Bicuspid Aortic Foundation View From Here July 2010

When I read about a 48 year old man with BAV in England,
I remembered what happened to my husband twenty years ago.

This man had a heart murmur as a child, but was "reassured and discharged from clinic" at the age of 16. At the age of 48, he was seen for a chest infection. Six months later, when he had more obvious symptoms, he was already in big trouble. - his kidneys, liver, and heart all were failing.

He ultimately underwent emergency surgery. His BAV was so calcified the opening was described as a pinhole. His liver and kidneys improved, but the muscle of his main pumping chamber, his left ventricle, did not come back.

He made his living as a builder, and I wonder what his life is like now - how limited he is by the weakness of his heart muscle.

My husband was roughly that age when what we supposed to be a chest flu became raging double pneumonia. It was the year Jim Henson, the creator of the Muppets, died of pneumonia, and I remember the fear of losing him. He pulled through, but my fear persisted as he was sent to a cardiologist who told us about BAV, and then had aortic valve replacement surgery. Unlike this builder in England, my husband's heart did come back. Eventually his left ventricle "remodeled" itself, losing its abnormal thickness and boot-like shape. He was very fortunate.

Cardiac physicians at London's Heart Hospital wrote about two men with BAV - this 48 year old builder and a 46 year old postman, who also had a heart murmur as a child. Along with a severely leaking valve, the postman also had a 5 cm ascending aortic aneurysm that was replaced during his surgery.

Following is the name of this paper, and a quotation from it:

"An undiagnosed bicuspid aortic valve can result in severe left ventricular failure,"
Sagar Saha, cardiology clinical fellow, Rachel Bastiaenen, cardiology clinical fellow, Martin Hayward, consultant cardiothoracic surgeon, and Jean R McEwan, consultant cardiologist, Heart Hospital, London, BMJ. 2007 February 24; 334(7590): 420-422.

"Our cases highlight the potential complications of a bicuspid valve, which could have been prevented by regular monitoring and earlier surgery. Avoiding urgent and emergency surgery has advantages in terms of surgical mortality (perioperative mortality for first time aortic valve replacement in the United Kingdom: emergency surgery 12%; urgent 5%; elective 1.9%; personal communication, Bruce Keogh, Society of Cardiothoracic Surgeons and AD Cunningham, Central Cardiac Audit Database) and the longer term effects of ventricular impairment. Bicuspid valves are present in many people who were reassured in early life or lost to follow-up and in many "healthy" middle aged men whose hearts have never been examined."

As the authors clearly state, there may be many adults today who were told they had heart murmurs as children. As they reach their middle years, the reason behind what seemed "innocent" murmurs in childhood may express itself, sometimes in a life-threatening way. Those with BAV have more than one reason to be on guard - in addition to the heart damage mentioned in this paper from London, there are other risks - of the aorta tearing or rupturing, as well as the possibility of brain aneurysms.

Recently I found that I can still be taken by surprise when a BAV is discovered. I never knew that someone so close to us - a healthy, active man - the best athlete in his high school class - had a heart murmur as a child. Fortunately for him, some bouts of rapid heartbeat caught his attention and sent him to the doctor. His surgery is behind him now - the BAV and the aneurysm are gone, before they had time to injure him. It was a fresh reminder of all the healthy, athletic adults today who may have BAV. Finding these people early gives them the best possible opportunity for appropriately timed surgery followed by a return to their healthy, active lives.

Heart murmurs,
perhaps forgotten over the years,
are important clues to finding BAVs and aneurysms early,
before there are urgent symptoms or emergencies.

Undiagnosed BAV puts lives at risk.

Diagnosed BAV leads to informed decisions,
creating a climate of hope.

Sincerely,

Arlys Velebir

Chair, Board of Directors
Bicuspid Aortic Foundation

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