The Hypertophic Cardiomyopathy Research Foundation

Diagnosis and Treatment

An echo being administerd

An 'echo tech' (diagnostic medical sonographer) using an echo machine.

HCM is cardiology’s great masquerader because there is no single symptom to look for so patients may be misdiagnosed with more familiar conditions like asthma, mitral valve prolapse, or anxiety-panic disorder. Symptoms or signs you should be concerned about are shortness of breath, chest pain, unexplained blackouts, unexplained heart murmur, EKG abnormalities, and a family history of sudden death at a young age. The initial diagnosis of HCM is made with an echocardiogram. Echocardiography is a non-invasive, non-X-ray imaging technique that uses sound waves to picture the heart.

Normally, heart walls are 11 mm or less in thickness. Patients with HCM have a characteristic thickening of the heart walls ranging from 14 to 50 mm which is visible on echocardiography.

This illustrates a normal parasternal echo with a normal thin septum and mitral valve function. The yellow arrow points to the septum.
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This is an illustration of a very thick septum. The yellow arrow delineates the septum.
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Here we see the SAM (systolic anterior motion) of the mitral valve is obstructing the outflow from the left ventricle, thus reducing the flow of oxygenated blood to the body and increasing the left ventricular work.
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After diagnosis, each patient’s treatment plan is based on the severity of symptoms, diagnosis of obstructive or non-obstructive HCM, and results of further evaluation. Since sudden death is a potentially catastrophic complication; further tests may be ordered to assess an individual’s risk. Most patients can be treated successfully with medications. Patients with serious obstruction and symptoms that do not respond to medication benefit from cardiac surgery.

Here we see the preoperative heart with SAM and mitral-septal contact causing obstruction.
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This shows the heart after surgical septal myectomy . The septum has been thinned by myectomy surgery. There is now no SAM of the mitral valve--- no mitral septal contact and no obstruction.
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Patients at risk for sudden cardiac death are treated with an implantable cardioverter defibrillator that detects and aborts episodes of chaotic heart rhythm known as ventricular fibrillation. Treatment is often a multidisciplinary approach: employing the efforts of an HCM specialist physician, echocardiographer, electrophysiologist, pacemaker specialist, and cardiac surgeon.