Aortic coarctation is defined as a congenital obstruction of the aorta located near the insertion of the ductus arteriosus. It can be localised or diffuse and it is associated with some degree of hypoplasia of the aortic arch and/or isthmus.
Coarctation is a relatively frequent cardiovascular malformation, accounting for approximately 5% to 7% of all congenital heart defects. The incidence of aortic coarctation is 4 cases per 10,000 births 11. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890-900. Link.
Coarctation can occur as an isolated cardiovascular malformation, but it is also frequently associated with a bicuspid aortic valve or any simple or complex congenital heart disease.
There are cases of atypical aortic coarctation, such as those associated with PHACE syndrome (posterior fossa brain malformations, haemangiomas of the face, arterial anomalies, cardiac anomalies, and eye abnormalities). Finally, there is a less frequent type of aortic coarctation that affects the descending aorta, either the thoracic or abdominal, called MAS (middle aortic syndrome) which will not be discussed in this chapter.
Presentation during neonatal life can be fatal. However, beyond neonatal age, aortic coarctation may be discovered because of a heart murmur, absent...