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Atypical coarctation of aorta

Orpha number ORPHA1456
Synonym(s) Coarctation of the abdominal aorta
Mid-aortic dysplastic syndrome
Mid-aortic syndrome
Midaortic syndrome
Middle aortic syndrome
Prevalence <1 / 1 000 000
Inheritance
  • Sporadic
Age of onset Childhood
ICD-10
  • Q25.1
OMIM -
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

Atypical coarctation of aorta (CoA) is characterized by localized or extended narrowing of the ascending aorta, of the descending thoracic aorta at the level of the diaphragm, or of the abdominal aorta. Symptoms are attributed to upper body arterial hypertension and may include headache, abdominal angina, leg fatigue at exercise, cold feet and intermittent claudication. A pressure difference between arms and legs may exist as it is the case for the isthmic CoA. A systolic vascular murmur can be heard over the region of stenosis. This disease is often caused by an arteritis (Takayasu arteritis or aortitis) or a fibromuscular dysplasia. Some forms may be congenital. It can be associated with neurofibromatosis such as von Recklinghausen disease or Williams syndrome. This rare condition affects 0.5% to 2% of individuals with CoA. The symptomatic treatment consists of antihypertensive medication. The causal treatment is either percutaneous transluminal angioplasty with or without endovascular stent placement or surgery.

Expert reviewer(s)

  • Dr A HAGER
  • Pr Harald KAEMMERER
Last update: January 2003

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