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Practice

Bicuspid aortic valve disease

Katie L. Losenno and Michael W.A. Chu
CMAJ December 10, 2013 185 (18) 1599; DOI: https://doi.org/10.1503/cmaj.121875
Katie L. Losenno
Division of Cardiac Surgery, Department of Surgery, Western University, London, Ont.
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Michael W.A. Chu
Division of Cardiac Surgery, Department of Surgery, Western University, London, Ont.
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  • For correspondence: michael.chu@lhsc.on.ca
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Bicuspid aortic valve disease is the most common congenital heart disease in developed nations

Bicuspid aortic valves are present in 1%–2% of the population, with men being 3 times more commonly affected than women. This disease is more than just anatomic variation and may be associated with other heart and aorta abnormalities as a result of molecular and connective tissue derangements.1

Bicuspid aortic valves are heritable

Although the exact cause is unknown, familial clustering of bicuspid aortic valves and associated aortopathy has revealed genetic components. Clinical and echocardiographic screening of first-degree relatives of affected patients is warranted to rule out bicuspid aortic valves and thoracic aortic disease.2

Valvular and aortic complications are common among patients with bicuspid aortic valves

The clinical presentation is heterogeneous. Many patients with bicuspid aortic valves are asymptomatic and have minimal valvular dysfunction. However, with increasing age, aortic stenosis (Figure 1) and insufficiency (Appendix 1, available at www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.121875/-/DC1) are common, with over one-third of initially asymptomatic patients experiencing a major cardiovascular event or requiring cardiac surgery over 20 years.3 An estimated 15%–45% of patients may also have ascending aortic aneurysms;3,4 however, the risk of aortic dissection is relatively low (1%–6%).3–5

Figure 1:
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Figure 1:

Severely calcified bicuspid aortic valve showing the most common pattern in bicuspid aortic valve disease: fusion of the left (L) and right (R) cusps with a prominent calcified raphe (arrow), opposed to a calcified noncoronary cusp (N).

Lifelong clinical follow-up and serial echocardiography are required

Patients with aortic stenosis and a mean/peak gradient of more than 30/50 mm Hg or aortic root diameter of more than 40 mm should have annual follow-up. For those with less severe asymptomatic disease, clinical follow-up may be performed every 3 years.2

Aortic valve surgery is needed after symptoms develop (angina, dyspnea, syncope)

Other indications for surgery include left ventricular dysfunction and concomitant need for heart surgery in patients with severe aortic stenosis or insufficiency. Surgical outcomes are excellent, and most patients return to a normal quality of life. Surgery for an associated aneurysm is recommended if the diameter of the ascending aorta is more than 50 mm or if there is more dilation than 5 mm/yr, regardless of symptoms.2

CMAJ invites submissions to “Five things to know about …” Submit manuscripts online at http://mc.manuscriptcentral.com/cmaj

Footnotes

  • Competing interests: Michael Chu has received financial support from Medtronic Canada to teach minimally invasive mitral valve repair. No competing interests declared for Katie Losenno.

  • This article has been peer reviewed.

References

  1. ↵
    1. Niwa K,
    2. Perloff JK,
    3. Bhuta SM,
    4. et al
    . Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Circulation 2001;103:393–400.
    OpenUrlAbstract/FREE Full Text
  2. ↵
    1. Warnes CA,
    2. Williams RG,
    3. Bashore TM,
    4. et al
    . ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease). Circulation 2008;118:e714–833.
    OpenUrlFREE Full Text
  3. ↵
    1. Michelena HI,
    2. Desjardins VA,
    3. Avierinos JF,
    4. et al
    . Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation 2008; 117:2776–84.
    OpenUrlAbstract/FREE Full Text
  4. ↵
    1. Tzemos N,
    2. Therrien J,
    3. Yip J,
    4. et al
    . Outcomes in adults with bicuspid aortic valves. JAMA 2008;300: 1317–25.
    OpenUrlCrossRefPubMed
  5. ↵
    1. Davies RR,
    2. Kaple RK,
    3. Mandapati D,
    4. et al
    . Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg 2007;83:1338–44.
    OpenUrlCrossRefPubMed
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Canadian Medical Association Journal: 185 (18)
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Vol. 185, Issue 18
10 Dec 2013
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Bicuspid aortic valve disease
Katie L. Losenno, Michael W.A. Chu
CMAJ Dec 2013, 185 (18) 1599; DOI: 10.1503/cmaj.121875

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Bicuspid aortic valve disease
Katie L. Losenno, Michael W.A. Chu
CMAJ Dec 2013, 185 (18) 1599; DOI: 10.1503/cmaj.121875
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    • Bicuspid aortic valve disease is the most common congenital heart disease in developed nations
    • Bicuspid aortic valves are heritable
    • Valvular and aortic complications are common among patients with bicuspid aortic valves
    • Lifelong clinical follow-up and serial echocardiography are required
    • Aortic valve surgery is needed after symptoms develop (angina, dyspnea, syncope)
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