I don't know if people heard about this, but a participant in the Olympic trials prior to the NY marathon died suddenly:
A triumphant United States Olympic trials marathon turned somber yesterday morning when Ryan Shay, a 28-year-old veteran marathoner, collapsed during the race in Central Park and was pronounced dead at Lenox Hill Hospital.
It put a terrible twist on the victory by Ryan Hall, who exulted in the emotion of winning the race and capturing an Olympic berth. But he had no idea that the ambulance that had passed him on the course was carrying Shay, his good friend and occasional training partner, a man whose wedding he had helped celebrate in July.
Shay collapsed at the five-and-a-half-mile mark near the Central Park boathouse, relatively early in the 26.2-mile race, and he was pronounced dead at 8:46 a.m., stunning the sport on a cool, crisp morning that seemed perfect for a marathon. The death was announced by Mary Wittenberg, the president of the New York Road Runners, which staged the race. No cause of death was given. The medical examiner's office said an autopsy would be performed today.His former longtime coach, Joe Vigil, said Shay had no health problems. But Shay's father, Joe, said in a telephone interview last night that a doctor found Ryan had an enlarged heart when he was 14. He said the doctor was concerned at the time and recommended keeping an eye on his condition.
"You always think, why didn't we see this coming?" said Joe Shay, who was not in New York for the race. "Ryan never complained about anything. He said everything like that was an excuse for lack of preparation."
Joe Shay said Ryan told him he had another heart test this spring and a doctor said he might need a pacemaker when he was older.
It is always terribly sad when something like this happens. A lot of non-medical people ask me why something like this could happen to someone who was in such visibly good shape.
A differential diagnosis is a list of things that can cause a particular symptom or illness. The differential diagnosis for sudden death in young athlete is a very short list:
- Hypertrophic cardiomyopathy -- Hypertrophic cardiomyopathy is on the top of the list; it is the most common cause with this presentation. It is a rare inherited condition where the walls of the heart to grow into the ventricles. During times of vigorous pumping such as running a marathon the overgrown walls can sometimes obstruct the outflow of the heart causing sudden death. The doctors in this case appear to believe that Mr. Shay had this condition because he had a large heart. People ask, "can't you screen for that?" Well, yes, but competitive athletes often have enlarged hearts anyway, and in general this is not a problem for them. This is probably why the doctor who screened him did not think it would be an issue. (More on hypertrophic cardiomyopathy in this post.)
Congenital long QT syndrome -- Long QT syndrome is a inherited problem with the electrical system in the heart. The term QT refers to the length of time in a particular part of the EKG -- what the doctor reads to understand the electrical activity in someone's heart. To the right is an example of an EKG. Basically the QT interval is the time it takes for ventricular depolarization -- leading to contraction -- and repolarization to occur. If the QT interval is long, it suggests that the action potential leading to the contraction is also long. The heart muscle is not repolarizing quickly enough. This can cause problems if some parts of the heart are repolarizing faster than others; they may be able to contract again -- i.e. not be refractory -- while other parts are still depolarized. This can cause a circling depolarization in the ventricles that is no longer paying attention to the pacemaker of the heart -- cardiologist call this a "re-entrant phenomena." It can result polymorphic ventricular tachycardia or torsade de pointes -- this is often the stuff on TV shows that precedes someone having to be shocked with paddles to resuscitate them. Torsades looks like the EKG trace below.- Aberrant coronary origins -- Aberrant coronary arteries constitutes a range of developmental abnormalities where the coronary arteries that service the heart are not connected in the normal way to the aorta. Usually this is clinically silent, but sometimes they are connected in a way that during extreme exertion blood is cut off to the heart causing sudden death.
- Arrhythmogenic right ventricular dysplasia (ARVD) -- This is a rare autosomal dominant disease where part of the ventricular wall undergoes programmed cell death -- apoptosis -- and is replaced with fat or scar tissue. The replacement of heart tissue with non-conducting fat or scar changes the conduction system in the heart making the individual prone to arrhythymia.
Brugada syndrome -- I was talking to my Dad who is an ER doc about this, and he said, "You will be doing a public service if you advertise Brugada syndrome." So here goes. Brugada syndrome is a recently recognized (like the last 10 years) heritable mutation in a sodium channel in the heart. Though there is some debate about the pathogenesis, it is believed that this causes a shortening of the action potential in some places in the heart as opposed to others. This would shorten the refractory period for those portions and lead to a re-entrant phenomena as described above. The tricky part is that Brugada is temperature and exertion dependent. Sometimes there is no change in the EKG; sometimes there is. The one good thing is that Brugada has a very characteristic EKG signature that looks like a slide. If you see an EKG like the one on the right in leads V1-3, you have someone with Brugada syndrome.
Other choices for sudden death in a marathon runner are hyponatremia, Marfan's syndrome, asthma, and heat stroke. Hyponatremia has killed several runners in recent years, but most of them are not professionals and do not know how much water to drink during the race. I don't think that was this guys deal. Marfan's is a connective tissue disorder where among other things your blood vessels do not hold together well. This can sometimes cause an aortic dissection and sudden death. However, patients with Marfan's are really easy to recognize because they are really tall with long fingers. This guy doesn't really fit that profile either.
If you would like to read more about the differential diagnosis of downed runners and what to do about them, I recommend these two papers.
I think that is about it. Can anyone think of other things that would be on the differential?
- Log in to post comments
Thanks for this. When I read about the runner's death, I hoped you'd address some of the possible causes. :)
Of, course, the DDx would include non-cardiac causes of sudden death in a young person, such as ruptured brain aneurysm or AVM, or pulmonary embolism. Also, cardiac causes would still include premature coronary thrombosis. Wasn't there a young Russian figure skater who died in his late 20's of CAD a few years ago?
Awesome post: I really like how you help me review a lot of the material that I've learned long ago. Keep it up.
Performance enhancing drugs. But I'm a cynic that way...
I think you are right, Bruce, though I hadn't heard about the Russian person. You could have a genetic hyperlipidemia and get a heart attack in your 20s. But then again, I feel like most people with hyperlipidemia show it, and they aren't generally running marathons.
The thing with AVMs and berry aneurysms is that you will get the worst headache of your life and you may die on the way to the hospital, but I feel like this was more sudden. That is why I emphasized cardiac causes.
It seems like most of these cardiac causes can be screened for, but I don't get the sense that the U.S. is even yet having the discussion of screening young people for these types of things. I remember reading that Italy had a program for screening young athletes (for them, particularly soccer hopefuls) which could result in sidelining--and therefore probably saving--some of the young players.
Here is a fact from the study with the pre-screening: "From 1979-1980 to 2003-2004, the annual rate of sudden heart death in athletes decreased by 89 percent, the study found." (link)
A slightly longer write-up is is here
Just a reminder: Your child does not have to be an "athlete" to have these heart conditions.
We (as parents) find ourselves quite lucky that our oldest son was diagnosed with hypertrophic cardiomyopathy with obstruction because of the detection of a heart murmur and subsequent echocardiogram, rather than the norm: sudden cardiac death.
One of his classmates was also found to have Long-QT syndrome basically through the same method, a regular well child check-up, both at the age of 14.
Here is the message: Well child check-ups do not end at age 10, or whatever! They continue until the child is an adult, which when they continue monitoring their health with annual adult exams.
(I say this even though Child #2 has been sent to have an X-ray for the THIRD time for his scoliosis... give it up, doc! He is 17, the scoliosis is only 10%, it is not worth worrying about! Though the kid is happy that he is within 1/8 of an inch of six feet tall!).
By the way, there is a website and organizaition for HCM. I find it a bit depressing to visit (the forum has parents who have had children die), but here it is:
http://www.4hcm.org/WCMS/index.php?overview
By the way, our son was attending a soccer camp when the "well child" check-up occured. He had been complaining that his arm hurt when he was running, and even then he seem to literally be slowing down.
After the murmur was detected at his 14 year old check-up he was sent to have an echocardiogram. After that exam we received a phone call from our family doctor to not have him participate in soccer camp (No more attempts at running!). It turns out that the echo tech called the family doc with what she had seen on the echo before the "official" doctor had examined the tape.
I have gained a great deal of respect for the technicians who conduct echocardiograms and other screening tests from this experience.
By the way, at the time my son was diagnosed with HCM, a young lady died at a track meet from the same thing. There was a discussion in the newspaper about screening all athletes for heart conditions, but it was concluded that it was still too expensive for the risk. I know for a fact that echocardiograms cost between $1800 to $2200.
Yes, they are expensive. Due to the fact that one child has HCM, our insurance paid for the echocardiograms for our other two children. Both of them seem to have escaped the HCM gene (even the one with 10% scoliosis).
When do we make echocardiograms a qualifying test for school sports?
(side note: Bikemonkey, you are being foolish. HCM is genetic, it is not due to any performance enhancing chemical. My aunt died of HCM as a newborn in the 1920s, and my son is not exactly an athlete. You should do a bit more research before posting silly statements)
Marvelous post. The issue is always what do we do to prevent this. In the US the issue of sports preparedness physicals is serious and the potential patients are sadly underserved. We only check for hernias and put a stethoscope on their hearts. No diagnostics are usually done. In a loud crowded room, no murmer of IHSS can be heard and many of us wouldn't recognize it or be able to actively elicit it. An ECG is cheap and slightly more effective in ruling out Brugada syndrome and IHSS but is rarely if ever done. These physicals are batched, and done in odd places such as community gyms by volunteer doctors. Invasive diagnostics do not occur in the main.
Prudent parents should ask for and receive a more thorough evaluation including at least an ECG, a thorough past history and family history and perhaps ECG and other diagnostics to protect their children. Contact or non-contact sports have their own risks and those risks should be planned for. The regrettable deaths we see every day from sports are largely preventable deaths. The prevention can be from protective apparatus such as sensing helmets in football, or from protective parents and physicians. Standards exist but are rarely followed because we want speed, a signature on the form for the coach, and little else. Coaches are not thoroughly trained in prevention and they by and large don't care much. Action in the arena of sports death prevention requires a concensus from the community and community effort.
Thanks to all who posted. I am a nurse practitioner student and work with electrophysiologists. The heart is an electrical/mechanical pump and an amazing organ. Ryan Shay had an enlarged heart since age 14 -- does that mean he had thickened muscle or smaller chambers -- was his septum thicker than normal or did he have a toned heart or the beginnings of a viral cardiomyopathy? I had a patient who was a tremendous athlete, he ran, he played ice hockey, tennis, etc and one day woke up with a stroke -- he had cardiomyopathy and the stroke was the first indication. He ended up with a heart transplant but everyone was perplexed at how this young healthy post grad student could have a stroke and be so active. Well, guess what...it was familial. His dad had an ICD as well. Sudden death for anyone is a horrible experience -- but our society does not anticipate it in young athletes. I think it is disappointing that we are one of the richest countries in the world and we don't screen as much as we should -- but the economists say it is financially prohibitive. Tell that to the parent of the kid who drops dead. An ECG is cheap and could be a preliminary step for at least some form of screening -- most kids don't even get that much.
: I really like how you help me review a lot of the material that I've learned long ago.
I have often been shocked how many people (doctors particularly) have never heard of Brugada syndrome. I know that I tend towards the zebras, but it isn't such a zebra!