We have studied retrospectively 103 patients with congenital heart disease from july 1989 to december 1991. The mean age is 7.8 years +/- 8.6. Epidemiological factors, clinical, morphological and prognostic data have been reviewed. All the patients had been evaluated with echocardiography based on an anatomical and segmental approach. Sex-ratio is 1.11. Situs is solitus in 101 cases (98%). Atrio-ventricular connexions are concordant in 97 patients (94%). The ventriculo-arterial connexions are concordant in 91 patients (88%). We found an anomaly of the spatial relation between great vessels in 9 cases (8.7%). Echocardiographic diagnosis have been confirmed by catheterism and at surgery in 10 patients, and by post-mortem examination in 2 cases. Isolated ventricular septal defect is the most frequent anomaly (19.4%), followed by Tetralogy of Fallot (17.5%). Only 13 patients among the 75 (17.3%) needing surgery have been operated. Six patients (5.8%) died following complications of the malformation. The prognosis without surgery is poor for the majority of our patients with congenital heart disease. Improved early detection and acquisition of adequate diagnosis technology are needed to realize surgical corrections.