Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy is a disease of the heart in which the heart muscle becomes thick and inflexible without any apparent cause. This condition is more important as the cause of abrupt and unforeseen death due to cardiac reasons or disability-causing symptoms in patients of any age.
Hypertrophic Cardiomyopathy
In general, a cardiomyopathy is any disease that attacks the heart muscles. In hypertrophic cardiomyopathy, the heart muscle cells are pushed out of their normal alignment and this condition is known as myocardial disarray. Another effect of hypertrophic cardiomyopathy is the disruption of the electrical signaling arrangement of the heart.
It is believed that the roots of hypertrophic cardiomyopathy lie in the mutation of a gene (one of those responsible for a mutation of the myosin heavy chain) which forms a part of the muscle cell of the heart (also called a myocyte).
Hypertrophic cardiomyopathy usually causes a certain amount of thickening of the left ventricle wall which can be obstructive or non-obstructive, depending on the amount of blood it allows to flow.
The people who carry the maximum risk for hypertrophic cardiomyopathy are
- Those patients who have a prior 'heart attack' (cardiac arrest)
- People with a close relative who has dies due to hypertrophic cardiomyopathy
- people who exhibit fainting spells with no satisfactory explanation. This is especially true in young people or while undertaking physical activity
- People who experience brief episodes of tachycardia
- Patients whose blood pressure does not rise during exercise. This is especially significant for people less than 50 years
As far as treatment for hypertrophic cardiomyopathy is concerned, except some beta-blockers, the most effective by far has been the Implantable Cardioverter Defibrillator (ICD). This is an implantable device which automatically senses potentially life-threatening heart rhythm disturbances and anomalies and corrects them at the outset itself. This fast and effective response makes them the treatment of choice for young people with few symptoms of hypertrophic cardiomyopathy and definitely for those who have survived a heart attack.
The implantable cardioverter-defibrillator (ICD) is the most reliable and effective treatment for HCM patients at high-risk. It has the potential to alter the disease course by automatically sensing and terminating lethal disturbances of heart rhythm, often in young people with little or no symptoms. ICDs are clearly warranted for those who survive a cardiac arrest, but should also be considered as a preventive measure for other high-risk HCM patients after taking into consideration the strength of their risk factors(s), the level of risk acceptable to patient and family, and the access to ICDs.