Cardiac diagnosis and severity for all children in the cohort.

Notes:*adapted from Wren [14];**adapted from Lane [15].Assignment of primary CHD diagnosis: The methodology for assigning primary diagnoses to 1,768 children with multiple defects was validated independently by three raters (RK, CB, CW). Based on cardiac diagnoses in medical records, children assigned a primary diagnosis were 1,738 (98%), 1,610 (91%) and 1,146 (65%) for each rater; this increased to 1,761 (99.7%), 1,689 (95.5%) and 1,658 (93.8%) respectively using records of surgical procedures (Interrater agreement: k = 0.83). A ‘miscellaneous’ category included defects found in fewer than 40 children: congenitally corrected transposition of the great arteries (n = 24), partial atrioventricular septal defect (n = 20), aortopulmonary window (n = 26), atrial septal defect (n = 36) and rarer diagnoses (n = 83).CPS groups were: no intervention-children who received no surgical intervention prior to death during first year of life; curative-children who had successful repair of atrial or ventricular septal defect, pulmonary stenosis or total anomalous pulmonary veins and had no additional cardiac defects; corrective-children who had a procedure which approximated normal anatomy and restored biventricular function, with no expectation of future surgery during childhood; palliative-children whose surgery did not restore biventricular function, including children for whom all stages of multi-stage repair were not achieved, who had a valve replacement which would require later revision, or for whom only a single functional ventricle circulation was possible.Cardiac diagnosis and severity for all children in the cohort.