Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births) in which the aorta

Aorta

The aorta is the largest artery in the body, originating from the left ventricle of the heart and extending down to the abdomen, where it branches off into two smaller arteries...

 is not completely developed. There is a gap between the ascending and descending thoracic aorta

Thoracic aorta

The thoracic aorta is contained in the posterior mediastinal cavity.It begins at the lower border of the fourth thoracic vertebra where it is continuous with the aortic arch, and ends in front of the lower border of the twelfth thoracic vertebra, at the aortic hiatus in the diaphragm where it...

. Almost all patients also have other cardiac anomalies. Interrupted aortic arch is often associated with DiGeorge Syndrome

DiGeorge syndrome

22q11.2 deletion syndrome, which has several presentations including DiGeorge syndrome , DiGeorge anomaly, velo-cardio-facial syndrome, Shprintzen syndrome, conotruncal anomaly face syndrome, Strong syndrome, congenital thymic aplasia, and thymic hypoplasia is a syndrome caused by the deletion of a...

.

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

Open Heart Surgery

Open Heart Surgery was released on August 8, 2000 by rock band Virginwool. The band signed to Breaking/Atlantic Records after initially beginning signed to Universal Records. The album was produced and mixed by Brad Wood....

 soon after birth. Awaiting surgery, prostaglandin

Prostaglandin

A prostaglandin is any member of a group of lipid compounds that are derived enzymatically from fatty acids and have important functions in the animal body. Every prostaglandin contains 20 carbon atoms, including a 5-carbon ring....

 can be administered to keep the ductus arteriosus

Ductus arteriosus

In the developing fetus, the ductus arteriosus , also called the ductus Botalli, is a shunt connecting the pulmonary artery to the aortic arch. It allows most of the blood from the right ventricle to bypass the fetus's fluid-filled lungs. Upon closure at birth, it becomes the ligamentum arteriosum...

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.

External links

• C.S. Mott Children's Hospital, Congenital Heart Center: Interrupted Aortic Arch at umich.edu
• Heart center encyclopedia at cincinnatichildrens.orgcincinnatichildrens.org