Key result
Congenital heart disease was associated with a significantly increased risk of cancer compared to matched controls (1.95% vs 0.94%; HR 2.24; 95% CI 2.01-2.48).
Why the study?
Adult patients with congenital heart disease have an increased cancer incidence, but the cancer risk from birth up to age 42 years compared to matched controls needed investigation.
Does congenital heart disease increase the risk of developing cancer in young patients compared to matched controls?
Cohort (n=241,798)
Does congenital heart disease increase the risk of developing cancer in young patients compared to matched controls?
Hazard Ratio: 2.24 (95% CI 2.01–2.48)
Absolute Event Rate: 1.95% vs 0.94%
Young patients with congenital heart disease have a more than twofold increased risk of developing cancer compared to the general population, suggesting a potential need for systematic screening in this vulnerable group.
May support enhanced cancer surveillance in congenital heart disease; hypothesis-generating and requires prospective validation.
Background Adult patients with congenital heart disease (CHD) have an increased incidence of cancer presumably due to repeated radiation exposure, genetic predisposition or a continued stress factor during heart interventions. Purpose We aimed to investigate the risk to be diagnosed with cancer from birth up to the age of 42 years in patients with CHD and compared to matched controls. Methods Using data from the Patient and Cause of Death Registers in Sweden, we identified successive cohorts of patients with CHD born 1970–79, 1980–89 and 1990–93. Each CHD patient was matched for birth year, sex and county with 10 controls without CHD from the general population with follow-up data and comorbidities collected from birth until 2011. Results We identified 21,982 patients with CHD and 219,816 matched controls of whom 428 (1.95%) and 2,072 (0.94%), respectively, were diagnosed with cancer over a mean follow-up of 26.2 and 27.5 years. The overall hazard ratio (HR) for cancer was 2.24 times (95% confidence interval [CI], 2.01–2.48) in children and young adults with CHD compared to controls; higher in men (HR 2.41 (95% CI, 2.08–2.79)) and among patients with isolated atrial or ventricular defects, as well as in the large group of miscellaneous anomalies. The cumulative incidence of cancer in patients with CHD increased by birth cohort with almost 2% among those born 1990–93 been diagnosed with cancer on reaching adulthood. Conclusions Young patients with CHD had more than 2 timmes increased risk to develop cancer compared to matched controls. At the age of 42, 1 out of 50 patients with CHD developed cancer and the risk was significantly higher in men with CHD. The patient groups with atrial and ventricular septal defects had a significantly increased risk of cancer and a systematic screening for cancer could be considered to this vulnerable group of patients. Acknowledgement/Funding By grants from the Swedish state under the agreement concerning research and education of doctors, the Swedish Research Council.
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Mandalenakis et al. (2019) conducted a cohort in Congenital heart disease (n=241,798). Congenital heart disease vs. Matched controls without congenital heart disease was evaluated on Cancer diagnosis (HR 2.24, 95% CI 2.01-2.48). Congenital heart disease was associated with a significantly increased risk of cancer compared to matched controls (1.95% vs 0.94%; HR 2.24; 95% CI 2.01-2.48).
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