Bicuspid Aortic Valve Follow-up

  • Author: Edward J Bayne, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Sep 27, 2011
 

Complications

Overall complication rates in patients with bicuspid aortic valves vary.[16] 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.[22] 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;[35] 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.[36] 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.[12]
    • 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.[37]
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Prognosis

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.

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Patient Education

  • 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.
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Contributor Information and Disclosures
Author

Edward J Bayne, MD  Assistant Professor, Division of Pediatric Cardiology, Emory University School of Medicine; Consulting Staff, Sibley Heart Center Cardiology, Children's Healthcare of Atlanta

Edward J Bayne, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul M Seib, MD  Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

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Bicuspid aortic valve with unequal cusp size. Note eccentric commissure and raphe.
Parasternal long-axis echocardiogram showing doming of a bicuspid aortic valve.
Parasternal short-axis echocardiographic view in diastole, showing bicuspid aortic valve with nearly equal cusp size and right-left orientation of the commissure. Note the 2 color signals showing minimal aortic insufficiency.
Two-dimensional echocardiogram of typical bicuspid aortic valve in diastole and systole. Valve margins are thin and pliable and open widely, creating the fishmouth appearance.
Long-axis and short-axis transthoracic echocardiograms showing a bicuspid aortic valve. In diastole, hammocking (prolapse) of the valve cusps occurs. The short-axis view shows the irregular sclerotic margins. This type of bicuspid valve is the most commonly replaced, typically because of insufficiency.
Basilar oblique transesophageal image showing congenitally bicuspid aortic valve with vegetation (due to Streptococcus viridans endocarditis).
 
 
 
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