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Introduction Hypoplastic left heart syndrome (HLHS) occurs in 0.1 : 1000 to 0.6 : 1000 live births and accounts for 2% to 3% of all congenital heart diseases (CHDs). HLHS is one of the most severe CHDs, with a high rate of perinatal death.…

Introduction Aortic stenosis (AS) affects 1.4 : 10,000 newborns. The spectrum of clinical manifestations varies widely according to the degree of valve stenosis. Disease Definition AS and aortic atresia encompass a group of congenital heart diseases (CHDs) characterized by left ventricular outflow…

Introduction Pulmonary stenosis (PS) and pulmonary atresia (PA) with intact ventricular septum have a low incidence in the fetus. In PS, there is a narrowing of the right ventricular outflow tract, whereas PA is characterized by an absent ventriculoarterial connection.…

Introduction Ebstein anomaly (EA) and tricuspid dysplasia (TD) are congenital heart diseases (CHDs) affecting the tricuspid valve and consequently all of the right heart. Although these anomalies are two different entities from an anatomic point of view, they share many…

Introduction Tricuspid atresia (TA) is a rare form of congenital heart disease (CHD) that usually involves severe hypoplasia of the right ventricle. Prenatal diagnosis of TA and associated intracardiac and extracardiac anomalies is possible with a high degree of accuracy.…

Introduction Atrioventricular septal defect (AVSD) is one of the most commonly diagnosed congenital heart diseases (CHDs) during fetal life and affects 4% to 5% of newborns with a CHD. AVSDs have a strong association with Down syndrome and frequently occur…

Introduction Ventricular septal defect (VSD) is the most common congenital heart disease (CHD) in children and adults. VSD is present in 25% to 30% of newborns with a CHD. VSD can frequently occur in combination with other CHDs, as part…

Introduction Congenital heart diseases (CHDs) are the most common severe congenital malformations, affecting 0.8% to 1% of newborns. Approximately half of the CHDs are severe defects. The overall mortality of a CHD diagnosed prenatally ranges from 25% to 35%, and…

Introduction Congenital high airway obstruction syndrome (CHAOS) is a rare fetal anomaly that is associated with a high mortality (80%–100%). Prenatal diagnosis is clinically relevant in parental counseling and decision making regarding fetal surveillance, planning of fetal intervention, method of…

Introduction The prenatal diagnosis of fetal goiter was first described in 1980. Advances in prenatal imaging and fetal hormonal physiology have enabled the identification of some severe but treatable disorders in the fetus. The potential benefits to the fetus of…