When athletes die young

This is my second posting on Off-Wing Opinion. I will continue to periodically discuss sports related medical issues in dual postings – there and here

We shudder when we hear or read about an athlete dying young. Let me share three names – Hank Gaithers, Flo Hyman, and Pistol Pete Maravich. All died young. All died on the court. Why does this happen? Could we screen for these deaths?

They died from different causes, all heart related, but very different etiologies. None had a classic heart attack. Prevention of Sudden Death During Exercise.

Hank Gaithers had hypertrophic cardiomyopathy, which often causes abnormal heart rhythms. He technically died from an arrhythmia, which was clearly secondary to the hypertrophic cardiomyopthy.

Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden cardiac death in young American athletes. In HCM, there is abnormal thickening of the left ventricle (heart main pumping chamber), making it harder for the heart to pump blood into the aorta. Hypertrophic cardiomyopathy can be fatal when it causes malignant heart rhythms.

We do not know what causes this problem, and as I will discuss later, we often do not make this diagnosis prior to death.

Flo Hyman died from Marfan’s syndrome – which lead to a ruptured aortic aneurysm. Marfan Syndrome: A Silent Killer Again quoting from the Prevention of Sudden Death article

A connective tissue disorder that causes weakening of he blood vessels seen in people who are all tall- nicknamed Abe Lincoln disease. I the walls of the aorta are damaged by Marfan?s syndrome, they can tear during strenuous exercise as happened to Flo Hyman.

I remember a University of Maryland basketball player named Chris Patton who died of Marfan’s. We could possibly screen tall athletes for Marfan’s – and some have been found and managed – but they must avoid strenuous exercise.

The most bizarre cause happened to Pete Maravich. He had a congenital abnormality of his coronary arteries. Most are born with a right coronary artery, and a left coronary artery – which subdivides into two major branches. Thus, we generally have 3 coronary arteries. Maravich had a rare congenital abnormality – a single coronary artery. This represents one of a variety of unusual developmental abnormalities.

The blood vessels that run into the heart muscle do so in an irregular manner. In some cases, the patient has a single blood vessel instead of two, as was the care with Pete Maravich.

So we now have discussed how three famous athletes died suddenly. What new message do we have?

An article in the March 19, 2003 Journal of the American College of Cardiology discusses sudden death in athletes – Hypertrophic Cardiomyopathy Often Undiagnosed in African Americans

The findings are based on a study of 584 male athletes who had died suddenly and were entered in a national registry. The comparison group consisted of 1986 patients who were clinically diagnosed with HCM at one of four major medical centers.

Of the athlete deaths, 286 were due to cardiovascular diseases, lead author Dr. Barry J. Maron, from the Minneapolis Heart Institute, and colleagues note. Fifty-five percent of these deaths involved white athletes and 42% involved black athletes.
The most common cause of cardiovascular death was HCM, responsible for 36% of the cases, the authors note. The next most common etiology was anomalous coronary artery of wrong sinus origin, accounting for 13% of cases.

Although most cardiovascular deaths involved whites, 55% of deaths due to HCM involved black athletes. “In contrast, of 1986 clinically identified HCM patients, only 158 (8%) were African American (p < 0.001)," the researchers report.
Disproportionate access to healthcare could explain the racial disparities identified, the authors note. "Alternatively, it is possible that HCM in African Americans may represent a more virulent form of the disease, possibly due to a malignant genetic substrate when associated with exercise, and, thereby, predisposing to sudden death on the athletic field in susceptible individuals," they add.

“Regardless of these considerations, it is our aspiration that the present report will trigger greater awareness that HCM not uncommonly occurs and is an important cause of sudden death in young African American males,” the researchers write. This should create “a higher index of suspicion and ultimately more frequent clinical HCM diagnoses in such athletes.”

This article raises the question of how we should screen prospective athletes. The American Heart Association has a position paper on this topic – Cardiovascular Preparticipation Screening of Competitive Athletes . These recommendations are complex, and probably incompletely followed. Each time an athlete dies on the field or court, we must ask why. One could argue that we should perform a much more complete and complex evaluation to save some of these lives. These are difficult medical decisions; these are difficult societal decisions. Screening for rare conditions costs significant dollars. How much are we willing to spend?