Abstract

BackgroundThe relationship between congenital heart disease (CHD) and malignancies has not been determined. This study aimed to explore the association of CHD with malignancies and examine the risk factors for the development of cancer after a diagnosis of CHD.Patients and MethodsThis nationwide, population-based cohort study on cancer risk evaluated 31,961 patients with newly diagnosed CHD using the Taiwan National Health Insurance Research Database (NHIRD) between 1998 and 2006. The standardized incidence ratios (SIRs) for all and specific cancer types were analyzed, while the Cox proportional hazard model was used to evaluate risk factors of cancer occurrence.ResultsAmong patients with newly diagnosed CHD regardless of ages, 187 (0.6%) subsequently developed cancers after a diagnosis of CHD. Patients with CHD had increased risk of cancer (SIR, 1.45; 95% CI, 1.25–1.67), as well as significantly elevated risks of hematologic (SIR, 4.04; 95% CI, 2.76–5.70), central nervous system (CNS) (SIR, 3.51; 95% CI, 1.92–5.89), and head and neck (SIR, 1.81; 95% CI, 1.03–2.94) malignancies. Age (HR, 1.06; 95% CI, 1.05–1.06) and co-morbid chronic liver disease (HR, 1.91; 95% CI, 1.27–2.87) were independent risk factors for cancer occurrence among CHD patients.ConclusionPatients with CHD have significantly increased cancer risk, particularly hematologic, CNS, and head and neck malignancies. Physicians who care for patients with CHD should be aware of their predisposition to malignancy after the diagnosis of CHD. Further studies are warranted to clarify the association between CHD and malignancies.

Highlights

  • Congenital heart disease (CHD) is a gross structural abnormality of the heart or intra-thoracic great vessels that is present at birth and manifests as with actual or potential functional significance [1]

  • Patients with congenital heart disease (CHD) had increased risk of cancer (SIR, 1.45; 95% confidence interval (CI), 1.25–1.67), as well as significantly elevated risks of hematologic (SIR, 4.04; 95% CI, 2.76–5.70), central nervous system (CNS) (SIR, 3.51; 95% CI, 1.92–5.89), and PLOS ONE | DOI:10.1371/journal.pone

  • Cancer Risk and Congenital Heart Disease head and neck (SIR, 1.81; 95% CI, 1.03–2.94) malignancies

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Summary

Introduction

Congenital heart disease (CHD) is a gross structural abnormality of the heart or intra-thoracic great vessels that is present at birth and manifests as with actual or potential functional significance [1]. Previous studies have shown that patients with congenital anomalies have increased risk of developing cancer, such as leukemia, lymphoma, brain tumor, neuroblastoma, germ cell tumor, retinoblastoma, and soft tissue sarcoma [14,15,16,18,19,20,21,22,23,24,25]. Most of these studies have focused on the association of all categories of congenital anomalies with cancer.

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