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Marfan Syndrome: A Silent Killer
Richard Demak
February 17, 1986
A hidden congenital disorder claimed the life of volleyball star Flo Hyman and has sidelined three college basketball players
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February 17, 1986

Marfan Syndrome: A Silent Killer

A hidden congenital disorder claimed the life of volleyball star Flo Hyman and has sidelined three college basketball players

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Flo Hyman, the best American woman volleyball player ever, was spending this winter as the star of a talented team in a top-class Japanese league. On the night of Friday, Jan. 24, Hyman's team, Daiei, was playing in Matsue, a city on the shores of the Sea of Japan, some 380 miles west of Tokyo. During the third game, Hyman was taken out in a routine substitution. She sat down on the bench. Seconds later she slid silently to the floor and lay there, still. She was dead. An hour later, doctors at the Red Cross Hospital in Matsue ruled that the cause of death was a "heart attack."

Flo Hyman had died of no such thing. An autopsy performed in Culver City, Calif. at the request of Hyman's family six days after her death revealed that what killed this strong and vital 31-year-old athlete was a disorder much less common than a heart attack. Hyman, the star of the 1984 U.S. silver medal-winning team, had died of a ruptured aorta caused by Marfan syndrome, a congenital condition that exacts a disproportionately large toll among tall, lanky people such as Hyman, who was 6'5". For this reason, Marfan syndrome is—or should be—of special interest to physicians who treat basketball and volleyball players and other athletes who tend to be tall.

The pathologist who performed the autopsy, Dr. Victor Rosen, was given permission by Hyman's family to talk to SI about his findings. Rosen, once an assistant in the office of Los Angeles County coroner Thomas Noguchi, said that Hyman had been in superb condition except for a single fatal flaw—a dime-sized weak spot in her aorta, the massive artery that carries the entire flow of blood leaving the heart. That small spot, less than an inch above her heart, had been there since her birth, and there the artery had burst, exploding inside her chest as she sat on the sideline in Matsue. It was the result of Marfan syndrome.

The malady was first described in 1896 by Antoine Marfan, a French pediatrician. It is a genetic abnormality that affects connective tissue—the stuff that binds and supports all the cells in the human body. The defective genes that cause Marfan syndrome result in critical changes in the protein that gives connective tissue its strength. This weakens and, in effect, loosens the tissue, producing, in ways that are not always clear, characteristics by which victims of Marfan syndrome are commonly identified: tallness, long fingers, deformities of the breastbone (in some cases protruded, or pigeon-breasted, and in other cases indented) and nearsightedness. Hyman was nearsighted and wore glasses off the court. To some experts, Abraham Lincoln's long fingers and great height (he was 6'4") indicate that he may have suffered from the syndrome. It has also been suggested that the long fingers that helped account for Niccolò Paganini's dexterity on the violin were the result of Marfan syndrome.

Of all the symptoms, tallness is the most obvious. The average height of white males with the disorder is 6'2". People with Marfan syndrome are often tall even when other members of their families are not. (However, Hyman's father is 6'1" and her mother, who died several years ago, was tall—5'11".) Also, the arms tend to be disproportionately long. With most people, when the arms are extended from the sides, the distance between the fingertips of the left and right hands is about equal to one's height. For someone with Marfan's, the arm span is usually much greater than the height.

Why the genes that control the connective tissue malfunction in this way is a mystery. Men and women of any race can be affected, and in two-thirds of the cases, the syndrome is clearly inherited, but not necessarily from both parents. Every child of a carrier of the defective gene has a 50% chance of having Marfan's and some of its characteristics.

Marfan syndrome is particularly frightening because its diagnosis is very difficult. Most people who are very tall or have supple fingers or are nearsighted do not have it. It affects only one person in 10,000. There is no laboratory test that will detect it. It doesn't show up in blood tests or biopsies. Dr. Reed Pyeritz, director of the Medical Genetics Clinic at Johns Hopkins Medical Institutions in Baltimore, is an expert on Marfan's. Pyeritz, an associate professor of medicine and pediatrics, treats 400 people for the syndrome every year, but he says there are many others, including, presumably, some playing sports at various levels, who are Marfan sufferers but don't know it. Pyeritz says, "Often the first person to make the diagnosis of Marfan's is the coroner."

Tragically, such was the case with Hyman. A similar case involved a University of Maryland basketball player 10 years ago. Chris Patton, a 6'9" sophomore, had just finished his second season with the Terrapins. Patton's coach, Lefty Driesell, recalls what happened: "The season was over, and the nets were up on some outdoor courts near the stadium. Chris lived nearby. It was a pickup game, and he went up for a dunk. They told me he was dead before he hit the ground." An autopsy showed young Patton's aorta had burst because of the lifelong weakening effects of Marfan's. No hint of the illness had been detected before he died.

It was the same with Hyman. In spite of all the physical exams she received in her athletic career, not even the tiniest sign had surfaced. She had been a member of the U.S. volleyball team since 1974—one of seven players who stayed with the team after America's 1980 boycott of the Moscow Olympics. The hard-driving U.S. coach, Arie Selinger, purposely used techniques that were brutal in order to condition his team, and he chose players accordingly. He wanted only the best and seemingly fittest of athletes, even if they weren't the most polished volleyball players. Hyman was sensitive to heat and suffered occasional fainting spells, but this is not believed to be related to Marfan syndrome.

In March 1982 Hyman was given a physical at the Olympic Training Center in Colorado Springs. "Her exam revealed nothing," said Jenny Stone, the head athletic trainer at the center. Hyman had at least one more thorough physical from doctors connected with the U.S. Olympic program, and in 1984, she, like all Olympic athletes, was examined upon arrival at the Olympic Village in Los Angeles. All was apparently normal. She continued to give the appearance of perfect health right up to the moment of her death. "The pathologist told me she had a beautiful body," says Hyman's sister, Suzanne Jett. "He said she had the heart of a baby." Rosen told SI, "There was no fat on her. She had tremendous muscle development. She had what we call a time-bomb lesion. Only no ticking is heard."

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