Which cardiovascular findings are characteristic of relapsing polychondritis (RP)?

Updated: Dec 24, 2020
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
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Relapsing polychondritis has been reported to affect the cardiovascular system in 24% of patients. Aortic and mitral valve regurgitation, aortic aneurysm, aortitis, aortic thrombosis, pericarditis, first- to third-degree heart block, and myocardial infarction, at times mediated through ostial stenosis of a coronary artery or arteries, have been reported.

Relapsing polychondritis aortitis exhibits inflammation in the media, leading to loss of glycosaminoglycans and elastic tissue. [34] Any region of the aorta and more than one region simultaneously may be affected. In descending order of frequency, they include the following:

  • Ascending aorta
  • Aortic ring
  • Descending thoracic portion
  • Abdominal aorta

The most common clinical presentations include aortic arch syndrome, abdominal aortic aneurysm, and aortic regurgitation. Silent rupture and death may occur.

The clinical presentation of aortic regurgitation (resulting from ascending aorta involvement) may include left ventricular failure. Aortic regurgitation may result from damage to the aortic cusps or from annular dilatation due to destruction of supporting tissues.

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