الفهرس 
IntroductionOnly 14 pages are availabe for public view
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Abstract
Congenital heart defects occur in 0.81.2% of all live births and comprise approximately 40% of all congenital defects. As many as 85% of all CHD are of unknown etiology. Only 3% of cases follow a clear Mendelian inheritance. Although environmental agents, such as viruses and drugs can cause CHD they account for only a small percentage of cases. A positive family history of CHD is one of the known major risk factors for CHD compared with the risk to offspring of healthy individuals .(1) CHD that becomes apparent in the first year of life present unique diagnostic and management problems .(2) It differ from CHD manifesting in adults and older children because, more serious diseases tend to come to medical attention early in life but the relatively minor forms may go undetected till later.(306) Also CHD that becomes apparent in the first year of life affect the ongoing child care and may complicate bonding, feeding, growth , development and immunization .(29) Most CHD are well tolerated in the fetus because of the parallel nature of the fetal circulation. (307) The cardiac defects that become evident in newborn differ greatly from those firstly recognized later in infancy. It is therefore practical to subdivide the problem of CHD in the first year of life into four classifications based on the age of presentation as the following: 0 to3 days; 4 to14 days; 2 to18 weeks; and 4 to12 months. However, this subdivision does not mean that the diagnostic and management issues of CHD change abruptly at a given day or week of life .(3) Factors influencing the usual age of presentation of infants with CHDs : (3) 1) 03 days: changes of the transitional circulation such as parallel nonmixing circulations and critically obstructed series circulation. 2) 414 days :closing ductus arteriosus that causes obstructed pulmonary or descending aortic blood flow or falling of pulmonary vascular resistance that may results in pulmonary edema. 3) 2 18 weeks: Chronic low pulmonary vascular resistance that may results in pulmonary edema also. 4) 412 months :CHD presenting at that age may show more subtle signs of heart disease such as growth failure, or persistent heart murmur . CHD presenting in the first three days of life include (TGA with intact ventricular septum, HLHS, pulmonary valve atresia with intact ventricular septum (HRHS) ,severe Ebstein ?s anomaly, critical AS, critical PS, IAA and obstructed TAPVR. Although a few asymptomatic patients with nonlifethreatening condi<U+00AC>tions come to attention within the first 3 days because of a heart murmur, the three primary modes of presentation for critical CHD in the neonate are cyanosis, shock, and pulmonary edema. (2) The aim of this work is to highlight some important information about CHD specially that presents in the first year of life to clarify the modes , the expected age of presentation of each CHD and the factors influencing its presentation at each age group. The following are some guidelines to help the physicians to adopt a clear strategy for CHD diagnosis and management . The patients with a potential twoventricle circulation should have a corrective procedure rather than a palliative procedure that although life saving and with lower risk does not correct the underlying pathophysiology and may cause secondary damage to the heart, while corrective procedures on cardiopulmonary bypass (open) can now be performed even in the premature and low birth weight neonates as small as 1200 to 1500 gm with acceptable mortality rate. However, the prolonged medical therapy to achieve further weight gain does not improve survival and continues to expose the neonate to the risk of NICU complications and the weight gain may be marginal due to the underlying CHD.