Congenital heart disease (CHD) is a cardiac abnormality that occur during the formation of the heart during intrauterine life [1] . They are extremely diverse, ranging from simple benign abnormalities with the growth of the child without major problems until the malformation make the survival of the newborn (NN) impossible. Their impact is estimated between 6 and 9 ‰ live births (NV) [2]. Abstract Background: Congenital heart disease is the most common congenital disorder in newborns represents

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... a major cause of perinatal death. The prevalence of these malformations is still unrecognized in Tunisia because of the lack of a national register. The aim of this study was describe the epidemiological and clinical aspects of the heart diseases with neonatal diagnosis in Tunisia. Methods: A retrospective multicentrc study has been undergone. All the neonates with congenitalheart diseases have been collected in seven neonatal departments in Tunisia, a period of two years (2015 and 2016). Results: Three hundred cases of congenital heart defects were collected with a prevalence of 3.4 / 1000 live births. These abnormalities are divided into four groups according to pathophysiological mechanisms: Left-right shunt heart disease: (99 cases or 33%), cyanotic heart disease (129 cases or 43%), obstructive heart disease (42 cases or 14%) and other heart diseases (30 boxes or 10%). Among all of these heart diseases, 44.7% had a prenatal diagnosis at a median gestational age of 30 weeks of amenorrhea. 23.1% of newborns were from consanguineous parents. The mean maternal age was 32 years old. Pre-existing diabetes and gestational diabetes were present in 5.3% and 9.6% of cases respectively. The sex ratio was 1.14. Prematurity was found in 19.4%. low birth weight was denoted in 16.9% of neonates. 23.3% of newborns had associated congenital malformations. The average age at diagnosis was 6.69 days. Respiratory distress was the mostcommon symptom (47.7%). The association with a heart murmur was recorded in 39% of cases. Conclusion: Congenital heart diseases are burdened by a significant rate of morbidity and mortality the perinatal period. Their prevalence in our country is still underestimated despite the progress of techniques and means of diagnosis. It is therefore necessary to insist on the need for the creation of a national register of congenital heart diseases listing the various cases reported for the real estimation of the prevalence of these malformations in Tunisia.