Date:           Wednesday, Feb 19th 1997
Subject:     Cardio Report ... The Analysis
                    Updated on September 2nd, 1997

After this weekend I feel ten times more knowledgable about what's going with this heart of mine. My brother-in-law, who is a general physician as well as a good friend, took a call from my cardiologist, Dr. Jayne on Friday the 14th. He then in turn spent hours with my sister and I, getting me to understand the entire situation from top to bottom. And I must say I am relieved! Not that my cardiologist has changed his opinion about surgery, mind you, but I can now explain how it is I feel so healthy in spite of the problems.

In this addendum I hope to provide a better description of the condition of my heart as I understand it. I also want to clear up any inaccuracies originating from the three parts I've written thus far. You'll have to bear with me as I offer a little lesson in human physiology. It was necessary for me to understand it all and is needed here in order to describe the strange developments that occurred during the first year of my life.

Get this. Out of 1000 babies born, 6 to 8 of them have congenital heart disease. About 1% of this group (or 1 out of 14) will have coronary artery defects such as I did at birth. But you'd have to find about 1800 children with CHD before you would encounter 1 case exhibiting the unusual arrangement that I, in fact, inherited. And virtually none would have survived it without corrective surgery. In researching similar cases, the oldest patient Dr. Jayne could come up with was a girl who underwent the surgery at the age of sixteen. (I later came across a study done in 1996, Adult-type ALCAPA, stating there were only 300 cases reported worldwide ). I do indeed feel privileged having made it 42 1/2 years now without!

The great arteries, as they are described in medical literature, located on top of the heart, are the aorta and the pulmonary artery. The aorta carries oxygenated blood originating from the lungs via the left ventrical out to the body's vascular system. The pulmonary artery, on the other hand, carries deoxygenated blood from the heart via the right ventrical out to the lungs for replenishment. In a normal heart there are two major coronary arteries which supply the heart muscle with oxygenated blood. Looking at the front of the heart from left to right, they are the right coronary artery (RCA) and the left main artery located behind the pulmonary artery. After a very short distance the left main branches to form the left anterial descending artery (LAD) and the left circumflex artery (LCA). The roots of both coronary arteries originate from the base of the aorta.

Within that group of infants born with anomalies or defects in the coronary arteries there are cases that bear resemblance to mine in one way or another. The anomaly of misplaced or missing connections is possible within that smallest group, but the combination of various defects is at least interesting, if not just plain rare. In my case, the left main artery does not exist. The fact that my LCA is directly attached to the aorta is unusual and aided in my survival. It is highly unusual, however, that it is connected to the base of the aorta on the right side, branching from the RCA and wrapping around the posterior of the heart, reaching over to and feeding the left side of the myocardium as the circumflex artery should have been. The LAD, then, is completely isolated and was essentially non-functional at birth. What I did not understand previously is that my LAD is connected to the pulmonary artery which is totally contrary to it's design. In a normal heart system it would be conducting oxygen-rich blood to the heart muscle from the aorta, not oxygen-poor blood from the pulmonary artery. An additonal defect exists in the form of a blockage due to a narrowing of the artery wall (stenosis) near this junction. In particular, the mislocation of the LAD is unusual and certainly fatal if not corrected surgically within the first year of life! The direct flow of deoxygenated blood through this artery and entrance into heart muscle tissue would weaken the heart in a short time. So how did I escape this fate? It was the stenosis, that second defect, near the top of the LAD that restricted this toxic flow into my heart!

My Heart  Closed For Renovations - Check Back Later!

The miracle continued as I developed physically. In order to feed the central area of the heart muscle, the only functioning coronary arteries eventually responded by taking on more blood volume. Over time the RCA and LCA expanded well beyond the normal size for these vessels. When Dr. Jayne described them as the largest the cardiology staff had ever seen I couldn't comprehend his meaning. But the numbers explain it well ... a normal diameter for these arteries would be in the range of 2 to 4 mm. Mine yielded measurements of 10 mm! (This was in fact the source of my heart murmur all along from the first diagnosis when I was sixteen. Not the sound of a leaking mitral valve, but instead it was the sound of high volume blood flow through these two monsterous arteries). Now I know that what appeared on the video monitor during the catheterization as large coil-shaped structures was truly my right coronary artery and not some unassociated part of my vascular system as I had assumed at the time. The rest of the phenomena which led to the complete distribution of blood to my heart occurred during the expansion process. Large collateral arteries developed between all three vessels, sending blood to the under-utilized LAD. The bizarre part is how the flow became strong enough to invade the LAD and reverse the normal downward direction of flow. This could only happen by virtue of the stenosis that was severe enough to not only limit the flow of oxygen-poor blood from the pulmonary artery, but also limit the leakage of oxygen-rich blood now opposing it from the collateral arteries.

With such a strange plumbing system on board, no template existed for Dartmouth's computer modelling software. Due to the complexity of the data generated during my cardiac cath. procedure, rendering images by computer was not possible, so Dr. Jayne had to present his own hand-drawn diagrams to the small conference last Friday. In combination with so many video images, physical dimensions and blood pressures taken at various points within the arteries, I'm told there were lots of "ooohs" and "aaahs" coming from all those present. Overall, everyone agrees that my heart has compensated beautifully during all these years. It works pretty darn well if I can maintain the level of running I've done over the past eleven years. In the end, however, the recommendation was for surgery. The main point of concern being the possible shunt effect which could occur as the reversed flow in the LAD increases to an extent that the leakage of oxygenated blood to the lungs becomes serious. That seems possible under extreme, sustained physical stress. How much stress was involved during those hard 400m intervals? I'm not clear on this.

Obviously the choice for or against surgery will ultimately be mine.The only question that remains in my mind is if I forgo surgery should I continue running competitvely? And what kind of running should that be, long slow distance or the brief intensive sprints that I'm fond of? I'll certainly need to study this further.

ObRN:  Oh yes! That 'R' does stand for Running (8 friggin' weeks is long enough!). I did run 1 1/2 miles yesterday with only a little bit of pain. Though I used BOTH legs this time, I got tired pretty quick. Starting over is NEVER a fun experience.

Part Three

Conclusion

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