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Worldwide Congenital heart defects (CHD) are the leading cause of infant deaths owingto congenital anomalies.Knowing the actual incidence of severe CHD is important for defining the requirement for resources and the burden of disease within the total population.Objectives The aim of the study is to estimate the incidence of severe CHD at the province ofAl Qassim as a reflection of that in the KSA.Study Design: A retrospective database review of all cases diagnosed to have severe CHD at Prince Sultan Cardiac Center (PSCC) -Qassim during a three year period from Jan 2008 to Dec 2010.Results During the study period 316 patients were diagnosed to have severe CHD.During the three year study period the total live birth at the province of Alqassim was 58908.The incidence of severe CHD is 5.4/1000 live birth/year.65% of cases were diagnosed at less than twomonths of age, with a median age at diagnosis of 45 days (range of 1 day to 360 1 yeardays).VSD was the commonest lesion diagnosed (22.5%).Collectively prostaglandin dependant lesions constitute 38% of cases.15% of patients were proved to have a syndrome.The commonest syndrome was Down syndrome.We Cconclusionde that Ssevere CHD is a major health problem at the KSA and worldwide.At the province of AlQassim the incidence of sever CHD is 5.4 per 1000 live birth.