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Glossary

Alcohol Septal Ablation (ASA)

Ablation is the removal of material from the surface of an object. ASA, performed in the cardiac catheterization laboratory under mild sedation, utilizes a small amount of alcohol is injected into the small area of the thickened heart muscle causing the obstruction. Almost immediately, there is a reduction in obstruction in properly selected patients, with continued improvement of symptoms over the ensuing two years, at which point it plateaus. Over 90% of patients show significant improvement in symptoms.

Amiodarone

Amiodarone is and antiarrhythmic medication used to treat and prevent certain types of serious, life-threatening abnormal heart rhythms when other medications do not help or can not be tolerated.

Angina

Angina, is chest pain due to a lack of blood supply to the heart muscle.

Aortic Valve

The aortic valve is one of the heart valves. It is located between the left ventricle, the heart’s largest chamber, and the aorta, the body’s largest artery.

Apical HCM

Apical hypertrophic cardiomyopathy (HCM) is characterized by thickening of the heart muscle of the left ventricle. It is a relatively rare, usually non-obstructive variant of HCM.

Arrhythmia

Arrhythmias are problems with the heart beat rate. During an arrhythmia, the heart can beat too fast, too slow, or with an irregular rhythm.

Arrhythmia Ablation Procedures

Ablation, which removes material from the surface of an object, is used to treat abnormal heart rhythms (arrhythmias). The type of arrhythmia and the presence of other heart disease determine whether ablation can be performed surgically or non-surgically. In non-surgical ablation, a catheter is inserted through a small incision in the groin and guided into a specific area of the heart. A special device directs energy through the catheter to area of the heart muscle causing the abnormal heart rhythm. This energy "disconnects" the source of the abnormal rhythm from the rest of the heart. Surgical ablation procedures used for treating atrial fibrillation can be "minimally invasive" or traditional "open" surgery and may be combined with other surgical therapies, such as bypass surgery, valve repair or valve replacement. This form of ablation for arrhythmias is distinct from alcohol septal ablation, which is a treatment for outflow tract obstruction.

Atherosclerosis

Atherosclerosis, also known as hardening of the arteries, occurs when fat, cholesterol and other substances build up in the walls of arteries and form plaque, which can block the arteries and cause symptoms and problems throughout the body over time.

Atrial Fibrillation (AF)

Atrial fibrillation is the most common irregular heart beat. It may be asymptomatic, but is often associated with palpitations, fainting, chest pain or congestive heart failure. AF can increase the risk of stroke.

Beta-Blockers

Beta-blockers are a class of drugs particularly useful in managing cardiac arrhythmias, blood pressure or outflow tract obstruction in HCM.

Calcium Antagonists

Calcium antagonists, also known as calcium channel blockers, are medications that prevent calcium from entering heart cells and blood vessel walls. They relax and widen the vessels and can help lower blood pressure, reduce heart rate, relieve angina and control arrhythmia. As such, they are also oftentimes useful in HCM.

Cardiac MRI

Cardiac MRI produces high-quality images of the beating heart. It is considered the “gold standard” for evaluating heart muscle viability and anatomy. Because the technology evaluates the size of the heart chambers and thickness of the walls, it is the test of choice for the diagnosis of HCM.

Cardiopulmonary Testing (CPX)

Cardiopulmonary exercise testing is a noninvasive and objective approach to evaluating heart and lung function. It provides physicians with data on gas exchanges in the lungs at rest and during exercise, and yields breath-by-breath measures of oxygen uptake. It is especially useful in assessing heart failure patients, as well as assessing an HCM patient’s need for heart transplantation.

Cardiovascular Magnetic Resonance Imaging

See Cardiac MRI

Chordal Apparatus

The chordal apparatus consists of cord-like bands of fibrous tissue that attach the small muscles (papillary) within the heart that serve to anchor the valves to the edges of the valves themselves.

Coumadin

Coumadin (warfarin) is in a class of blood-thinners that decrease the clotting ability of the blood and prevent the formation or enlargement of blood clots. It is generally used in patients with certain types of arrhythmia and prosthetic heart valves.

Diastolic Function

Diastole (dilation) is the period of time when the heart chambers are relaxed in order to refill with blood after the muscle has contracted. The level of diastolic dysfunction is an indicator of disease severity, and is common in patients with HCM with and without outflow tract obstruction.

Disopyramide

Disopyramide is a drug used to treat arrhythmias. However, in HCM, it is oftentimes used to relax the heart and relieve outflow tract obstruction.

Dyspnea

Dyspnea is shortness of breath, and may be due to multiple factors.

ECG (Electrocardiography)

ECG is a noninvasive method of recording the electrical activity of the heart over a period of time. It measures the rate and regularity of heartbeats, as well as the size and function of the chambers, the presence of any damage to the heart, and the effects of drugs or devices used to regulate the heart (such as a pacemaker).

ECG Gating

ECG gating is a diagnostic technique in which image acquisition is triggered by a start pulse derived from an ECG taken from the patient while imaging. ECG gating techniques are useful whenever data acquisition is too slow to occur during a short fraction of the cardiac cycle. This technique is also very promising for evaluating vessel-wall motion of the aortic artery.

Echocardiogram

An echocardiogram uses sound waves (ultrasound) to produce images of the heart that show how the heart is beating and pumping blood. It can identify various abnormalities of the heart muscle and valves.

Electrophysiology (EP) Ablations

Cardiac electrophysiology focuses on the heart’s electrical system. While often used as a diagnostic tool, it is also employed to help correct arrhythmias through ablation (tissue removal), which cauterizes cells to eliminate rhythm abnormalities.

Electrophysiology (EP) Evaluation

Cardiac electrophysiology focuses on the heart’s electrical system, and is often used as a diagnostic tool.

Heart Failure (HF)

Heart failure, often called congestive heart failure, is the inability of the heart to pump sufficient blood to meet the body’s needs. Some patients may have heart failure that manifests as excessive fatigue, even though there is no congestion (fluid) buildup.

Holter Monitor

A Holter monitor is a battery-operated device that records the heart's rhythms continuously. Prescribed to see if there have been any irregular heart rhythms, the monitor is usually worn for 24 - 48 hours during normal activity. Some monitors may be worn longer.

Internal Cardioverter Defibrillator (ICD)

An implantable cardioverter-defibrillator is a small battery-powered electrical impulse generator implanted in patients at risk for sudden cardiac death due to ventricular fibrillation and ventricular tachycardia. The device is programmed to detect these irregular heart rhythms and correct them by delivering a jolt of electricity.

Inter-Ventricular Septum

The inter-ventricular (or ventricular) septum is the wall that separates the lower chambers (ventricles) of the heart. It is the area most commonly thickened in HCM.

Intramyocardial Scarring

Intramyocardial scarring is fibrous tissue that replaces normal tissue destroyed by heart muscle disease or injury to the heart’s muscular tissue. Such scarring can disrupt the heart’s electrical conduction system and possibly lead to heart failure. Scarring is usually detected by MRI.

Left Ventricular Outflow Tract (LVOT)

A ventricular outflow tract is the portion of a ventricle through which blood passes in order to enter the great arteries. The left ventricular outflow tract (LVOT) connects to the aorta.

Mitral Valve Regurgitation (MVR)

Mitral valve regurgitation is a disorder in which the mitral valve does not close properly when the heart pumps out blood, causing abnormal blood leakage (regurgitation) from the left ventricle, through the mitral valve, and into the left atrium.

Myocardial Disarray

Myocardial disarray is the loss of the normal parallel alignment of the muscle cells of the heart. This structural abnormality — found in hypertrophic cardiomyopathy — leads to a lack of coordination of the heart chambers and is a cause of life-threatening arrhythmias.

Myocardium

The myocardium is the heart muscle.

Non-Sustained Ventricular Tachycardia

Ventricular tachycardia is a rapid heart rhythm, originating in one of the ventricles. If the fast rhythm ends within 30 seconds, it is considered a non-sustained ventricular tachycardia. If it lasts more than 30 seconds, it is known as a sustained ventricular tachycardia.

Palpitations

Palpitations are heartbeat abnormalities that can range from unnoticed skipped beats or accelerated heart rate to very noticeable changes accompanied by dizziness or difficulty breathing.

Papillary Muscles

Papillary muscles are small structures within the heart that anchor the heart valves. Located in the heart’s ventricles, they attach to the cusps of the mitral and tricuspid valves via the chordal apparatus and contract to prevent valves from falling out of place (leaking) or prolapsing.

Permanent Pacemaker

A pacemaker is a small, battery-powered device that sends small electrical impulses to the heart inorder to regulate the beat and normalize its speed. A permanent pacemaker is surgically implanted under the skin of the chest.

Pulmonary Hypertension

Pulmonary hypertension is a lung disorder in which the arteries that carry blood from the heart to the lungs become narrowed, making it difficult for blood to flow through the vessels. As a result, blood pressure in the pulmonary arteries exceeds normal levels, straining the right ventricle of the heart and causing it to enlarge. The right ventricle then gradually becomes weaker and loses its ability to pump enough blood to the lungs. This could lead to heart failure, especially in patients who have lived with HCM for many years.

Radiofrequency Ablation (RFA)

Radiofrequency ablation involves removing (ablating) dysfunctional tissue by using the heat generated from the high-frequency alternating current. The procedure can be used to locate and destroy abnormal electrical circuits in the heart that generate recurrent arrhythmias.

Sarcomere

A sarcomere is the basic microscopic unit of a muscle. Sarcomeres give skeletal and cardiac muscles their striated appearance.

Septum

The septum is the wall that separates the heart’s lower chambers (ventricles).

Stress Testing (Exercise and Nuclear Perfusion Imaging)

Cardiac stress testing is used to measure the heart's ability to respond to external stress under controlled conditions. The stress response is induced through exercise or drug stimulation, with the test comparing coronary circulation while the patient is at rest and during maximum physical exertion. The objective is to identify abnormal blood flow to the heart's muscle, such as atherosclerosis. In HCM, patients undergo stress testing to also evaluate their risk annually for sudden cardiac death.

Supravalvular/Subvalvular Obstruction

  • A supravalvular obstruction occurs when the obstruction is located above the aortic valve usually due to presence of excess tissue (membrane); it is not a form of HCM.
  • A subvalvular obstruction occurs when the obstruction is located below the aortic valve. There are two forms of this condition attributed to HCM. Surgery is usually required when the amount of excess tissue (membrane) is abnormal.

Supraventricular Tachycardia

Supraventricular tachycardia is an unusually rapid heart rhythm originating above the ventricular tissue.

Surgical (Septal) Myectomy

Septal myectomy is a complex surgical procedure that removes a portion of the thickened wall separating the ventricles and obstructing blood flow. Once thickness of the septum is reduced, the left ventricle enlarges and blood flow improves. It has been around for over 40 years and considered the gold-standard procedure for patients with symptomatic HCM uncontrolled by medications. Young patients and those with complex anatomy are well-suited for surgical myectomy.

Syncope

Syncope is the temporary loss of consciousness (fainting) caused by inadequate blood supply to the brain, either due to arrhythmia or outflow tract obstruction.

Tachycardia

Tachycardia is the unusually fast – but regular – beating of the heart.

Thrombus

A thrombus is a blood clot that forms in a vessel or chamber of the heart and remains there.

Transesophageal Echocardiography (TEE)

Transesophageal echocardiogrphy is an alternative to standard cardiac echocardiography. It uses high-frequency sound waves to produce a graphic outline of the heart’s movement. TEE utilizes a specialized probe containing an transducer at its tip that is passed into the patient's esophagus and allows for a close look at the heart’s valves and chambers without interference from the ribs or lungs.

Transthoracic Echocardiography (TTE)

Transthoracic echocardiography is the standard form of cardiac ultrasound. The transducer (probe) is placed on the patient’s chest, and images are taken through the chest wall, producing a noninvasive, accurate and quick assessment of the overall health of the heart. The procedure can assess heart valves and degree of heart muscle contraction, as well as evaluate all four chambers of the heart.

Ventricular Fibrillation

Ventricular fibrillation is the uncoordinated contraction of the cardiac muscle of the heart’s ventricles, making them quiver rather than contract properly. The condition causes an extremely irregular, serious heart-rhythm disturbance in the lower chambers of the heart.

Ventricular Hypertrophy

Ventricular hypertrophy is the enlargement (thickening) of ventricles.

Ventricular Tachycardia

Ventricular tachycardia, a rapid heart beat originating in one of the ventricles, is a potentially life-threatening arrhythmia because it can lead to ventricular fibrillation.
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