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Coarctation of Aorta

Coarctation of Aorta is a topic covered in the Select 5-Minute Pediatrics Topics.

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Basics

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

Prevalence

  • ∼6–8% of patients with congenital heart disease have coarctation.
  • Male > female (1.5–4.0:1)

Risk Factors

Genetics

  • Multifactorial: occurs in 35% of patients with Turner syndrome (XO)
  • Has been described in cases of monozygotic twins
  • Many studies document the prevalence of a microdeletion at 22q11 in patients with arch anomalies and ventricular septal defects.

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.

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Citation

Cabana, Michael D., editor. "Coarctation of Aorta." Select 5-Minute Pediatrics Topics, 7th ed., Wolters Kluwer Health, 2015. Medicine Central, im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14107/all/Coarctation_of_Aorta.
Coarctation of Aorta. In: Cabana MD, ed. Select 5-Minute Pediatrics Topics. 7th ed. Wolters Kluwer Health; 2015. https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14107/all/Coarctation_of_Aorta. Accessed April 24, 2019.
Coarctation of Aorta. (2015). In Cabana, M. D. (Ed.), Select 5-Minute Pediatrics Topics. Available from https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14107/all/Coarctation_of_Aorta
Coarctation of Aorta [Internet]. In: Cabana MD, editors. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. [cited 2019 April 24]. Available from: https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14107/all/Coarctation_of_Aorta.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Coarctation of Aorta ID - 14107 ED - Cabana,Michael D, BT - Select 5-Minute Pediatrics Topics UR - https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14107/all/Coarctation_of_Aorta PB - Wolters Kluwer Health ET - 7 DB - Medicine Central DP - Unbound Medicine ER -