**1. Introduction**

Congenital heart defect (CHD) may be defined as an anatomic malformation of the heart or great vessels which occurs during intrauterine development, irrespective of the age at presentation. Ventricular septal defect and coarctation of the aorta are typical examples of CHDs. In this chapter, a brief review of incidence, etiology and classification of CHD, and an overview of the most common congenital cardiac anomalies and their management will be presented. Cardiac abnormalities, generally considered not congenital in origin but important cardiac problems in children, namely rheumatic heart disease, Kawasaki syndrome and cardiomyopathy will not be discussed in this review. Also, discussion of important symptoms/findings/issues with which the children are referred to pediatric cardiologists such as cardiac murmur, chest pain, syncope/dizziness, palpitation, arrhythmia, hypertension, clearance for participation in sports, coronary risk factors, bacterial endocarditis prophylaxis, ADHD medication use, clearance for non-cardiac surgery and others will not be included in the this chapter.

#### **Incidence of congenital heart defects**

The reported incidence of congenital cardiac defects varies between 0.47 to 1.17% of live births, but 0.6% to 0.8% of live births is considered typical. This would result in birth of 25,000 to 35,000 infants with CHD each year in the United States alone. Congenital heart defects are more common than well-known congenital anomalies such as congenital pyloric stenosis, cleft lip, Down syndrome and congenital dislocation of the hip.
