This study consists of a prevalence congenital heart disease (CHD) registered in the Pediatric Cardiology Department of Umberto I General Hospital in Rome between January 1st 1992 and December 31-th 1993. Cases recorded in this period have been taken part of a larger study called Italian Multicentric Study for recording and follow-up of congenital heart disease (IMS-CHD); the purpose is to determine the prevalence of CHD in Italy and discover the outcome of affected children. In this duration, 187 new cases have been recorded, in which 63.6% had a single defect while 36.4% had multiple defects. These isolated defects were most frequently occurred (51.9%) following the stenosis of the pulmonary artery (15.5% and the defects of the interatrial septum (15%).