Epidemiologia delle malformazioni cardiovascolari. III. Prevalenza e decorso in 46.895 nati vivi alla Maternità di Careggi, Firenze nel periodo 1975-1984

BACKGROUND. To evaluate the prevalence of congenital heart disease in a homogeneous population we examined 46,895 liveborns in the period from January 1975 to December 1984 in the Careggi Maternity Hospital in Florence. METHODS. The diagnosis of congenital heart disease was made in 579 newborns within five days from birth. All newborns were examined clinically by two neonatologists and referred to the pediatric cardiologist in case of cardiac abnormalities. An ECG was recorded in each of them, chest x-ray in 87% and echo in those who were born after 1980. The children were followed up until December 1989. Mean follow-up period was 6 years. RESULTS. The annual incidence ranged from 9.5% to 15.7% (average 12.3%). Chromosomic anomalies and extra-cardiac malformations occurred in 102 children (17.6%), respectively in 50 (8.6%) and 52 (9.0%) cases. Ventricular septal defect (VSD) and the patent ductus arteriosus (PDA), isolated or associated, were the most frequently recognized congenital heart diseases. 52 children (9.0%) were lost at follow-up. The initial diagnosis was changed in 11/579 cases. In 187 children (32.3%) there was a spontaneous disappearance of clinical and/or instrumental findings that had suggested the presence of congenital heart disease at birth: in 144 the diagnosis was VSD, and in 43 PDA. The prevalence of VSD was 4.7 per thousand in the period 1975-80 and 8.6 in the period 1981-84. 131 children (22.6%) died, 127 (96.9%) of them in the first year of life. 52 children were operated on and pulmonary valvuloplasty was performed in 5. CONCLUSIONS. The prevalence of congenital heart disease does not change during a long observation period, while the number of VSDs increases. VSD and PDA spontaneously close in a high percentage of cases. The study suggests the usefulness of an intensive care unit for newborns with severe congenital heart disease, especially for those in the first year of life.