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“Marvin, should we head south or stick around for Thanksgiving?” ©      Sue Gioulis cartoon for Blogfinger.

“..then the birds got together to chirp about the weather…..lah de dah, ’tis autumn.”

*This lyric is from the 1941 song “Tis’ Autumn” by Henry Nemo. Nat King Cole had the first recording of this composition. The Stacey Kent version can be found in our post below.  Here is Stan Getz:

STAN GETZ  “Tis’ Autumn.”

Hurricane Cleo hits Miami Beach in August 1964, demolishing the lobby of the Deauville  Hotel. Paul Goldfinger award winning photograph. ©

GLORIA ESTEFAN AND THE MIAMI SOUND MACHINE:  “Anything For You.”

Arrhythmogenic right ventricular dysplasia.

By Paul Goldfinger, MD, FACC

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.

But beyond the history and physical,  there is controversy as to what else should be done routinely  to screen these athletes.

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?  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 get screened themselves.