Hypertrophic Cardiomyopathy (Hypertrophic Obstructive Cardiomyopathy)

Hypertrophic cardiomyopathy is a disorder of the heart muscle, in which the walls of the ventricles thicken (hypertrophy) and become stiff, without any additional work load on the heart.

  • Hypertrophic cardiomyopathy usually results from an inherited genetic defect.
  • Chest pain (angina), shortness of breath, and palpitations and fainting bouts are the usual symptoms.
  • An echocardiography can confirm the initial diagnosis made through physical examination.
  • Drugs to reduce the force of the heart’s contractions help those with hypertrophic cardiomyopathy.

The incidence of hypertrophic cardiomyopathy is equal in men and women except among the older age group, where women outnumber men simply because the general life span of women is longer. In this age group, four people out of every hundred have this condition.

Causes

Congenital hypertrophic cardiomyopathy is almost always the result of a genetic defect that is inherited. Even when this heart condition develops later in life, the cause could be traced to this genetic factor.

Acquired hypertrophic cardiomyopathy may be the result of certain disorders due to over production of hormones. For example; acromegaly, resulting from an excess of pituitary growth hormone, and pheochromocytoma resulting from the excess of the hormone epinephrine, can often lead to hypertrophic cardiomyopathy. It may also be caused by another hereditary disorder called neurofibromatosis.

Symptoms

  • Fainting (syncope) during exertion is a common symptom.
  • Awareness of heartbeats (palpitations) may be present due to an abnormal heart rhythm (arrhythmia).
  • Chest pain (angina) is also very common symptom associated with this condition.
  • When the hypertrophied heart resists being refilled with blood from the lungs, it results in stagnation of blood in the lungs, leading to shortness of breath.

When the walls of the ventricle thicken, the mitral valve between the left atrium and the left ventricle may not close properly, due to which blood leaks back into the atrium.

In some cases, when the thickening of the heart muscle cause obstruction to the flow of blood out of the heart below the aortic valve, it results in a condition called hypertrophic obstructive cardiomyopathy.

Diagnosis

Hypertrophic cardiomyopathy is easy to diagnose from the sounds picked up by the stethoscope. Echocardiography is the ideal test to confirm the initial diagnosis. Chest x-ray and ECG are additional tests usually prescribed by doctors.

Prognosis

The fatality rate of hypertrophic cardiomyopathy is 4% among those who suffer from it. Most deaths tend to be sudden; an abnormal heart rhythm (arrhythmia) being the usual cause. Death due to chronic heart failure is a comparatively rare occurrence in those with this condition. Hypertrophic cardiomyopathy is the most usual cause of sudden death in young athletes.

Genetic Counseling

Since the usual cause of hypertrophic cardiomyopathy is an inherited genetic defect, family members of those who are known to have this disorder can opt for genetic testing to confirm their status. Those who are likely to inherit this disorder are advised to go for genetic counseling when they plan a family.

Treatment

Drug therapy for hypertrophic cardiomyopathy focuses on reducing the resistance of the heart to refilling with blood after every contraction.

Beta-blockers reduce the extent to which the heart muscle contracts, so that it can return to relaxed position more easily allowing the heart to fill better. When the contraction is limited, it can also improve the blood flow out of the heart, in case a thickened muscle is blocking the blood flow.

The calcium channel blocker verapamil also works along similar lines and it is often prescribed along with beta blockers. They also help slow down the heart, giving it extra time to fill. Disopyramide is another useful drug which help reduce the strength of heart contractions.

When treatment with drugs does not improve the condition, the thickened heart muscle can be surgically removed (myectomy) to improve the blood flow from the heart. Surgery may reduce the symptoms, but not the risk of death.

Cardiac catheterization is a minimally invasive procedure by which selective destruction of thickened tissue can be achieved, by injecting a small amount of absolute alcohol (alcohol ablation) into a small, carefully ear marked area. Killing the thickened tissue causing obstruction improves the blood flow. This method is preferred over open heart surgery due to the reduced risk associated.

A cardioverter-defibrillator for better control of heart beat can be implanted in people with an increased risk of sudden death due to arrhythmia.

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Yasser Elnahas

MD, PHD, Professor Of CardioVascular Surgery
Dr. Yasser Elnahas, Is an associate Professor of Cardiovascular Surgery. Dr. Elnahas was trained as a fellow At Texas Heart Institute And Mayo Clinic Foundation.Dr. Elnahas is dedicated to educating the general public about different disease conditions and simplifying the medical knowledge in an easy to understand terminology.

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