based cohort study.

Setting and participants: CHD patients born between 1970 and 2017 were identified using Swedish Health Registers. Each was matched by birth year and sex with 10 non-CHD controls. Included were those born in Sweden with a cancer diagnosis.

Results: Cancer developed in 758 out of 67814 CHD patients (1.1%), with 139 deaths (18.3%)-of which 41 deaths occurred in patients with genetic syndromes. Cancer was the cause of death in 71.9% of cases. Across all CHD patients, cancer accounted for 1.8% of deaths. Excluding patients with genetic syndromes and transplant recipients, mortality risk between CHD patients with cancer and controls showed no significant difference (adjusted HR 1.17; 95% CI 0.93 to 1.49). CHD patients had a lower median age at cancer diagnosis-13.0 years (IQR 2.9-30.0) in CHD versus 24.6 years (IQR 8.6-35.1) in controls. Median age at death was 15.1 years (IQR 3.6-30.7) in CHD patients versus 18.5 years (IQR 6.1-32.7) in controls. The top three fatal cancer diagnoses were ill-defined, secondary and unspecified, eye and central nervous system tumours and haematological malignancies.

Conclusions: Cancer-related deaths constituted 1.8% of all mortalities across all CHD patients. Among CHD patients with cancer, 18.3% died, with cancer being the cause in 71.9% of cases. Although CHD patients have an increased cancer risk, their mortality risk post-diagnosis does not significantly differ from non-CHD patients after adjustements and exclusion of patients with genetic syndromes and transplant recipients. However, CHD patients with genetic syndromes and concurrent cancer appear to be a vulnerable group.

Keywords: CARDIOLOGY; Congenital heart disease; Epidemiology; ONCOLOGY; Prognosis.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Cancer diagnoses in (A) congenital heart disease (CHD) patients and (B) controls without CHD.
See this image and copyright information in PMC

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