Interrupted aortic arch

Interrupted aortic arch
Classification and external resources
Specialty	medical genetics
ICD-10	Q25.2 (EUROCAT)
ICD-9-CM	747.11
eMedicine	ped/2515

Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births)^[1] 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.^[2]

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.^[1]

References

1 2 Messner, Greg; Reul, George J.; Flamm, Scott D.; Gregoric, Igor D.; Opfermann, Ulrich Tim (2002). "Interrupted aortic arch in an adult single-stage extra-anatomic repair". Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital 29 (2): 118–21. PMC 116738. PMID 12075868.
↑ Chin, Alvin J (Oct 2, 2007). "Interrupted Aortic Arch". eMedicine. Retrieved May 27, 2009.

External links

C.S. Mott Children's Hospital, Congenital Heart Center: Interrupted Aortic Arch at umich.edu
Heart center encyclopedia at cincinnatichildrens.orgcincinnatichildrens.org
Collins-Nakai, RL; Dick, M; Parisi-Buckley, L; Fyler, DC; Castaneda, AR (1976). "Interrupted aortic arch in infancy". The Journal of Pediatrics 88 (6): 959–62. doi:10.1016/S0022-3476(76)81049-9. PMID 1271195.

Congenital vascular defects / Vascular malformation (Q25–Q28, 747)

Great arteries/
other arteries

Aorta	Patent ductus arteriosus Coarctation of the aorta Interrupted aortic arch Double aortic arch Right-sided aortic arch Overriding aorta Aneurysm of sinus of Valsalva Vascular ring

Pulmonary artery	Pulmonary atresia Stenosis of pulmonary artery

Subclavian artery	Aberrant subclavian artery

Umbilical artery	Single umbilical artery

Great veins

Superior/inferior vena cava	Congenital stenosis of vena cava Persistent left superior vena cava

Pulmonary vein	Anomalous pulmonary venous connection (Total, Partial) Scimitar syndrome

Arteriovenous malformation

Cerebral arteriovenous malformation

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