COMPLETENESS OF ASCERTAINMENT AND PREVALE NCE OF MAJOR CONGENITAL HEART-DISEASES AT LIVEBIRTH IN CAMPANIA

We evaluated the completeness of ascertainment and the diagnostic accu racy of major congenital heart diseases reported to the Birth Defects Registry of Campania, during 1991. The population consisted of 37775 l ive births. The heart diseases included in the study were: Transpositi on of the Great Arteries, Truncus Arteriosus, Tetralogy of Fallot, Hyp oplastic Left Heart Syndrome, Pulmonary Stenosis, Coarctation of the A orta, Anomalous Pulmonary Venous Connections, Aortic Stenosis, Single Ventricle, Atrioventricular Canal Defects. We estimated the total numb er of cases using two sources of data: the delivery centres that notif y the birth defects to the Registry and the only regional Cardiosurger y Department. The capture-recapture techniques allowed to calculate th e ascertainment-corrected rates using information provided by duplicat e cases (cases for found in both sources). The cases reported to the R egistry were 38; the cases known by the Cardiosurgery Department were 42; the estimate of the total number of major congenital heart disease s resulted 70 (Cl95% = 59 divided by 85), with a rate of 18.5 per 10,0 00 live births (Cl95% = 15 divided by 24). The sensitivity of Registry was 54%; the second source increased the sensitivity by 27%. The pred ictive value of the delivery centre's diagnosis was 97.5%. The sensiti vity of major congenital heart diseases surveillance system is low, bu t it improves greatly adding a second source. There is a high probabil ity that the diagnosis of heart disease by the birth centre is correct .