Heart Disorder: The Disorder All Athletes Should Know About !
The heart disorder, called hypertrophic cardiomyopathy, is an abnormal thickening of the muscles in the walls of the heart. It causes half of the sudden death cases involving athletes under age 35.
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Because of some possible similarities between early repolarisation and this syndrome, and because some patients with the syndrome have been misdiagnosed as early repolarisation, a discussion of the similarities, differences and electrophysiologic mechanisms of these two syndromes is justified.
Early repolarisation is a frequent finding in healthy, usually sportive, young men. The Brugada syndrome also frequently affects active, sportive, and apparently healthy young men. During exercise, there is a normalization of the ST segment in both situations. Adrenergic stimulation with isoproterenol normalizes the ST segment in both syndromes, while beta-adrenergic blockade increases the ST segment elevation.
Which are the differences between the two syndromes then? The first and very much important difference is that normal individuals with early repolarisation do not develop sudden death because of ventricular arrhythmias.
There exist also electrocardiographic differences between the two syndromes, like the type and localization in the precordial leads of the ST segment elevation (picture 12).
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Picture 12
Early repolarization. Compare with the electrocardiograms of patients with Brugada syndrome to note the important differences..
Although the underlying cellular electrophysiologic mechanisms of early repolarisation are not completely clear, some electrophysiologic similarities between the two syndromes may exist.
From a discussion with Dr. Antzelevitch, picture 13 was drawn to try to understand the differences between early repolarisation, long QT syndrome and Brugada syndrome, and why arrhythmias do not occur in the first situation but do occur in the other two.
Picture 13
Electrophysiologic differences between early repolarization, long QT syndrome and Brugada syndrome. In early repolarization there exists a voltage gradient but no dispersion of duration of action potentials. That is why these patients show ST elevation but do not develop arrhythmias.
In the long QT syndrome there exist a voltage gradient because of prolongation of the action potential in some areas because of early afterdepolarizations. The voltage gradient results in phase 2 re-entry. In Brugada syndrome re-entry occurs because of the voltage gradient caused by the shortening of the duration of the action potential.