When someone is diagnosed with hypertrophic cardiomyopathy it means that they have an abnormal thickening of the heart muscle wall. When someone has hypertrophic cardiomyopathy it also means that the blood flow of the heart may be somewhat obstructed because of this thickening.
Hypertrophic cardiomyopathy is the leading cause of sudden heart attack and death in young athletics during physical activity. Hypertrophic cardiomyopathy can cause disruptions in electrical functions of the heart. This condition can happen in all racial groups and affects 2% – 5% of the human race. This is a disease in which the contractile blocks in the heart begin to duplicate themselves and cause the thickening of the heart muscle. It will eventually lead to a disorder called myocardial disarray which affects the configuration of the heart muscle cells. It is believed that hypertrophic cardiomyopathy occurs because a mutation in sarcomeric genes that mutate the protein component that is present in the sarcomere.
This disease has several symptoms that include angina, dizziness, palpitations, dyspnea, and sudden death from heart attack. Syncope can also occur in children and young teenagers who have a small left ventricle. This is because of the decrease of blood flow from the heart. Children with who have hypertrophic cardiomyopathy will also experience pain in the chest known as angina. People who have hypertrophic cardiomyopathy also experience noticeable heart palpitations. Most will experience other symptoms like fatigue and breathlessness. High blood pressure is also present in some cases of patients who have hypertrophic cardiomyopathy. However, there are some people who have hypertrophic cardiomyopathy who do not have any symptoms at all. In this case, the condition may be only be found during a routine medical exam.
Many children are diagnosed with hypertrophic cardiomyopathy through genetic screening and EKG testing when the condition is expected. It is known to be a complication of genetic origination and is an inherited disease. Children who have this disease generally will also have other heath problems and complications like Danon’s disease and Noonan Syndrome and other metabolic disorders. Most children are not diagnosed with this disease for many years because of the lack of symptoms in the beginning. The first symptoms that will begin to appear will be when the child complains of angina and dyspnea. When the child exercises they may feel lethargic and unusually fatigued. Arrhythmias and heart palpitations will become common and arrhythmia can be detected by an EKG. It is important for the child to have a thorough medical evaluation by a doctor as soon as hypertrophic cardiomyopathy is suspected. People of all ages can have hypertrophic cardiomyopathy however and it is not just confined to children.
Treatment for this disease includes the use of medications that can help to relax the heart muscle. Drugs like beta blockers and calcium channel blockers are normally used too. Many times these medications are sufficient to relieve the symptoms and no further treatments are necessary. If an arrhythmia is present blood thinners may be used to reduce the risk of blood clots. Permanent pacemakers may be implanted also, but this is not done as frequently today as it was in the past. If the blood flow from the heart is dangerously blocked they can do an operation called a myectomy. This surgery involves the removal of the thickened part of the heart wall so the blood can flow freely again. Nowadays a procedure called alcohol septal ablation may be done. This is a procedure where an injection of alcohol is given into the arteries that feed the thickened part of the heart. High risk patients can also be implanted with a cardioverter defibrillator to prevent sudden death. High risk patients exhibit a significantly lowered blood pressure during exercise and have a family history of cardiac arrest or episodes of unexplained fainting.
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One major problem in getting the public to understand HCM is knowning the difference between a “heart attack” and a cardiac arrest. I was disappointed to see this “medical” article refer to HCM as the case of a “heart attack”, HCM does not cause “heart attacks” it causes sudden cardiac arrest. It would better serve the public to accurately explain this important issue. If you have a family history of sudden unexpected death under the age of 50 screening for HCM with an echocardiogram should be done.
Thank you,
Lisa Salberg
CEO and Founder
Hypertrophic Cardiomyopathy Association
Please explain the difference between a heart attack and sudden cardiac arrest. I was diagnosed with HCM last yr. Just trying to learn as much as possible about it…thanks.