Interrupted Aortic Arch (IAA)

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This is an uncommon defect in which the aorta is either atretic (severely narrowed) or interrupted, creating disruption in the normal connection between the ascending aorta and the descending aorta.  It is nearly always associated with a large VSD (opening between the lower two chambers of the heart).  The patent ductus arteriosus or PDA (the blood vessel between the aorta and pulmonary artery) allows some blood to get to the body. If a VSD is present, blood from the left ventricle will shunt to the right ventricle causing increased blood flow to the lungs, which leads to congestive heart failure. These children can present with cardiovascular collapse or shock if the PDA closes. The infant is maintained on a medication, prostaglandin (PGE1), intravenously from birth to maintain blood flow to the body and lungs until surgery can be performed.  This defect is also associated with DiGeorge Syndrome or 22q11 chromosome deletion.

Surgical correction:  Consists of sewing the descending portion of the aorta to the ascending aorta and closing the ventricular septal defect (VSD) with a patch.  Typically this operation is done in the first week of life.