**1. Introduction**

Congenital heart disease (CHD) is the most common malformation detected prenatally and at birth. Generally, estimated incidence is approximately 10/1000 of live births and is significantly higher in premature infants and in stillborn [1]. Although remarkable progress has been made in the diagnosis and treatment of this condition, it still remains a significant cause of neonatal morbidity and mortality. Congenital malformations of the heart are a broad spectrum of defects varying from mild lesions that produce only minimal or no symptoms and might be incidentally detected in adult life to severe anomalies that cause premature death. There are many factors that increase the risk of having a child with CHD. The etiology of CHD can be separated into genetic and non-genetic forms. Epidemiological studies have suggested that a

genetic or environmental cause can be identified in approximately 30% of CHD cases [2]. Approximately 17% of CHD occurs in association with a well-defined syndrome such as trisomies 13, 15, 18, 21 and Turner syndrome [3]. Some environmental factors have been identified as responsible for CHD such as congenital rubella infection or teratogenic drugs [2]. However, the majority of cases remain unexplained, probably due to some combination of genetic and environmental factors [4].

The main classification of CHD includes:


In this chapter, we will discuss in detail the importance of fetal echocardiography and the fundamental impact of early detection and interventions during fetal life on postnatal outcomes. We will also discuss the importance of adequate counseling in order to allow parents to understand the condition, to support them in the most difficult decision to interrupt or keep pregnancy, to offer extensive information on available therapeutic options, and prevision of data on outcome and quality of life.
