Bicuspid Aortic Valve Follow-up

Updated: Jan 08, 2016
  • Author: Edward J Bayne, MD; Chief Editor: Steven R Neish, MD, SM  more...
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Overall complication rates in patients with bicuspid aortic valves vary. [20] In general, bicuspid aortic valve may be a common reason for acceleration of the normal aging process (eg, valve sclerosis, calcification). Four specific complications are related to the congenitally bicuspid aortic valve.

  • Aortic stenosis

    • Sclerosis of the bicuspid aortic valve generally begins in the second decade of life, and calcification becomes more concerning during and after the fourth decade of life. [26] The presence of coronary risk factors (eg, smoking, hypercholesterolemia) may accelerate these processes.

    • Approximately 50% of adults with severe aortic stenosis have a congenitally bicuspid valve.

    • Historically, rheumatic fever was the most common cause of aortic stenosis. With significantly decreasing incidence of rheumatic fever in developed nations, bicuspid aortic valve is the most common cause of aortic stenosis in adults and is probably the most common etiology of valve insufficiency as well. Acute rheumatic fever and its recurrences are still a major problem in developing countries, and, in these areas, long-term effects of rheumatic fever are still more significant than bicuspid valve in the etiology of aortic stenosis and insufficiency. Rheumatic aortic valve damage can be confirmed only at surgery or autopsy by the presence of Aschoff bodies.

  • Aortic insufficiency

    • Most cases of severe aortic insufficiency are related, either directly or indirectly, to a congenitally bicuspid valve.

    • Numerous factors may contribute to development of aortic valve insufficiency. These include cusp prolapse, erosion of irregular commissure lines, aortic root dilatation (particularly at the sinotubular junction or supra-aortic ridge), infective endocarditis, and systemic hypertension (particularly with coarctation).

  • Bacterial (eg, infective) endocarditis

    • The risk of developing infective endocarditis on a bicuspid aortic valve is 10-30% over a lifetime.

    • Bicuspid aortic valve is the second most common congenital etiology for infective endocarditis in infants and children; [39] overall, approximately 25% of endocarditis infections develop on a bicuspid valve.

  • Aortic root dissection

    • Findings on histologic studies of the aortic root in individuals with bicuspid aortic valve are controversial. [40] Enlargement of the root is often attributed to poststenotic dilatation. However, the root may dilate without significant valve stenosis, and abnormal histology with broken elastic fibers and other findings suggestive of Marfan syndrome has been identified in numerous studies. [13]

    • The risk of aortic root dissection is much higher for individuals with Marfan syndrome (approximately 40%) than for those with bicuspid aortic valve (approximately 5%). However, because bicuspid aortic valve is more prevalent in the general population, this disorder is more commonly associated with aortic root dissection.

    • A population-based, retrospective cohort study assessed the complications of patients with bicuspid aortic valve living in Olmsted County, Minnesota. Of the 416 patients studied over a mean follow-up of 16 years, reported incidence of aortic dissection was low (2 of 416), but it was significantly higher than in the general population. [41]



Overall prognosis for the individual with bicuspid aortic valve is good. Reviews and reports in the past have emphasized the fairly benign course for patients with bicuspid valves. However, more recent reports on the natural history of these valves suggest numerous more serious problems and an acceleration of normal valvular wear and tear. These problems may not develop until adulthood. Routine and regular follow-up for the child or adolescent with bicuspid aortic valve is recommended.


Patient Education

See the list below:

  • Patient and family education should emphasize the fairly benign course for the child with bicuspid aortic valve.

  • Older children and adolescents should begin to be made aware of the accelerated aging processes (ie, progressive stenosis), with particular attention to coronary risk factors.

  • The importance of bicuspid aortic valve as a potential substrate for infective endocarditis should be emphasized. Good oral and dental hygiene is important.

  • Most young individuals with bicuspid aortic valve should not require restrictions in physical activity or sports participation, unless they have stenosis or insufficiency. Routine examination is recommended prior to sports participation at least once.