Specialty cardiology ICD-9-CM 747.11 | ICD-10 Q25.2 (EUROCAT) eMedicine ped/2515 | |
Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births) in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. Interrupted aortic arch is often associated with DiGeorge syndrome.
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Diagnosis
It can be diagnosed with an echocardiogram. Patients will have a loss of appetite, turn pale, may feel cold in the lower half of the body due to not enough blood flow.
Treatment
Treatment consists of open heart surgery soon after birth. Awaiting surgery, prostaglandin can be administered to keep the ductus arteriosus open, thereby allowing blood flow to the lower body. Failure to treat the condition yields a mortality rate of 90% at a median age of 4 days.