This case highlights that severe echocardiographic findings of mid-cavity obstruction in pediatric hypertrophic cardiomyopathy may not always correlate with severe clinical symptoms or impaired development.
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Case highlights rare pediatric HCM with obstruction; leaves open optimal management strategies.
Hypertrophic cardiomyopathy (HCM) is very common and can affect people of any age. About one out of every 500 people has HCM. It affects men and women equally. HCM is a common cause of sudden cardiac arrest (SCA) in young people, including young athletes. HCM occurs if heart muscle cells enlarge and cause the walls of the ventricles (usually the left ventricle) to thicken. Despite this thickening, the ventricle size often remains normal. However, the thickening may block blood flow out of the ventricle. If this happens, the condition is called obstructive hypertrophic cardiomyopathy. HCM also can affect the heart's mitral valve, causing blood to leak backward through the valve. Sometimes, the thickened heart muscle doesn't block blood flow out of the left ventricle. This is called non-obstructive hypertrophic cardiomyopathy. On this presentation we report a case of a child, with a rare form of the idiopathic hypertrophic cardiomyopathy, associated with mid-cavity obstruction and high intraventricular peak pressure. Cardiomyopathy, diagnosed antenataly, was followed postnataly and, despite of a lot echocardiographic findings - the growing, development and clinical signs are minimal.
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Ragip Retkoceri (2015) studied this question.
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