The front page of today’s Asbury Park Press is about the sudden cardiac death (SCD) of a 16 year old male high school varsity lacrosse player from Howell Township. The event occurred during a game being held at Rutgers.
The autopsy revealed the presence of a rare genetic heart muscle disorder called arrhythmogenic right ventricular dysplasia (ARVD)
The pathology of this condition (see drawing above) consists of a weakening of the walls of the main pumping chamber on the right side of the heart (right ventricle) causing that chamber to dilate (enlarge). The muscle of the right ventricular wall deteriorates and becomes replaced by fatty tissue. Dangerous cardiac rhythm disturbances can occur and result in SCD.
But we also want to talk about how we are screening our high school and college athletes for cardiac risk before allowing them to participate in strenuous sports.
ARVD is one of a number of congenital disorders that can cause SCD. The most common one, albeit rare, is also a familial condition called hypertrophic cardiomyopathy.
The National Institutes of Health (NIH) says, “The sudden death rate among high school athletes is 1:100,000–200,000; among marathon runners 1:50, 000; and among recreational joggers 1:15, 000. Thus, exercise-triggered sudden cardiac death is a rare event.”
Not everyone with ARVD will experience a sudden death, but those who do often have a history of preceding symptoms. Such symptoms may include fainting, dizziness and/or palpitations. Medical personnel who perform evaluations of athletes must be alert to such warning indicators of risk. The physical examinations of those with ARVD are often unrevealing. But certain tests may suggest a cardiac problem such as an electrocardiogram (ECG). And a cardiac ultrasound (echocardiogram) is often diagnostic.
In addition, the victim may have no relatives who are known to have this familial disorder.
All students at high schools and colleges undergo a screening history and physical examination before being allowed to participate in a strenuous sport such as lacrosse. But such an evaluation may fail to find something worrisome in many cases of ARVD with subsequent SCD. Or a clue may be missed—for example if the examiner doesn’t ask about symptoms such as palpitations.
Of course, if a student has symptoms such a palpitations or fainting; or a worrisome family history; or something of concern on the physical exam, then a referral must be made to a cardiologist, at which point more testing should be done which may include electrocardiography (ECG), echocardiography (ultrasound,) MRI of the heart, heart muscle biopsy, angiography, stress testing, or genetic analysis.
But is there anything else that should be done by way of routine risk assessment in all athletes besides the history and physical exam? This is a controversial subject.
In my opinion, an ECG is a must. Some experts believe that an echocardiogram should also be done routinely on athletes.
The problem is that those hidden conditions, such as ARVD, which may cause sudden death are extremely rare as is the incidence of SCD in apparently healthy young athletes. And the yield of checking thousands of athletes with ECG’s and ECHO tests is extremely low and very expensive. And even with such testing, certain diagnoses can be missed, so in the US, most screening does not include more than the history and physical exam.
But in Europe they do routine ECG’s and find it to be very useful in locating some high risk individuals. I don’t know if any programs use routine echocardiograms.
If I had my way, and money were no object, all these screening exams would be conducted by cardiologists, but the reality is that general practitioners and even physician extenders such as nurse practitioners are usually given the job; however, they might overlook something.
I would definitely do an ECG regardless of cost on each student being screened. And the ECG must be read by a cardiologist. No official public health organization has suggested a routine echocardiogram.
But even for rare conditions, if you could make the diagnosis of, for example, arrhythmogenic RV dysplasia, you could offer the patient an opportunity for counseling and therapy, even though ARVD is not curable. Some such patients have even received a heart transplant.
To put this in perspective, SCD in young athletes is a rare event among the many hundreds of thousands of students who participate in strenuous sports, and when such an event occurs, it always appears in the news, so the concerns get amplified.
But that doesn’t mean we shouldn’t aggressively try and identify those who are at risk, even if such screening is not 100% successful.
Parents could get some peace of mind by having an ECG and echocardiogram done, even if they have to pay for it themselves. And, don’t forget that close relatives of SCD victims should also get screened.
You could try the office of the medical examiner in that town.
Thank you, Paul. Natural disease is all that they told us. It was also written on the death certificate. After all these years, I assume it’s impossible to go back to the original autopsy report.
Paul, I just read your farmers market & medical articles. They were excellent. The farmers market was fun & the medical was very informative. I hope some parents take this seriously & have their children checked out. E
Carol. This disease (ARVD) was first described and named in a medical report in 1982.
But the abnormalities should have been mentioned in the autopsy report if your brother had ARVD, even if they could not have put a name to it back then.
I have no idea what the pathologist meant by “natural disease” if that is what is written in the autopsy report. But you should know that sometimes an autopsy will not reveal the cause of SCD.
My brother died at 16 years old in 1971. He was playing in a soccer game. They performed an autopsy, but all they could tell us was that he died of “natural disease.” Would this condition have been detected in an autopsy at that time? His death came one month after my mother’s sudden death from a cerebral hemorrhage and devastated our family. It was puzzling to us that they could not find a reason.