Doony's heart condition: Aortic Stenosis

Normal Valve

Doony was born with a bicuspid instead of a tricuspid valve in his heart. Normally, an aortic valve is made up of three leaves. Doon was dreaming of butterflies when he picked his out or something, because he got a weird one which has only two leaves. This means the ventricle which pumps blood through the valve has to work harder -- the valve doesn't open as wide as it should.

Bicuspid Valve

For many people with a bicuspid valve (it's a common defect) there's no problem beyond that. Some never find out they even have the defect until late in life, and their left ventricle just works a little bit harder. Heart conditions late in life are more common among people with bicuspid valve, but with proper diet and exercise it doesn't necessarily lead to a decreased life span.

Doony went through a series of tests, culminating in a 3-D echogram in February. This confirmed the valve was bicuspid, but the condition was listed as moderate: and that meant better to just leave it alone than risk any kind of an intervention, since it wasn't serious. The one concern was that no heart murmur had been heard when Doony was born. If somebody had just missed it, no problem. But if the murmur hadn't been there, and it had been detected because it was getting louder, we might have a progressing rather than a static condition -- and that would be cause for worry.

Worry is the word today. June 23rd we went back for a follow up echogram. This revealed an increase in the systolic pressure from 55 to 80 and a thickening of the heart wall to 30% above normal. The condition moved from "moderate" to "significant".

"Significant" means the risk of just leaving it alone is now about on par with the risk of an intervention. Because the ventricle works harder and harder, this means the walls of the heart thicken and the chamber hypertrophies -- i.e. the ventricle expands and can start to take over room from the other chambers. This increases the chance of a sudden heart failure.

Normally, the first intervention is to insert a catheter in Doon's leg and thread it up into the chamber, where a tiny balloon is inflated inside the valve to try and expand it gently. The risk of this procedure is tearing the valve. Other options are surgery to reach the valve and use a similar procedure, or replacement with an artificial valve. That of course isn't usual in a child Doony's age because he'd have to trade in for a bigger heart valve as he grows up. And if that happens at the rate he goes through shoes, that would probably mean an awful lot of heart valves.

Current situation is that we go back in September for another test, where Doony will be sedated. He was fighting the echogram this time, so his heart was working harder, which made an accurate measure difficult. They know he's on the borderline between intervention and leaving it alone, but they want to be sure he's over that line before we take any action. So a few more months to see if it continues to progress, a sedated test, and we'll know if we're looking at an immediate procedure in September or a further wait and see.

In the meantime, if Doony is sick he doesn't know it. He's a tough little guy and we're confident he is going to be just fine. We'll be complete wrecks worrying, or course, but he'll be fine.

September 21, 2000 UPDATE: We just got the test results, and his systolic pressure was measured at 60-70. The thickening of the heart wall was 20% over normal. So the verdict is just watch the condition for the next couple years, and the doctor considers it unlikely he'll need a intervention in the next five years, though all depends on the semiannual tests. He fought the echogram again, but not so fiercely that he needed to be sedated. Personally, Marth and I could have used a little sedation, but we're hugely relieved at the results.