ACYANOTIC DISEASE

Classification of CHD There are many different ways to categorize CHD. One of the most frequently used is: Cyanotic Heart Disease - where there is mixing of pure oxygen-rich blood with venous blood, resulting in a bluish or violet discoloration of the patient's lips and finger-tips. Acyanotic Heart Disease - where there is no cyanosis. In general, cyanotic heart birth defects are more complex, and have a combination of defects. Other ways to group CHD are based on lung blood flow (high, normal or low) part or side of the heart affected (left, right, both). Acyanotic Heart Disease The common forms of acyanotic CHD are those where there is a defect in one of the walls separating the chambers of the heart, or obstruction to one valve or artery. Common examples of acyanotic CHD are: Atrial Septal Defect (ASD) - a hole between the upper chambers of the heart. Ventricular Septal Defect (VSD) - a hole between the lower chambers of the heart. Patent Ductus Arteriosus (PDA) - an abnormal communication between the aorta and pulmonary artery. Coarctation of the Aorta - an obstruction within the large artery of the body. Pulmonary Stenosis - a narrowing of the valve between the right ventricle and pulmonary artery.

Atrial Septal Defect (ASD)

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What It Is The septum is a wall that separates the heart's left and right sides. Septal defects are sometimes called a "hole" in the heart. A defect between the heart's two upper chambers (the atria) is called an atrial septal defect (ASD). When there is a large defect between the atria, a large amount of oxygen-rich (red) blood leaks from the heart's left side back to the right side. Then this blood is pumped back to the lungs, despite already having been refreshed with oxygen. This is inefficient, because already-oxygenated blood displaces blood that needs oxygen. Many people with this defect have few, if any, symptoms. Surgical Therapy Closing an atrial septal defect in childhood can prevent serious problems later in life. The long-term outlook is excellent. If atrial septal defects are diagnosed in adulthood, the defect is also repaired. Rarely, the defect is left unrepaired if there's pulmonary hypertension (high blood pressure in the lungs). Your cardiologist can determine if the defect should be closed.

Ongoing Care Medical After an ASD is closed, patients need follow-up with a cardiologist. Only rarely will they need to take medicine. Your cardiologist can monitor you with noninvasive tests if needed. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms. They will help show if more procedures, such as a cardiac catheterization, are needed.

Activity Restrictions Activity restrictions are almost never needed unless there are associated problems that you and your doctor have discussed. Endocarditis Prevention Not needed. Problems You May Have People with repaired atrial septal defects rarely have any problems. Those who have palpitations or faint need to be reevaluated by their cardiologist and may need medical therapy. Also, if the ASD is diagnosed late in life, the heart may be less able to pump. This can require diuretics, drugs to help the heart pump better and drugs to control blood pressure. If pulmonary hypertension develops (which is rare), some people may need more medications. Will You Need More Surgery? Once an ASD has been closed, it's unlikely that more surgery will be needed. Rarely, a patient may have a residual hole. Whether it will need to be closed depends upon its size.

Ventricular Septal Defect (VSD)

What It Is The septum is a wall that separates the heart's left and right sides. Septal defects are sometimes called a "hole" in the heart. A defect between the heart's two lower chambers (the ventricles) is called a ventricular septal defect (VSD). When there is a large opening between the ventricles, a large amount of oxygen-rich (red) blood from the heart's left side is forced through the defect into the right side. Then it's pumped back to the lungs, even though it's already been refreshed with oxygen. This is inefficient, because already-oxygenated blood displaces blood that needs oxygen. This means the heart, which must pump more blood, may enlarge from the added work. High blood pressure may occur in the lungs' blood vessels because more blood is there. Over time, this increased pulmonary hypertension may permanently damage the blood vessel walls. If the opening between the ventricles is small, it doesn't strain the heart. In that case, the only abnormal finding is a loud murmur. Surgical Therapy Closing small ventricular septal defects may not be needed. They often close on their own in childhood or adolescence. But if the opening is large, even in patients with few symptoms, closing the hole in the first two years of life is recommended to

prevent serious problems later. Usually the defect is closed with a patch. Over time the normal heart lining tissue covers the patch, so it becomes a permanent part of the heart. Some defects can be sewn closed without a patch. Repairing a VSD restores the blood circulation to normal. The long-term outlook is good. Ongoing Care Medical After their VSD is closed, patients should be examined regularly by a cardiologist. He or she will make sure that the heart is working normally. Activity Restrictions Most patients won't need to limit their activity. However, if you have pulmonary hypertension or your heart doesn't pump as well as it used to, you may need to limit your activity to how much you can endure. Your cardiologist will help determine if you need to limit your activity. Endocarditis Prevention Unclosed VSDs require endocarditis prophylaxis. After the VSD is successfully closed, preventive treatment is needed only during a six-month healing period. Problems You May Have Most people whose uncomplicated ventricular septal defects are repaired early in life don't have any significant long-term problems. In some people, the heart muscle may be less able to contract. This requires diuretics, agents to help the heart pump better and drugs to control blood pressure. Also, if pulmonary hypertension develops (it's uncommon), you may need more medical therapy. Will You Need More Surgery? People whose ventricular septal defects are repaired rarely need more surgery unless residual defects are seen afterwards. If this occurs, whether you'll need surgery depends upon the size of the residual defects.

Pulmonary Valve Stenosis

What It Is Oxygen-depleted blood is pumped through the pulmonary valve to the lungs to pick up oxygen. Normally the pulmonary valve has three leaflets. If these leaflets are malformed, the valve may become narrowed (stenotic) or leaky (insufficient). The stenosis, insufficiency or both can be mild to severe. Surgical Treatment If the stenosis is severe, the pulmonary valve must be opened to increase blood flow to the lungs. A balloon-tipped catheter is used to do this. It dilates (widens) the valve when the balloon is inflated. The valve also may be opened during open-heart surgery. If the insufficiency is severe, an operation is required to repair or replace the pulmonary valve. Ongoing Care Medical If your pulmonary stenosis or insufficiency was severe, you'll need ongoing care to check for restenosis or increasing insufficiency. If it's mild or moderate, you probably won't need surgical treatment at first. But you will need ongoing follow-up so your cardiologist can check for arrhythmias or any signs of strain or dilation of your right

ventricle. (The right ventricle is the heart chamber that pumps blood through the pulmonary valve.) Activity Restrictions If you have mild to moderate pulmonary stenosis or insufficiency, you probably won't need to limit your physical activity. If it's severe, you may need to limit your activity because of strain or dilation of the right ventricle or arrhythmias. Ask your cardiologist about exercise recommendations. Endocarditis Prevention If you have a stenotic or insufficient pulmonary valve, you'll need to take antibiotics to prevent endocarditis. Problems You May Have Symptoms If you have mild pulmonary stenosis or insufficiency, you probably won't have any symptoms. If it's moderate or severe, you may not tolerate exercise well and may have shortness of breath or palpitations. Pregnancy You'll likely handle pregnancy well if you have mild or moderate stenosis or regurgitation. If your lesion is severe, pregnancy may be higher risk, particularly if you have an artificial valve. Your cardiologist should be involved in your care during pregnancy and delivery. Will You Need More Surgery? If you develop problems with your right ventricle due to pulmonary stenosis or insufficiency (see above), or you have significant heart rhythm problems, you may need surgery again. Your pulmonary valve may be repaired or replaced during openheart surgery. A stenotic valve also may be dilated with a balloon catheter in the cardiac catheterization laboratory. Sometimes an ablation procedure is also required to control arrhythmias.

Coarctation of the Aorta

What It Is The aorta is the body's main artery. It distributes oxygen-rich blood to all parts of the body except the lungs. The first branches of the aorta go to the upper body (arms and head). After that, blood goes to the lower body (abdomen and legs). Coarctation of the aorta is a narrowing of the aorta between the upper-body artery branches and the branches to the lower body. This blockage can increase blood pressure in your arms and head, reduce pressure in your legs and seriously strain your heart. Aortic valve abnormalities often accompany coarctation. Surgical Treatment The narrowing can be removed by surgery or sometimes by a nonsurgical balloon dilation in the cardiac catheterization lab. Aortic coarctation may return even after successful surgery or balloon dilation. This isn't uncommon if your repair was done when you were a newborn. (It's uncommon if it was repaired when you were a child.) If you've reached your full adult size and have no blood pressure difference between your arms and legs, it's highly unlikely that your aorta will become obstructed again. Recurrent coarctation is usually treated with nonsurgical balloon dilation or by implanting a stent using cardiac catheterization. Ongoing Care

Medical After the coarctation is repaired, you'll need your blood pressure checked every 1-2 years. The reason is that you're at higher risk of developing generalized high blood pressure or problems with your aortic valve. Both of these can be checked for during your routine cardiology visits. Activity Restrictions Depending on your blood pressure at rest or during exercise, you may be advised to avoid some forms of strenuous exercise. Heavy isometric exercise, such as power weightlifting, may be a particular concern if your pressure is elevated. In general, you don't need to restrict activity if your arm and leg blood pressures are normal. Ask your cardiologist if you should limit any activity. Endocarditis Prevention You may need antibiotics before certain dental or surgical procedures if you have an aortic obstruction or aortic valve abnormality. Pregnancy Most women with repaired coarctation shouldn't have any difficulties, unless there's residual aortic obstruction or generalized high blood pressure. However, if you have persistent coarctation or any associated problems that might affect you or your baby, check with your physician before considering getting pregnant. Problems You May Have Symptoms Coarctation of the aorta usually doesn't have symptoms. However, if the obstruction becomes severe, you may not tolerate exercise well. You could have a headache or leg pains after exertion. You also might have chest pain or palpitations. Tell your cardiologist promptly about any activity-related symptoms. Will You Need More Surgery? The need for surgery or catheterization depends mostly on the level of pressure in your arms and legs when you're resting and, under some circumstances, during exercise. If your arm and leg blood pressures are normal, you probably won't need more intervention.

Patent Ductus Arteriosus

Definition:

Patent ductus arteriosus (PDA) is a condition where the ductus arteriosus, a blood vessel that allows blood to bypass the baby's lungs before birth, fails to close after birth. The word "patent" means open. Prior to birth, bloodflow in the fetus bypasses its lungs because the fetus gets oxygen through the placenta. After birth, the ductus arteriosus and foramen ovale close because blood must then go to the infant's lungs. Alternative Names: PDA Causes, incidence, and risk factors: PDA occurs in about 1 in 2,000 infants. Premature infants and those with respiratory distress syndrome are at higher risk. PDA is also associated with coarctation of the aorta, transposition of the great vessels, and ventricular septal defect. Symptoms: poor feeding habits shortness of breath Note: There may be no symptoms. Signs and tests: Patients with PDA have a characteristic heart murmur that can be heart with a stethoscope. The diagnosis is confirmed with an echocardiogram .

Heart, section through the middle. The interior of the heart is composed of valves, chambers, and associated vessels.

Patent ductus arteriosis (PDA). The heart pumps blood throughout the body. It is located in the thorax. Treatment: In newborns, fluid restriction and medications (indomethacin, ibuprofen) are used initially to close PDAs. If these measures do not work or can't be used, surgical ligation (clipping) is performed. Surgical treatment of PDAs is performed on older children even if they have no symptoms because the PDA will not close by itself. Endovascular coils placed in the PDA have been used successfully as an alternative to surgery. Expectations (prognosis): The surgery has a low risk of complications in experienced hands, and the outcome is mostly related to the patient's pre-existing medical condition(s). Complications: If the patent ductus is not closed, the infant has a risk of developing heart failure or infective endocarditis.