Abstract
Congenital malformations of the respiratory system consist of a wide range of fetal defects that can affect the developing fetal lung vasculature, airways, and parenchyma. These anomalies include congenital diaphragmatic hernia (CDH), congenital bronchial atresia (BA), congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestrations (BPS), and congenital high airway obstruction syndrome (CHAOS). Congenital malformations of the lungs can lead to complications including pulmonary hypoplasia, pulmonary arterial hypertension, fetal heart failure, and hydrops, in which fetal intervention may be indicated. In this chapter, we discuss various etiologies and diagnostic strategies for these respiratory abnormalities as well as the possible fetal interventions to correct these conditions. The management options can be nonsurgical or surgical, depending on the disease presentation and severity.