Critical Aortic Stenosis and Heart Failure

"Sunset scene from a little hill . . . ."
                                                                          - Seven Angels by Paul Bernhard Rogers, sung by Sissel

I mentioned that I was not overly afraid of a heart murmur. 

But I was not the one who had grown up in its shadow.
Perhaps only those who live with this knowledge from a young age understand.

My husband was not allowed to play sports with his classmates. He was on the sidelines, as the team manager, when he wanted to be out there playing. Yes, he had lived with the thought through out his life that his heart was less than perfect. But he never let it stop him from living his life. As a young man with BAV who had surgery in his teens told us, "I refuse to live in fear."

For decades my husband had proved that it was entirely possible to live a healthy, active lifestyle. In those years, if he had seen a doctor, which he did not, they might easily have told him not to think about it. He might never need anything done.

But never is a long, long time.

I avoid the words always and never when talking about bicuspid aortic valves.

Now, it could not be denied. His aortic valve was barely opening, and his heart could no longer compensate. He was in what is called congestive heart failure. It affects your whole body.

What is Congestive Heart Failure Like?

It was as though he had aged decades in the wink of an eye. An active, strong man had turned into an elderly shadow of himself almost overnight. With the pneumonia, he had lost between 10 and 20 pounds of muscle. What do I remember about him? He had no strength, no energy. He was short of breath, even at rest, and could not breathe lying flat. Going for a gentle walk on a flat surface, he had to sit down and rest. He had to sit down to tie his shoes; bending over took too much effort.

His face had no color whatsoever. They checked his oxygen levels many times, thinking they would be low. The levels were fine, although he was so pale he looked like he had no blood at all. Our friends who saw him at that time never forgot that deathly looking pallor he had. He was on a diuretic to keep the fluid out of his lungs. Interestingly, although fluid wanted to gather in his lungs, he never had even a hint of swelling in his ankles. He never had any pain (some people do, it is called angina).

I never said it out loud, but icy fingers of fear gripped me. Oh yes, I was afraid now. Was this what happened to someone with a heart murmur? They had told us his heart muscle had distorted itself from working too hard. He now had a boot-shaped heart.

Over the years I have asked others with aortic valve stenosis about their experience, looking for someone whose heart failed like my husband's. I found two of them, both men, similar to his experience. And about 19 years later, his own sister went into heart failure suddenly, just like her brother. Again, there is variation in how each person's body reacts to a narrowed aortic valve.

What the Angiogram Showed

We did not understand the details of it all very well back then. I am looking at the report of his angiogram, written in two parts, by both the cardiologist and radiologist. This test is what the internist told us was the "gold standard" to understand what was going on inside him. Today there are CT angiograms, which may provide enough information without putting a catheter up into the heart.

• His aortic valve narrowing was described as critical, the opening calculated at 0.5 cm squared, and the valve was heavily calcified.
• He had moderate to severe pulmonary hypertension.
• His overworked, thickened left ventricle had global mild to moderate hypokenesis. The muscle was under-active, not squeezing as vigorously as it should. 
• There are two different numbers for his ejection fraction, 30% and 40%, measuring how much blood is "ejected" with each squeeze. Regardless of which was correct, not enough blood was getting through.
• Pressure build up across the valve was 75 mm with a mean of 57 mm.

All of this proved why his body was screaming. His left heart muscle was getting too weak to push his blood out through a narrow, highly calcified valve to circulate through his body. His lungs were under pressure.

There was some good news, his coronary arteries were normal, with no signs of blockage! Of the many bicuspids I have met since, this has usually been the case. It is a great thing to be spared the trouble of clogged arteries of the heart, which is what is usually called heart disease, and the main reason for heart attacks. Bicuspids have enough to cope with, without that also. (However, remember, there are always exceptions!) Each person's body will ultimately tell them and their doctors just who they are in the spectrum of possibilities for those with BAV.

There is one other thing noted in the reports by both the radiologist and the cardiologist: post stenotic dilatation. The cardiologist called it "significant". The radiologist writes it was "of the aortic root". Nothing was ever said to us about this. These many years later, I have learned why. More about this later.

Waiting for Surgery

It seems ridiculous to me now, but he briefly went back to work before he had surgery. The doctors did not suggest that he stay home, and we did not think to ask. In hindsight, we were not thinking very clearly! One day he called me, telling me that he had some kind of "episode". We did not bother to ask, but made the decision right then - no more work until after surgery. One day, still at home waiting, his heart rate jumped up. We called the cardiologist for guidance, who was not concerned unless it went even higher. Thankfully, it calmed down on its own.

I have spoken with three women who suddenly lost their husbands to stenosis of their bicuspid aortic valves. Two of them knew they had it, and were under some level of medical care. Each one of these families have gone through shocking tragedies. It should not have happened to them. All these men needed was surgery. 

No one warned us those many years ago about the risk of sudden death from aortic stenosis. I reflect on where we were at that time, and I realize that my husband too must have been at risk of his heart just stopping. I wonder why quite so much time went by before his surgery, which was scheduled just over 5 weeks after that first echocardiogram. If I could go back to that time today, I would question the delay. 

And so it was high time for surgery. He would not get any better, only continue to decline. It was a relief to at last have him safely admitted to the hospital, the night before surgery. Would his heart recover its strength? Could he come back to being that same strong man in the prime of life once again? That is the next part of the story.