Pulmonary Hypertension in Congenital Diaphragmatic Hernia
Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly, where a defect in the diaphragm results in herniation of the abdominal contents into the chest and compression of the intrathoracic structures. In utero compression of the lungs results in pulmonary hypoplasia and pulmonary hypertension after birth. Along with the degree of lung hypoplasia and pulmonary hypertension, underdevelopment of the left ventricle also determines severity in CDH and contributes to adverse outcomes. After birth, CDH patients present with severe hypoxemic respiratory failure and shock, secondary to structural and functional changes in the heart, pulmonary circulation, airways, and lung parenchyma. The optimal approach to management of respiratory failure, pulmonary hypertension, and shock changes as the underlying pathophysiology evolves in the days and weeks after birth. In this chapter, we describe the approach to CDH management as shock which predominates early resolves and systemic hemodynamics stabilize and pulmonary vascular abnormalities progress from acute to subacute (late) and finally chronic stages.
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