HEART DISEASES
How much does your heart weigh?
CORONARY HEART DISEASE (CHD)
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DEFINITION
The coronary arteries, which supply the heart with nutrients, oxygen and blood become diseased or damaged, usually because of plaque deposits (cholesterol-containing deposits). Plaque accumulation narrows the coronary arteries and the heart gets less oxygen.
SIGN AND SYMPTOMS
The most common symptoms of coronary heart disease (CHD) are chest pain (angina) and a heart attack. You can also experience other symptoms, such as heart palpitations and unusual breathlessness. In some cases, people may not have any symptoms before they are diagnosed.
CAUSES AND RISK FACTORS
Coronary heart disease (CHD) is usually caused by a build-up of fatty deposits on the walls of the arteries around the heart (coronary arteries). The fatty deposits, called atheroma, are made up of cholesterol and other waste substances.
The build-up of atheroma on the walls of the coronary arteries makes the arteries narrower, restricting the flow of blood to the heart muscle. This process is called atherosclerosis. Your risk of developing atherosclerosis is significantly increased if you:
DIAGNOSIS
Risk assessment
If your doctor thinks you may be at risk of developing CHD, they may carry out a risk assessment for cardiovascular disease, heart attack or stroke. This may be carried out as part of an NHS Health Check. Your doctor will ask about your medical and family history, check your blood pressure, and do a blood test to assess your cholesterol level. All these factors will be considered as part of the diagnosis.
Further tests
To confirm a suspected diagnosis, you may be referred for more tests. A number of different tests are used to diagnose heart-related problems, including:
TREATMENT
Although coronary heart disease (CHD) cannot be cured, treatment can help manage the symptoms and reduce the risk of further problems.
Many different medicines are used to treat CHD. Usually they either aim to reduce blood pressure or widen your arteries. Heart medicines should not be stopped suddenly without the advice of your doctor as there is a risk this may make your symptoms worse. For example,
Some of the main procedures used to treat blocked arteries are outlined below.
EXTRA INFORMATION
Coronary heart disease (CHD) is the leading cause of death both in the UK and worldwide. It's responsible for more than 73,000 deaths in the UK each year. About 1 in 6 men and 1 in 10 women die from CHD. In the UK, there are an estimated 2.3 million people living with CHD and around 2 million people affected by angina, which is the chest pain that occurs when the blood supply to the muscles of the heart is restricted. It usually happens because the arteries supplying the heart become hardened and narrowed.
CHD generally affects more men than women, although from the age of 50 the chances of developing the condition are similar for both sexes.
The coronary arteries, which supply the heart with nutrients, oxygen and blood become diseased or damaged, usually because of plaque deposits (cholesterol-containing deposits). Plaque accumulation narrows the coronary arteries and the heart gets less oxygen.
SIGN AND SYMPTOMS
The most common symptoms of coronary heart disease (CHD) are chest pain (angina) and a heart attack. You can also experience other symptoms, such as heart palpitations and unusual breathlessness. In some cases, people may not have any symptoms before they are diagnosed.
CAUSES AND RISK FACTORS
Coronary heart disease (CHD) is usually caused by a build-up of fatty deposits on the walls of the arteries around the heart (coronary arteries). The fatty deposits, called atheroma, are made up of cholesterol and other waste substances.
The build-up of atheroma on the walls of the coronary arteries makes the arteries narrower, restricting the flow of blood to the heart muscle. This process is called atherosclerosis. Your risk of developing atherosclerosis is significantly increased if you:
- Smoke
- have high blood pressure (hypertension)
- have diabetes
DIAGNOSIS
Risk assessment
If your doctor thinks you may be at risk of developing CHD, they may carry out a risk assessment for cardiovascular disease, heart attack or stroke. This may be carried out as part of an NHS Health Check. Your doctor will ask about your medical and family history, check your blood pressure, and do a blood test to assess your cholesterol level. All these factors will be considered as part of the diagnosis.
Further tests
To confirm a suspected diagnosis, you may be referred for more tests. A number of different tests are used to diagnose heart-related problems, including:
- electrocardiogram (ECG)
- exercise stress tests
- X-rays
- echocardiogram
- radionuclide tests
- magnetic resonance imaging (MRI) scans
- computerized tomography (CT) scans
TREATMENT
Although coronary heart disease (CHD) cannot be cured, treatment can help manage the symptoms and reduce the risk of further problems.
Many different medicines are used to treat CHD. Usually they either aim to reduce blood pressure or widen your arteries. Heart medicines should not be stopped suddenly without the advice of your doctor as there is a risk this may make your symptoms worse. For example,
- Antiplatelet
- Statins
- Beta-blockers
- Angiotensin II receptor antagonists
- Calcium channel blockers
- Diuretics
Some of the main procedures used to treat blocked arteries are outlined below.
- Coronary angioplasty
- Coronary artery bypass graft
- Heart transplant
EXTRA INFORMATION
Coronary heart disease (CHD) is the leading cause of death both in the UK and worldwide. It's responsible for more than 73,000 deaths in the UK each year. About 1 in 6 men and 1 in 10 women die from CHD. In the UK, there are an estimated 2.3 million people living with CHD and around 2 million people affected by angina, which is the chest pain that occurs when the blood supply to the muscles of the heart is restricted. It usually happens because the arteries supplying the heart become hardened and narrowed.
CHD generally affects more men than women, although from the age of 50 the chances of developing the condition are similar for both sexes.
MARFAN SYNDROME
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DEFINITION
Marfan syndrome is an inherited disease that affects the body's connective tissue, which provides the strength, support, and elasticity to tendons, cartilage, heart valves, blood vessels, and other vital parts of the body.For people with Marfan syndrome, the connective tissue lacks strength due to its abnormal chemical makeup. One of the biggest threats of Marfan syndrome is damage to the aorta, the artery that carries blood from the heart to the rest of the body. Marfan syndrome can rupture the inner layers of the aorta, causing dissection that leads to bleeding in the wall of the vessel. Aortic dissection can be deadly. Surgery may be required to replace the affected part of the aorta.
SIGN AND SYMPTOMS
Some people with Marfan syndrome also have mitral valve prolapse, a billowing of the heart valve that may be associated with irregular or rapid heart beats and shortness of breath. It may require surgery. The syndrome affects the bones, eyes, skin, lungs, and nervous system along with the heart and blood vessels.
CAUSES AND RISK FACTOR
Marfan syndrome is caused by a change in the gene that controls how the body makes fibrillin, an essential component of connective tissue that contributes to its strength and elasticity. In most cases, Marfan syndrome is inherited from a parent, but 1 in 4 cases occurs in people with no known family history of the disease. It occurs equally in men and women, who have a 50% risk of passing on the gene to their children. Marfan syndrome is present at birth, but may not be diagnosed until adolescence or later. Everyone with Marfan syndrome has the same defective gene, but not everyone experiences the same symptoms to the same degree.
DIAGNOSIS
Genetic testing alone cannot tell you if you have Marfan syndrome, as there are other connective tissue disorders. If the disease is suspected, the doctor will perform a thorough physical exam of the eyes, heart and blood vessels, and muscle and skeletal system, obtain a history of symptoms and information about family members that may have had the disorder, to determine if you have it. Other tests, such as a chest X-ray, an electrocardiogram (ECG), and an echocardiogram will be used to evaluate changes in the heart and blood vessels, and detect heart rhythm problems. If sections of the aorta are unable to be visualized through echocardiogram, or a dissection is already suspected, a transesophageal echocardiogram (TEE), MRI, or CT scan may be needed. The scans can also be used to check the lower back for signs of dural ectasia, a back problem that is common in people with Marfan syndrome. Other diagnostic tests for Marfan syndrome include a slit lamp eye exam in which the doctor will check for dislocated lenses.
TREATMENT
Medications are typically not used to treat Marfan syndrome. However, your doctor may prescribe a beta-blocker, which decreases the forcefulness of the heartbeat and the pressure within the arteries, thus preventing or slowing the enlargement of the aorta. Beta-blocker therapy is usually started when the person with Marfan syndrome is young. Some people are unable to take beta-blockers because they have asthma or because of the medication's side effects, which may include drowsiness or weakness, headaches, slowed heartbeat, swelling of the hands and feet or trouble breathing and sleeping. In these cases, another medication called a calcium channel blocker may be recommended. An ongoing clinical trial that began in 2007 is looking at the how two drugs, atenolol, a beta-blocker that may slow the growth of the aorta, and losartan, an angiotension receptor blocker used to lower blood pressure, can be used to manage Marfan syndrome.
Surgery for Marfan syndrome is to prevent aortic dissection or rupture and to treat problems affecting the heart's valves, which control the flow of blood in and out of the heart and between the heart's chambers. The decision to perform surgery is based on the size of the aorta, expected normal size of the aorta, rate of aortic growth, age, height, gender, and family history of aortic dissection. Surgery involves replacing the dilated portion of the aorta with a graft, a piece of man-made material that is inserted to replace the damaged or weak area of the blood vessel. A leaky aortic or mitral valve (the valve that controls the flow of blood between the two left chambers of the heart) can damage the left ventricle (the lower chamber of the heart that is the main pumping chamber) or cause heart failure. In these cases, surgery to replace or repair the affected valve is necessary. If surgery is performed early, before the valves are damaged, the aortic or mitral valve may be repaired and preserved. If the valves are damaged, they may need to be replaced. If surgery is needed, you should consult with a surgeon who is experienced in surgery for Marfan syndrome. People who have surgery for Marfan syndrome still require life-long follow-up care to prevent future complications associated with the disease.
EXTRA INFORMATION
In the past, the average age of death for people with Marfan syndrome was 32 years old. Today, with the help of early diagnosis, appropriate management, and long-term follow-up care by an experienced team of health care providers, most people with the disorder live active, healthy lives with a life expectancy similar to that of the general population. The condition is fairly common, affecting 1 in 5,000 Americans. It is found in people of all races and ethnic backgrounds.
MYOCARDIAL INFARCTION
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DEFINITION
It is known as heart attack, cardiac infarction and coronary thrombosis. Interrupted blood flow (lack of oxygen) damages or destroys part of the heart muscle. This is usually caused by a blood clot that develops in one of the coronary arteries (blood vessels that supply the heart with blood). It can also occur if an artery suddenly narrows (spasm).
SIGN AND SYMPTOMS
The common symptoms include chest pain, (a sensation of pressure, tightness or squeezing in the centre of your chest, pain in other parts of the body – it can feel as if the pain is travelling from your chest to your arms (usually the left arm is affected, but it can affect both arms), jaw, neck, back and abdomen, feeling lightheaded or dizzy, sweating, shortness of breath, nausea or vomiting, an overwhelming sense of anxiety (similar to having a panic attack) and also coughing or wheezing.
CAUSES AND RISK FACTOR
Heart attacks are caused by the blood supply to the heart being suddenly interrupted. Like all other tissues and organs in the body, the heart needs a constant supply of oxygen-rich blood. Without this supply, heart muscles may be damaged and begin to die. If this is not treated, heart muscles will experience irreversible damage. If a large portion of the heart is damaged in this way, the heart will stop beating (known as a cardiac arrest), resulting in death.
DIAGNOSIS
If a heart attack is suspected, you should be admitted to hospital immediately. You will usually be admitted to an acute cardiac care unit (ACCU), or directly to the cardiac catheterisation unit, to confirm the diagnosis and begin treatment. An electrocardiogram (ECG) is an important test in suspected heart attacks. It helps determine what type of heart attack you have had, which will help determine the most effective treatment.
TREATMENT
The treatment options for a heart attack depend on whether you have had an ST segment elevation myocardial infarction (STEMI), or another type of heart attack. A STEMI is the most serious form of heart attack and requires emergency assessment and treatment. It is important that you are treated quickly to minimise damage to your heart. If you have symptoms of a heart attack and an electrocardiogram (ECG) shows you have a STEMI, you will be assessed for treatment to unblock the coronary arteries.
EXTRA INFORMATION
Heart attacks are one of the most common reasons why a person requires emergency medical treatment. Men are more likely to have a heart attack than women. The British Heart Foundation estimates that around 50,000 men and 32,000 women have a heart attack each year in England. Most heart attacks occur in people aged over 45.
DEFINITION
It is known as heart attack, cardiac infarction and coronary thrombosis. Interrupted blood flow (lack of oxygen) damages or destroys part of the heart muscle. This is usually caused by a blood clot that develops in one of the coronary arteries (blood vessels that supply the heart with blood). It can also occur if an artery suddenly narrows (spasm).
SIGN AND SYMPTOMS
The common symptoms include chest pain, (a sensation of pressure, tightness or squeezing in the centre of your chest, pain in other parts of the body – it can feel as if the pain is travelling from your chest to your arms (usually the left arm is affected, but it can affect both arms), jaw, neck, back and abdomen, feeling lightheaded or dizzy, sweating, shortness of breath, nausea or vomiting, an overwhelming sense of anxiety (similar to having a panic attack) and also coughing or wheezing.
CAUSES AND RISK FACTOR
Heart attacks are caused by the blood supply to the heart being suddenly interrupted. Like all other tissues and organs in the body, the heart needs a constant supply of oxygen-rich blood. Without this supply, heart muscles may be damaged and begin to die. If this is not treated, heart muscles will experience irreversible damage. If a large portion of the heart is damaged in this way, the heart will stop beating (known as a cardiac arrest), resulting in death.
DIAGNOSIS
If a heart attack is suspected, you should be admitted to hospital immediately. You will usually be admitted to an acute cardiac care unit (ACCU), or directly to the cardiac catheterisation unit, to confirm the diagnosis and begin treatment. An electrocardiogram (ECG) is an important test in suspected heart attacks. It helps determine what type of heart attack you have had, which will help determine the most effective treatment.
TREATMENT
The treatment options for a heart attack depend on whether you have had an ST segment elevation myocardial infarction (STEMI), or another type of heart attack. A STEMI is the most serious form of heart attack and requires emergency assessment and treatment. It is important that you are treated quickly to minimise damage to your heart. If you have symptoms of a heart attack and an electrocardiogram (ECG) shows you have a STEMI, you will be assessed for treatment to unblock the coronary arteries.
EXTRA INFORMATION
Heart attacks are one of the most common reasons why a person requires emergency medical treatment. Men are more likely to have a heart attack than women. The British Heart Foundation estimates that around 50,000 men and 32,000 women have a heart attack each year in England. Most heart attacks occur in people aged over 45.
DILATED CARDIOMYOPATHY (DCM)
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DEFINITION
Dilated cardiomyopathy (DCM) is a condition in which the heart's ability to pump blood is decreased because the heart's main pumping chamber, the left ventricle, is enlarged and weakened. In some cases, it prevents the heart from relaxing and filling with blood as it should. Over time, it can affect the other heart chambers.
SYMPTOMS OF DCM
Many people with dilated cardiomyopathy have no symptoms or only minor symptoms, and live a normal life. Other people developsymptoms, which may progress and worsen as heart function worsens.
Symptoms of DCM can occur at any age and may include:
- Heart failure symptoms (shortness of breath and fatigue)
- Swelling of the lower extremities
- Fatigue (feeling overly tired)
- Weight gain
- Fainting (caused by conditions such as irregular heart rhythms, abnormal responses of the blood vessels during exercise, or no cause may be found)
- Palpitations (fluttering in the chest due to abnormal heart rhythms)
- Dizziness or lightheadedness
- Blood clots can form in the dilated left ventricle as a result of pooling of the blood; if a blood clot breaks off, it can lodge in an artery and disrupt blood flow to the brain, causing stroke. A clot can also block blood flow to the organs in the abdomen or legs.
- Chest pain or pressure
- Sudden death
CAUSES OF DCM
DCM can be inherited, but it is primarily caused by a variety of other factors, including:
- Severe coronary artery disease
- Alcoholism
- Thyroid disease
- Diabetes
- Viral infections of the heart
- Heart valve abnormalities
- Drugs that are toxic (or cause damage) to the heart
DIAGNOSIS
The most common tests for cardiomyopathy are an ECG, which records the electrical activity of your heart, and an echocardiogram which shows the pumping action of your heart.
TREATMENT FOR DCM
Medications: To manage heart failure, most people improve by taking drugs, such as a beta-blocker,ACE inhibitor or an ARB, and/or diuretics. If you have an arrhythmia (irregular heart beat), your doctor may give you a medication to control your heart rate or lessen the occurrence of arrhythmias. Blood thinners may be used to prevent blood clots from occurring.
Lifestyle changes: If you have heart failure, you should reduce sodium in your diet based on your doctor's recommendations. Your doctor may recommend aerobic exercise, but don't do heavy weight lifting.
People with severe DCM may need one of the following surgeries:
- Cardiac resynchronization by biventricular pacemaker. For some people with DCM, stimulating both the right and left ventricles with this pacemaker improves the heart's ability to contract with more force, thereby improving symptoms and increasing the length of time you can exercise. This pacemaker also will help people with heart block or some bradycardias (slow heart rates).
- Implantable cardioverter defibrillators (ICD). ICDs are suggested for people at risk for life-threatening arrhythmias or sudden cardiac death. The ICD constantly monitors the heart rhythm. When it detects a very fast, abnormal heart rhythm, it ''shocks'' the heart muscle into returning to a normal rhythm.
- Surgery. Conventional surgeries for coronary artery disease or valvular disease may be recommended. Some people may be eligible for surgical repair of the left ventricle or placement of a cardiac assist device.
- Heart transplant
EXTRA INFORMATION
Dilated cardiomyopathy (DCM) is the most common type, occurring mostly in adults 20 to 60. Men are more likely in men than in women. It affects the heart's ventricles and atria, the lower and upper chambers of the heart, respectively.
HYPERTROPHIC CARDIOMYOPATHY (HCM)
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DEFINITION
Hypertrophic cardiomyopathy (HCM) is associated with thickening of the heart muscle, most commonly at the septum between the ventricles, below the aortic valve. This leads to stiffening of the walls of the heart and abnormal aortic and mitral heart valve function, both of which may impede normal blood flow out of the heart.
SYMPTOMS OF HCM
Many people with HCM have no symptoms or only minor symptoms, and live a normal life. Other people develop symptoms, which progress and worsen as heart function worsens.
Symptoms of HCM can occur at any age and may include:
- Chest pain or pressure (occurs usually with exercise or physical activity, but can also occur with rest or after meals)
- Shortness of breath (dyspnea), especially with exertion
- Fatigue (feeling overly tired)
- Fainting (caused by irregular heart rhythms, abnormal responses of the blood vessels during exercise, or no cause may be found)
- Palpitations (fluttering in the chest) due to abnormal heart rhythms (arrhythmias), such as atrial fibrillation or ventricular tachycardia
- Sudden death (occurs in a small number of patients with HCM)
CAUSES
HCM can run in families, but the condition may also be acquired as a part of aging or high blood pressure. In other instances, the cause is unknown.How Is Hypertrophic Cardiomyopathy Diagnosed?HCM is diagnosed based on medical history (your symptoms and family history), a physical exam, and echocardiogram results. Additional tests may include blood tests, electrocardiogram, chest X-ray, exercise stress test, cardiac catheterization, CT scan, and MRI.
DIAGNOSIS
HCM is diagnosed based on medical history (your symptoms and family history), a physical exam, and echocardiogram results. Additional tests may include blood tests, electrocardiogram, chest X-ray, exercise stress test, cardiac catheterization, CT scan, and MRI.
TREATMENT
Treatment of HCM depends on whether there is narrowing in the path that blood takes to leave the heart (called the outflow tract); how the heart is functioning; and if arrhythmias are present. Treatment is aimed at preventing symptoms and complications and includes risk identification and regular follow-up, lifestyle changes, medications, and procedures as needed.
EXTRA INFORMATION
Hypertrophic cardiomyopathy (HCM) is very common and can affect people of any age. It affects men and women equally. It is a common cause of sudden cardiac arrest in young people, including young athletes.
RESTRICTIVE CARDIOMYOPATHY
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DEFINITION
Restrictive cardiomyopathy, the rarest form of cardiomyopathy, is a condition in which the walls of the lower chambers of the heart (the ventricles) are abnormally rigid and lack the flexibility to expand as the ventricles fill with blood.The pumping or systolic function of the ventricle may be normal, but the diastolic function (the ability of the heart to fill with blood) is abnormal. Therefore, it is harder for the ventricles to fill with blood, and with time, the heart loses the ability to pump blood properly, leading to heart failure.
SIGNS AND SYMPTOMS
Many people with restrictive cardiomyopathy have no symptoms or only minor symptoms, and live a normal life. Other people develop symptoms, which progress and worsen as heart function worsens.
Symptoms of restrictive cardiomyopathy can occur at any age and may include:
- Shortness of breath (at first with exercise; but over time it occurs at rest)
- Fatigue (feeling overly tired)
- Inability to exercise
- Swelling of the legs and feet
- Weight gain
- Nausea, bloating, and poor appetite (related to fluid retention)
- Palpitations (fluttering in the chest due to abnormal heart rhythms)
- Fainting (usually caused by irregular heart rhythms or abnormal responses of the blood vessels during exercise)
- Chest pain or pressure (occurs usually with exercise or physical activity, but can also occur with rest or after meals)
CAUSES
Restrictive cardiomyopathy is not usually inherited and its cause is often unknown. Known causes of restrictive cardiomyopathy may include:
- Build-up of scar tissue (often for no known reason)
- Build-up of abnormal proteins (amyloidosis) in the heart muscle
- Chemotherapy or chest exposure to radiation
- Excess iron (hemochromatosis) in the heart
- Other systemic diseases (e.g. (sarcoidosis)
DIAGNOSIS
The size of the heart may remain normal with restrictive cardiomyopathy. In some cases, restrictive cardiomyopathy may be confused with constrictive pericarditis, a condition in which the layers of the pericardium (the sac that surrounds the heart) become thickened, calcified, and stiff.Restrictive cardiomyopathy is diagnosed based on medical history (your symptoms and family history), physical exam, and tests: such as blood tests, electrocardiogram, chest X-ray, echocardiogram, exercise stress test, cardiac catheterization, CT scan, and MRI.A myocardial biopsy, or biopsy of the heart muscle, occasionally is done to determine the cause of cardiomyopathy. During a myocardial biopsy, a small tissue sample is taken from the heart and examined under a microscope to examine the cause of the symptoms.
TREATMENT
Treatment of restrictive cardiomyopathy is difficult. Treatment is usually focused on treating the cause of this condition. Doctors recommend lifestyle changes and medications to treat heart failure.
EXTRA INFORMATION
Restrictive cardiomyopathy tends to affect older adults. The heart's ventricles become rigid because abnormal tissue, such as scar tissue, replaces the normal heart muscle.
ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATY (ARVC)
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DEFINITION
ARVC is an inherited condition, which means that it's passed on through families. It is caused by a change or mutation in one or more genes. The chances of inheriting ARVC vary, and you might inherit the mutation but not develop the condition.
SYMPTOMS
ARVC has four phases. Not everyone will develop all of the phases or be affected in the same way. Many people with the condition never develop any serious problems.
1. Concealed phase
There are small changes in the right ventricle.
2. Overt phase
There are noticeable structural changes to your heart muscle. These changes affect the pumping action of the heart and cause abnormal heart rhythms.
3. Weakening of the right ventricle
Your right ventricle becomes stretched and its pumping action becomes weaker.
4. Weakening of the left ventricle
Your left ventricle is also affected. The pumping action of both your ventricles becomes weaker.
If you have ARVC, you may have one or more of the following symptoms:
- Palpitations
- Light-headedness
- Fainting
- Breathlessness
- Abnormal heart rhythms
- Swollen ankles or legs
- Swelling in the abdomen
- Risk of sudden death on exertion
The cause of ARVD is unknown. It occurs in about 1 in 5,000 people. ARVD can occur with no family history, although often it runs in families. A family history of ARVD is present in at least 30 to 50 percent of cases. Therefore, it is recommended that all first- and second-degree family members (parents, siblings, children, grandchildren, uncle, aunt, nephew, niece) be evaluated carefully for this form of cardiomyopathy, even in the absence of symptoms.
Researchers have found two patterns of inheritance for ARVD:
- Autosomal dominant - When one parent has ARVD. Studies show that in these families, the family members have a 50 percent chance of inheriting the condition, although the symptoms and age of onset may be different between family members. ARVD is more prevalent in some geographic locations, such as Italy.
- Autosomal recessive (one form is called Naxos disease) - Characterized by the symptoms of ARVD listed above. Naxos disease is associated with a thickening of the outer layer of skin on the palms of the hands and soles of the feet (hyperkeratosis) and thick, "wool-like" hair.
ARVD may also be related to non-genetic causes such as congenital abnormalities (affecting the right ventricle), viral or inflammatory myocarditis.
DIAGNONSIS
It can be difficult to diagnose ARVC, because the changes to the heart muscle are often very minor or build up in a 'patchy' pattern. Many of the changes and symptoms could also be caused by a number of other conditions, making diagnosis even more difficult. Your doctor may suspect that you have ARVC because of your symptoms, or because of the results of a chest x-ray. Or you may be going for tests because someone else in your family has been diagnosed with the condition. The most common tests for this cardiomyopathy are an ECG, which records the electrical activity of your heart, and an echocardiogram, which shows the pumping action of your heart. Your doctor may also arrange for you to have an exercise test, signal averaged electrocardiogram or MRI scan.
TREATMENT
There are three aims for treatment:
- To identify those at risk of sudden cardiac death
- To prevent or control arrhythmias (abnormal heart rhythms)
- To improve the pumping action of the heart muscle
EXTRA INFORMATION
In the 1980s and 90s, the same changes in the heart muscle were noticed at the post-mortem examinations of some young people who had died suddenly, especially in athletes. Researchers also noticed that there was a tendency for the condition to run in families.
BICUSPID AORTIC VALVE DISEASE
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DEFINITION
The aortic valve is a one-way valve between the heart and the aorta, the main artery from the heart that distributes oxygen-rich blood to the body. Normally, the aortic valve has three small flaps or leaflets that open widely and close securely to regulate blood flow, allowing blood to flow from the heart to the aorta and preventing blood from flowing backwards into the heart.
In bicuspid aortic valve disease (BAVD), the valve has only two leaflets. With this deformity, the valve doesn’t function perfectly, but it may function adequately for years without causing symptoms or obvious signs of a problem.
The latest studies suggest that bicuspid aortic valve disease is caused by a connective tissue disorder that also causes other circulatory system problems. People with bicuspid aortic valve disease also may have abnormal coronary arteries, aortic aneurysm or an abnormal thoracic aorta (the portion of the aorta that passes through the upper chest) and unstable (labile) high blood pressure.
SIGNS AND SYMPTOMS
Although bicuspid aortic valve disease is present at birth, it usually is not diagnosed until adulthood because the defective valve can function for years without causing symptoms. Rarely, the disease is so severe at birth that the baby develops congestive heart failure early in life. More commonly, patients will have a history of having a childhood murmur and symptoms develop in mid-life as the valve ages.
Calcium deposits on and around the leaflets eventually cause the valve to stiffen and narrow, a condition known as stenosis. As stenosis develops, the heart must pump increasingly harder to force the blood through the valve. Symptoms of a stenotic valve include chest pain, shortness of breath and dizziness or fainting caused by inadequate blood flow to the brain.
If the bicuspid valve does not close completely, blood can flow backwards into the heart. This is regurgitation, also called aortic valve insufficiency. The heart then must pump that same blood out again, causing strain on the heart’s lower left chamber, the left ventricle. Over time, the ventricle will dilate, or over-expand. The main symptom of aortic valve regurgitation is shortness of breath during exertion, like walking up stairs.As the disease progresses, these symptoms start occurring more frequently, even without exercise. When a physician listens to the person's heart, characteristic murmurs can be heard.
CAUSES
The actual cause of bicuspid aortic valve disease is not completely clear. We do know that the two-leaflet valve develops in the early stages of pregnancy, and the defect is present at birth. About 2% of the population has BAVD, and it is twice as common in males as in females.
DIAGNOSIS
Individuals experiencing symptoms such as shortness of breath typically begin with their family doctor, who will perform a physical examination that includes listening to the person's heart. When the patient’s symptoms and the sounds heard on the cardiac exam raise the suspicion of bicuspid aortic valve disease, the patient needs to be referred to a specialist in valvular heart disease.CT scans, and magnetic resonance imaging (MRI) of the heart to evaluate the heart, valves and aorta.
Other tests such as an electrocardiogram (to test the heart’s electrical activity) or coronary angiography (an X-ray of the heart's blood vessels using a special dye) can help diagnose other problems with the heart and arteries that may be associated with bicuspid aortic valve disease.
TREATMENT
In the most severe cases, when symptoms are present at birth or in early infancy, surgical repair of the valve must be performed immediately. In other cases, people can go their whole lives without knowing they have BAVD.
For the patient who has stenosis and symptoms, the preferred treatment is to replace the valve with a mechanical or biological valve. The mortality rate for this procedure at Cleveland Clinic is less than 1%.
EXTRA INFORMATION
Aortic stenosis mainly affects older people - the result of scarring and calcium buildup in the valve cusp (flap or fold). Age-related aortic stenosis usually begins after age 60, but often does not show symptoms until ages 70 or 80.