eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Aortic Valve, Bicuspid: Follow-up

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
Contributor Information and Disclosures

Updated: Nov 13, 2009

Follow-up

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.

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.

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.

Miscellaneous

Medicolegal Pitfalls

  • The bicuspid aortic valve may not be recognized as a source for emboli in cases of unexplained fever and focal CNS deficits.

Special Concerns

  • Pregnancy in women with bicuspid aortic valve should pose no special concerns unless complications of progressive aortic stenosis or aortic root dilation occur.
 


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References
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Further Reading

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Keywords

bicuspid valve, bicuspid aortic valve, bicommissural aortic valve, rheumatic fever, truncus arteriosus, coarctation of the aorta, interrupted aortic arch, Marfan syndrome, aortic insufficiency, hypoplastic left heart syndrome, patent ductus arteriosus, Turner syndrome, short stature, Williams syndrome

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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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

CME Editor

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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