This can occur in children of any age, but is most often seen in adolescents. Nearly one-half have abnormal ECG findings, usually of a supraventricular origin (atrial flutter or fibrillation are most common). The disease is caused by a primary hypertrophied, undilated left ventricle without structural lesion and is usually inherited, so a family history will be helpful. Typically, most children present with chest pain, exertional dyspnea, and syncope. Occasionally, sudden death is the only clinical finding.
The cause of most cases of sudden death is unknown and may be the consequence of hemodynamic, structural, and electrophysiologic consequences. Although supraventricular rhythms are more common, electrophysiologic studies indicate that ventricular dysrhythmias are the cause of sudden death.
Once recognized, therapy should be instituted with b blockade to ameliorate symptoms. The goal of therapy is to improve left ventricular filling during diastole. b Blockade results in heart rate reduction to allow for more time for diastolic filling. Blockade also decreases myocardial muscle tension and improves compliance and eases filling. Other agents may be useful, but are usually instituted after conduction studies are performed. Surgery may be useful to eliminate some of the symptoms. Neither surgery nor medical management will prevent sudden death. There is not enough evidence to suggest that implantable defibrillation devices will prevent the sudden death of these children.31
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