Q. I just read about an awful tragedy - a young man died unexpectedly while playing sports. Why does that happen?
A. Fortunately, this dreadful occurrence is quite rare. But when an otherwise apparently healthy youngster dies suddenly (especially during physical activity), there are about a half-dozen conditions that we consider.
The most common cause is called hypertrophic cardiomyopathy, or HCM, which is an abnormal thickening of the walls of the main pumping chamber of the heart (the left ventricle). This condition typically is genetically transmitted, and it is important to identify because siblings of the affected patient may have the condition as well.
Another genetically transmitted condition affects the transport of compounds into the cells of the heart and can lead to fatal irregularities in the heartbeat. This condition - called the long QT syndrome or LQTS - also runs in families.
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Other subjects have inflammation of the heart, usually because of a viral infection; we call this condition myocarditis. And some patients have abnormally positioned arteries (the coronary arteries) that feed the heart muscle. Others may have structural problems with their hearts, such as holes in the walls separating the heart chambers, but these patients usually are identified beforehand, because of the presence of a heart murmur or symptoms.
Last, some cases of sudden death are from the side effects of drugs, either over-the-counter or prescribed medications, or illicit drugs like cocaine.
Q. How can youngsters who might have problems be identified ahead of time? And if a problem is detected, what can be done to prevent subsequent sudden death?
A. How to identify these conditions ahead of time is a controversial and contested topic. While a few patients with one of these conditions will be identified by a routine history and physical examination, most will not. And how to identify them is the real challenge.
The two most useful tests are the electrocardiogram (where leads are attached to the skin and a recording is made of the electrical activity of the heart) and the echocardiogram (where ultrasound is used to generate images of the heart chambers and valves). But there are problems with both screening tests - they cost money; they don’t catch everyone with a potentially fatal condition; and they incorrectly identify many subjects as at risk who on further testing are found to have nothing wrong with their hearts.
So there is no consensus as to whether these screening tests are worth the effort. In Italy, screening is routine, but not so in the United States.
If you are concerned, the best advice I can give you is to talk with your primary care provider for further guidance on this complicated subject.
As to prevention, if we do identify one of these conditions, the best treatment that we have is a special type of pacemaker called a defibrillator that can be implanted surgically in the upper chest and will shock an abnormal (and potentially fatal) heart rhythm irregularity back to normal if it occurs.
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Wynne is vice president for health affairs at UND, dean of the School of Medicine and Health Sciences and a professor of medicine. He is a cardiologist by training.
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The content of this column is for informational purposes only and does not substitute for professional medical advice or care. The information provided herein should not be used for diagnosing or treating a health problem or disease.
If you have or suspect you may have a health problem, you should consult your health care provider. Never disregard professional medical advice or delay in seeking it because of something you have read in this column.
