What causes chronic aortic regurgitation (AR)?

Updated: Nov 19, 2018
  • Author: Stanley S Wang, JD, MD, MPH; Chief Editor: Terrence X O'Brien, MD, MS, FACC  more...
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Answer

Bicuspid aortic valve is the most common congenital lesion of the human heart. Although it leads more often to progressive aortic stenosis than to AR, it is nonetheless the most common cause of isolated AR requiring aortic valve surgery. In patients with bicuspid aortic valve, an associated aortopathy may be present, resulting in aortic dilation and/or dissection that worsens the AR. [8]  Current American College of Cardiology/American Heart Association (ACC/AHA) guidelines recommend consideration of surgical intervention when the aortic diameter reaches 5.0 cm (or 4.5 cm in patients who are undergoing cardiac surgery for reasons other than aortic enlargement). [9]

Certain weight loss medications, such as fenfluramine and dexfenfluramine (commonly referred to as Phen-Fen), may induce degenerative valvular changes that result in chronic AR.

Rheumatic fever, a common cause of AR in the first half of the 20th century, has become less common in the United States, although it remains prevalent in some immigrant populations. Fibrotic changes cause thickening and retraction of the aortic valve leaflets, resulting in central valvular regurgitation. Leaflet fusion may occur, leading to concurrent aortic stenosis. Associated rheumatic mitral valve disease is almost always present.

Ankylosing spondylitis often causes an aortitis, which most frequently involves the aortic root, with associated AR. [10] Further extension of the subaortic fibrotic process into the intraventricular septum may result in conduction system disease. Coronary and more distal aortic abnormalities are also seen in this condition.

Behçet disease causes cardiac complications in less than 5% of patients, but potential findings include proximal aortitis with AR, as well as coronary artery disease. [11]

Giant cell arteritis is a systemic vasculitis that typically affects the extracranial branches of the carotid artery but may also cause aortic inflammation and AR (as well as coronary artery disease and LV dysfunction). [12]

Rheumatoid arthritis uncommonly causes granulomatous nodules to form within the aortic valve leaflets. In rare cases, this may lead to clinical AR, although it is more commonly an incidental finding postmortem. [13]

Systemic lupus erythematosus can cause valvular fibrosis and consequent dysfunction, including AR. [14] Lupus is also associated with Libman-Sacks endocarditis, resulting in sterile, verrucous valvular vegetations that can cause AR. [15, 16]

Takayasu arteritis, in addition to having aortic valvular (and coronary) involvement, can produce an aortitis. The aortitis may increase the risk of prosthetic valve detachment, leading some to advocate for concurrent aortic root replacement in patients undergoing valve surgery. [17]

Whipple disease has been reported in the literature in association with AR or aortic valve endocarditis. [18]


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