Coarctation of Aorta
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- 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.
- ~6–8% of patients with congenital heart disease have coarctation.
- More common in males
- 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.