Abstract 3305: Cancer mortality in children surviving congenital heart surgery: A study from the pediatric cardiac care consortium
Authors: Thomas Amanda S. , Spector Logan , Kochilas Lazaros K. 
Published: August 2019

Abstract
Introduction: Children with congenital heart defects (CHD) are a unique population with abbreviated survival compared to the general population. Case-control studies have suggested an elevated risk of childhood cancer in children with CHD. This has not been evaluated in prospective studies nor has risk of cancer in adult survivors of CHD. This study describes mortality from cancer among survivors of CHD in the Pediatric Cardiac Care Consortium (PCCC).
Methods: We performed a retrospective cohort study of individuals who underwent surgery for CHD at 0-21 years of age in the PCCC during 1982-2003. Patients surviving their first CHD surgery (n= 35,998) were linked to the National Death Index (NDI) through 2014. Cancer mortality was compared to that in the general population using standardized mortality ratios (SMRs) matched on age, year, and sex.
Results: Seventy patients were identified as having an underlying cause of death as cancer and surviving a CHD operation in the PCCC (males, n=38). Of these, 17 (24.3%) had a diagnosis of Trisomy 21. Deaths occurred over a median follow-up of 8.81 years (IQR: 5.9-15.8) post hospital discharge with median age at death of 13.5 years (IQR: 7.0-20.0). Overall adjusted SMRs for deaths relating to cancer were 2.75 (95% CI: 2.11-3.40) and increased for those with a chromosomal abnormality (2.31 to 6.76). Decrease in mortality was observed the older the patient was at CHD surgery (neonate to adolescence: 5.03 to 1.65 respectively). Increased mortality in surgically severe CHDs (SMR: 4.75; 95% CI: 2.26-7.23) was seen.
Conclusions: The PCCC-NDI linked database leverages the largest cohort of children with CHD in the U.S. We found an increased risk of death from cancer, especially in those with chromosomal abnormalities. This may demonstrate an increased risk of cancer, but could indicate higher risk of death from cancer for children who previously had CHD. To resolve these possibilities we will link to cancer registries to obtain incident disease.
N (%)SMR95% CIOVERALL702.75(2.11-3.40)Lesion GroupLeft-to-Right Lesions36 (51.4)2.82(1.90-3.75)Impaired Systemic Flow13 (18.5)2.71(1.23-4.18)Impaired Pulmonary Flow7 (10.0)2.52(0.65-4.39)Complex Lesions3 (4.3)2.07(0.0-4.42)Anomalous Pulmonary Venous Return2 (2.9)5.92(0.0-14.12)Transposition Physiology2 (2.9)2.74(0.0-6.55)Other4 (5.7)2.38(0.05-4.71)Single Ventricle3 (4.3)3.74(0.0-7.98)Age at SurgeryNeonate12 (17.2)5.03(2.19-7.88)Infant17 (24.3)3.14(1.65-4.63)Child22 (31.4)3.59(2.09-5.10)Adolescent19 (27.1)1.65(0.91-2.40)Chromosomal AbnormalityYes17 (24.3)6.76(3.55-9.97)No53 (75.7)2.31(1.69-2.94)SeverityModerate28 (40.0)2.99(1.88-4.10)Mild22 (31.4)2.21(1.29-3.13)Severe14 (20.0)4.75(2.26-7.23)N/A6 (8.6)1.91(0.38-3.43)
Citation Format: Amanda S. Thomas, Logan Spector, Lazaros K. Kochilas. Cancer mortality in children surviving congenital heart surgery: A study from the pediatric cardiac care consortium [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2019; 2019 Mar 29-Apr 3; Atlanta, GA. Philadelphia (PA): AACR; Cancer Res 2019;79(13 Suppl):Abstract nr 3305.