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Complications

In a minority of cases a number of specific complications can occur:

Arrhythmias

Arrhythmias, irregularities of the heart beat, are a common complication. Symptoms such as palpitation may occur, but not often. Exercise testing or Holter monitoring may detect them. The arrhthymias called ventricular tachycardia (arising from the ventricles) or atrial fibrillation (described below) are particularly important and may require treatment. Atrial Fibrillation, the normal regular rhythm of the heart beat is lost and replaced by an irregular rhythm which may be episodic or persistent. The loss of normal atrial (the top of the heart) contraction produces a risk of clot formation in the atria. Anticoagulants and drugs to slow the heart rate are required.

Endocarditis

This is an infection of the heart which occurs rarely in Hypertrophic Cardiomyopathy. Bacteria in the bloodstream can stick to the inside of the heart where it has been roughened by turbulent blood flow. **As uncommon as this is, the President of the HCMA fell victim to this. She had been MISINFORMED by a dentist who said "Genetic disorders do not need to be premedicated". The HCMA advises extreme caution when undergoing any "invasive" medical procedure**

Heart Block

The normal electrical signal may travel down to the ventricles slowly or may even be completely blocked, "heart block". If this occurs, a pacemaker is implanted (see "other forms of therapy").

Sudden Death

Overall, in patients with Hypertrophic Cardiomyopathy there is an increased risk of premature death, which can occur with little or no warning. Sudden death can strike at any age. In the past the risk of sudden death was thought to be much higher than we believe it to be today. It is estimated that the risk of sudden death is between 1 and 2 % in the HCM population. There are members of the HCM population at a higher risk for sudden death and for those at higher risk it is advised they consult with their health care provider about receiving an implantable defibrillator.

“Burnt out” or “End Stage” HCM

The terms associated with this part of the HCM disease process are misleading and rather depressing. It is our goal to help readers gain a better understand this rare but important potential consequence of HCM, occurring in under 3% of the total HCM population. It is poorly understood at this time why this occurs. Some theories suggest it is the individual genetic mutation that determines the progression of the disease.

While “End stage”/”Burnt out” is not precisely the same it is remarkably similar to the dilated form of cardiomyopathy. In dilated cardiomyopathy (often referred to as congestive cardiomyopathy), the heart reaches a point of generalized weakening and thinning of the muscle walls with dilated chambers, particularly the left ventricle. While the walls may have previously been normal or thickened from overwork through trying to compensate for an inadequate ability to pump, (or in the case of HCM), previously “thick” muscle now changes to a different form of damaged tissue. The dilation and thinning of the cardiac chambers, especially of the left ventricle, is often referred to as “remodeling”. The weakening and dilation of the heart muscle eventually leads to heart failure. previously normal or in the case of HCM, previously “thick” heart muscle changes to a different form of damaged tissue, leading to a generalized weakening of the walls of the cardiac chambers. To compensate for the weakening of their muscular walls, the cardiac chambers dilate. (The dilation of the cardiac chambers, especially of the left ventricle, is often referred to as "remodeling.") The weakening and the dilation of the heart muscle eventually leads to heart failure.

Is a cure available?

At present there is no cure for Hypertrophic Cardiomyopathy, HCM. There is a slight possibility that some drugs may decrease the degree of muscle thickening. However, no treatment has been shown to return the heart to normal. Research continues in this area. Developments are most likely to come from the early detection of persons carrying the gene for Hypertrophic Cardiomyopathy and treating them to prevent the development of hypertrophy. The achievement of this goal will require much further research and is many years away from reality.



CREATED: 2004/11/24 LAST UPDATED: 2008/06/24