RHEUMATIC HEART DISEASE Rheumatic Heart Disease What is rheumatic heart disease?

Rheumatic heart disease is a condition in which permanent damage to heart valves is caused by rheumatic fever. The heart valve is damaged by a disease process that generally begins with a strep throat caused by bacteria called Streptococcus, and may eventually cause rheumatic fever. Who is at risk for rheumatic fever? Rheumatic fever is uncommon in the US, except in children who have had strep infections that were untreated or inadequately treated. Children ages 5 to 15, particularly if they experience frequent strep throat infections, are most at risk for developing rheumatic fever. Why is rheumatic fever a concern? Rheumatic fever, an inflammatory disease, can affect many connective tissues, especially in the heart, joints, skin, or brain. The infection often causes heart damage, particularly scarring of the heart valves, forcing the heart to work harder to pump blood. The damage may resolve on its own, or it may be permanent, eventually causing congestive heart failure (a condition in which the heart cannot pump out all of the blood that enters it, which leads to an accumulation of blood in the vessels leading to the heart and fluid in the body tissues). What are the symptoms of rheumatic fever? The symptoms of rheumatic fever usually start about one to five weeks after your child has been infected with Streptococcus bacteria. The following are the most common symptoms of rheumatic fever. However, each child may experience symptoms differently. Symptoms may include:

joint inflammation - including swelling, tenderness, and redness over multiple joints. The joints affected are usually the larger joints in the knees or ankles. The inflammation "moves" from one joint to another over several days. small nodules or hard, round bumps under the skin a change in your child's neuromuscular movements (this is usually noted by a change in your child's handwriting and may also include jerky movements) rash (a pink rash with odd edges that is usually seen on the trunk of the body or arms and legs) fever weight loss fatigue

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stomach pains

The symptoms of rheumatic fever may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis. Treatment for rheumatic heart disease: Specific treatment for rheumatic heart disease will be determined by your child's physician based on: • • • • • your child's overall health and medical history extent of the disease your child's tolerance for specific medications, procedures, or therapies expectations for the course of the disease your opinion or preference

The best treatment for rheumatic heart disease is prevention. Antibiotics can usually treat strep throat (a Streptococcus bacterial infection) and stop acute rheumatic fever from developing. Antibiotic therapy has sharply reduced the incidence and mortality rate of rheumatic fever and rheumatic heart disease. Children who have previously contracted rheumatic fever are often given continuous (daily or monthly) antibiotic treatments to prevent future attacks of rheumatic fever and lower the risk of heart damage. If inflammation of the heart has developed, children may be placed on bed rest. Medications are given to reduce the inflammation, as well as antibiotics to treat the Streptococcus infection. Other medications may be necessary to handle congestive heart failure. If heart valve damage occurs, surgical repair or replacement of the valve may be considered. Pathophysiology Rheumatic fever develops in children and adolescents following pharyngitis with group A beta-hemolytic Streptococcus (ie, Streptococcus pyogenes). The organisms attach to the epithelial cells of the upper respiratory tract and produce a battery of enzymes allowing them to damage and invade human tissues. After an incubation period of 2-4 days, the invading organisms elicit an acute inflammatory response with 3-5 days of sore throat, fever, malaise, headache, and an elevated leukocyte count. In 0.3-3% of cases, infection leads to rheumatic fever several weeks after the sore throat has resolved. Only infections of the pharynx initiate or reactivate rheumatic fever. The organism spreads by direct contact with oral or respiratory secretions, and spread is enhanced by crowded living conditions. Patients remain infected for weeks after symptomatic resolution of pharyngitis and may serve as a reservoir for infecting others. Penicillin treatment shortens the clinical course of streptococcal pharyngitis and, more importantly, prevents the major sequelae.

Group A Streptococcus is a gram-positive coccus that frequently colonizes the skin and oropharynx. This organism may cause suppurative disease, such as pharyngitis, impetigo, cellulitis, myositis, pneumonia, and puerperal sepsis. It also may be associated with nonsuppurative disease, such as rheumatic fever and acute poststreptococcal glomerulonephritis. Group A streptococci elaborate the cytolytic toxins streptolysins S and O. Of these, streptolysin O induces persistently high antibody titers that provide a useful marker of group A streptococcal infection and its nonsuppurative complications. Group A Streptococcus, as identified using the Lancefield classification, has a group A carbohydrate antigen in the cell wall that is composed of a branched polymer of L- rhamnose and N- acetyl-D-glucosamine in a 2:1 ratio. Group A streptococci may be subserotyped by surface proteins on the cell wall of the organism. The presence of the M protein is the most important virulence factor for group A streptococcal infection in humans. More than 90 M serotypes have been identified, some of which have a long terminal antigenic domain (epitopes) similar to antigens in various components of the human heart. Rheumatogenic strains often are encapsulated mucoid strains rich in M proteins and resistant to phagocytosis. These strains are strongly immunogenic, and M-binding antibodies and T cells against the streptococcal infection may cross react with heart tissue. Streptococcal antigens that are structurally similar to those in the heart include hyaluronate in the bacterial capsule, cell wall polysaccharides (similar to glycoproteins in heart valves), and membrane antigens that share epitopes with the sarcolemma and smooth muscle. Acute rheumatic heart disease often produces a pancarditis characterized by endocarditis, myocarditis, and pericarditis. Endocarditis is manifested as valve insufficiency. The mitral valve is most commonly and severely affected (65-70% of patients), and the aortic valve is second in frequency (25%). The tricuspid valve is deformed in only 10% of patients and is almost always associated with mitral and aortic lesions. The pulmonary valve is rarely affected. Severe valve insufficiency during the acute phase may result in congestive heart failure and even death (1% of patients). Whether myocardial dysfunction during acute rheumatic fever is primarily related to myocarditis or is secondary to congestive heart failure from severe valve insufficiency is not known. Pericarditis, when present, rarely affects cardiac function or results in constrictive pericarditis. Chronic manifestations due to residual and progressive valve deformity occur in 9-39% of adults with previous rheumatic heart disease. Fusion of the valve apparatus resulting in stenosis or a combination of stenosis and insufficiency develops 2-10 years after an episode of acute rheumatic fever, and recurrent episodes may cause progressive damage to the valves. Fusion occurs at the level of the valve commissures, cusps, chordal attachments, or any combination of these. Rheumatic heart disease is responsible for 99% of

mitral valve stenosis in adults in the United States. Associated atrial fibrillation or left atrial thrombus formation from chronic mitral valve involvement and atrial enlargement may be observed.

Tetralogy of Fallot (Note: before reading the specific defect information and the image associated with it, it will be helpful to review normal heart function.) What is it? Tetralogy of Fallot refers to a combination of abnormalities with four key features: 1) A ventricular septal defect (a hole between the ventricles) and 2) obstruction of blood flow from the right ventricle to the lungs (pulmonary stenosis) are the most important. Sometimes the pulmonary valve isn’t just narrowed but is completely obstructed (pulmonary atresia). Also, 3) the aorta (major artery from the heart to the body) lies directly over the ventricular septal defect and 4) the right ventricle develops hypertrophy (thickened muscle). Because of the pulmonary stenosis, blood can’t get to the lungs easily, so the blood doesn’t get as much oxygen as it should. Because the aorta overrides the ventricular septal defect, blood from both ventricles (oxygenrich and oxygen-poor) is pumped into the body. People with unrepaired tetralogy of Fallot are often blue (cyanotic) because of the oxygen-poor blood that’s pumped to the body.

What causes it? In most cases, the cause isn’t known although in some patients, genetic factors play a role. It’s a common type of heart defect. It may be seen more commonly in patients with Down syndrome (in association with AV canal

defects) or DiGeorge syndrome. Some patients can have other heart defects along with tetralogy of Fallot. How does it affect the heart? Normally the left side of the heart only pumps blood to the body, and the heart’s right side only pumps blood to the lungs. In a patient with tetralogy of Fallot, blood can travel across the hole (VSD) from the right pumping chamber (right ventricle) to the left pumping chamber (left ventricle) and out into the body artery (aorta). Obstruction in the pulmonary valve leading from the right ventricle to the lung artery prevents the normal amount of blood from being pumped to the lungs. Sometimes the pulmonary valve is completely obstructed (pulmonary atresia). How does tetralogy of Fallot affect me? Most patients are diagnosed with tetralogy of Fallot as infants or young children. Patients with unrepaired tetralogy of Fallot are usually blue (cyanotic). This is true in infants, children and adults with unrepaired tetralogy of Fallot. Most adults with tetralogy of Fallot have had it repaired in childhood. Many people are symptom free but may have residual or recurrent problems. These include valve leakage of blood into the heart’s right side, blockage of blood leaving the heart’s right side and heart rhythm problems. Some patients with these problems may have limited exercise tolerance and may require medicines, repeat operations and/or a special pacemaker to lower the risk of heart rhythm problems. What can be done about tetralogy of Fallot? Tetralogy of Fallot is treated with two kinds of surgery. One provides temporary improvement by a shunt to give more blood flow to the lungs. The other is a complete repair of the two most important abnormalities that make up tetralogy of Fallot. Patients might have one or both surgeries in their lifetime. Shunt Operation In some patients, a shunt operation blood flow to the lungs. This is not the inside of the heart. The shunt is material sewn between a body pulmonary artery. The shunt is intracardiac repair is done later. allow them grow big enough to done in adults if a complete repair adults had shunts as children, but patients may still be able to get a Complete Repair may be done first to provide more open-heart surgery and doesn’t fix usually a small tube of synthetic artery (or the aorta) and the removed when a complete This is usually done in babies to have a full repair but is occasionally isn’t an immediate option. Some never got a complete repair. Those complete repair, even as adults.

Complete repair tends to be done early in life, but in some cases it can be done in adulthood. The surgeon closes the ventricular septal defect with a patch and relieves the obstruction to blood flow going to the lungs. This may be done by removing some thickened muscle below the pulmonary valve, repairing or removing the obstructed pulmonary valve and, if needed, enlarging the pulmonary artery branches that go to each lung. Sometimes a tube (conduit) with a valve in it is placed between the right ventricle and the pulmonary artery. This is sometimes called a Rastelli repair. It’s similar to the type of repair used for some other heart defects. Repeat Operations The surgery to open up the pulmonary valve often leads to a leaky pulmonary valve. Although this is better tolerated than the original abnormality, the leaky valve can eventually cause problems. There also can be obstructed pathways that are left behind or develop as the patient grows. Both leaky valves and obstructed pathways can cause problems for adult patients with tetralogy of Fallot. In many cases, another surgery to replace the pulmonary valve may be needed in adolescence or adulthood. Ongoing Care: Medical If you’ve had tetralogy of Fallot repaired, you’ll need regular follow-up with a cardiologist who’s had special training in congenital heart defects. You may need to take medicine before or after your operation to help your heart muscle contract or to control heart rhythm abnormalities. Your cardiologist will follow your progress with various tests. These include electrocardiograms, Holter monitors, exercise stress tests, echocardiograms and cardiac MRIs. This will help determine if you need another procedure, such as a cardiac catheterization or more surgery. You should also consult a cardiologist with expertise in caring for adults with congenital heart disease if you’re undergoing any type of non-heart surgery or invasive procedure. Activity Restrictions If tetralogy of Fallot has been repaired with surgery, and there’s no obstruction or leak in the pulmonary valve, you may be able to participate in normal activities without much increased risk. You may need to limit your activity if there is leftover obstruction or a pulmonary valve leak, which is common after repair. This limitation may be especially necessary for competitive sports. Patients with decreased heart function or rhythm abnormalities may need to limit their activity more. Your cardiologist will help decide if you need limits. See the Physical Activity section for more information. What will I need in the future?

If you’ve had tetralogy of Fallot repaired, you’ll need regular lifelong regular follow-up with a cardiologist who’s had special training in congenital heart defects. Some long-term problems can include leftover or worsening obstruction between the right pumping chamber and the lung arteries. Another problem can be a leaky pulmonary valve and enlargement of the heart’s right side. Patients with repaired tetralogy of Fallot have a higher risk of heart rhythm disturbances called arrhythmias. Sometimes these may cause dizziness or fainting. Generally, the long-term outlook is good, but some patients may need medicines, heart catheterization or even more surgery. Endocarditis Prevention People with tetralogy of Fallot are at increased risk for developing endocarditis. People with unrepaired and partially repaired tetralogy will need antibiotics to prevent endocarditis before certain dental procedures. If your tetralogy of Fallot has been repaired, your cardiologist will let you know if you need to continue to receive these routine antibiotics. See the section on Endocarditis for more information. Problems You May Have: Heart Function In the long-term postoperative period, your heart muscle function may decrease and you’ll need medication. This usually reflects weakening function of the right side of the heart, but in rare instances decreased function of the left ventricle may also occur. Medicines that may be needed include diuretics (water pills), drugs to help your heart pump better and drugs to control your blood pressure. Heart Rhythm Disturbances People with repaired tetralogy of Fallot have a higher risk of heart rhythm disturbances called arrhythmias. These can originate from the atria or the ventricles. Sometimes they may cause dizziness or fainting. You may need medicine to control them. Sometimes patients also need blood thinners to reduce the risk of stroke related to the arrhythmias. In rare cases, you may need a procedure in the cardiac catheterization laboratory or the operating room to eliminate these arrhythmias and control symptoms. See the Arrhythmias section for more information. Some patients need pacemakers or implantable defibrillators to treat their arrhythmias. Pregnancy In many cases, women with repaired tetralogy of Fallot have successful, fullterm pregnancies. Some woman with significant residual heart problems may be at risk for a difficult pregnancy. See the section on Pregnancy for more information. Once tetralogy of Fallot has been repaired, the pregnancy risk is low unless the woman has some side effects or residual effect like irregular

heart rhythms or persistent narrowing of the pulmonary valve. Any woman considering pregnancy should have a complete physical examination by her cardiologist in consultation with a multi-disciplinary team before becoming pregnant to find out what, if any risk there might be with the pregnancy. Pregnancy is considered to be high-risk and not recommended for women with unoperated tetralogy of Fallot who remain “cyanotic” (blue). Will I need more surgery? After the first complete repair, residual problems may require you to have more open-heart surgeries or procedures in the cardiac catheterization laboratory. In some cases, a procedure done in the cardiac catheterization laboratory using a balloon-tipped catheter to dilate and/or place an expandable stent in narrowed areas may be needed. Anatomy of the Heart Your heart is located under the ribcage in the center of your chest between your right and left lungs. Its muscular walls beat, or contract, pumping blood continuously to all parts of your body. The size of your heart can vary depending on your age, size, and the condition of your heart. A normal, healthy, adult heart most often is the size of an average clenched adult fist. Some diseases of the heart can cause it to become larger. The Exterior of the Heart Below is a picture of the outside of a normal, healthy, human heart. Heart Exterior

The illustration shows the front surface of a heart, including the coronary arteries and major blood vessels. The heart is the muscle in the lower half of the picture. The heart has four chambers. The right and left atria (AY-tree-uh) are shown in purple. The right and left ventricles (VEN-trih-kuls) are shown in red. Some of the main blood vessels—arteries and veins—that make up your blood circulatory system are directly connected to the heart. The ventricle on the right side of your heart pumps blood from your heart to your lungs. When you breathe air in, oxygen passes from your lungs through your blood vessels and into your blood. Carbon dioxide, a waste product, is passed from your blood through blood vessels to your lungs and is removed from your body when you breathe out. The left atrium receives oxygen-rich blood from your lungs. The pumping action of your left ventricle sends this oxygen-rich blood through the aorta (a main artery) to the rest of your body. The Right Side of Your Heart The superior and inferior vena cavae are in blue to the left of the heart muscle as you look at the picture. These veins are the largest veins in your body. After your body's organs and tissues have used the oxygen in your blood, the vena cavae carry the oxygen-poor blood back to the right atrium of your heart. The superior vena cava carries oxygen-poor blood from the upper parts of your body, including your head, chest, arms, and neck. The inferior vena cava carries oxygen-poor blood from the lower parts of your body. The oxygen-poor blood from the vena cavae flows into your heart's right atrium and then on to the right ventricle. From the right ventricle, the blood is pumped through the pulmonary (PULL-mun-ary) arteries (in blue in the center of the picture) to your lungs. There, through many small, thin blood vessels called capillaries, the blood picks up more oxygen. The oxygen-rich blood passes from your lungs back to your heart through the pulmonary veins (in red to the left of the right atrium in the picture). The Left Side of Your Heart Oxygen-rich blood from your lungs passes through the pulmonary veins (in red to the right of the left atrium in the picture). It enters the left atrium and is pumped into the left ventricle. From the left ventricle, the oxygen-rich blood is pumped to the rest of your body through the aorta. Like all of your organs, your heart needs blood rich with oxygen. This oxygen is supplied through the coronary arteries as blood is pumped out of your heart's left ventricle.

Your coronary arteries are located on your heart's surface at the beginning of the aorta. Your coronary arteries (shown in red in the drawing) carry oxygenrich blood to all parts of your heart. The Interior of the Heart Below is a picture of the inside of a normal, healthy, human heart. Heart Interior

The illustration shows a cross-section of a healthy heart and its inside structures. The blue arrow shows the direction in which oxygen-poor blood flows from the body to the lungs. The red arrow shows the direction in which oxygen-rich blood flows from the lungs to the rest of the body. The Septum The right and left sides of your heart are divided by an internal wall of tissue called the septum. The area of the septum that divides the atria (the two upper chambers of your heart) is called the atrial or interatrial septum. The area of the septum that divides the ventricles (the two lower chambers of your heart) is called the ventricular or interventricular septum. Heart Chambers The picture shows the inside of your heart and how it's divided into four chambers. The two upper chambers of your heart are called atria. The atria receive and collect blood. The two lower chambers of your heart are called ventricles. The ventricles pump blood out of your heart into the circulatory system to other parts of your body.

Heart Valves The picture shows your heart's four valves. Shown counterclockwise in the picture, the valves include the aortic (ay-OR-tik) valve, the tricuspid (triCUSS-pid) valve, the pulmonary valve, and the mitral (MI-trul) valve. Blood Flow The arrows in the drawing show the direction that blood flows through your heart. The light blue arrows show that blood enters the right atrium of your heart from the superior and inferior vena cavae. From the right atrium, blood is pumped into the right ventricle. From the right ventricle, blood is pumped to your lungs through the pulmonary arteries. The light red arrows show the oxygen-rich blood coming in from your lungs through the pulmonary veins into your heart's left atrium. From the left atrium, the blood is pumped into the left ventricle. The left ventricle pumps the blood to the rest of your body through the aorta. For the heart to work properly, your blood must flow in only one direction. Your heart's valves make this possible. Both of your heart's ventricles have an "in" (inlet) valve from the atria and an "out" (outlet) valve leading to your arteries. Healthy valves open and close in very exact coordination with the pumping action of your heart's atria and ventricles. Each valve has a set of flaps called leaflets or cusps that seal or open the valves. This allows pumped blood to pass through the chambers and into your arteries without backing up or flowing backward. PHISIOLOGY The heart is the muscular organ of the circulatory system that constantly pumps blood throughout the body. Approximately the size of a clenched fist, the heart is composed of cardiac muscle tissue that is very strong and able to contract and relax rhythmically throughout a person's lifetime.

The heart has four separate compartments or chambers. The upper chamber on each side of the heart, which is called an atrium, receives and collects the blood coming to the heart. The atrium then delivers blood to the powerful lower chamber, called a ventricle, which pumps blood away from the heart through powerful, rhythmic contractions. The human heart is actually two pumps in one. The right side receives oxygenpoor blood from the various regions of the body and delivers it to the lungs. In the lungs, oxygen is absorbed in the blood. The left side of the heartreceives the oxygen-rich blood from the lungs and delivers it to the rest of the body. Systole The contraction of the cardiac muscle tissue in the ventricles is called systole. When the ventricles contract, they force the blood from their chambers into the arteries leaving the heart. The left ventricle empties into the aorta and the right ventricle into the pulmonary artery. The increased pressure due to the contraction of the ventricles is called systolic pressure. Diastole The relaxation of the cardiac muscle tissue in the ventricles is called diastole. When the ventricles relax, they make room to accept the blood from the atria. The decreased pressure due to the relaxation of the ventricles is called diastolic pressure. Electrical Conduction System The heart is composed primarily of muscle tissue. A network of nerve fibers coordinates the contraction and relaxation of the cardiac muscle tissue to obtain an efficient, wave-like pumping action of the heart.

The Sinoatrial Node (often called the SA node or sinus node) serves as the natural pacemaker for the heart. Nestled in the upper area of the right atrium, it sends the electrical impulse that triggers each heartbeat. The impulse spreads through the atria, prompting the cardiac muscle tissue to contract in a coordinated wave-like manner. The impulse that originates from the sinoatrial node strikes the Atrioventricular node (or AV node) which is situated in the lower portion of the right atrium. The atrioventricular node in turn sends an impulse through the nerve network to the ventricles, initiating the same wave-like 1. Sinoatrial node (SA contraction of the ventricles. node) The electrical network serving the ventricles leaves the atrioventricular node through the Right and 2. Atrioventricular Left Bundle Branches. These nerve fibers send node (AV node) impulses that cause the cardiac 3. Common AV Bundle 4. Right & Left Bundle Branches