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Hypertrophic cardiomyopathy

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When the muscles of the heart start to thicken, making it difficult for the blood to flow from it, the heart has to work double time to be able to pump blood for the rest of the body. This condition is known as Hypertrophic Cardiomyopathy or HCM, and it is a disorder that affects nearly 1.5 million people in the United States each year. It is a disease that is also known in medical circles as Hypertrophic Obstructive Cardiomyopathy (HOCM) or Idiopathic Hypertropic Subaortic Stenosis (IHSS)

HCM is one of the factors that lead to sudden cardiac death in young adults below the age of 30. It causes massive cardiac arrest, and subsequently, death. HCM is one of the culprits in the mortalities among professional athletes.

HYPERTROPHIC CARDIOMYOPATHY SIGNS AND SYMPTOMS

This type of complex heart disease is characterized by a thickening of the coronary muscle. It causes stiffness of the left ventricle, and various changes in the heart's mitral valve and cells. The thickening of the heart muscle or myocardium occurs in the area found below the aortic bulb called the septum. The septum is wall composed mainly of muscle and it separates the left and right sides of the heart. When the septum starts to thicken, it pushes against the heart's lower chambers and ventricles, significantly reducing or blocking the flow of blood to the heart's aorta. This type of blood impediment is known as an outflow tract obstruction, and it puts additional pressure on the ventricles to pump harder to maintain adequate blood flow.

HYPERTROPHIC CARDIOMYOPATHY CAUSES

Apart from the thickening of the muscular septum, Hypertrophic Cardiomyopathy can also cause other parts of the heart muscle to thicken, such as the right and left ventricles, as well as its apex. When this occurs, the heart starts to assume an asymmetrical shape, as is the case when one portion of the heart becomes thicker than the others.

Hypertrophic Cardiomyopathy is an inherited disease that is passed down through families. Scientific theory points toward evidence that this condition is a result of genetic problems with the genes that are responsible for controlling the growth of the heart muscle. When there is a genetic defect in the gene code governing the characteristics of the heart, abnormalities occur that develop within the coronary muscles of a family. This is not an absolute rule, however, as some people who possess these abnormal gene codes never acquire the disease during their lifetime.

Hypertrophic Cardiomyopathy is also an acquired disease, and elderly individuals, as well as people who have a history of high blood pressure have a greater tendency of acquiring HCM. Younger adults can also be affected, and in some cases, the causes of HCM are unknown.

Due to the great variety of causes of HCM, the medical community is still unable to accurately pinpoint any segment of the population considered to be at high risk for developing this type of coronary disease.

A majority of people who have acquired Hypertrophic Cardiomyopathy usually experience very minor or no symptoms of the disease, and they are able to lead a relatively normal life. Other people develop a full range of symptoms that steadily progresses until their condition worsens and their heart functions degenerate.
Hypertrophic cardiomyopathy
Image: Hypertrophic cardiomyopathy


HCM symptoms include a sensation of pressure in the chest and instances of chest pain that occur whenever a person engages in exercise or other physical activity that requires a degree of exertion. However, it may also crop up unexpectedly when a person is at rest or after having eaten a meal. Dyspnea or shortness of breath accompanies the chest pain, and it is followed by fatigue, especially among adult sufferers. These symptoms are caused by pressure from blockage that is felt in the left atrium and radiates to the lungs.

About 15% to 20% of people suffering from Hypertrophic Cardiomyopathy experience syncope or fainting. This is due in part to irregular heart rhythms and the reaction of a person's blood vessels to exercise. An HCM sufferer will also experience heart palpitations or arrhythmias. HCM arrhythmias may either be ventricular tachycardia or atrial fibrillations, which occur in some 25% of people with HCM and puts them at a higher risk for developing blood clots and subsequent heart failure. A small percentage of patients succumb to sudden death due to the disease.

Other symptoms include dizziness, light-headedness after any physical exertion, shortness of breath while in a reclining position, and a lower tolerance for physical activity. Among many young adults, Hypertrophic Cardiomyopathy causes a sudden collapse and subsequent death without any warning. On the other hand, some patients do not manifest any symptoms at all and only discover that they have HCM during any number of routine medical examinations.

Upon medical consultation, the doctor will take down a list of all the symptoms a patient has, their medical history, and any family history of the disease. The patient will also be subjected to a thorough physical exam, with particular concentration on the heart and lungs. The doctor will determine whether the patient has Hypertrophic Obstructive Cardiomyopathy by listening to the patient's heartbeat with a stethoscope or taking the pulse at the arms and neck to check for signs of a heart murmur or abnormal heartbeat.

HYPERTROPHIC CARDIOMYOPATHY DIAGNOSIS

The doctor will also take an echocardiogram of the patient's heart, looking for the telltale thickening of the heart walls in the images created through echo sound waves. This electronic imaging technique is one of the most common diagnostic tests used to determine the presence of HCM.

Other tests that the patient will be subject to will include blood tests, chest x-rays, an exercise stress test, magnetic resonance imaging or MRI, cardiac catherization, and installation of a portable 24-hour Holt monitor to make a record of the patient's heart movements.

HYPERTROPHIC CARDIOMYOPATHY TREATMENT

Treating Hypertrophic Cardiomyopathy involves controlling the symptoms of the disease in order to avoid complications. In some cases, patients with severe symptoms are required to be hospitalized until their condition is stabilized with appropriate treatment.

HCM symptoms respond favorably to medications that relax the heart and help it to contract. These medications include beta-blockers and calcium channel blockers that the doctor may prescribe to reduce the symptoms of chest pain during physical activity. With milder manifestations of the disease, these drugs can help relieve symptoms without patients having to undergo invasive treatments such as surgery.

Patients who have been diagnosed with accompanying arrhythmias will be prescribed with medication that regulates their heartbeat. If the arrhythmia stems from atrial fibrillation, the doctor may prescribe the use of blood thinners, such as low dose aspirin, to reduce the risk of a patient developing blood clots.


More severe symptoms will require invasive treatment such as an insertion of a pacemaker, or a procedure called a surgical myectomy which involves cutting into and removing thickened portions of the heart the blocks the blood flow. Heart valve replacement may also be undertaken to repair or replace a leaking mitral valve.

Other procedures include injecting alcohol into the arteries (alcohol septal ablation) that lead to the thickened heart muscle, and inserting an implantable-cardioverter defibrillator (ICD) for patients who are at high risk for sudden death.

While some people with Hypertrophic Cardiomyopathy do not manifest any symptoms of the disease, other people may experience a whole range of symptoms that may worsen over the course of time. The risk for sudden death is high, and this can occur even when the sufferer is at a young age. Nearly 50% of all deaths relating to HCM occur after or during physical activity, especially among professional athletes.
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