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An undiagnosed bicuspid aortic valve can result in severe left ventricular failure

BMJ 2007; 334 doi: (Published 22 February 2007) Cite this as: BMJ 2007;334:420
  1. Sagar Saha, cardiology clinical fellow,
  2. Rachel Bastiaenen, cardiology clinical fellow,
  3. Martin Hayward, consultant cardiothoracic surgeon,
  4. Jean R McEwan, consultant cardiologist
  1. Heart Hospital, London W1G 8PH
  1. Correspondence to: J R McEwan j.mcewan{at}
  • Accepted 11 December 2006

Asymptomatic bicuspid aortic valves diagnosed in childhood need regular monitoring to allow early surgical intervention and prevention of left ventricular failure

Bicuspid aortic valve occurs in 0.8-2% of European and North American populations.12 It is the most common reason for a predisposition to severe aortic regurgitation or stenosis in middle life, but patients are asymptomatic until late in the disease. Failure to present until clinical symptoms develop—either because the murmur is not detected or is lost to follow-up—can have important consequences. Early detection and continued surveillance are crucial to allow early intervention and preservation of cardiac function. We describe two patients with heart murmurs diagnosed in childhood who were lost to follow-up. Both presented as adults with heart failure and complications of bicuspid aortic valves. One developed aortic stenosis, the other aortic incompetence. Both needed urgent surgery. Many people in the general population with bicuspid aortic valves have never been diagnosed or have been reassured in childhood and are no longer under review.

Case 1

A 48 year old builder presented with shortness of breath at rest and mild icterus. He had been prescribed antibiotics for a presumed chest infection when he visited his general practitioner six months earlier. A heart murmur was noted during that consultation but not followed up. He later recalled that as a child he underwent annual review for a heart murmur. At age 16 he was reassured and discharged from clinic.

On clinical examination the patient had severe aortic stenosis with a slow rising pulse, narrow pulse pressure, and an ejection systolic murmur radiating to the carotids. …

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