Saturday, August 2, 2014

There Are No Perfect Numbers - Making Decisions

In mathematics, there are perfect numbers.
The first one happens to be 6.
 But in aortic disease, there are no perfect numbers.

Just 2 millimeters more
How big is 2 millimeters? It is the thickness of a US dollar coin. Looking at it, one wonders how such a small distance could make so much difference. For one man, it was the difference between having surgery or not. His aorta was 5.8 cm. The doctors told him surgery is done at 6.0 cm. The difference - just 2 mm - the thickness of a coin.

When "6" was the Number
For Louie Lopez, this tiny length became a distance too far, a point never reached. He was in the hospital, talking with the surgeon, when his aorta tore and fully ruptured. Massive bleeding, massive damage. Rushed to surgery, he had no chance. The year was 1995. Yes, "6" was "the number" for aortic aneurysm surgery back then.

Not long ago, Terry Lopez talked about that time, almost 20 years ago, when she lost her husband, Louie. "They were waiting for just 2 mm more. Just 2 mm....surely, 20 years later they can do better than that."

Reading papers, watching medical conference videos from thought leaders in thoracic aortic disease, I marvel at the paucity of answers, the preponderance of uncertainty still, as they continue to debate aneurysm size and the timing of surgery. There is still more unknown than known. Aortic disease remains a more than worthy opponent for those who do battle with it.

The Numbers
Physicians look for numbers to guide them in making decisions about aortic disease. For aneurysms, the most prominent number is the size (diameter) of the aneurysm. There is a great deal of debate about the number, the size when surgery should be done. Some argue to protect from what can be serious complications of surgery, urging that patients wait for the aorta to grow larger. Others promote earlier surgery, at smaller sizes, to protect from life-threatening, deadly events.

The following paper from 2013 is an example of the debate, giving pro and con perspectives from physicians around the globe, Germany, the US, and Australia:

"Aortic Surgery for Ascending Aortic Aneurysms Under 5.0 cm in Diameter in the Presence of Bicuspid Aortic Valve",  authored by Klaus Kallenbach, MD, PhD,, Thoralf M. Sundt, MD, and Thomas H. Marwick, MD, PhD, MPH. 

In this paper, it is noted that very few patients were studied (with differing underlying causes of aortic disease), in coming up with the number for surgery, although used to guide the care of millions.  "The number" has generally been coming down over the years as surgery became safer, but the uncertainty of what the number should be remains.

Some individuals suffer aortic tearing, perhaps death, at smaller sizes. On the other hand, surgery has risk, and offering surgery at smaller sizes may injure someone whose aorta would not have torn or ruptured if allowed to grow larger.

 The size of the aneurysm alone is just not enough. Other measures, other ways to understand, are needed, but not available today.

If there were "perfect numbers" for the aorta, our friend Richard would not have dissected at about the same age and the same aneurysm size as my husband (who did not dissect, but had preventive surgery at 5.2 cm). Richard barely survived and has paid a high price in additional surgery because of his original dissection 10 years ago.

For the narrowed aortic valve, aortic stenosis, there are also numbers: the size of the valve opening and the pressure build up. There are measures for the leaking aortic valve also. Are they perfect numbers, that each individual can trust with their life? Is there a promise that nothing bad will happen before you reach the "guideline" numbers? Nothing bad will happen until you have obvious symptoms?

If there were "perfect numbers" for aortic valves, Chuck Doherty would still be with his family. So would  Chad Rogers.

Guidelines, Not Guarantees 
Those with BAV and other forms of aortic disease need to understand there are no guarantees, no solid promises. There are guidelines based on information available today. To our surprise we may find them quite limited, once we understand what they are based upon. As a patient, you need to research for yourself, understanding as much as you can.

Making Decisions
Part of that research can be searching for and choosing physicians/surgeons with a philosophy and approach to risk that is in agreement with your own. There are things to think about, such as your lifestyle, frequency of foreign travel, and general comfort with the risk of surgery versus the risk of waiting. These decisions should be individualized within the context of guidelines and statistics; decisions made in conjunction with physicians whose philosophy and approach to life and risk are in harmony with your own. Physicians who clearly share their outcome statistics with you.

Handling Uncertainty, Owning Decisions
In 2013, Aortic Valve and Ascending Aorta Guidelines for Management and Quality Measures were published. Including references, there are 66 pages. In the summary on page 54 are these words,"The choice of the best procedure or valve is dependent on many factors as discussed above and no procedure or device is ideal. Ultimately it is up to the patient, the cardiologist, and surgeon to reach a decision on appropriate treatment." 

As these guidelines tell us, it is very important that you, the patient, are a partner in these decisions, and that you understand the basis on which you and your physicians together are making these decisions.

Wouldn't it be wonderful if there were perfect numbers, perfect devices, and simple decision making? Yes, but as in much in life, that is rarely the case. There are pros and cons, trade offs, in most decisions that must be made. But we only have one heart, one aorta, so these are very important decisions.

The more you know, the more you understand the pros and cons, you can make decisions that belong to you, that you can own. Not knowing leaves us vulnerable, caught by surprise. Perhaps many of us have already been there, rudely discovering the imperfections and limitations regarding aortic valve options.

Over 20 years ago, the mechanical valve that saved my husband's life was called a lifetime solution. The night I walked into our home and found my fallen husband, terribly injured by that valve and its complications, I began to understand the terrible depths of untruth in that "promise" that we had happily once believed. I will never forget him saying to me, "I did everything I was supposed to do."

If you understand there are no "perfect numbers", no "ideal" devices and solutions, it will at least help you to be proactive, to question, to research, to seek more opinions when things don't make sense, and to partner with your physicians in the decisions that are made.

And when something does not feel right, when an echo or other test results do not make sense, don't let it go. Keep asking questions, seeking answers.

No one cares more than you do.
 Because it is you, the patient,
 above all others,
 who will "own" the outcome.

May this help you to read,
 and make informed decisions,
while living in a climate of hope.

Best wishes to all,
Arlys Velebir
Bicuspid Aortic Foundation