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Pediatric Cardiologist Discusses Sudden Cardiac Death in Young
The headline is simultaneously dramatic, tragic and unexpected: “Young athlete dies suddenly.” While rare, such events inevitably cause extreme anxiety and concern amongst parents, peers and the public alike. Many related questions are often directed to those of us in the healthcare community. How can individuals seemingly so healthy, so full of promise and potential, succumb so quickly? More importantly, how can this be prevented?
There is approximately a 1 in 200,000 chance of an apparently healthy adolescent dying suddenly from cardiac causes during a year of sports participation. Medical screening evaluations are aimed at determining those that may be at risk.
The most common cause of sudden death in young athletes is hypertrophic cardiomyopathy. This disorder occurs in 0.2% of the population, and is often inherited in an autosomal dominant pattern. Patients may be asymptomatic, but a variety of distinctive symptoms and signs may be associated (discussed below). Echocardiography confirms the diagnosis. The next most common cause, obviously more difficult to detect, is a congenital coronary artery abnormality such as an anomalous origin of the left main coronary artery from the right aortic sinus.
Less common causes include myocarditis and dilated cardiomyopathies, Marfan syndrome with aortic rupture, ventricular arrhythmias (such as those occurring in long Q-T syndrome and arrhythmogenic right ventricle), and a number of types of valvar congenital heart disease (such as aortic stenosis).
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In determining those adolescents at risk, a background history is of most importance, and for this reason information from both parent and child should be sought. Any past occurrence of chest pain, dizziness, syncope, fatigue, and/or dyspnea may be of significance. Also pertinent are any past indications of an abnormal heart murmur or high blood pressure, and a family history of syncope, seizures, sudden death, or other signs of significant heart disease in males before 55 years of age or females before 65 years of age.
A comprehensive physical examination should be performed by a practitioner qualified to recognize pediatric heart disease, and specifically identify heart murmurs, abnormal blood pressures, and phenotypical features that can be associated with heart and circulation abnormalities (such as the physical characteristics of Marfan syndrome). Any significant findings should be further investigated through a referral to a pediatric cardiologist.
Since these medical evaluations cannot identify all those who are vulnerable, could additional screening tests be of assistance? The answer lies in the number of such tests that would be necessary, considering the millions of school age children involved in athletics in this country. The performance of reliable electrocardiography and echocardiography in all these individuals would be a practical impossibility. Ramifications would include labeling those individuals with trivial findings as having heart disease - perhaps outnumbering those identified with real disease. Various senior advisory groups, including the American Academy of Pediatrics, do not currently recommend such testing. In the future, it is possible that genetic testing may offer a better method of determining who may be at risk.
Screening, however, is only one approach to decreasing potential cardiac problems. Many sudden deaths, no matter what the underlying cause, are the direct result of sudden ventricular tachycardia or ventricular fibrillation. These are arrhythmias that are routinely recognized and potentially successfully treated by automatic external defibrillators (AEDs) found increasingly in public areas such as airports, schools and shopping centers. In fact, the Food and Drug Administration has recently approved AEDs for home use.
There is a strong case to be made for such devices to be available at school athletic venues. Healthcare professionals can do only so much in the hospital, office or clinic setting. In the absence of better screening methods, it is important that all means at our disposal are used to attempt to reduce the impact of this tragic problem.
Niels Giddins MD, FAAP, FACC, Chair, Pediatric Cardiology
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