AORTIC OBSTRUCTIONS IN INFANTS AND CHILDREN - PATHOPHYSIOLOGY AND CLINICAL PRESENTATION OF INTERRUPTED AORTIC-ARCH

Interrupted aortic arch is an uncommon form of congenital heart diseas e in which there is almost always an associated ventricular septal def ect or aorticopulmonary window. There may also be other cardiac abnorm alities or DiGeorge anomaly. Presentation usually occurs as the ductus closes and is related to decreased perfusion to the lower body, left- to-right shunting, and increased left ventricular end-diastolic pressu res. Diagnosis can generally be made by echocardiography, but catheter ization may provide additional helpful data. Temporary palliation may be achieved with prostaglandin E1 infusion to maintain ductal patency, but urgent operation is required. Long-term results are good after re pair of the arch and associated defects, but subaortic stenosis or res tenosis at the site of the arch repair may occur.