What is Hypertrophic Cardiomyopathy (HCM)?

 

Cardiomyopathy is a condition in which the muscle of the heart is abnormal in the absence of an apparent cause. This terminology is purely descriptive and is based on the Latin deviation. HCM is a primary and usually familial cardiac disorder with heterogeneous expression, unique pathophysiology, and a diverse clinical course, for which several disease causing mutations in the genes encoding proteins of the cardiac sacomere have been reported. While HCM has typically been recognized by its structure i.e., hypertrophy, the electrical function of the heart, is also adversely affected. There are four types of cardiomyopathy: hypertrophic, ischemic, dilated and restrictive. The main feature of HCM is an excessive thickening of the heart muscle (hypertrophy literally means to thicken). Thickening is seen in the ventricular septal measurement (normal range .08-1.2cm), and in weight. In HCM, septal measurements may be in the range of 1.3cm to 6.0+cm. Heart muscle may also thicken in normal individuals as a result of high blood pressure or prolonged athletic training. Furthermore, there is a fine line between and athletic heart and a heart with HCM. In Hypertrophic Cardiomyopathy (HCM), the muscle thickening occurs without an obvious cause. In addition, microscopic examination of the heart muscle in HCM is abnormal. The normal alignment of muscle cells is absent and this abnormality is called "myocardial disarray". Myocardial DisarrayThe contrast between the regular, parallel alignment of muscle cells in a normal heart with the irregular, disorganized alignment of muscle cells or "myocardial disarray" found in some parts of the heart in hypertrophic cardiomyopathy is only obvious under microscopic examination. HCM is also known by several other names. These names include IHSS (or idiopathic hypertrophic subaortic stenosis), which was the first popular term used in the United States; "stenosis" means obstruction. The same can be said for HOCM (hypertrophic obstructive cardiomyopathy), which is, still used in the United Kingdom...largely out of habit and convenience. Nevertheless, virtually all HCM experts and other cardiovascular specialists now regard as the best single name for this broad disease spectrum --- hypertrophic cardiomyopathy or HCM. This term emphasizes the hypertrophy, which is the diagnostic marker in most patients and the fact that this disease is a form of cardiomyopathy -- or heart muscle disorder... without mentioning obstruction. Therefore, it is preferable to describe the disease as either "HCM with obstruction" or "HCM without obstruction." "Cardiomyopathy" itself is a very general term referring to any condition (and there are many) importantly affecting the heart muscle itself while "hypertrophic cardiomyopathy" refers to a specific and genetic condition, which usually shows a familial pattern. The most characteristic feature of HCM is a hypertrophied left ventricle (asymmetric thickening of the wall usually most prominently involving the ventricular septum) without abnormal enlargement of the ventricular cavities.What is Dilated Cardiomyopathy (DCM)?Dilated Cardiomyopathy (DCM) is a condition in which the heart becomes weakened and enlarged, and it cannot pump blood efficiently. The decreased heart function can affect the lungs, liver, and other body systems. The treatment for DCM is to treat the underlying cause. 

Disease Facts

What is Polycythemia Vera (PV)?

 

Polycythemia Vera (POL-e-si-THE-me-ah VE-ra), or PV, is a rare blood disease in which your body makes too many red blood cells. These extra red blood cells make your blood thicker than normal. The thickened blood flows more slowly through your small blood vessels and can form clots. These clots can cause heart attack and stroke.Blood cells are formed in your bone marrow—the soft tissue inside bones. In addition to red blood cells, your blood contains two other types of cells: white blood cells to help fight infection and platelets to help your blood clot. If you have PV, your bone marrow produces too many red blood cells, but it also can make too many white blood cells and platelets.Red blood cells also are called RBCs or erythrocytes (eh-RITH-ro-sites). Normal red blood cells look like doughnuts without holes in the center and have an average lifespan of 120 days. Red blood cells contain hemoglobin (HEE-muh-glow-bin), an iron-rich protein that gives blood its red color and carries oxygen to the body. Red blood cells also remove carbon dioxide, a waste product, from cells and carry it to the lungs to be exhaled.IMPORTANT GENERAL INFORMATION ABOUT PVPV is a rare, chronic disease that can be fatal if not diagnosed and treated. The cause of PV is not known. It develops slowly and may not produce symptoms for many years. Sometimes, your symptoms can be vague and nonspecific. Many people find out they have PV from blood tests done for other reasons. It is more common in adult males 60 years or older. It is very rare in people younger than 20 years.With PV, thicker than normal blood slows down the flow of blood to all parts of your body. Clots can form more easily, which can block blood flow through arteries or veins. The slower flow of blood means your organs don’t receive enough oxygen. The shortage of oxygen can lead to angina, congestive heart failure, and gout. Slower blood flow also deprives your arms, legs, lungs, and eyes of the oxygen they need to perform normally. This can cause headaches, dizziness, itching, and problems with your vision, such as blurred or double vision. PV may also cause you to develop stomach ulcers and kidney stones.A small number of people with PV may develop myelofibrosis (MY-e-lo-fi-BRO-sis), a condition in which your bone marrow is replaced by fibrous (scar) tissue. The abnormal bone marrow cells may begin to grow out of control. This abnormal growth can lead to acute myelogenous (my-e-LOJ-e-nus) leukemia (AML), a disease that worsens very quickly. In AML, too many immature white blood cells are found in the blood and bone marrow.HEART DISEASE FACTSHeart disease is the leading cause of death in the United States and is a major cause of disability. Almost 700,000 people die of heart disease in the U.S. each year. That is about 29% of all U.S. deaths. Heart disease is a term that includes several more specific heart conditions. The most common heart disease in the United States is coronary heart disease, which can lead to heart attack.·Heart disease is the leading cause of death for both women and men in the United States. ·In 2002, 696,947 people died of heart disease (51% of them women). This was 29% of all U.S. deaths. The age-adjusted death rate was 241 per 100,000 population. ·Heart disease is the leading cause of death for American Indians and Alaska Natives, blacks, Hispanics, and whites. For Asians and Pacific Islanders, cancer is the leading cause of death (accounting for 26.1% of all deaths), heart disease is a close second (26.0%). ·Heart disease crude death rates per 100,000 population for the five largest U.S. racial/ethnic groups are as follows: Hispanics, 72; Asians and Pacific Islanders, 78; American Indians, 80; blacks, 206; and whites, 259. ·In 2002, age-adjusted death rates for diseases of the heart were 30% higher among African Americans than among whites. Age-adjusted rates are used to compare populations with differing age distributions. ·By state, age-adjusted death rates per 100,000 for diseases of the heart ranged from 165 (Minnesota) to 327 (Mississippi) in 2002. ·Coronary heart disease is the principal type of heart disease. There were 494,392 people that died from coronary heart disease in 2002. That is about 71% of all heart disease deaths. ·It is estimated that about 47% of cardiac deaths occur before emergency services or transport to a hospital. ·In 2006, heart disease is projected to cost more than $258 billion, including health care services, medications, and lost productivity. ·Worldwide, coronary heart disease kills more than 7 million people each year. ·Risk factors for heart disease among adults (for years 1999–2002 unless noted):·Percentage of persons aged 20 years and older with hypertension or taking hypertension medications: 30.2%·Percentage of persons aged 20 years and older with high blood cholesterol: 17.3%·Percentage of persons aged 20 years and older with physician-diagnosed diabetes: 6.5%·Percentage of persons aged 20 years and older who are obese: 30.5%·Percentage of adults aged 18 years and older who are current cigarette smokers (2003): 21.6%·Percentage of adults aged 18 years and older who engage in no leisure-time physical activity (2003): 37.6% ·In 2003, approximately 37% of adults reported having two or more of six risk factors for heart disease and stroke (high blood pressure, high cholesterol, diabetes, current smoking, physical inactivity, and obesity). ·Timely access to emergency cardiac care and survival is partly dependent on early recognition of heart attack symptoms and immediate action by calling emergency services. In a 2001 survey, most persons (95%) recognized chest pain as a heart attack symptom, but only 11% correctly classified all symptoms and knew to call 9–1–1 when someone was having a heart attack. ·Studies among people with heart disease have shown that lowering high blood cholesterol and high blood pressure can reduce the risk of dying of heart disease, having a nonfatal heart attack, and needing heart bypass surgery or angioplasty. ·Studies among people without heart disease have shown that lowering high blood cholesterol and high blood pressure can reduce the risk of developing heart disease. 

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