Rheumatic fever

Rheumatic fever is an inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever). The disease can affect the heart, joints, skin, and brain.

Causes

Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. It is not common in the United States, and usually occurs in isolated outbreaks. The latest outbreak was in the 1980s.

Rheumatic fever mainly affects children ages 6 -15, and occurs approximately 20 days after strep throat or scarlet fever.

Symptoms

• Abdominal pain
• Fever
• Heart (cardiac) problems, which may not have symptoms, or may result in shortness of breath and chest pain
• Joint pain, arthritis (mainly in the knees, elbows, ankles, and wrists)
• Joint swelling; redness or warmth
• Nosebleeds (epistaxis)
• Skin nodules
• Skin rash (erythema marginatum)
  • Skin eruption on the trunk and upper part of the arms or legs
  • Eruptions that look ring-shaped or snake-like
• Sydenham chorea (emotional instability, muscle weakness and quick, uncoordinated jerky movements that mainly affect the face, feet, and hands)

Exams and Tests

Because this disease has different forms, no one test can firmly diagnose it. Your doctor will perform a careful exam, which includes checking your heart sounds, skin, and joints.

Tests may include:

• Blood test for recurrent strep infection (such as an ASO test)
• Complete blood count
• Electrocardiogram
• Sedimentation rate (ESR)

Several major and minor criteria have been developed to help standardize rheumatic fever diagnosis. Meeting these criteria, as well as having evidence of a recent streptococcal infection, can help confirm that you have rheumatic fever.

The major criteria for diagnosis include:

• Arthritis in several joints (polyarthritis)
• Heart inflammation (carditis)
• Nodules under the skin (subcutaneous skin nodules)
• Rapid, jerky movements (chorea, Sydenham chorea)
• Skin rash (erythema marginatum)

The minor criteria include:

• Fever
• High ESR
• Joint pain
• Other laboratory findings

You'll likely be diagnosed with rheumatic fever if you meet two major criteria, or one major and two minor criteria, and have signs that you've had a previous strep infection.

Treatment of Rheumatic Fever

There is no actual cure for rheumatic fever but medication can be provided to alleviate the pain caused by some of the symptoms. Recurrent occurrence of the streptococcal infection can be prevented by administering penicillin to the patient. Aspirin is usually given to reduce fever and sometimes, corticosteroids are given to the patient to combat joint pain and swelling.

Administering low doses of antibiotics such as penicillin, erythromycin or sulfadiazine over a long-term period will help in preventing strep throat from occurring again.

Bed rest will be recommended for some patients in order to give the body some time to heal. Intake of fluids should be increased and the patient should have at least 6-8 glasses of water per day.

Complications of Rheumatic Fever

Depending on the severity of the initial attack of the disease, some patients may develop the following complications:

• Heart inflammation: inflammation of the muscle or lining of the heart
• Rheumatic Heart Disease: problems causes in the condition of the heart due to previous episode of rheumatic fever
• Sydenham Chorea: involuntary / spasmodic movements of the body
• Arrhythmias: heart rhythm disorder
• Endocarditis: deals with abnormalities caused to the endocardium (innermost tissues that lines the heart’s chambers) – inflammation of the heart valves or the lining of its chambers can be caused due to rheumatic fever
• Congestive heart failure: a very serious condition where the heart is unable to pump sufficient blood to the rest of the body
• Pericarditis: inflammation of the pericardium or the sac-like covering of the heart

Prevention of Rheumatic Fever

Physicians do not know how to reduce the pace of the damage caused to heart valves due to rheumatic fever. Therefore, it is best to nip this problem in the bud and promptly treat diseases caused due to streptococcal infections with the timely intake of antibiotics.

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Posted in: CVS,Rheumatic fever

Pericarditis - constrictive

Constrictive pericarditis is long-term (chronic) inflammation of the sac-like covering of the heart (the pericardium) with thickening, scarring, and muscle tightening (contracture).

See also:

• Bacterial pericarditis
• Pericarditis
• Pericarditis after heart attack

Causes

Constrictive pericarditis is most commonly caused by conditions or events that cause inflammation to develop around the heart, including:

• Heart surgery
• Radiation therapy to the chest
• Tuberculosis

Less common causes include:

• Abnormal fluid buildup in the covering of the heart due to bacterial or viral infection or surgical complications
• Mesothelioma

Constrictive pericarditis may also develop without apparent cause.

The inflammation causes the covering of the heart to become thick and rigid, making it hard for the heart to stretch properly when it beats. As a result, the heart chambers don't fill up with enough blood. Blood backs up behind the heart, causing heart swelling and other symptoms of heart failure.

The condition is relatively rare in children.

Symptoms

Symptoms of chronic constrictive pericarditis include:

• Difficulty breathing (dyspnea) that develops slowly and gets worse
• Fatigue, excessive tiredness
• Long-term swelling (edema) of the legs and ankles
• Swollen abdomen
• Weakness

Exams and Tests

Constrictive pericarditis is very difficult to diagnose. Signs and symptoms are similar to restrictive cardiomyopathy and cardiac tamponade. Your doctor will need to rule out these conditions when making a diagnosis.

A physical exam may show that your neck veins stick out, suggesting increased blood pressure in the area. This is called Kussmaul's sign. The doctor may note weak or distant heart sounds when listening to your chest with a stethoscope.

The physical exam may also reveal liver swelling and fluid in the belly area.

The following tests may be ordered:

• Chest MRI
• Chest CT scan
• Chest x-ray
• Coronary angiography or cardiac catheterization
• ECG
• Echocardiogram

Treatment

The goal of treatment is to improve heart function. The cause must be identified and treated. This may include antibiotics, antituberculosis medications, or other treatments.

Diuretics ("water pills") are commonly prescribed in small doses to help the body remove excess fluid. Analgesics may be needed to control pain.

Decreased activity may be recommended for some patients.

A low-sodium diet may also be recommended.

The definitive treatment is a type of surgery called a pericardiectomy. This involves cutting or removing the scarring and part of the sac-like covering of the heart.

Outlook (Prognosis)

Constrictive pericarditis may be life threatening if untreated.

However, surgery to treat the condition is associated with a relatively high complication rate and is usually reserved for patients who have severe symptoms.

Possible Complications

• Cardiac tamponade
• Damage to the coronary arteries
• Heart failure
• Pulmonary edema
• Scarring of the heart muscle

When to Contact a Medical Professional

Call your health care provider if you have symptoms of constrictive pericarditis.

Prevention

Constrictive pericarditis in some cases is not preventable.

However, conditions that can lead to constrictive pericarditis should be adequately treated.

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Posted in: CVS,Pericarditis - constrictive

Pericardial effusion

Definition

Pericardial effusion (per-ih-KAHR-de-ul uh-FU-zhun) is the accumulation of excess fluid around the heart.

The heart is surrounded by a double-layered, sac-like structure called the pericardium. The space between the layers normally contains a very small amount of fluid.

Pericardial effusion is often related to inflammation of the pericardium that's caused by disease or injury, but pericardial effusion can also occur without inflammation. Sometimes, pericardial effusion can be caused by the accumulation of blood after a surgical procedure or injury.

When the volume of fluid exceeds the pericardium's "full" level, pericardial effusion puts pressure on the heart, causing poor heart function. If left untreated, pericardial effusion can cause heart failure or death.

What causes pericardial effusion?

Pericardial effusion, and the possible inflammation of the pericardium resulting from it (called pericarditis), can have many possible causes, including:

• Infection such as viral, bacterial or tuberculous
• Inflammatory disorders, such as lupus
• Cancer that has spread (metastasized) to the pericardium
• Kidney failure with excessive blood levels of nitrogen
• Heart surgery

Who is affected by pericardial effusions?

Since pericardial effusions are a result of many different diseases or conditions, anyone who develops one of the many conditions that can produce an effusion may be affected.

Is pericardial effusion serious?

The seriousness of the condition depends on the primary cause and size of the effusion and whether it can be treated effectively. Causes that can be treated or controlled, such as an infection due to a virus or heart failure, allows the patient to be effectively treated and remain free of pericardial effusions.

Pericardial effusion caused by other conditions, such as cancer, is very serious and should be diagnosed and treated promptly.

Additionally, rapid fluid accumulation in the pericardium can cause cardiac tamponade, a severe compression of the heart that impairs its ability to function. Cardiac tamponade resulting from pericardial effusion can be life-threatening.

What are the symptoms of pericardial effusion?

Many patients with pericardial effusion have no symptoms. The condition is often discovered on a chest x-ray or echocardiogram that was performed for another reason. Initially, the pericardium may stretch to accommodate excess fluid build-up. Therefore, signs and symptoms may not occur until a large amount of fluid has collected over time.

If symptoms do occur, they may result from compression of surrounding structures, such as the lung, stomach or phrenic nerve (a nerve that connects to the diaphragm). Symptoms also may occur due to diastolic heart failure (heart failure that occurs because the heart is unable to relax normally between each contraction due to the added compression).

Symptoms of pericardial effusion include:

• Chest pressure or pain
• Shortness of breath
• Nausea
• Abdominal fullness
• Difficulty in swallowing

Symptoms that pericardial effusion is causing cardiac tamponade include:

• Blue tinge to the lips and skin
• Shock
• Change in mental status

Cardiac tamponade is a severe compression of the heart that impairs its ability to function. Cardiac tamponade resulting from pericardial effusion can be life-threatening and is a medical emergency, requiring urgent drainage of the fluid.

How is pericardial effusion diagnosed?

The tests most commonly used to diagnose and evaluate pericardial effusion include:

• Chest x-ray
• Computed tomography (CT) scan of the chest
• Echocardiogram
• Pericardiocentesis: a procedure that uses a needle to remove fluid from the pericardium; the fluid is then examined to determine the cause of the effusion

How is pericardial effusion treated?

Treatment of pericardial effusion is based on the underlying condition that is causing it and if the effusion is leading to severe symptoms, such as shortness of breath or difficulty breathing.

Depending on the cause, the excess fluid may be either rich in protein (exudate) or watery (transudate). These two categories help physicians determine the best way to treat the cause of a pericardial effusion.

Medical management

The goal of medical management for pericardial effusions is to treat the underlying cause. Medical therapies for pericardial effusions include:

• Nonsteroidal antiinflammatory medications (NSAIDs) can be used to treat pericardial effusions caused by inflammation.
• Diuretics and other heart failure medications can be used to treat pericardial effusions caused by heart failure.
• Antibiotics are used to treat pericardial effusions caused by an infection.
• If a pericardial effusion is related to the presence of cancer, treatment may include chemotherapy, radiation therapy, or medication infused within the chest.

Procedures to treat pericardial effusion

Regardless if the pericardial effusion is transudative (consisting of watery fluid) or exudative (made up of protein-rich fluid), a large pericardial effusion causing respiratory symptoms or cardiac tamponade should be drained to remove the excess fluid, prevent its re-accumulation, or treat the underlying cause of the fluid buildup.

Large pericardial effusions may be drained through:

• Ultrasound-guided pericardiocentesis, a safe and effective procedure to remove excess fluid from the pericardium.
• Video-assisted thoracoscopic surgery (VATS), also known as thoracoscopy is a minimally-invasive technique performed under general anesthesia. VATS allows for visual evaluation of the pericardium and is used when the diagnosis of pericardial effusion has remained undiagnosed despite previous, less-invasive tests. It is also used to drain the excess fluid and prevent its reaccumulation.

Pericardial effusions that cannot be managed through medical management or drainage of excess fluid may require percutaneous (nonsurgical) or surgical treatment.

Percutaneous Balloon Pericardiotomy is a nonsurgical procedure performed using x-ray guidance to view the pericardium and place a balloon dilating catheter. Percutaneous balloon pericardiotomy is 85 to 92 percent successful at relieving reaccumulation of pericardial fluid 30 days following the procedure.

Pericardial Window (Subxyphoid Pericardiostomy) is a minimally invasive procedure in which an opening is made in the pericardium to drain fluid that has accumulated around the heart. A pericardial window can be completed through a small incision below t

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Posted in: CVS,Pericardial effusion

Dextrocardia

Dextrocardia is a condition in which the heart is pointed toward the right side of the chest instead of normally pointing to the left. It is present at birth (congenital).

Causes

During the early weeks of pregnancy, the baby's heart develops. Sometimes, for reasons that are unclear, the heart develops and turns so that it points to the right side of the chest instead of the left side.

There are several types of dextrocardia. Most involve other defects of the heart and abdomen area.

The simplest type of dextrocardia is one in which the heart is a mirror image of the normal heart, and no other problems exist. This condition is rare. Usually in this case, the organs of the abdomen and the lungs will also be arranged in a mirror image of their normal position. For example, the liver will be on the left side instead of the right.

Some people with mirror-image dextrocardia have a problem with the fine hairs (cilia) that filter the air going into their nose and air passages. This condition is called Kartagener syndrome.

In the more common types of dextrocardia, heart defects are present in addition to the abnormal location of the heart. The most common heart defects seen with dextrocardia include:

• Double outlet right ventricle
• Endocardial cushion defect
• Pulmonary stenosis or atresia
• Single ventricle
• Transposition of the great vessels
• Ventricular septal defect

The abdominal and chest organs in babies with dextrocardia may be abnormal and may not work correctly. A very serious syndrome that appears with dextrocardia is called heterotaxy. Heterotaxy means the organs (atria of the heart and abdominal organs) are not in their usual places.

In heterotaxy, the spleen may be completely missing. Because the spleen is an extremely important part of the immune system, babies born without a spleen are in danger of severe bacterial infections and death. In another form of heterotaxy several small spleens exist, but may not work correctly.

Heterotaxy may also include:

• Abnormal gallbladder system
• Problems with the lungs
• Problems with the structure of the intestines
• Severe heart defects

Possible risk factors for dextrocardia include:

• Family history of the condition
• Mother with diabetes (may play a role in some forms of dextrocardia)

Symptoms

There are no symptoms of dextrocardia if the heart is normal.

Conditions that may include dextrocardia may cause the following symptoms:

• Bluish skin
• Difficulty breathing
• Failure to grow and gain weight
• Fatigue
• Jaundice (yellow skin and eyes)
• Pale skin (pallor)
• Repeated sinus or lung infections

Exams and Tests

There are no signs of dextrocardia if the heart is normal.

Conditions that can include dextrocardia may cause the following signs:

• Abnormal arrangement and structure of the organs in the abdomen
• Enlarged heart
• Problems with the structure of the chest and lungs, seen on x-rays
• Rapid breathing or problems breathing
• Rapid pulse

Tests to diagnose dextrocardia include:

• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI) of the heart
• Ultrasound of the heart (echocardiogram)
• X-rays

Treatment

A complete mirror image dextrocardia with no heart defects requires no treatment. It is important, however, to let the child's health care provider know the heart is on the right side of the chest. This information can be important in some exams and tests.

Treatment for conditions that include dextrocardia depends on whether the infant has other heart or physical problems in addition to dextrocardia.

If heart defects are present with dextrocardia, the baby will most likely need surgery. Critically ill babies may need treatment with medication before surgery. These medications help the baby grow larger so surgery is less difficult to perform.

Medications include:

• "Water pills" (diuretics)
• Medications that help the heart muscle pump more forcefully (inotropic agents)
• Medications that lower blood pressure and ease the workload on the heart (ACE inhibitors)

The baby might also need surgery to correct problems in the organs of the abdomen.

Children with Kartagener syndrome will need repeated treatment with antibiotics for sinus infections.

Children with a missing or abnormal spleen need long-term antibiotics.

All children with heart defects should get antibiotics before surgeries or dental treatments.

See also:

• Congenital heart defect corrective surgery
• Pediatric heart surgery

Outlook (Prognosis)

Babies with simple dextrocardia have a normal life expectancy and should have no problems related to the location of the heart.

When dextrocardia appears with other defects in the heart and elsewhere in the body, how well the baby does depends on the severity of the problems.

The death rate in babies and children without a spleen may be high due to infections. This is at least partially preventable with daily antibiotics.

Possible Complications

Complications depend on whether dextrocardia is part of a larger syndrome, and whether other problems exist in the body. Complications include:

• Bacteria in the blood (septic shock)
• Blocked intestines (due to a condition called intestinal malrotation)
• Congestive heart failure
• Death
• Infection (heterotaxy with no spleen)
• Infertility in males (Kartagener syndrome)
• Repeated pneumonias
• Repeated sinus infections (Kartagener syndrome)

When to Contact a Medical Professional

Call your health care provider if your baby:

• Is often ill
• Does not seem to gain weight
• Tires easily

Seek emergency care if your baby has:

• A bluish tinge to the skin
• Trouble breathing
• Yellow skin (jaundice)

Prevention

Some syndromes that include dextrocardia may run in families. If you have a family history of heterotaxy, talk to your health care provider before becoming pregnant.

While there are no known ways to prevent dextrocardia, avoiding the use of illegal drugs (especially cocaine) before and during pregnancy may lower the risk of this problem.

Talk to your health care provider if you have diabetes, because it may contribute to your risk of having a child with certain forms of dextrocardia.

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Posted in: CVS,Dextrocardia

Marfan syndrome

What is Marfan syndrome?

Marfan syndrome is a heritable condition that affects the connective tissue. The primary purpose of connective tissue is to hold the body together and provide a framework for growth and development. In Marfan syndrome, the connective tissue is defective and does not act as it should. Because connective tissue is found throughout the body, Marfan syndrome can affect many body systems, including the skeleton, eyes, heart and blood vessels, nervous system, skin, and lungs.

Marfan syndrome affects men, women, and children, and has been found among people of all races and ethnic backgrounds. It is estimated that at least 1 in 5,000 people in the United States have the disorder.

What are the symptoms of Marfan syndrome?

Marfan syndrome affects different people in different ways. Some people have only mild symptoms, while others are more severely affected. In most cases, the symptoms progress as the person ages. The body systems most often affected by Marfan syndrome are:

• Skeleton - People with Marfan syndrome are typically very tall, slender, and loose-jointed. Because Marfan syndrome affects the long bones of the skeleton, a person's arms, legs, fingers, and toes may be disproportionately long in relation to the rest of the body. A person with Marfan syndrome often has a long, narrow face, and the roof of the mouth may be arched, causing the teeth to be crowded. Other skeletal problems include a sternum (breastbone) that is either protruding or indented, curvature of the spine (scoliosis), and flat feet.
  
  
• Eyes - More than half of all people with Marfan syndrome experience dislocation of one or both lenses of the eye. The lens may be slightly higher or lower than normal, and may be shifted off to one side. The dislocation may be minimal, or it may be pronounced and obvious. One serious complication that may occur with this disorder is retinal detachment. Many people with Marfan syndrome are also nearsighted (myopic), and some can develop early glaucoma (high pressure within the eye) or cataracts (the eye's lens loses its clearness).
  
  
• Heart and blood vessels (cardiovascular system) - Most people with Marfan syndrome have problems associated with the heart and blood vessels. Because of faulty connective tissue, the wall of the aorta (the large artery that carries blood from the heart to the rest of the body) may be weakened and stretch, a process called aortic dilatation. Aortic dilatation increases the risk that the aorta will tear (aortic dissection) or rupture, causing serious heart problems or sometimes sudden death. Sometimes, defects in heart valves can also cause problems. In some cases, certain valves may leak, creating a "heart murmur," which a doctor can hear with a stethoscope. Small leaks may not result in any symptoms, but larger ones may cause shortness of breath, fatigue, and palpitations (a very fast or irregular heart rate).
  
  
• Nervous system - The brain and spinal cord are surrounded by fluid contained by a membrane called the dura, which is composed of connective tissue. As someone with Marfan syndrome gets older, the dura often weakens and stretches, then begins to weigh on the vertebrae in the lower spine and wear away the bone surrounding the spinal cord. This is called dural ectasia. These changes may cause only mild discomfort; or they may lead to radiated pain in the abdomen; or to pain, numbness, or weakness in the legs.
  
  
• Skin - Many people with Marfan syndrome develop stretch marks on their skin, even without any weight change. These stretch marks can occur at any age and pose no health risk. However, people with Marfan syndrome are also at increased risk for developing an abdominal or inguinal hernia, in which a bulge develops that contains part of the intestines.
  
  
• Lungs - Although connective tissue problems make the tiny air sacs within the lungs less elastic, people with Marfan syndrome generally do not experience noticeable problems with their lungs. If, however, these tiny air sacs become stretched or swollen, the risk of lung collapse may increase. Rarely, people with Marfan syndrome may have sleep-related breathing disorders such as snoring, or sleep apnea (which is characterized by brief periods when breathing stops).

What causes Marfan syndrome?

Marfan syndrome is caused by a defect, or mutation, in the gene that determines the structure of fibrillin-1, a protein that is an important part of connective tissue. A person with Marfan syndrome is born with the disorder, even though it may not be diagnosed until later in life.

The defective gene that causes Marfan syndrome can be inherited: The child of a person who has Marfan syndrome has a 50 percent chance of inheriting the disease. Sometimes a new gene defect occurs during the formation of sperm or egg cells, making it possible for two parents without the disease to have a child with the disease. But this is rare. Two unaffected parents have only a 1 in 10,000 chance of having a child with Marfan syndrome. Possibly 25 percent of cases are due to a spontaneous mutation at the time of conception.

Although everyone with Marfan syndrome has a defect in the same gene, different mutations are found in different families, and not everyone experiences the same characteristics to the same degree. In other words, the defective gene expresses itself in different ways in different people. This phenomena is known as variable expression. Scientists do not yet understand why variable expression occurs in people with Marfan syndrome.

How Marfan syndrome diagnosed?

There is no specific laboratory test, such as a blood test or skin biopsy, to diagnose Marfan syndrome. The doctor and/or geneticist (a doctor with special knowledge about inherited diseases) relies on observation and a complete medical history, including:

• information about any family members who may have the disorder or who had an early, unexplained, heart-related death
  
  
• a thorough physical examination, including an evaluation of the skeletal frame for the ratio of arm/leg size to trunk size
  
  
• an eye examination, including a "slit lamp" evaluation
  
  
• heart tests such as an echocardiogram (a test that uses ultrasound waves to examine the heart and aorta).

The doctor may diagnose Marfan syndrome if the patient has a family history of the disease, and if there are specific problems in at least two of the body systems known to be affected. For a patient with no family history of the disease, at least three body systems must be affected before a diagnosis is made. Moreover, two of the systems must show clear signs that are relatively specific for Marfan syndrome.

In some cases, a genetic analysis may be useful in making a diagnosis of Marfan syndrome, but such analyses are often time consuming and may not provide any additional helpful information. Family members of a person diagnosed with Marfan syndrome should not assume they are not affected if there is no knowledge that the disorder existed in previous generations of the family. After a clinical diagnosis of a family member, a genetic study might identify the specific mutation for which a test can be performed to determine if other family members are affected.

Recently, doctors discovered a connective tissue disorder known as Loeys-Dietz syndrome, which has several characteristics that overlap with those of Marfan syndrome. When making a diagnosis, it is important to distinguish between the two disorders: Loeys-Dietz is more likely to cause fatal aortic aneurysms, and treatment for the two is different. A diagnostic test for Loeys-Dietz syndrome is available.

Treatment

Vision problems should be treated when possible.

Take care to monitor for scoliosis, especially during adolescence.

Medicine to slow the heart rate may help prevent stress on the aorta. Avoid participating in competitive athletics and contact sports to avoid injuring the heart. Some people may need surgical replacement of the aortic root and valve.

People with Marfan syndrome should take antibiotics before dental procedures to prevent endocarditis. Pregnant women with Marfan syndrome must be monitored very closely because of the increased stress on the heart and aorta.

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Posted in: CVS,Marfan syndrome

Coarctation of the aorta

Definition

Coarctation (ko-ahrk-TAY-shun) of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrow part of your aorta.

Coarctation of the aorta is generally present at birth (congenital). Coarctation of the aorta may range from mild to severe, and may not be detected until adulthood, depending on how narrowed the aorta is.

Coarctation of the aorta often occurs along with other heart defects. While treatment for coarctation of the aorta is usually successful, it's a condition that requires careful follow-up through infancy and throughout adulthood.

Symptoms

The signs and symptoms of coarctation of the aorta depend on its seriousness. Children with serious aortic narrowing tend to show signs and symptoms earlier in life, while mild cases may not be diagnosed until adulthood.

Babies with severe coarctation of the aorta usually begin having signs and symptoms shortly after birth. These include:

• Pale skin
• Irritability
• Heavy sweating
• Difficulty breathing

Left untreated, aortic coarctation in babies may lead to heart failure and death.

Older children and adults with the condition often don't have symptoms, because they tend to have less severe narrowing of the aorta. If signs or symptoms appear, the most common sign is high blood pressure (hypertension) measured in the arm. Signs and symptoms may include:

• High blood pressure
• Shortness of breath, especially during exercise
• Headache
• Muscle weakness
• Leg cramps or cold feet
• Nosebleeds

When to see a doctor
Seek medical help if you or your child has the following signs or symptoms:

• Severe chest pain
• Fainting
• Sudden shortness of breath
• Unexplained high blood pressure

While experiencing these signs or symptoms doesn't necessarily mean that you have a serious problem, it's best to get checked out quickly. Early detection and treatment may help save your life.

Risk factors

Coarctation of the aorta often occurs along with other congenital heart defects, although doctors don't know what causes multiple heart defects to form together. If you or your child has any of the following heart conditions, you or your child is more likely to have aortic coarctation:

• Bicuspid aortic valve. The aortic valve separates the lower left chamber (left ventricle) of the heart from the aorta. A bicuspid aortic valve has two leaflets instead of the usual three.
• Ventricular septal defect. In this condition, there's a hole in the wall that separates the two lower chambers (ventricles) of the heart. Oxygen-poor blood from the right ventricle mixes with oxygen-rich blood from the left ventricle, which pumps blood out to the body.
• Patent ductus arteriosus. While a baby is still in the womb, the ductus arteriosus is a blood vessel connecting the left pulmonary artery to the aorta, allowing blood to bypass the lungs. Shortly after birth, the ductus arteriosus usually closes. If it remains open, it's called a patent ductus arteriosus.
• Aortic valve stenosis. This is a narrowing of the valve that separates the left ventricle of the heart from the aorta. This means your heart has to pump harder to get adequate blood flow to your body. Over time, this can thicken the muscle of your heart and lead to heart failure.
• Mitral valve stenosis. This is a narrowing of the valve that lets blood flow through the left side of your heart. This means blood may back up into your lungs, causing shortness of breath or lung congestion. Like aortic valve stenosis, this condition can also lead to heart failure.
• cause:
  

Doctors aren't certain what causes aortic coarctation in most cases. For unknown reasons, mild to severe narrowing develops in part of the aorta. Although aortic coarctation can occur anywhere along the aorta, the coarctation is most often located near a blood vessel called the ductus arteriosus. The condition generally begins before birth.

Rarely, coarctation of the aorta may develop later in life. Severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu's arteritis) may narrow your aorta, leading to aortic coarctation. In rare cases, trauma may lead to coarctation of the aorta.

Coarctation of the aorta usually occurs beyond the blood vessels that branch off to your upper body and before the blood vessels that lead to your lower body. This often means you'll have high blood pressure in your arms, but low blood pressure in your legs and ankles.

How is coarctation of the aorta diagnosed?

Your child's physician may have heard a heart murmur during a physical exam and referred your child to a pediatric cardiologist for testing. A heart murmur is simply a noise caused by the turbulence of blood flowing through a narrow region. Symptoms your child exhibits also will help with the diagnosis.

• A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood.
• The cardiologist will perform a physical exam, listen to your child's heart and lungs, and make other observations that help in the diagnosis.

The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur will give the cardiologist an initial idea of which heart problem your child may have. Other tests are needed to help with the diagnosis, and may include:

• chest x-ray
• echocardiogram (echo)
• electrocardiogram (ECG or EKG)
• magnetic resonance imaging (MRI)

Treatment & Care

Coarctation of the aorta is treated with repair of the narrowed vessel. This may be done through a cardiac catheterization procedure or through surgery in an operating room.

Very sick infants who require care in our intensive care unit (ICU), may need emergency repair of the coarctation. Others with few symptoms may have the repair scheduled on a less urgent basis.

• Cardiac catheterization
  • During the procedure, your child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart.
  • Once the catheter is in the heart, the cardiologist will pass an inflated balloon through the narrowed section of the aorta to stretch the area open.
  • A small device, called a stent, may also be placed in the narrowed area after the balloon dilation to keep the aorta open.
• Surgery
  • The surgical repair is done through an incision on the left side of the chest below your child’s armpit.
  • The ribs are spread, the lung moved aside, and the aorta exposed near the heart.
  • The aorta is clamped on either side of the narrowing and the narrow segment is cut out.
  • The two ends of aorta are then sewn together, clamps are removed and flow through the aorta is reestablished.

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Posted in: Coarctation of the aorta,CVS

Takayasu disease

What is Takayasu disease?

Takayasu disease or Takayasu arteritis is a chronic inflammation of the large blood vessel that distributes blood from the heart, called the aorta. It is most common in women (90%) of Asian descent. It usually begins between 10 and 30 years of age. The cause is unknown.

Takayasu disease has also been referred to as pulseless disease and aortic arch syndrome.

What are symptoms of Takayasu disease?

The inflammation of the aorta and its branch arteries can lead to poor blood supply to tissues of the body in patients with Takayasu disease. This can cause painful, cool or blanched extremities, dizziness, headaches, chest pain, and abdominal pain. Other early symptoms can include fatigue, weight loss, and low-grade fever.

How is Takayasu disease diagnosed?

The doctor can find elevated blood pressure in over half of the patients with Takayasu disease. Abnormal sounds of blood movement through blood vessels can sometimes be heard with a stethoscope. Small notches in the ribs of the back of the chest have sometimes been detected on routine chest x-rays and are felt to be a result of abnormal pulsations of blood vessels in these areas.

The diagnosis is supported by the blood tests, such as a sedimentation rate (sed rate), that suggest inflammation in the body. In fact, elevated blood pressure with an elevated sedimentation rate is distinctly uncommon in children and very helpful in suggesting Takayasu disease as a possible cause. Anemia (low red blood cell count) is frequent.

Takayasu arteritis is ultimately diagnosed with an angiogram of the arteries (arteriogram) whereby a contrast material is injected into the blood vessels which makes them visible by x-ray. With the arteriogram, the doctor can visualize the abnormally narrowed and constricted arteries. Studies have shown successful diagnosis of Takayasu disease using magnetic resonance angiography (MRA). MRA, the combination of an MRI scan with angiogram, could be used as a noninvasive method of diagnosing and monitoring patients with Takayasu disease.

How is Takayasu disease treated?

Takayasu disease is treated by suppressing the inflammation with cortisone medication. While most patients can improve, some do not or relapse. In cortisone-resistant patients, stronger medications which suppress the immune system (immunosuppressive drugs), thereby further decreasing active inflammation of the arteries, have been used. Examples include prednisone, prednisolone, methotrexate (Rheumatrex, Trexall), cyclosporine, cyclophosphamide (Cytoxan), and azathioprine (Imuran). Strict control of elevated blood pressure (hypertension) is important.

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Posted in: CVS,Takayasu disease

Atrial Fibrillation

Atrial Fibrillation

What is atrial fibrillation (AF)?

Atrial fibrillation is a disorder found in about 2.2 million Americans. During atrial fibrillation, the heart's two small upper chambers (the atria) quiver instead of beating effectively. Blood isn't pumped completely out of them, so it may pool and clot. If a piece of a blood clot in the atria leaves the heart and becomes lodged in an artery in the brain, a stroke results. About 15 percent of strokes occur in people with atrial fibrillation.

The likelihood of developing atrial fibrillation increases with age. Three to five percent of people over 65 have atrial fibrillation.

How is atrial fibrillation treated?

Several approaches are used to treat and prevent abnormal beating:

• Medications are used to slow down rapid heart rate associated with AF. These treatments may include drugs such as digoxin, beta blockers (atenolol, metoprolol, propranolol), amiodarone, disopyramide, calcium antagonists (verapamil, diltiazam), sotalol, flecainide, procainamide, quinidine, propafenone, etc.
• Electrical cardioversion may be used to restore normal heart rhythm with an electric shock, when medication doesn't improve symptoms.
• Drugs (such as ibutilide) can sometimes restore the heart's normal rhythm. These drugs are given under medical supervision, and are delivered through an IV tube into a vein, usually in the patient's arm.
• Radiofrequency ablation may be effective in some patients when medications don't work. In this procedure, thin and flexible tubes are introduced through a blood vessel and directed to the heart muscle. Then a burst of radiofrequency energy is delivered to destroy tissue that triggers abnormal electrical signals or to block abnormal electrical pathways.
• Surgery can be used to disrupt electrical pathways that generate AF.
• Atrial pacemakers can be implanted under the skin to regulate the heart rhythm.

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Posted in: Atrial Fibrillation,CVS

Atrial Fibrillation

Atrial Fibrillation

What is atrial fibrillation (AF)?

Atrial fibrillation is a disorder found in about 2.2 million Americans. During atrial fibrillation, the heart's two small upper chambers (the atria) quiver instead of beating effectively. Blood isn't pumped completely out of them, so it may pool and clot. If a piece of a blood clot in the atria leaves the heart and becomes lodged in an artery in the brain, a stroke results. About 15 percent of strokes occur in people with atrial fibrillation.

The likelihood of developing atrial fibrillation increases with age. Three to five percent of people over 65 have atrial fibrillation.

How is atrial fibrillation treated?

Several approaches are used to treat and prevent abnormal beating:

• Medications are used to slow down rapid heart rate associated with AF. These treatments may include drugs such as digoxin, beta blockers (atenolol, metoprolol, propranolol), amiodarone, disopyramide, calcium antagonists (verapamil, diltiazam), sotalol, flecainide, procainamide, quinidine, propafenone, etc.
• Electrical cardioversion may be used to restore normal heart rhythm with an electric shock, when medication doesn't improve symptoms.
• Drugs (such as ibutilide) can sometimes restore the heart's normal rhythm. These drugs are given under medical supervision, and are delivered through an IV tube into a vein, usually in the patient's arm.
• Radiofrequency ablation may be effective in some patients when medications don't work. In this procedure, thin and flexible tubes are introduced through a blood vessel and directed to the heart muscle. Then a burst of radiofrequency energy is delivered to destroy tissue that triggers abnormal electrical signals or to block abnormal electrical pathways.
• Surgery can be used to disrupt electrical pathways that generate AF.
• Atrial pacemakers can be implanted under the skin to regulate the heart rhythm.

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Posted in: Atrial Fibrillation,CVS

Atrial septal defect

Atrial septal defect (ASD) is a congenital heart defect in which the wall that separates the upper heart chambers (atria) does not close completely. Congenital means the defect is present at birth.

Causes, incidence, and risk factors

In fetal circulation, there is normally an opening between the two atria (the upper chambers of the heart) to allow blood to bypass the lungs. This opening usually closes around the time the baby is born.

If the ASD is persistent, blood continues to flow from the left to the right atria. This is called a shunt. If too much blood moves to the right side of the heart, pressures in the lungs build up. The shunt can be reversed so that blood flows from right to left. Small atrial septal defects often cause very few problems and may be found much later in life. Many problems can occur if the shunt is large, however. In advanced and severe cases with large shunts the increased pressure on the right side of the heart would result in reversal of blood flow (now from right to left). This usually results in significant shortness of breath.

ASD is not very common. When the person has no other congenital defect, symptoms may be absent, particularly in children. Symptoms may begin any time after birth through childhood. Individuals with ASD are at an increased risk for developing a number of complications including:

• Atrial fibrillation (in adults)

• Heart failure

• Pulmonary overcirculation

• Pulmonary hypertension

• Stroke

Symptoms

Small to moderate sized defects may produce no symptoms, or not until middle age or later. Symptoms that may occur can include:

• Difficulty breathing (dyspnea)

• Frequent respiratory infections in children

• Sensation of feeling the heart beat (palpitations) in adults

• Shortness of breath with activity

Signs and tests

The doctor may hear abnormal heart sounds when listening to the chest with a stethoscope. A murmur may be heard only in certain body positions, and sometimes a murmur may not be heard at all. The physical exam may also reveal signs of heart failure in some adults.

If the shunt is large, increased blood flow across the tricuspid valve may create an additional murmur when the heart relaxes between beats.

Tests that may done include:

• Cardiac catheterization

• Chest x-ray

• Coronary angiography (for patients over 35 years old)

• Doppler study of the heart

• ECG

• Echocardiography

• Heart MRI

• Transesophageal echocardiography (TEE)

Treatments and drugs

If your child has an atrial septal defect, your doctor may recommend monitoring it for a period of time to see if it closes on its own, while treating any symptoms with medications. Many atrial septal defects close on their own during childhood. For those that don't close, some small atrial septal defects don't cause any problems and may not require any intervention. But, many atrial septal defects eventually require surgery to be corrected.

If your child needs treatment, the timing of it depends on your child's condition and whether your child has any other congenital heart defects.

Medications
Medications won't repair the hole, but they may be used to reduce some of the signs and symptoms that can accompany an atrial septal defect. Drugs may also be used to reduce the risk of complications after surgery. Medications may include those to:

• Keep the heartbeat regular. Examples include beta blockers (Lopressor, Inderal) and digoxin (Lanoxin).
• Reduce the risk of blood clots. Anticoagulants, often called blood thinners, can help reduce the chances of developing a blood clot and having a stroke. Anticoagulants include warfarin (Coumadin) and anti-platelet agents, such as aspirin.

Surgery
Many doctors recommend repairing an atrial septal defect diagnosed during childhood to prevent complications as an adult. For adults and children, surgery involves plugging or patching the abnormal opening between the atria. Doctors can do this through two methods:

• Cardiac catheterization. A thin tube (catheter) is inserted into a blood vessel in the groin and guided to the heart. Through the catheter, a mesh patch or plug is put into place to close the hole. The heart tissue grows around the mesh, permanently sealing the hole.
• Open-heart surgery. This type of surgery is done under general anesthesia and requires the use of a heart-lung machine. Through an incision in the chest, surgeons use patches or stitches to close the hole.

Follow-up care depends on the type of defect and whether other defects are present. For simple atrial septal defects closed during childhood, only occasional follow-up care is needed. For adults, follow-up care may depend on any resulting complications.

Lifestyle and home remedies

If you find out you have a congenital heart defect, or you've had surgery to correct one, you may wonder about limitations on activities and other issues.

• Exercise. Having an atrial septal defect usually doesn't restrict you from activities or exercise. If you have complications, such as heart failure or pulmonary hypertension, you may not be able to do some activities or exercises. Your cardiologist can help you learn what is safe.
• Preventing infection. Some heart defects, and the repair of defects, create changes to the surface of the heart in which bacteria can become stuck and grow into an infection (infective endocarditis). Atrial septal defects generally aren't associated with infective endocarditis. But if you have other heart defects in addition to an atrial septal defect, or if you've recently had atrial septal defect repair, you may need to take antibiotics before certain dental or surgical procedures.

Prevention

By Mayo Clinic staff In most cases, atrial septal defects can't be prevented. If you have a family history of heart defects or other genetic disorders, consider talking with a genetic counselor to assess what the risk might be before getting pregnant

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Posted in: Atrial septal defect,CVS

Ventricular septal defect

Ventricular septal defect describes one or more holes in the wall that separates the right and left ventricles of the heart. Ventricular septal defect is one of the most common congenital (present from birth) heart defects. It may occur by itself or with other congenital diseases.

Causes, incidence, and risk factors

Before a baby is born, the right and left ventricles of its heart are not separate. As the fetus grows, a wall forms to separate these two ventricles. If the wall does not completely form, a hole remains. This hole is known as a ventricular septal defect, or a VSD.

Ventricular septal defect is one of the most common congenital heart defects. The baby may have no symptoms, and the hole can eventually close as the wall continues to grow after birth. If the hole is large, too much blood will be pumped to the lungs, leading to heart failure.

The cause of VSD is not yet known. This defect often occurs along with other congenital heart defects.

In adults, ventricular septal defects are a rare but serious complication of heart attacks. These holes are related to heart attacks and do not result from a birth defect.

Symptoms

Patients with ventricular septal defects may not have symptoms. However, if the hole is large, the baby often has symptoms related to heart failure.

The most common symptoms include:

• Shortness of breath

• Fast breathing

• Hard breathing

• Paleness

• Failure to gain weight

• Fast heart rate

• Sweating while feeding

• Frequent respiratory infections

Signs and tests

Listening with a stethoscope usually reveals a heart murmur (the sound of the blood crossing the hole). The loudness of the murmur is related to the size of the defect and amount of blood crossing the defect.

Tests may include:

• Chest x-ray -- looks to see if there is a large heart with fluid in the lungs

• ECG -- shows signs of an enlarged left ventricle

• Echocardiogram -- used to make a definite diagnosis

• Cardiac catheterization (rarely needed, unless there are concerns of high blood pressure in the lungs)

• MRI of the heart -- used to find out how much blood is getting to the lungs

Treatment

If the defect is small, no treatment is usually needed. However, the baby should be closely monitored by a health care provider to make sure that the hole eventually closes properly and signs of heart failure do not occur.

Babies with a large VSD who have symptoms related to heart failure may need medicine to control the symptoms and surgery to close the hole. Medications may include digitalis (digoxin) and diuretics.

If symptoms continue despite medication, surgery to close the defect with a Gore-tex patch is needed. Some VSDs can be closed with a special device during a cardiac catheterization, although this is infrequently done.

Surgery for a VSD with no symptoms is controversial. This should be carefully discussed with your health care provider.

Expectations (prognosis)

Many small defects will close on their own. For those defects that do not spontaneously close, the outcome is good with surgical repair. Complications may result if a large defect is not treated.

Complications

• Heart failure

• Infective endocarditis (bacterial infection of the heart)

• Aortic insufficiency (leaking of the valve that separates the left ventricle from the aorta)

• Damage to the electrical conduction system of the heart during surgery (causing arrhythmias)

• Delayed growth and development (failure to thrive in infancy)

• Pulmonary hypertension (high blood pressure in the lungs) leading to failure of the right side of the heart

Calling your health care provider

Most often, this condition is diagnosed during routine examination of an infant. Call your infant's health care provider if the baby seems to be having difficulty breathing, or if the baby seems to have an unusual number of respiratory infections.

Prevention

Except for the case of heart-attack-associated VSD, this condition is always present at birth.

Drinking alcohol and using the antiseizure medicines depakote and dilantin during pregnancy have been associated with increased incidence of VSDs. Other than avoiding these things during pregnancy, there is no known way to prevent a VSD.

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Posted in: CVS,Ventricular septal defect

Tricuspid valve disease

For more information on valve disease and its treatment,
The tricuspid valve is located between the right atrium (top chamber) and right ventricle (bottom chamber). Its role is to make sure blood flows in a forward direction from the right atrium to the ventricle.
Tricuspid valve disease refers to abnormal function of the tricuspid valve. Two types of tricuspid disease include:

• Tricuspid regurgitation - the valve is leaky or doesn't close tight enough, causing blood to leak backwards across the valve
• Tricuspid stenosis - the valve leaflets are stiff and do not open widely enough, causing a restriction in the forward flow of blood. Your physician may refer to this as an increased pressure gradient across the valve, found by echocardiogram or cardiac catheterization.

Tricuspid valve disease can be caused by

Tricuspid Regurgitation

• Infection, such as rheumatic fever or infective endocarditis
• A dilated right ventricle, causing the annulus (a ring of tough fibrous tissue which is attached to and supports the leaflets of the valve) of the tricuspid valve to enlarge
• Increased pressure through the tricuspid valve (seen with pulmonary hypertension)
• Less common causes include congenital defects, trauma, carcinoid heart disease, tumor, tricuspid valve prolapse, Ebstein's anomaly, systemic lupus, and trauma.

Tricuspid valve disease, if caused by rheumatic fever, is often combined with mitral and/or aortic valve disease.

What are the symptoms?

Tricuspid valve disease may be tolerated for a long time without any symptoms. Symptoms may include:

Edwards MC ³ Annuloplasty System

• Irregular heart rhythm (atrial fibrillation)
• Easily tired (fatigue)
• A fluttering discomfort in the neck
• With severe disease, heart failure symptoms (right abdominal pain, shortness of breath, swelling in the legs or abdomen, cold skin)

How is Tricuspid Valve Disease diagnosed?

Tricuspid valve disease may first be diagnosed during a physical exam. The doctor will often hear a murmur (abnormal blood flow through the valve). Other signs your doctor may find are an irregular pulse and a fluttering or abnormal pulsation in your neck (jugular vein).
Tests used to diagnose valve disease may include:

• Electrocardiography (ECG)
• Chest X-ray
• Echocardiography
• Transesophageal echocardiography
• Cardiac Catheterization (cardiac cath or angiogram)
• Radionuclide scans
• Magnetic resonance imaging (MRI)

Rx

Medical Management

• Your doctor will want to monitor the progress of your valve disease with regular appointments. They may be spaced once a year or more often, if your doctor feels you need to be followed more closely.
  

  Lanyard and template handle assists with placement of ring
• Your appointment will include a medical exam. Diagnostic studies may be repeated at regular intervals.
• Your physician may prescribe medications to treat your symptoms. These medications may include drugs to treat heart failure or medications to control irregular heart rhythms.

Surgical Management:

Tricuspid valve repair

When valve disease is severe, it may be necessary to repair or replace the diseased valve. Tricuspid valve repair using an annuloplasty ring is the preferred surgical approach for tricuspid regurgitation and may be performed for primary tricuspid disease or for combined cases with other valve surgery (mitral, aortic). See illustrations to the right. For more information and to view actual surgery,
When the valve can not be repaired, a valve replacement will be performed. Find more information about valve surgery.

Endocarditis prevention

Anatomically correct design conforms to the 3-D tricuspid valve opening.

If you have tricuspid valve disease, you are at risk for getting endocarditis, an infection that causes damage to the heart valves (even if your valve has been repaired or replaced with surgery). You will need to follow these guidelines:

• Tell your doctors and dentist you have valve disease. You may want to carry a card with this information.
• Call your doctor if you have symptoms of an infection (sore throat, general body achiness, and fever). Colds and flus do not cause endocarditis. But, infections, which may have the same symptoms, do. So, to be safe, call your doctor.
• Take good care of your teeth and gums to prevent infections. See your dentist for regular visits.
• Take antibiotics before you undergo any procedure that may cause bleeding:
  • any dental work (even a basic teeth cleaning)
  • invasive tests
  • most major or minor surgery

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Posted in: CVS,Tricuspid valve disease

Tricuspid Regurgitation

Background:

Mild Tricuspid regurgitation may be detected in over 90% of the normal population by color Doppler echocardiogram. This is usually a benign finding and does not require any follow up or treatment. Virtually all of the normal population will have a mild degree of leakage in one, two, or three of the heart valves by echocardiogram. We call this physiologic regurgitation and many cardiologists prefer not to mention it to parents, as they may become concerned about a common and benign echocardiogram finding.

Pathologic tricuspid regurgitation is a disorder involving backward flow of blood across the tricuspid valve from the right ventricle (lower heart chamber) to the right atrium (upper heart chamber). Leakage occurs during contraction of the right ventricle and may be caused by damage or malformation of the tricuspid valve or and/or by significant enlargement of the right heart. The tricuspid valve may have been damaged by infection (endocarditis). In other cases, it may be a congenital malformation in the valve itself such as a dysplastic pulmonary valve or Ebstein’s anomaly of the tricuspid valve.

 

Tricuspid regurgitation may also be present in cases of distal anatomic obstructions such as pulmonary valve atresia or in cases of pulmonary hypertension (high pressures in the lungs). Rarely it may be caused by an unusual tumor called a carcinoid, rheumatoid arthritis, radiation therapy, Marfan’s syndrome, or chest trauma. Finally, tricuspid regurgitation is found in many patients with a single ventricle, corrected transposition of the great arteries or those who underwent the Fontan procedure in which the right ventricle is acting as the main pump of the heart. Those patients require lifetime follow up with serial echocardiograms. The tricuspid regurgitation may become severe enough to require heart surgery.

 

Other potential causes of significant tricuspid regurgitation include restrictive cardiomyopathy and constrictive pericarditis.

 

Symptoms:

Mild to moderate tricuspid regurgitation may not produce any symptoms at all in patients with normal pulmonary pressures. Patients with pulmonary hypertension and/or severe tricuspid regurgitation may experience these symptoms:

 Fatigue, tiredness

 Weakness

 Difficulty breathing

 Shortness of breath, especially on exertion

 General swelling

 Swelling of the abdomen

 Swelling of the feet and ankles

 Active pulsing in the neck veins

 Palpitations or “racing heart”

 Weight loss

 Loss of appetite

 Heart failure

Diagnosis and Cardiovascular Tests:

In cases of mild tricuspid regurgitation, the physical examination may be completely normal without an audible heart murmur. In cases of moderate severe tricuspid regurgitation, a heart murmur may be present and the liver may be enlarged. The abdomen may be distended and edema (swollen extremities) may be present. The electrocardiogram and chest x-ray may be abnormal. The echocardiogram is very helpful in determining the degree of tricuspid regurgitation, the size of the right heart, and its function. In addition, it may show the veins draining into the heart as being dilated. An echo may show any malformation or damage to the tricuspid valve or if an associated heart defect is present. Doppler echocardiography is used to estimate the pressures inside the heart and lungs. In more severe cases, the patient may require an MRI or cardiac catheterization.

Treatment:

Most patients with mild tricuspid regurgitation will not require any medical treatment. Patients with a normal heart and very mild forms of tricuspid regurgitation do not require any follow up. In more severe cases, the patients may require diuretics (water pills), while other patients may benefit from other medications that help improve the contractility of the heart. Medical treatment may depend on the underlying condition. For example, patients with pulmonary hypertension may require specific medications to lower lung pressures.

In general, patients with a single ventricle and the Fontan procedure may be on a few medications that may help release some of the volume overload or workload of the right ventricle and others may help improve the contractility of the heart pump.

Patients with an anatomical or structural problem of the tricuspid valve may require heart surgery. Some patients with progressive tricuspid regurgitation may also require surgery to prevent further deterioration of heart function.

Outcome:

Patients with an otherwise normal heart and mild to moderate tricuspid regurgitation lead a normal life and have no restrictions. Most of the restrictions to sports are associated with an underlying associated heart defect or underlying condition such as pulmonary hypertension. Therefore, prognosis in general may depend on the underlying condition and potential risk factors and not as much on the severity of the tricuspid regurgitation. Long-term complications may include heart failure, endocarditis, weight loss, and liver damage (cirrhosis).

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Posted in: CVS,Tricuspid Regurgitation

Aortic Regurgitation

Aortic Regurgitation

The aortic valve is between the heart's left ventricle (lower chamber that pumps blood to the body) and the aorta (the large artery that receives blood from the heart's left ventricle and distributes it to the body). Regurgitation means the valve doesn't close properly, and blood can leak backward through it. This means the left ventricle must pump more blood than normal, and will gradually get bigger because of the extra workload. Aortic regurgitation can range from mild to severe. Some people may have no symptoms for years. But as the condition worsens, symptoms will appear. These can include

• fatigue (especially during times of increased activity)
• shortness of breath
• edema (retention of fluid) in certain parts of the body such as the ankles
• heart arrhythmias (abnormal heartbeats)
• angina pectoris (chest pain or discomfort caused by reduced blood supply to the heart muscle)

What causes aortic regurgitation?

Aortic regurgitation can be caused by several things. It may be due to a bicuspid aortic valve. This is a congenital (existing at birth) deformity of the valve. In it, the valve has two cusps (flaps) rather than the normal three cusps. It can also be found in other kinds of congenital heart disease. Aortic regurgitation can also be caused by infections of the heart, such as rheumatic fever or infective endocarditis. Diseases that can cause the aortic root (the part of the aorta attached to the ventricle) to widen, such as the Marfan syndrome or high blood pressure, are other causes.

What should be done?

Patients with mild aortic regurgitation who have few or no symptoms need to see their physician regularly. As conditions worsen, medications may be used. These drugs can help regulate the heart rhythm, rid the body of fluids to control edema, and/or help the left ventricle pump better.
Serious cases may require surgical treatment. This involves replacing the diseased valve with an artificial one.
People with aortic regurgitation are at increased risk for developing an infection of the heart valve or lining of the heart (endocarditis). In the past, the American Heart Association has recommended that patients with aortic regurgitation take a dose of antibiotics before certain dental or surgical procedures. However, our association no longer recommends antibiotics before dental procedures except for patients at the highest level of risk for bad outcomes from endocarditis, such as

• patients with a prosthetic cardiac valve,
• patients who have had endocarditis before,
• patients with certain kinds of congenital heart disease, or
• heart transplant patients who develop a problem with a heart valve. 

Also, the American Heart Association no longer recommends routine antibiotics to prevent endocarditis in patients undergoing gastrointestinal (GI) or genitourinary (GU) tract procedures
.

Frequency

United States

Rheumatic fever and syphilis used to be major causes of aortic regurgitation, but these diseases have diminished in recent years because of the introduction of new antibiotics.

Mortality/Morbidity

• Three fourths of patients with significant aortic regurgitation survive 5 years after diagnosis; half survive for 10 years. Patients with mild-to-moderate regurgitation survive 10 years in 80-95% of the cases.
• Average survival after onset of congestive heart failure (CHF) is less than 2 years.
• Acute aortic regurgitation is associated with significant morbidity, which can progress from pulmonary edema to refractory heart failure and cardiogenic shock.

Age

Chronic aortic regurgitation often begins in the late 50s and is documented most frequently in patients older than 80 years.

History

• General

  • The clinical signs of aortic regurgitation are caused by forward and backward flow of blood across the aortic valve, leading to increased stroke volume.
  • The degree of regurgitation is determined by the degree of valvular incompetence; left ventricular compliance; and end-ventricular, end-diastolic volume.
• Acute aortic regurgitation: Symptoms are manifestations of cardiovascular collapse.

  • Weakness
  • Severe dyspnea
  • Hypotension
  • Angina
• Chronic aortic regurgitation

  • Exertional dyspnea
  • Nocturnal dyspnea
  • Orthopnea
  • Diaphoresis
  • Abdominal discomfort
  • Uncomfortable awareness of heartbeat
  • Palpitations

Differentials

• Abdominal Trauma, Blunt
• Acute Coronary Syndrome
• Congestive Heart Failure and Pulmonary Edema
• Endocarditis
• Mitral Regurgitation
• Mitral Stenosis
• Myocardial Infarction

Laboratory Studies

• CBC
• Prothrombin time (PT)/activated partial thromboplastin time (aPPT)
• Type and screen
• Electrolytes
• Myocardial muscle creatine kinase isoenzyme (CK-MB)
• Lactate dehydrogenase panel
• Isoenzymes
   

  Emergency Department Care

  • General

    • Provide adequate airway management.
    • Intubate when necessary.
    • Consider prompt surgical intervention in acute aortic regurgitation.
  • Acute aortic regurgitation

    • Administer a positive inotrope (eg, dopamine, dobutamine) and a vasodilator (eg, nitroprusside). Rarely, administration of cardiac glycosides (eg, digoxin) for rate control may be necessary.
    • Avoid beta-blockers in the acute setting.
    • Administration of vasodilators may be appropriate to improve systolic function and to decrease afterload.
  • Chronic aortic regurgitation

    • Consider antibiotic prophylaxis for patients with endocarditis when performing procedures likely to result in bacteremia.
    • Administration of pressors and/or vasodilators may be appropriate.
  • Hemodynamically significant aortic regurgitation may require surgical intervention according to the following criteria:

    • Cardiac-thoracic ratio >0.64
    • Fractional shortening < 25-29%
    • End-systolic diameter >55 mm
    • End-diastolic radius to myocardial wall thickness ratio >4.0
    • Ejection fraction < 0.45
    • Cardiac index < 2.2-2.5 L/min/m²

   

  What are the treatments for aortic regurgitation?

  If the backflow of blood is mild and you have no symptoms then you may not need any treatment. If you develop symptoms or complications, various medicines may be advised to ease the symptoms. Surgery may be advised if symptoms become worse.

  Medication

  Medication may be advised to help ease symptoms of heart failure if heart failure develops. For example:
  

  • Diuretics (water tablets) usually help if you are breathless. They make the kidneys produce more urine. This gets rid of excess blood and fluid which may build up in the lungs or other parts of the body with heart failure.
  • Angiotensin-converting enzyme (ACE) inhibitors are medicines which help to reduce the amount of work the heart does and to ease symptoms of heart failure.

  Valve replacement surgery

  This may be with a mechanical or a tissue valve. Mechanical valves are made of materials which are not likely to react with your body, such as titanium. Tissue valves are made from treated animal tissue, such as valves from a pig. If you need surgery, a surgeon will advise on which is the best option for your situation.
  
  Surgical treatment has greatly improved the outlook in most people with more severe regurgitation. Surgery to replace the valve has a very good success rate. The outlook is good if the valve is treated before the heart becomes badly damaged.

  Antibiotics to prevent endocarditis

  Antibiotics used to be offered to all people with heart valve disease before dental treatment and some surgical procedures to prevent the development of endocarditis. However, the National Institute for Health and Clinical Excellence (NICE) issued guidance in 2008 which advised that people at risk of endocarditis only need to take antibiotics if they actually have an infection at the time that dental or surgical procedures are undertaken.

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Posted in: Aortic Regurgitation,CVS

Aortic Stenosis

Aortic Stenosis

Aortic stenosis is abnormal narrowing of the aortic valve. A number of conditions cause disease resulting in narrowing of the aortic valve. When the degree of narrowing becomes significant enough to impede the flow of blood from the left ventricle to the arteries, heart problems develop. The basic mechanism is as follows:

• The heart is a muscular pump with four chambers and four heart valves.
  
• The upper chambers, the right atrium and left atrium (atria - plural for atrium), are thin walled filling chambers.
  
• Blood flows from the right and left atria across the tricuspid and mitral valves into the lower chambers (right and left ventricles).
  
• The right and left ventricles have thick muscular walls for pumping blood across the pulmonic and aortic valves into the circulation.
  
• Heart valves are thin leaflets of tissue which open and close at the proper time during each heart beat cycle.
  
• The main function of these heart valves is to prevent blood from flowing backwards.
  
• Blood circulates through the arteries to provide oxygen and other nutrients to the body, and then returns with carbon dioxide waste through the veins to the right atrium; when the ventricles relax, blood from the right atrium passes through the tricuspid valve into the right ventricle.
  
  
• When the ventricles contract, blood from the right ventricle is pumped through the pulmonic valve into the lungs to reload on oxygen and remove carbon dioxide.
  
  
• The oxygenated blood then returns to the left atrium and passes through the mitral valve into the left ventricle.
  
  
• Blood is pumped by the left ventricle across the aortic valve into the aorta and the arteries of the body.

The flow of blood to the arteries of the body is impaired when aortic stenosis exists. Ultimately, this can lead to heart failure. Aortic stenosis occurs three times more commonly in men than women.
 

Symptoms

Aortic stenosis may have no symptoms (asymptomatic) for many years. This is why the condition, which may have been congenital (present from before birth) is often diagnosed during teenage years. Symptoms may appear later in life after decades of gradual progressive narrowing. The onset of symptoms may be gradual or abrupt with:

• Breathlessness
• Breathing problems worsened by physical activity
• Coughing at night when lying down in bed
• Fainting
• Heart palpitations
• Pains in the chest, from the heart (angina)
• Fatigue
• Visual problems.

A range of causes

Some of the causes of aortic stenosis include:

• Congenital heart disease - the baby is born with heart abnormalities. For example, the aortic valve may be smaller than it should be.
• Valve abnormalities - some people are born with minor abnormalities of the aortic valve. Over time, these abnormalities may cause the valve to narrow.
• Rheumatic heart disease - a condition that can scar the aortic valve and narrow its opening.
• Calcium deposits - a build-up of calcium can stiffen the aortic valve and interfere with its proper functioning. This is the most common cause of aortic stenosis in people aged 70 years and over.

Possible complications

Aortic stenosis can be a serious and potentially life threatening condition. Some of the possible complications include:

• Pulmonary oedema - the back pressure of blood inside the heart changes the pressure in the blood vessels of the lungs. This causes congestion and breathing difficulties.
• Cardiomegaly - without treatment, the left ventricle may thicken and enlarge. This reduces the ventricle’s ability to pump blood.
• Congestive heart failure - aortic stenosis compromises the functioning of the heart’s left side. Congestive heart failure occurs when the right side of the heart also stops working properly.
• Heart arrhythmia - an irregular heartbeat. Some arrhythmias in the ventricles may be associated with cardiac death, such as ‘ventricular fibrillation’ when the ventricles are reduced to quivering rather than beating.

Diagnosis methods

Aortic stenosis is diagnosed using a number of tests including:

• Physical examination including listening to the heart with a stethoscope.
• Chest x-ray.
• Electrocardiogram (ECG) to monitor the heart rate and pick up any unusual rhythms and to assess thickening of the left ventricle.
• Echocardiograph (ultrasound scan) of the heart to assess the functioning of the aortic valve and of the left ventricle.
• Cardiac catheterisation (a slender tube is inserted into a blood vessel of the groin and threaded up to the heart).
• Left ventriculography, which includes using a dye so that the heart shows up more clearly on x-ray.
• Coronary arteriography to assess whether there is coronary artery disease in addition to the recognised aortic valve disease.

Treatment options

Treatment for aortic stenosis may include:

• Monitoring - for asymptomatic or mild cases.
• Medications to prevent heart failure.
• Hospitalisation - for moderate to severe cases.
• Lifestyle patterns such as maintaining physical activity while avoiding hard physical exercise, control of weight and avoidance of smoking.
• Surgery.

Surgical procedures

There are two main surgical procedures for treating aortic stenosis:

• Balloon valvuloplasty - a catheter is inserted into a blood vessel in the groin and threaded up to the heart. The tip of the catheter is placed inside the aortic valve and then a balloon is inflated. This helps to stretch and widen the valve and improve blood flow into the aorta. This procedure doesn’t cure the condition and further surgical treatment may be needed later in life. This procedure is usually limited to those with congenital aortic stenosis - usually in children or adolescents.
• Aortic valve replacement - if the valve is too defective, it may be surgically replaced with an artificial valve. Sometimes the person’s own pulmonary valve may be used. The latter is known as a pulmonary autograft or Ross Operation.

Things to remember

• The aortic valve opens when the heart contracts to allow the passage of blood from the left ventricle into the aorta, the body’s main artery.
• Aortic stenosis is the abnormal narrowing of the aortic valve, which impedes the flow of blood from the ventricle into the aorta.
• Aortic stenosis may be a serious and potentially life threatening condition.
• Treatment options include medications to prevent heart failure and surgery to repair or replace the faulty valve.

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Posted in: Aortic Stenosis,CVS

Mitral valve prolapse

Mitral valve prolapse

Mitral valve prolapse (MVP) is the systolic billowing of one or both mitral leaflets into the left atrium during systole.It may occur in the setting of myxomatous valve disease or in persons with normal mitral valve leaflets.

Prevalence and Risk Factors

MVP is the most common valvular disorder in the United States, occurring in 2.4% of the general population. There is a similar prevalence in men and women, with a greater risk of complications in men.

Pathophysiology and Natural History

Many patients with MVP have normal mitral leaflets, with little or no mitral regurgitation, and a benign prognosis. Survival rates among affected patients are similar to those of age- and gender-matched individuals without MVP. In other patients, MVP is caused by myxomatous valve disease, with typical findings of elongated and thickened leaflets, interchordal hooding, and chordal elongation . Patients with myxomatous MVP are at increased risk for cardiovascular complications, particularly when prolapse is associated with at least moderate mitral regurgitation or LV dysfunction. Although most patients with MVP do not develop severe mitral regurgitation, MVP is a common underlying cause of progressive mitral regurgitation, often necessitating mitral valve repair or replacement^.
The causes of myxomatous mitral valve disease are not certain, but appear to involve dysregulation of extracellular matrix proteins. Myxomatous mitral valve disease usually occurs sporadically, although there are well-described cases of familial clustering that involve an autosomal dominant mode of inheritance^. Three genetic loci for autosomal dominant myxomatous mitral valve disease have been described, but the precise genes and mutations have not yet been identified. Myxomatous MVP also may occur in conjunction with certain connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome.

Signs and Symptoms

Most patients with MVP are asymptomatic. In the past, multiple nonspecific symptoms (atypical chest pain, dyspnea, palpitations, anxiety, and syncope) and clinical findings (low body weight, low blood pressure, and pectus excavatum) were associated with MVP and termed mitral valve prolapse syndrome. Prospective testing has failed to confirm most of these associations. The classic findings of MVP on physical examination are a midsystolic click, with a late systolic murmur, heard best at the cardiac apex.

Diagnosis

Two-dimensional echocardiography is the most important test for diagnosing MVP (Class I).¹ The diagnosis is made when there is displacement of one or both mitral leaflets by 2 mm or more into the left atrium during systole (Fig). Because the mitral annulus is known to have a saddle shape, a normal mitral valve can appear to prolapse in certain echocardiographic views, most notably in the apical two- and four-chamber views. Therefore, the diagnosis of MVP should be based on a long-axis parasternal or apical three-chamber view. In patients with MVP, echocardiography is also useful in determining the presence and severity of MR and assessing left atrial and ventricular chamber size, LV function, and leaflet thickening and redundancy. Unless severe mitral regurgitation is present, findings on the chest radiograph and ECG typically are unremarkable. A more detailed discussion of the diagnosis of mitral valve prolapse may be found in the AHA/ACC guidelines.

Summary

• Mitral valve prolapse is present if there is more than 2 mm displacement of the mitral valve leaflets into the left atrium during systole in a parasternal long-axis or apical three-chamber view on echocardiography.

Treatment

Medical Treatment

Asymptomatic patients require no specific treatment and they should be reassured of their excellent prognosis. Although antibiotic prophylaxis for endocarditis was once advocated for certain patients with MVP, more recent guidelines do not recommend antibiotic prophylaxis in this group of patients.Beta blockers are useful for alleviating symptoms of palpitations, anxiety, and chest pain in certain patients.

Figure

MVP patients without mitral regurgitation should be evaluated every 3 to 5 years. Echocardiography should be performed if the patient has new cardiovascular symptoms or if the physical examination suggests that significant mitral regurgitation has developed. Patients with severe mitral regurgitation or high-risk features should be reviewed with an echocardiogram yearly or more often if their clinical condition warrants it.

Surgery

In MVP patients with severe mitral regurgitation, the indications for mitral valve surgery are similar to those for patients with other causes of severe regurgitation. When surgery is required, mitral valve repair is usually feasible . Repair is characterized by low mortality and long-lasting durability; the 10-year reoperation-free survival rate ranges between 93% and 96%.A more detailed discussion of the management of mitral valve prolapse may be found in the AHA/ACC guidelines.

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Posted in: CVS,Mitral valve prolapse