Aortopathy in Congenital Heart Disease

Cardiol Clin. 2020 Aug;38(3):325-336. doi: 10.1016/j.ccl.2020.04.002. Epub 2020 Jun 3.

Abstract

Aortic dilatation is common in patients with congenital heart disease and is seen in patients with bicuspid aortic valve and those with conotruncal congenital heart defects. It is important to identify patients with bicuspid aortic valve at high risk for aortic dissection. High-risk patients include those with the aortic root phenotype and those with syndromic or familial aortopathies including Marfan syndrome, Loeys-Dietz syndrome, and Turner syndrome. Aortic dilatation is common in patients with conotruncal congenital heart defects and rarely results in aortic dissection.

Keywords: Aortic dissection; Aortopathy; Bicuspid aortic valve; Congenital heart disease.

Publication types

  • Review

MeSH terms

  • Aorta* / abnormalities
  • Aorta* / diagnostic imaging
  • Aorta* / physiopathology
  • Aortic Diseases / complications*
  • Aortic Diseases / diagnosis
  • Aortic Diseases / etiology
  • Aortic Diseases / physiopathology
  • Aortic Dissection* / diagnosis
  • Aortic Dissection* / etiology
  • Bicuspid Aortic Valve Disease / complications*
  • Dilatation, Pathologic / complications
  • Dilatation, Pathologic / diagnosis
  • Heart Defects, Congenital / complications*
  • Humans
  • Risk Assessment