Hypertrophic Cardiomyopathy

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Hypertrophic cardiomyopathy, also called HCM, is a condition where the heart muscle is a lot thicker than normal.  In particular, the excessive thickness is seen  in the septum of the heart. In this article, we shall talk about hypertrophic cardiomyopathy and how  it can be managed.

What is hypertrophic cardiomyopathy?

Hypertrophic cardiomyopathy is a condition that affects the heart muscle. The septum of the heart, which is a part of the heart that divides it into a left and right side,  is abnormally thickened.  The thickening of the heart muscle makes it very stiff.  Due to this, there can be problems with the way the heart contracts and  this can indirectly affect the aortic valve and the mitral valve.

The thickened heart muscle means that the blood cannot exit the heart easily.  This is responsible for the number of different symptoms.

What causes hypertrophic cardiomyopathy?

Hypertrophic cardiomyopathy is a genetic condition and runs in families. In other words, individuals who have a parent or a blood relative suffering from this condition may develop hypertrophic cardiomyopathy.

The primary problem that is associated with the genes in the body is that it causes the muscle fibers  to become arranged in a very disorganised manner. This is known as myocardial disarray.

Based on the degree of  enlargement of the heart muscle, hypertrophic cardiomyopathy can be of two kinds –

  • Non-obstructive hypertrophic cardiomyopathy – This is where the heart muscle is thickened but does not cause any problems with the flow of blood  from the left ventricle into the aorta. The heart muscle can be rather stiff and cannot hold a large amount of blood.
  • Obstructive hypertrophic cardiomyopathy – Here,  the septum of the heart is extremely thickened  and can obstruct the flow of blood into the aorta. 7 out of 10 patients with obstructive hypertrophic cardiomyopathy usually experience symptoms.

What are the risk factors for hypertrophic cardiomyopathy?

The primary risk factor is a family history of hypertrophic cardiomyopathy. children  of individuals who suffer from this condition have a 50% chance of developing it. In other words, as a parent, if you have hypertrophic cardiomyopathy and have two children, it is possible that one of them will develop this condition.

Due to this genetic risk, any patient who is diagnosed with hypertrophic cardiomyopathy will require their family members undergoing screening tests as well.

What are the symptoms?

Below is a short list of symptoms that patients may experience –

  • Shortness of breath  that is more  prominent during exercise
  • Chest pain on exertion
  • Fainting episodes or a feeling that one is about to faint
  • Tiredness
  • Dizziness
  • Palpitations i.e. a sensation that the heart is racing in the chest
  • A heart murmur which is heard when the patient is examined

What are the tests required?

The best test to diagnose hypertrophic cardiomyopathy  that is safe and painless is an echocardiogram.  This can offer sufficient information regarding the structure of the heart and whether it is causing obstruction  or not.

Echocardiogram tests can be of two kinds.  A trans-thoracic echocardiogram is one where a probe is placed on the chest and images are visualised on a screen. You can read more about this here. A trans-oesophageal echocardiogram  is a procedure where a tube is passed down through the food pipe halfway down it’s length. the images that are obtained are crystal clear through this test  but  it is a rarely performed as a trans-thoracic echocardiogram is often sufficient.

An echocardiogram may also be performed in family members to screen for hypertrophic cardiomyopathy.

There are other tests that may be performed  that will help aid making a diagnosis and in guiding treatment. These tests include –

  1. A treadmill test
  2. A Holter recording
  3. A coronary angiogram
  4. A cardiac MRI scan

Click on the links above to read more about these tests.

What is the treatment for hypertrophic cardiomyopathy?

Medical treatment that is prescribed for patients with this condition are aimed at reducing the stress upon the heart  and preventing the development of symptoms.  Below are some of the commonly adopted treatment strategies in managing patients with hypertrophic cardiomyopathy.

1. Medical therapy

The use of medication in treatment  is extremely beneficial.  Drugs that  can relax the heart muscle and reduce the rate at which the heart beats  can help decrease the stress that the heart is undergoing.  This in turn can help the heart pump strongly and more efficiently.

The commonly used drugs are beta-blockers, calcium channel blockers and disopyramide. Most patients can be managed effectively and safely with medical therapy alone.

2. Septal ablation

This is a specialised procedure where the heart muscle is injected with a small amount of alcohol through a small catheter inserted into the heart (through the leg). The alcohol breaks down the heart muscle tissue  and reduces the size of the septum. In turn, this can reduce the overall size of the heart and improve blood flow into the aorta. Read more about septal ablation here.

3. Septal myectomy

This is a surgical procedure where a part of the second septum is removed. By doing so, the blood flow through the valves improves and the heart function also improves. Surgery is only offered to patients if medical therapy has failed. Nearly 95% of patients who undergo this procedure can return to having a normal life. Read more about septal myectomy here.

4. Implantable Cardioverter Defibrillator

This is a special device there is placed in a small pocket under the skin.  Small wires attached to this device are inserted into the heart muscle. The wires continuously monitor the heart rate and if the heartbeat exceeds a particular limit or is fast and irregular, the device corrects it by controlling the rate or by delivering a small shock. This treatment is life-saving in those patients who have a family history of sudden cardiac death.

If you suffer from hypertrophic cardiomyopathy  or have a friend or family member who does,  take a look at some of the lifestyle changes that they need to make in this post here.

To read more about genetic screening, click here.


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