DESCRIPTION

• 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.

EPIDEMIOLOGY

RISK-FACTORS

PATHOPHYSIOLOGY

• 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.