THE CARDIOVASCULAR SYSTEM

The cardiovascular system includes the heart and the blood vessels. The heart pumps blood, and the blood vessels channel and deliver it throughout the body. Arteries carry blood filled with nutrients away from the heart to all parts of the body. The blood is sometimes compared to a river, but the arteries are more like a river in reverse. Arteries are thick-walled tubes with a circular covering of yellow, elastic fibers, which contain a filling of muscle that absorbs the tremendous pressure wave of a heartbeat and slows the blood down. This pressure can be felt in the arm and wrist - it is the pulse. Eventually arteries divide into smaller arterioles and then into even smaller capillaries, the smallest of all blood vessels. One arteriole can serve a hundred capillaries. Here, in every tissue of every organ, blood's work is done when it gives up what the cells need and takes away the waste products that they don't need. Now the river comparison really does apply. Capillaries join together to form small veins, which flow into larger main veins, and these deliver deoxygenated blood back to the heart. Veins, unlike arteries, have thin, slack walls, because the blood has lost the pressure which forced it out of the heart, so the dark, reddish-blue blood which flows through the veins on its way to the lungs oozes along very slowly on its way to be reoxygenated. Back at the heart, the veins enter a special vessel, called the pulmonary arteries, into the wall at right side of the heart. It flows along the pulmonary arteries to the lungs to collect oxygen, then back to the heart's left side to begin its journey around the body again.

Heart Anatomy

The heart weighs between 7 and 15 ounces (200 to 425 grams) and is a little larger than the size of your fist. By the end of a long life, a person's heart may have beat (expanded and contracted) more than 3.5 billion times. In fact, each day, the average heart beats 100,000 times, pumping about 2,000 gallons (7,571 liters) of blood.

Your heart is located between your lungs in the middle of your chest, behind and slightly to the left of your breastbone (sternum). A double-layered membrane called the pericardium surrounds your heart like a sac. The outer layer of the pericardium surrounds the roots of your heart's major blood vessels and is attached by ligaments to your spinal column, diaphragm, and other parts of your body. The inner layer of the pericardium is attached to the heart muscle. A coating of fluid separates the two layers of membrane, letting the heart move as it beats, yet still be attached to your body. Your heart has 4 chambers. The upper chambers are called the left and right atria, and the lower chambers are called the left and right ventricles. A wall of muscle called the septum separates the left and right atria and the left and right ventricles. The left ventricle is the largest and strongest chamber in your heart. The left ventricle's chamber walls are only about a half-inch thick, but they have enough force to push

blood through the aortic valve and into your body. The Heart Valves

Four types of valves regulate blood flow through your heart:

The tricuspid valve regulates blood flow between the right atrium and right ventricle. The pulmonary valve controls blood flow from the right ventricle into the pulmonary arteries, which carry blood to your lungs to pick up oxygen. The mitral valve lets oxygen-rich blood from your lungs pass from the left atrium into the left ventricle. The aortic valve opens the way for oxygen-rich blood to pass from the left ventricle into the aorta, your body's largest artery, where it is delivered to the rest of your body.

The Heartbeat
A heartbeat is a two-part pumping action that takes about a second. As blood collects in the upper chambers (the right and left atria), the heart's natural pacemaker (the SA node) sends out an electrical signal that causes the atria to contract. This contraction pushes blood through the tricuspid and mitral valves into the resting lower chambers (the right and left ventricles). This part of the two-part pumping phase (the longer of the two) is called diastole. The second part of the pumping phase begins when the ventricles are full of blood. The electrical signals from the SA node travel along a pathway of cells to the ventricles,

causing them to contract. This is called systole. As the tricuspid and mitral valves shut tight to prevent a back flow of blood, the pulmonary and aortic valves are pushed open. While blood is pushed from the right ventricle into the lungs to pick up oxygen, oxygenrich blood flows from the left ventricle to the heart and other parts of the body. After blood moves into the pulmonary artery and the aorta, the ventricles relax, and the pulmonary and aortic valves close. The lower pressure in the ventricles causes the tricuspid and mitral valves to open, and the cycle begins again. This series of contractions is repeated over and over again, increasing during times of exertion and decreasing while you are at rest. The heart normally beats about 60 to 80 times a minute when you are at rest, but this can vary. As you get older, your resting heart rate rises. Also, it is usually lower in people who are physically fit. Your heart does not work alone, though. Your brain tracks the conditions around you climate, stress, and level of physical activityand adjusts your cardiovascular system to meet those needs. The human heart is a muscle designed to remain strong and reliable for a hundred years or longer. By reducing your risk factors for cardiovascular disease, you may help your heart stay healthy longer.

The Conduction System

Electrical impulses from your heart muscle (the myocardium) cause your heart to contract. This electrical signal begins in the sinoatrial (SA) node, located at the top of the right atrium. The SA node is sometimes called the heart's "natural pacemaker." An electrical impulse from this natural pacemaker travels through the muscle fibers of the

atria and ventricles, causing them to contract. Although the SA node sends electrical impulses at a certain rate, your heart rate may still change depending on physical demands, stress, or hormonal factors. The Circulatory System Your heart and circulatory system make up your cardiovascular system. Your heart works as a pump that pushes blood to the organs, tissues, and cells of your body. Blood delivers oxygen and nutrients to every cell and removes the carbon dioxide and waste products made by those cells. Blood is carried from your heart to the rest of your body through a complex network of arteries, arterioles, and capillaries. Blood is returned to your heart through venules and veins. If all the vessels of this network in your body were laid end-to-end, they would extend for about 60,000 miles (more than 96,500 kilometers), which is far enough to circle the earth more than twice!

Common Abnormalities 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 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 Exams 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)

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 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

Exams 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 ATRIOVENTRICULAR CANAL DEFECT

Atrioventricular canal defect is a combination of several abnormalities in the heart present at birth (congenital abnormalities). This defect, which is sometimes called endocardial cushion defect or atrioventricular septal defect, occurs when there's a hole between the chambers of the heart and problems with the valves that regulate blood flow in the heart. Atrioventricular canal defect allows extra blood to circulate to the lungs. Ensuing problems overwork the heart and cause it to enlarge. Atrioventricular canal defect is often associated with Down syndrome. If left untreated, atrioventricular canal defect may cause heart failure and high blood pressure in the lungs. To fix this defect, doctors often recommend surgery during the first year of life to close the hole and reconstruct the valves. Symptoms There are two common types of atrioventricular canal defect partial and complete. The partial form involves only the two upper chambers of the heart. The complete form allows blood to travel freely among all four chambers of the heart. In either type, extra blood circulates in the lungs.

Signs and symptoms of complete atrioventricular canal defect usually develop in the first several weeks of life. They include: Difficulty breathing (dyspnea) Lack of appetite Poor weight gain

Bluish discoloration of the lips and skin (cyanosis) If your baby has complete atrioventricular canal defect, he or she may also develop signs and symptoms of heart failure, including: Fatigue Wheezing Swelling (edema) in the legs, ankles and feet Sudden weight gain from fluid retention Excessive sweating Decreased alertness Irregular or rapid heartbeat Partial Atrioventricular canal defect Atrioventricular canal defect is a combination of several abnormalities in the heart present at birth (congenital abnormalities). This defect, which is sometimes called endocardial cushion defect or atrioventricular septal defect, occurs when there's a hole between the chambers of the heart and problems with the valves that regulate blood flow in the heart.

Atrioventricular canal defect allows extra blood to circulate to the lungs. Ensuing problems overwork the heart and cause it to enlarge. Signs and symptoms of a partial atrioventricular canal defect may not appear until early adulthood. When they do become noticeable, signs and symptoms may be related to complications that develop as a result of the defect, and may include: Abnormal heartbeat (arrhythmia) Heart failure High blood pressure in the lungs (pulmonary hypertension) Causes Chambers and the valves of the heart

A normal heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.

Atrioventricular canal defect occurs during fetal growth when your baby's heart is developing. While some factors, such as Down syndrome, may increase the risk of atrioventricular canal defect, in most cases the cause is unknown. The normal-functioning heart

Your heart is divided into four chambers, two on the right and two on the left. In performing its basic job pumping blood throughout your body your heart uses its left and right sides for different tasks. The right side moves blood into vessels that lead to your lungs. In your lungs, oxygen enriches your blood, which circulates to your heart's left side. The left side of your heart pumps blood into a large vessel called the aorta, which circulates blood to the rest of your body. Valves control the flow of blood into and out of the chambers of your heart. These valves open to allow blood to move to the next chamber or to one of the arteries, and they close to keep blood from flowing backward. A hole in the wall

In partial atrioventricular canal defect, a hole exists in the wall (septum) that separates the upper chambers (atria) of the heart. Also, the mitral valve between the upper and lower left chambers may not close completely (mitral valve regurgitation). In complete atrioventricular canal defect, there's a large hole in the center of the heart where the walls between the upper chambers (atria) and lower chambers (ventricles) meet. Instead of two separate valves one on the right (tricuspid) and one on the left (mitral) one large common valve exists between the upper and lower chambers. And, this valve may not close tightly. Oxygen-rich and oxygen-poor blood mix through the hole in the septum, and the abnormal valves leak blood into the heart's lower chambers (ventricles). These problems make the heart work harder, causing it to enlarge. Complications Treating an atrioventricular canal defect helps your child avoid potential complications, such as: Pneumonia. If your baby has an untreated atrioventricular canal defect, he or she may have recurrent bouts of pneumonia a serious lung infection. Enlargement of the heart (cardiomegaly). Increased blood flow through the heart forces it to work harder than normal, causing it to enlarge. Heart failure. Untreated, atrioventricular canal defect will usually result in heart failure a condition in which the heart is unable to pump enough blood to meet the body's needs. High blood pressure in the lungs (pulmonary hypertension). When the heart's left ventricle weakens and can't pump out enough blood, the increase in pressure backs up through the pulmonary veins to the arteries in the lungs, causing high blood pressure in the lungs. Complications later in life

Although treatment greatly improves the outlook for children with atrioventricular canal defect, some who have corrective surgery may still be at risk of the following associated conditions later in life: Leaky heart valves (regurgitation) Narrowing of the heart valves (stenosis) Heart rhythm abnormalities (arrhythmias) Breathing difficulties associated with damage to the lungs (pulmonary vascular

disease) Common signs and symptoms of these complications include shortness of breath, fatigue and a rapid, fluttering heartbeat, among others. In some cases, such as a leaky heart valve, a second surgery may be needed. Tests and diagnosis

If your baby has a partial atrioventricular canal defect, his or her doctor may not detect that anything's wrong right away. However, if your baby has a complete atrioventricular canal defect, signs and symptoms usually become noticeable within the first few weeks of life. Tests that help diagnose an atrioventricular canal defect include: Physical exam. Your baby's doctor may suspect a heart defect such as atrioventricular canal defect if your baby is having trouble breathing or having difficulty feeding and gaining weight. Your doctor may also suspect a heart defect if he or she hears a heart murmur an abnormal whooshing sound caused by turbulent blood flow. Echocardiogram. Doctors typically use an echocardiogram to diagnose atrioventricular canal defect. This test uses high-pitched sound waves that bounce off the heart to produce moving images that the doctor can view on a video screen. In a baby with atrioventricular canal defect, the echocardiogram reveals a hole in the wall between the heart chambers and abnormal valves. Because this test can track blood flow, it also shows blood moving through the hole from the left side to the right side of the heart, allowing oxygen-rich and oxygen-poor blood to mix. Cardiac catheterization. In some cases, doctors may use cardiac catheterization to diagnose atrioventricular canal defect. During this procedure, the doctor inserts a thin flexible tube (catheter) into an artery or vein in the groin and advances it up to the heart. A dye is injected through the catheter to make the heart structures visible on X-ray pictures. The catheter also allows the doctor to measure pressure in the chambers of the heart and in the blood vessels.

Transposition of the great arteries

Transposition of the great arteries is a rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). Transposition of the great arteries changes the way blood circulates through the body, leaving a shortage of oxygen in blood flowing from the heart to the rest of the body. Without an adequate supply of oxygen-rich blood, the body can't function properly. Transposition of the great arteries is usually detected within the first few weeks of life. Corrective surgery soon after birth is the usual treatment for transposition of the great arteries. Having a baby with transposition of the great arteries is naturally worrisome to you as a parent, but with proper treatment, the outlook is promising. Causes Transposition of the great arteries occurs during fetal growth when your baby's heart is developing. Why this defect occurs is unknown in most cases. Normally, the pulmonary artery which carries blood from your heart to your lungs to receive oxygen is attached to the lower right chamber (right ventricle). From your

lungs, the oxygen-rich blood goes to your heart's upper left chamber (left atrium), through the mitral valve into the lower left chamber (left ventricle). The aorta is normally attached to the left ventricle. It carries oxygen-rich blood out of your heart back to the rest of your body. In transposition of the great arteries, the positions of the pulmonary artery and the aorta are switched. The pulmonary artery is connected to the left ventricle, and the aorta is connected to the right ventricle. Oxygen-poor blood circulates through the right side of the heart and back to the body without passing through the lungs. Oxygen-rich blood circulates through the left side of the heart and right back into the lungs without being circulated to the rest of the body. Circulation of oxygen-poor blood through the body causes the skin to have a blue tint (cyanosis). Because of this, transposition of the great arteries is called a congenital cyanotic heart defect. Although some factors, such as rubella or other viral illnesses during pregnancy, maternal age over 40, or maternal diabetes, may increase the risk of this condition, in most cases the cause is unknown. Symptoms Transposition of the great arteries symptoms include: Blue color of the skin (cyanosis) Shortness of breath Lack of appetite Poor weight gain Complications Potential complications of transposition of the great arteries include: Lack of oxygen to tissues. Your baby's tissues may be getting too little oxygen (hypoxia). Heart failure. Unless there's some mixing of oxygen-rich blood and oxygen-poor blood within the body, heart failure a condition in which the heart can't pump enough blood to meet the body's needs may develop. Lung damage. The lack of oxygenated blood causes damage to the lungs, making breathing difficult.

Tests and diagnosis Your baby's doctor may also suspect a heart defect if he or she hears a heart murmur an abnormal whooshing sound caused by turbulent blood flow. A physical exam alone isn't enough to accurately diagnose transposition of the great arteries, however. One or more of the following tests are necessary for an accurate diagnosis: Echocardiography. An echocardiogram is an ultrasound of the heart it uses sound waves that bounce off your baby's heart and produce moving images that can be viewed on a video screen. Doctors use this test to diagnose transposition of the great arteries by looking at the position of the aorta and the pulmonary artery. Echocardiograms can also identify associated heart defects, such as a ventricular septal defect or an atrial septal defect. Chest X-ray. Although a chest X-ray doesn't provide a definitive diagnosis of transposition of the great arteries, it does allow the doctor to see your baby's heart size and see the position of the aorta and pulmonary artery. Electrocardiogram. An electrocardiogram records the electrical activity in the heart each time it contracts. During this procedure, patches with wires (electrodes) are placed on your baby's chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper. Cardiac catheterization. Because of its invasive nature, this procedure is typically done only when other tests, such as echocardiography, don't yield enough information to make a diagnosis. During a cardiac catheterization, the doctor inserts a thin flexible tube (catheter) into an artery or vein in your baby's groin and weaves it up to his or her heart. A dye is injected through the catheter to make your baby's heart structures visible on X-ray pictures. The catheter also measures pressure in the chambers of your baby's heart and in the blood vessels.

Tetralogy of Fallot

Tetralogy of Fallot (fuh-LOE) is a rare condition caused by the combination of four heart defects that are present at birth. These defects, which affect the structure of the heart, cause oxygen-poor blood to flow out of the heart and into the rest of the body. Infants and children with tetralogy of Fallot usually have blue-tinged skin because their blood doesn't carry enough oxygen. Tetralogy of Fallot is often diagnosed during infancy or soon after. However, tetralogy of Fallot may not be detected until later in life, depending on the severity of the defects and symptoms. With early diagnosis followed by appropriate treatment, most children with tetralogy of Fallot live relatively normal lives, though they'll need regular medical care and may have restrictions on exercise. Causes Tetralogy of Fallot occurs during fetal growth, when the baby's heart is developing. While factors such as poor maternal nutrition, viral illness or genetic disorders may increase the risk of this condition, in most cases the cause of tetralogy of Fallot is unknown. The four abnormalities that make up the tetralogy of Fallot include:

Pulmonary valve stenosis. This is a narrowing of the pulmonary valve, the flap that separates the right ventricle of the heart from the pulmonary artery, the main blood vessel leading to the lungs. Constriction of the pulmonary valve reduces blood flow to the lungs. The narrowing may also affect the muscle beneath the pulmonary valve. Ventricular septal defect. This is a hole in the wall that separates the two lower chambers (ventricles) of the heart. The hole allows deoxygenated blood in the right ventricle blood that has circulated through the body and is en route to the lungs to replenish its oxygen supply to flow into the left ventricle and mix with oxygenated blood fresh from the lungs. Blood from the left ventricle also flows back to the right ventricle in an inefficient manner. This ability for blood to flow through the ventricular septal defect dilutes the supply of oxygenated blood to the body and eventually can weaken the heart. Overriding aorta. Normally the aorta, the main artery leading out to the body, branches off the left ventricle. In tetralogy of Fallot, the aorta is shifted slightly to the right and lies directly above the ventricular septal defect. In this position the aorta receives blood from both the right and left ventricles, mixing the oxygenpoor blood from the right ventricle with the oxygen-rich blood from the left ventricle. Right ventricular hypertrophy. When the heart's pumping action is overworked, it causes the muscular wall of the right ventricle to enlarge and thicken. Over time this may cause the heart to stiffen, become weak and eventually fail. Symptoms Tetralogy of Fallot symptoms vary, depending on the extent of obstruction of blood flow out of the right ventricle and into the lungs. Signs and symptoms may include: A bluish coloration of the skin caused by blood low in oxygen (cyanosis) Shortness of breath and rapid breathing, especially during feeding Loss of consciousness (fainting) Clubbing of fingers and toes an abnormal, rounded shape of the nail bed Poor weight gain Tiring easily during play Irritability Prolonged crying A heart murmur

Complications All babies with tetralogy of Fallot need corrective surgery. Without treatment, your baby may not grow and develop properly. He or she is also at increased risk of serious complications, such as infective endocarditis, an inflammation of the inner lining of the heart caused by a bacterial infection. Untreated cases of tetralogy of Fallot usually develop severe complications over time, which may result in death or disability by early adulthood. Tests and diagnosis After your baby is born, your baby's doctor may suspect tetralogy of Fallot if the baby has blue-tinged skin or if a heart murmur an abnormal whooshing sound caused by turbulent blood flow is heard in your child's chest. By using several tests, your doctor can confirm the diagnosis. Chest X-ray. A typical sign of tetralogy of Fallot on an X-ray is a "boot-shaped" heart, because the right ventricle is enlarged. Blood test. Your child will need a test that measures the number of each type of cell in the blood, called a complete blood count. In tetralogy of Fallot, the number of red blood cells may be abnormally high (erythrocytosis) as the body attempts to increase the oxygen level in the blood. Oxygen level measurement (pulse oximetry). This test uses a small sensor that can be placed on a finger or toe to measure the amount of oxygen in the blood. Echocardiography. Echocardiograms use high-pitched sound waves, inaudible to the human ear, to produce an image of the heart. Sound waves bounce off your baby's heart and produce moving images that can be viewed on a video screen. This test helps diagnose tetralogy of Fallot because it allows the doctor to see whether there is a ventricular septal defect, if the structure of the pulmonary valve is normal, if the right ventricle is functioning properly, and if the aorta is positioned properly. Electrocardiogram. An electrocardiogram records the electrical activity in the heart each time it contracts. During this procedure, patches with wires (electrodes) are placed on your baby's chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper. This test helps determine if your baby's right ventricle is enlarged (ventricular hypertrophy) and if the heart rhythm is regular. Cardiac catheterization. During this procedure, your doctor inserts a thin flexible tube (catheter) into an artery or vein in your baby's groin and threads it up to his or her heart. A dye is injected through the catheter to make your baby's heart structures visible on X-ray pictures. The catheter also measures pressure

and oxygen levels in the chambers of the heart and in the blood vessels.

DOUBLE OUTLET RIGHT VENTRICLE

Double outlet right ventricle (DORV) is a congenital heart disease in which the aorta rises from the right ventricle (the chamber of the heart that pumps blood to the lungs), instead of from the left ventricle (the normal pumping chamber to the body). Both the pulmonary artery (which carries oxygen-poor blood to the lungs) and aorta (which carries oxygen-rich blood from the heart to the body) come from the same pumping chamber. No arteries arise from the left ventricle (the normal pumping chamber to the body). Causes Normally, the aorta arises from the left ventricle (the chamber of the heart that usually pumps blood to the body). The pulmonary artery normally arises from the right ventricle. In DORV, both arteries arise from the right ventricle. This is a problem because the right ventricle carries oxygen-poor blood, which the aorta then carries throughout the body. DORV always includes a ventricular septal defect (VSD). Pulmonary valve stenosis or transposition of the great arteries may also be part of the defect. The presence of a VSD helps the infant with DORV, because oxygen-rich blood from the lungs flows from the left side of the heart, through the VSD opening and into the right chamber, mixing with the oxygen-poor blood. However, the body may still not get enough oxygen even with this mixture, and the heart has to work harder to try to bring more oxygen-rich blood to the body. In addition, because the pulmonary artery receives blood from both ventricles instead of just the right one, blood pressure in the lungs increases. There are several types of DORV. The difference between these types is the location of the VSD compared to the location of the pulmonary artery and aorta. The type of DORV, and the presence or absence of pulmonary valve stenosis, affect the severity of signs and symptoms the baby may have. Patients with DORV often have other heart abnormalities such as: Anomalous coronary arteries Coarctation of the aorta Mitral valve problems Pulmonary atresia Pulmonary valve stenosis

Right aortic arch Transposition of the great arteries Symptoms Symptoms of DORV may include: Baby tires easily, especially when feeding Bluish skin color (the lips may also be blue) Clubbing (thickening of the nail beds) on toes and fingers (late sign) Failure to gain weight and grow Pale coloring Sweating Swollen legs or abdomen Trouble breathing Exams and Tests Signs of DORV may include: Enlarged heart Heart murmur Rapid breathing Rapid heartbeat Tests to diagnose DORV include: Chest x-rays Passing a thin, flexible tube into the heart to measure blood pressure and inject dye for special pictures of the heart and arteries (cardiac catheterization) Ultrasound exam of the heart (echocardiogram) Using magnets to create images of the heart (MRI)

TRUNCUS ARTERIOSUS
Truncus arteriosus is a rare type of congenital heart disease in which a single blood vessel (truncus arteriosus) comes out of the right and left ventricles, instead of the normal two (pulmonary artery and aorta). There are different types of truncus arteriosus, depending on the anatomy of the single vessel. Causes In normal circulation, the pulmonary artery comes out of the right ventricle and the

aorta comes out of the left ventricle, which are separate from each other. Coronary arteries (which supply blood to the heart muscle) come out of the aorta just above the valve at the entrance of the aorta. In truncus arteriosus, a single artery comes out of the ventricles. There is usually also a large hole between the two ventricles (ventricular septal defect). As a result, the blue (without oxygen) and red (oxygen-rich) blood mix. Some of this mixed blood goes to the lungs, some goes to the coronary arteries, and the rest goes to the body. Usually, too much blood is sent to the lungs. If left untreated, two problems occur: Too much blood circulation in the lungs may cause extra fluid to build up in and around them, making it difficult to breathe. The blood vessels to the lungs become permanently damaged. Over time, it becomes very hard for the heart to force blood to them. This is called pulmonary hypertension and it can be life-threatening. Symptoms

Bluish skin (cyanosis) Delayed growth or growth failure Fatigue Lethargy Poor feeding Rapid breathing (tachypnea) Shortness of breath (dyspnea) Widening of the finger tips (clubbing)

Exams and Tests A murmur is usually heard when listening to the heart with a stethoscope. Tests include: ECG Echocardiogram Heart catheterization (only needed in rare cases to help with the diagnosis or in planning a treatment strategy) MRI of the heart X-ray of the chest

COARTION OF THE AORTA

Coarctation of the aorta is a narrowing, or constriction, in a portion of the aorta. The condition forces the heart to pump harder to get blood through the aorta and on to the rest of the body. 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 oxygenrich 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.

Causes

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. 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 Complications Untreated coarctation of the aorta frequently leads to complications. Several of the complications are a result of long-standing high blood pressure caused by the aortic coarctation. Complications of coarctation of the aorta include: High blood pressure Stroke Rupture of the aorta Premature coronary artery disease narrowing of the blood vessels that supply the heart Weakened or bulging artery in the brain (cerebral aneurysm) Tests and diagnosis The age at which people with coarctation of the aorta are diagnosed depends on the severity of the condition. If the aortic coarctation is severe, it's usually diagnosed during infancy. Diagnostic tests

Tests to confirm a diagnosis of coarctation of the aorta include: Chest X-ray. X-rays produce pictures by passing an X-ray beam through your body. A chest X-ray may show an enlarged heart or a narrowing in the aorta at the site of the coarctation. Echocardiogram. Echocardiograms use high-pitched sound waves to produce an image of your heart. Sound waves bounce off your heart and produce moving images that can be viewed on a video screen. An echocardiogram may detect the location and severity of the aortic coarctation and can show other heart defects, such as a bicuspid aortic valve. Electrocardiogram (ECG). An ECG test records the electrical activity in your heart each time it contracts. For this procedure, patches with wires (electrodes) are placed on your chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper or a computer monitor. If the coarctation of the aorta is severe, the ECG will show that you might have a thickened heart muscle (ventricular hypertrophy). Magnetic resonance imaging (MRI). An MRI scan is a test that uses a magnetic field and pulses of radio wave energy to make pictures of your body.

An MRI of your chest will reveal the location of the coarctation of the aorta and determine whether it affects other blood vessels in your body. Cardiac catheterization. During this procedure, your doctor inserts a thin flexible tube (catheter) into an artery or vein in your groin and threads it up to your heart. A dye is injected through the catheter to make your heart structures visible on X-ray pictures. Cardiac catheterization helps determine the severity of the aortic coarctation.

AORTIC VALVE STENOSIS

Aortic valve stenosis or aortic stenosis occurs when the heart's aortic valve narrows. This narrowing prevents the valve from opening fully, which obstructs blood flow from your heart into your aorta and onward to the rest of your body. When the aortic valve is obstructed, your heart needs to work harder to pump blood to your body. Eventually, this extra work weakens your heart and limits the amount of blood it can pump, leading to symptoms, such as fatigue and dizziness. If you have severe aortic valve stenosis, you'll usually need surgery to replace the valve. Left untreated, aortic valve stenosis can lead to serious heart problems.

Normal heart and aortic valve stenosis

Causes Aortic valve stenosis is narrowing of the aortic valve. Many things can narrow this passageway between your heart and aorta. Causes of aortic valve stenosis include: Congenital heart defect. Rarely, some babies are born with an already narrowed aortic valve. Others are born with an aortic valve that has only two flaps (leaflets) not three. Known as a bicuspid aortic valve, this deformity may not cause any problems until adulthood, at which time the valve may begin too narrow or leak and may need to be repaired or replaced. Having a bicuspid aortic valve requires regular evaluation by a doctor to watch for signs of valve malfunction. In most cases, doctors don't know why a heart valve fails to develop properly, so it isn't something you could have prevented. Calcium buildup on the valve. With age, heart valves may accumulate deposits of calcium (aortic valve calcification). Calcium is a mineral found in your blood. As blood repeatedly flows over the aortic valve, deposits of calcium can accumulate on the valve's leaflets. These deposits may never cause any problems. However, in some people particularly those with a bicuspid aortic

valve calcium deposits result in stiffening of the leaflets of the valve. This stiffening narrows the aortic valve. This cause of aortic valve stenosis is most common in people older than 65, and symptoms often don't appear until age 70. Rheumatic fever. A complication of strep throat infection, rheumatic fever may result in scar tissue forming on the aortic valve. Scar tissue alone can narrow the aortic valve and lead to aortic valve stenosis. Scar tissue can also create a rough surface on which calcium deposits can collect, contributing to aortic valve stenosis later in life. Rheumatic fever may damage more than one heart valve, and in more than one way. A damaged heart valve may not open fully or close fully or both. While rheumatic fever is rare in the United States, some older adults had rheumatic fever as children. Complications Aortic valve stenosis of any cause can be a serious condition because it can weaken the heart. If the aortic valve is narrowed, the left ventricle has to work harder to pump a sufficient amount of blood into the aorta and onward to the rest of your body. In response, the left ventricle may thicken and enlarge. At first these adaptations help the left ventricle pump blood with more force. But eventually these changes weaken the left ventricle and your heart overall. Left unchecked, aortic valve stenosis can lead to life-threatening heart problems, including:

Heart failure Irregular heart rhythms (arrhythmias) Cardiac arrest Chest pain (angina)

Aortic valve stenosis also increases the risk of bacteria entering your bloodstream and causing an infection in your heart (endocarditis). If the aortic valve is narrowed, it's more prone to infection than a healthy valve. If you have aortic valve stenosis, you may need to take antibiotics before certain dental or medical procedures that may increase the risk of endocarditis. Tests and diagnosis

Diagnostic tests Electrocardiogram (ECG). In this test, patches with wires (electrodes) are attached to your skin to measure the electrical impulses given off by your heart. Impulses are recorded as waves displayed on a monitor or printed on paper. An ECG can provide clues about whether the left ventricle is thickened or enlarged, a problem which can occur with aortic valve stenosis. Chest X-ray. An X-ray image of your chest allows your doctor to check the size and shape of your heart, to determine whether the left ventricle is enlarged a possible indicator of aortic valve stenosis. A chest X-ray can also reveal calcium deposits on the aortic valve. In addition, a chest X-ray helps your doctor check the condition of your lungs. Aortic valve stenosis may lead to blood and fluid backing up in your lungs, which causes congestion that may be visible on an Xray. Echocardiogram. This test uses sound waves to produce an image of your heart. In an echocardiogram, sound waves are directed at your heart from a wand-like device (transducer) held on your chest. The sound waves bounce off your heart and are reflected back through your chest wall and processed electronically to provide video images of your heart. An echocardiogram helps your doctor closely examine the aortic valve to check for problems. A specific type of echocardiogram, a Doppler echocardiogram, may be used to help your doctor determine the severity of your aortic valve stenosis and to check for any leakage (regurgitation). Cardiac catheterization. Your doctor may order this procedure if noninvasive tests haven't provided enough information to firmly diagnosis the type or severity of your heart condition. Your doctor threads a thin tube (catheter) through an artery in your arm or groin to an artery in your heart. A dye injected through the catheter fills your heart's arteries, and the arteries become visible on an X-ray. This test helps show blockages in arteries to your heart that can coexist with aortic valve stenosis and may need surgical treatment along with aortic valve stenosis.