In the last 30 years surgical advances have led to correction of congenital heart defects (CHD) in children who otherwise would not have lived to adulthood. This study has three major objectives: 1) to determine mortality, morbidity, and disability after surgical treatment of CHD and to identify factors associated with morbidity and mortality; 2) to assess the impact of """"""""corrected"""""""" CHD on pregnancy and birth outcome; and 3) to determine the recurrence risk of CHD in first-degree relatives and offspring of probands with repaired defects. This study sustains and expands a registry of all Oregon children (less than or equal to 18 years) undergoing surgical repair from 1958 to the present of 14 major congenital heart defects: tetralogy of Fallot (TOF), ventricular septal defect (VSD), atrial septal defect (ASD), coarctation of the aorta, pulmonic stenosis (PS), aortic stenosis (AS), transposition of the great arteries, patent ductus arteriosus (PDA), tricuspid atresia (TA), total anomalous pulmonary venous return (TAPVR), pulmonary atresia with intact ventricular septum (PA), PA with VSD, partial atrioventricular canal (AVC), and complete AVC. The first eight defects are included in the registry at present: TOF (n =438). VSD (n =402), ASD (n =496), COA (n =479), AS (n = 175), PS (n = 200), TGA (n = 169), and PDA (n = 533). To date, 87-97% follow-up is available for these cohorts after medians of 50-185 months. All cases of surgical treatment of TA, TAPVR, PA, PA with VSD, and partial and complete AVC from 1958 to the present will be added to the registry. Preoperative, operative, and postoperative data for each patient will be entered into the database from chart review. Each hospital is visited twice yearly to ascertain new cases. Individuals are followed every two years for intercurrent events by mailed questionnaire or phone interview. Questionnaires and interviews assess major morbidity, functional status, and reproduction. Pregnancy, major events, and recurrent CHD will be confirmed by medical records. Actuarial analysis and the Cox proportional hazards model will determine mortality, morbidity, and their risk factors, and compare observed survival with expected population survival. Reproductive data will be analyzed per years of fertility for cohort, and compared to Oregon population statistics. This geographically-based study will add knowledge of late survival and morbidity of a population rapidly growing in number, adults with corrected CHD. Additionally, we will gather the first prospective population data on pregnancy after repair of CHD.
