Coarctation of Aorta

Basics

Discrete stenosis of the upper thoracic aorta, usually just opposite the site of insertion of the ductus arteriosus (juxtaductal). A segment of tubular hypoplasia and/or a remnant of ductal tissue give rise to a prominent posterior infolding (“the posterior shelf”).
The hemodynamic lesion is most often discrete but may be a long segment or tortuous in nature. It is usually juxtaductal but may occur in other sites (i.e., the abdominal aorta).
The prevalence of other associations (bicuspid aortic valve) and long-term complications (hypertension) indicate that this lesion may be part of a broader spectrum arteriopathy and/or endothelial disorder.

Occurs in 35% of patients with Turner syndrome (XO)
Has been described in cases of monozygotic twins
Mutations of several genes, including NOTCH1 and MCTP2, have been identified in some patients.
Many studies document the prevalence of a microdeletion at 22q11 in patients with arch anomalies and ventricular septal defects.

Decreased systemic blood flow to lower body after ductal closure
Increased afterload to left ventricle (LV) causes LV hypertrophy. Relative underperfusion of the renal vessels, baroreceptors, and multiple other mechanisms combine to induce compensatory hypertension.
If the coarctation is severe, LV dysfunction and congestive heart failure (CHF) result, with low cardiac output and increased LV end-diastolic pressure.
Decreased myocardial perfusion may be present in cases of very low output.

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