Congenital heart disease Congenital heart disease is a problem with the heart's structure and function that is present

at birth. Causes, incidence, and risk factors Congenital heart disease (CHD) can describe a number of different problems affecting the heart. It is the most common type of birth defect. Congenital heart disease causes more deaths in the first year of life than any other birth defects.Congenital heart disease is often divided into two types: cyanotic (blue skin color caused by a lack of oxygen) and non-cyanotic. The following lists cover the most common congenital heart diseases: Cyanotic: Ebstein's anomaly, Hypoplastic left heart,Pulmonary atresia,Tetralogy of Fallot,Total anomalous pulmonary venous return, Transposition of the great vessels, Tricuspid atresia,Truncus arteriosus Non-cyanotic: Aortic stenosis, Atrial septal defect (ASD), Atrioventricular canal (endocardial cushion defect), Coarctation of the aorta, Patent ductus arteriosus (PDA), Pulmonic stenosis, Ventricular septal defect (VSD)
These problems may occur alone or together. Most children with congenital heart disease do not have other types of birth defects. However, heart defects can be part of genetic and chromosome syndromes. Some of these syndromes may be passed down through families.

Examples include: DiGeorge syndrome, Down syndrome, Marfan syndrome, Noonan syndrome, Trisomy 13, Turner syndrome
Often, no cause for the heart disease can be found. Congenital heart diseases continue to be investigated and researched. Drugs such as retinoic acid for acne, chemicals, alcohol, and infections (such as rubella) during pregnancy can contribute to some congenital heart problems. Poorly controlled blood sugar in women who have diabetes during pregnancy has also been linked to a high rate of congenital heart defects.

Symptoms
Symptoms depend on the condition. Although congenital heart disease is present at birth, the symptoms may not appear right away. Defects such as coarctation of the aorta may not cause problems for many years. Other problems, such as a small ventricular septal defect (VSD), may never cause any problems. Some people with a VSD have a normal activity level and lifespan.

Exams and Tests

Most congenital heart defects are found during a pregnancy ultrasound. When a defect is found, a pediatric heart doctor, surgeon, and other specialists can be there when the baby is delivered. Having medical care ready at the delivery can mean the difference between life and death for some babies. Which tests are done on the baby depend on the defect, and the symptoms.

Treatment

Which treatment is used, and how well the baby responds to it, depends on the condition. Many defects need to be followed carefully. Some will heal over time, while others will need to be treated. Some congenital heart diseases can be treated with medication alone. Others need to be treated with one or more heart surgeries.

Prevention
Women who are expecting should get good prenatal care: Avoid alcohol and illegal drugs during pregnancy. Tell your doctor that you are pregnant before taking any new medicines. Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery. Pregnant women who have diabetes should try to get good control over their blood sugar levels. Certain genes may play a role in congenital heart disease. Many family members may be affected. Talk to your health care provider about genetic screening if you have a family history of congenital heart disease.

CYANOTIC 1.) Ebstein's anomaly

Ebstein's anomaly is a rare heart defect in which parts of the tricuspid valve are abnormal. The tricuspid valve separates the right lower heart chamber (right ventricle) from the right upper heart chamber (right atrium). The condition is congenital,
which means it is present from birth. Causes
The tricuspid valve is normally made of three parts, called leaflets or flaps. The leaflets open to allow blood to move from the right atrium (top chamber) to the right ventricle (bottom chamber) while the heart relaxes. They close to prevent blood from moving from the right ventricle to the right atrium while the heart pumps. In persons with Ebstein's anomaly, the leaflets are unusually deep in the right ventricle. The leaflets are often larger than normal. The defect usually causes the valve to work poorly, and blood may go the wrong way back into the right atrium. The backup of blood flow can lead to heart swelling and fluid buildup in the lungs or liver. Sometimes, not enough blood gets out of the heart into the lungs and the person may appear blue. In most cases, patients also have a hole in the wall separating the heart's two upper chambers and blood flow across this hole may cause oxygen-poor blood to go to the body. There may be narrowing of the valve that leads to the lungs (pulmonary valve). Ebstein's anomaly occurs as a baby develops in the womb. The exact cause is unknown, although the use of certain drugs (such as lithium or benzodiazepines) during pregnancy may play a role. The condition is rare. It is more common in white people.

Symptoms
Symptoms range from mild to very severe. Often, symptoms develop soon after birth and include bluish-colored lips and nails due to low blood oxygen levels. In severe cases, the baby appears very sick and has trouble breathing.Symptoms in older children may include: Cough, Failure to grow, Fatigue, Rapid breathing,

Shortness of breath, Very fast heartbeat

Exams and Tests

Newborns who have a severe leakage across the tricuspid valve will have very low levels of oxygen in their blood and significant heart swelling. The doctor may hear abnormal heart sounds, such as murmur, when listening to the chest with a stethoscope. Tests that can help diagnose this condition include: Chest x-ray, Magnetic resonance imaging (MRI) of the heart, Measurement of the electrical activity of the heart (EKG), Ultrasound of the heart (echocardiogram)

Treatment
Treatment depends on the severity of the defect and the specific symptoms. Medical care may include: Medications to help with heart failure, Oxygen and other breathing support, Surgery to correct the valve may be needed for children who continue to worsen or who have more serious complications

Outlook (Prognosis)
In general, the earlier symptoms develop, the more severe the disease.Some patients may have either no symptoms or very mild symptoms. Others may worsen over time, developing blue coloring (cyanosis), heart failure, heart block, or dangerous heart rhythms.

Possible Complications
A severe leakage can lead to swelling of the heart and liver, and congestive heart failure.Other complications may include: Abnormal heart rhythms (arrhythmias), including abormally fast rhythms (tachyarrhythmias) and abnormally slow rhythms (bradyarrhythmias and heart block), Blood clots from the heart to other parts of the body, Brain abscess

Prevention There is no known prevention, other than talking with your doctor before a pregnancy if you are taking medicines that are thought to be related to developing this disease. You may be able to prevent some of the complications of the disease. For example, taking antibiotics before dental surgery may help prevent endocarditis. 2.) Hypoplastic left heart syndrome
Hypoplastic left heart syndrome occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely. The condition is present at birth (congenital).

Causes
Hypoplastic left heart is a rare type of congenital heart disease. It is more common in males than in females. As with most congenital heart defects, there is no known cause. About 10% of patients with hypoplastic left heart syndrome also have other birth defects. The problem develops before birth when the left ventricle and other structures do not grow properly, including the: Aorta -- the blood vessel that carries oxygen-rich blood from the left ventricle to the entire body, Entrance and exit of the ventricle, Mitral and aortic valves This causes the left ventricle and aorta to be poorly developed, or hypoplastic. In most cases, the left ventricle and aorta are much smaller than normal. In patients with this condition, the left side of the heart is unable to send enough

blood to the body. As a result, the right side of the heart must maintain the circulation for both the lungs and the body. The right ventricle can support the circulation to both the lungs and the body for a while, but this extra workload eventually causes the right side of the heart to fail. The only possibility of survival is a connection between the right and the left side of the heart, or between the arteries and pulmonary arteries (the blood vessels that carry blood to the lungs). Babies are normally born with two of these connections: Foramen ovale (a hole between the right and left atrium), Ductus arteriosus (a small blood vesel that connects the aorta to the pulmonary artery) Both of these connections normally close on their own a few days after birth. In babies with hypoplastic left heart syndrome, blood from the right side of the heart travels through the ductus arteriosus. This is the only way for blood to get to the body. If the ductus arteriosus is allowed to close in a baby with hypoplastic left heart syndrome, the patient may quickly die because no blood will be pumped to the body. Babies with known hypoplastic left heart syndrome are usually started on a medicine to keep the ductus arteriosus open. Because there is little or no flow out of the left heart, blood returning to the heart from the lungs needs to pass through the foramen ovale or an atrial septal defect (a hole connecting the collecting chambers on the left and right sides of the heart) back to the right side of the heart. If there is no foramen ovale, or if it is too small, the baby could die. Patients with this problem have the hole between their atria opened, either with surgery or using a thin, flexible tube (heart catheterization).

Symptoms
At first, a newborn with hypoplastic left heart may appear normal. Symptoms usually occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include: Bluish (cyanosis) or poor skin color, Cold hands and feet (extremities), Lethargy, Poor pulse, Poor suckling and feeding, Pounding heart, Rapid breathing, Shortness of breath. In healthy newborns, bluish color in the hands and feet is a response to cold (this reaction is called peripheral cyanosis). However, a bluish color in the chest or abdomen, lips, and tongue is abnormal (called central cyanosis). It is a sign that there is not enough oxygen in the blood. Central cyanosis often increases with crying.

Exams and Tests

A physical exam may show signs of heart failure: Faster than normal heart rate, Lethargy, Liver enlargement, Rapid breathing. Also, the pulse at various locations (wrist, groin, and others) may be very weak. There are usually (but not always) abnormal heart sounds when listening to the chest. Tests may include: Cardiac catheterization, ECG, Echocardiogram, X-ray of the chest.

Treatment
Once the diagnosis of hypoplastic left heart is made, the baby will be admitted to the neonatal intensive care unit. A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to keep blood circulating to the body by keeping the ductus arteriosus open. These measures do not solve the problem. The condition always requires surgery.

The first surgery, called the Norwood operation, occurs within the baby's first few days of life. Stage I of the Norwood procedure consists of building a new aorta by: Using the pulmonary valve and artery, Connecting the hypoplastic old aorta and coronary arteries to the new aorta, Removing the wall between the atria (atrial septum), Making an artificial connection from either the right ventricle or a body-wide artery to the pulmonary artery to maintain blood flow to the lungs (called a shunt). Afterwards, the baby usually goes home. The child will need to take daily medicines and be closely followed by a pediatric cardiologist, who will determine when the second stage of surgery should be done. Stage II of the operation is called the Glenn shunt or hemi-Fontan procedure. This procedure connects the major vein carrying blue blood from the top half of the body (the superior vena cava) directly to blood vessels to the lungs (pulmonary arteries) to get oxygen. The surgery is usually done when the child is 4 to 6 months of age. During stages I and II, the child may still appear somewhat blue (cyanotic). Stage III, the final step, is called the Fontan procedure. The rest of the veins that carry blue blood from the body (the inferior vena cava) are connected directly to the blood vessels to the lungs. The right ventricle now serves only as the pumping chamber for the body (no longer the lungs and the body). This surgery is usually performed when the baby is 18 months - 3 years old. After this final step, the baby is no longer blue. Some patients may need more surgeries in their 20s or 30s if they develop hard to control arrhythmias or other complications of the Fontan procedure. In some hospitals, heart transplantation is considered a better choice than the three-step surgery process. However, there are few donated hearts available for small infants.

Outlook (Prognosis)
If left untreated, hypoplastic left heart syndrome is fatal. Survival rates for the staged repair continue to rise as surgery techniques and care after surgery improve. Survival after the first stage is more than 75%. The child's outcome after surgery depends on the size and function of the right ventricle.

Possible Complications
Complications include: Blockage of the artificial shunt, Chronic diarrhea (from a disease called protein losing enteropathy), Fluid in the abdomen (ascites) and in the lungs (pleural effusion), Heart failure, Irregular, fast heart rhythms (arrhythmias), Strokes and other nervous system complications, Sudden death.

When to Contact a Medical Professional

Contact your health care provider immediately if your infant: Eats less (decreased feeding), Has blue (cyanotic) skin, Has new changes in breathing patterns

Prevention
There is no known prevention for hypoplastic left heart syndrome. As with many congenital diseases, the causes of hypoplastic left heart syndrome are uncertain and have not been linked to a mother's disease or behavior.

3.) Pulmonary atresia

Pulmonary atresia is a form of heart disease that occurs from birth (congenital heart disease), in which the pulmonary valve does not form properly. The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs.

In pulmonary atresia, a solid sheet of tissue forms where the valve opening should be, and the valve stays closed. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.

Causes, incidence, and risk factors

As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is associated with another type of congenital heart defect called a patent ductus arteriosus (PDA). Persons with pulmonary atresia may also have a poorly developed tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart. Pulmonary atresia may occur with or without a ventricular septal defect (VSD): If the person does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA/IVS) and If the person has both problems, the condition is called pulmonary atresia with VSD. This is an extreme form of tetralogy of Fallot. Although both conditions are called pulmonary atresia, they are actually different defects.

Symptoms
Symptoms usually occur in the first few hours of life, although it may take up to a few days. Symptoms may include: Bluish colored skin (cyanosis), Fast breathing, Fatigue, Poor eating habits (babies may get tired while nursing or sweat during feedings) and Shortness of breath.

Signs and tests

The health care provider will use a stethoscope to listen to the heart and lungs. Persons with a PDA have a heart murmur that can be heard with a stethoscope. The following tests may be ordered: Chest x-ray, Echocardiogram, Electrocardiogram (ECG), Heart catheterization, Pulse oximetry - shows the amount of oxygen in the blood

Treatment
A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs. This medicine keeps a blood vessel open between the pulmonary artery and aorta. The vessel is called a patent ductus arteriosus (PDA). Other treatments include: A thin, flexible tube (heart catheterization) to repair the problem, Open heart surgery to repair or replace the valve, or to place a tube between the right ventricle and the pulmonary (lung) arteries, Reconstructing the heart as a single ventricle (for some patients), Heart transplant

Expectations (prognosis)
Most cases can be helped with surgery. However, how well a baby does depends on: The size and connections of the pulmonary artery (the artery that takes blood to the lungs), How well the heart is beating, How much the other heart valves are leaking. Outcomes can vary because of the different forms of this defect. A baby may need only a single procedure, or could need three or more surgeries and have only a single working ventricle.

Complications Delayed growth and development, Seizures, Stroke, Infectious endocarditis, Heart failure and Death Calling your health care provider

Call your health care provider if the baby has: Problems breathing, Skin or nails that appear blue (cyanosis)

Prevention
There is no known way to prevent this condition. All pregnant women should receive routine prenatal care. Many congenital defects can be discovered on routine ultrasound examinations. If the defect is found before birth, medical specialists (such as a pediatric cardiologist, cardiothoracic surgeon, and neonatologist) can be present at the birth, and ready to help as needed. This preparation can mean the difference between life and death for some babies.

4.) Tetralogy of Fallot

Tetralogy of Fallot is a type of congenital heart defect. Congenital means that it is present at birth.

Causes
Tetralogy of Fallot causes low oxygen levels in the blood. This leads to cyanosis (a bluish-purple color to the skin). The classic form includes four defects of the heart and its major blood vessels: Ventricular septal defect (hole between the right and left ventricles), Narrowing of the pulmonary outflow tract (the valve and artery that connect the heart with the lungs), Overriding aorta (the artery that carries oxygen-rich blood to the body) that is shifted over the right ventricle and ventricular septal defect, instead of coming out only from the left ventricle, Thickened wall of the right ventricle (right ventricular hypertrophy) Tetralogy of Fallot is rare, but it is the most common form of cyanotic congenital heart disease. Patients with tetraology of Fallot are more likely to also have other congenital defects. The cause of most congenital heart defects is unknown. Many factors seem to be involved. Factors that increase the risk for this condition during pregnancy include: Alcoholism in the mother, Diabetes, Mother who is over 40 years old, Poor nutrition during pregnancy, Rubella or other viral illnesses during pregnancy Children with tetralogy of Fallot are more likely to have chromosome disorders, such as Down syndrome and DiGeorge syndrome (a condition that causes heart defects, low calcium levels, and poor immune function).

Symptoms
Blue color to the skin (cyanosis), which gets worse when the baby is upset, Clubbing of fingers (skin or bone enlargement around the fingernails), Difficulty feeding (poor feeding habits), Failure to gain weight, Passing out, Poor development, Squatting during episodes of cyanosis

Exams and Tests

A physical examination with a stethoscope almost always reveals a heart murmur. Tests may include: Chest x-ray, Complete blood count (CBC), Echocardiogram, Electrocardiogram (EKG), MRI of the heart (generally after surgery)

Treatment
Surgery to repair tetralogy of Fallot is done when the infant is very young. Sometimes more than one surgery is needed. When more than one surgery is used, the first surgery is done to help increase blood flow to the lungs. Surgery to correct the problem may be done at a later time. Often only one corrective surgery is performed in the first few months of life. Corrective surgery is done to widen part of the narrowed pulmonary tract and close the ventricular septal defect.

Outlook (Prognosis)

Most cases can be corrected with surgery. Babies who have surgery usually do well. More than 90% survive to adulthood and live active, healthy, and productive lives. Without surgery, death usually occurs by the time the person reaches age 20. Patients who have continued, severe leakiness of the pulmonary valve may need to have the valve replaced. Regular follow-up with a cardiologist to monitor for life-threatening irregular heart rhythms (arrhythmias) is recommended.

Possible Complications
Delayed growth and development, Irregular heart rhythms (arrhythmias), Seizures during periods when there is not enough oxygen and Death

When to Contact a Medical Professional

Call your health care provider if new unexplained symptoms develop or the patient is having an episode of cyanosis (blue skin). If a child with tetralogy of Fallot becomes blue, immediately place the child on his or her side or back and put the knees up to the chest. Calm the baby and seek medical attention immediately.

Prevention: There is no known prevention. 5.) Total anomalous pulmonary venous return
Total anomalous pulmonary venous return is a heart disease that is present at birth (congenital heart disease) in which none of the four veins that take blood from the lungs to the heart is attached to the left atrium (left upper chamber of the heart).

Causes
The cause of total anomalous pulmonary venous return (TAPVR) is unknown. In normal circulation, blood is sent from the right ventricle to pick up oxygen in the lungs. It then returns through the pulmonary (lung) veins to the left side of the heart, which sends blood out through the aorta and around the body. In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body. For the infant to live, a large atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body. How severe this condition is depends on whether the pulmonary veins are blocked. The pulmonary veins may run into the abdomen, passing through a muscle (diaphragm). This muscle squeezes the veins and narrows them, causing the blood to back up into the lungs. This type of TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.

Symptoms
The infant may appear very sick and may have the following symptoms: Bluish color of the skin (cyanosis), Frequent respiratory infections, Lethargy, Poor feeding, Poor growth, Rapid breathing Note: Sometimes, no symptoms may be present in infancy or early childhood.

Exams and Tests

Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached, ECG shows enlargement of the ventricles (ventricular hypertrophy), Echocardiogram may show that the pulmonary vessels

are attached, MRI of the heart can show the connections between the pulmonary vessels, X-ray of the chest shows a normal to small heart with fluid in the lungs

Treatment
Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.

Outlook (Prognosis)
If this condition is not treated, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart.

Possible Complications
Breathing difficulties, Heart failure, Irregular, fast heart rhythms (arrhythmias), Lung infections, Pulmonary hypertension

Prevention: There is no known way to prevent TAPVR. 6.) Transposition of the great vessels
Transposition of the great vessels is a heart defect that occurs from birth (congenital). The two major vessels that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched (transposed).

Causes
The cause of most congenital heart defects is unknown. Factors in the mother that may increase the risk of this condition include: Age over 40, Alcoholism, Diabetes, Poor nutrition during pregnancy (prenatal nutrition), Rubella or other viral illness during pregnancy Transposition of the great vessels is a cyanotic heart defect. This means there is decreased oxygen in the blood that is pumped from the heart to the rest of the body. In normal hearts, blood that returns from the body goes through the right side of the heart and pulmonary artery to the lungs to get oxygen. The blood then comes back to the left side of the heart and travels out the aorta to the body. In transposition of the great vessels, the blood goes to the lungs, picks up oxygen, returns to the heart, and then flows right back to the lungs without ever going to the body. Blood from the body returns to the heart and goes back to the body without ever picking up oxygen in the lungs. Symptoms appear at birth or very soon afterward. How bad the symptoms are depends on the type and size of heart defects (such as atrial septal defect or patent ductus arteriosus) and how much oxygen moves through the body's general blood flow. The condition is the second most common cyanotic heart defect.

Symptoms
Blueness of the skin, Clubbing of the fingers or toes, Poor feeding, Shortness of breath

Exams and Tests

The health care provider may detect a heart murmur while listening to the chest with a stethoscope. The baby's mouth and skin will be a blue color. Tests often include the following: Cardiac catheterization, Chest x-ray, ECG,

Echocardiogram (if done before birth, it is called a fetal echocardiogram), Pulse oximetry (to check blood oxygen level)

Treatment
The baby will immediately receive a medicine called prostaglandin through an IV (intravenous line). This medicine helps keep a blood vessel called the the ductus arteriosus open, allowing some mixing of the two blood circulations. A procedure using a long, thin flexible tube (balloon atrial septostomy) may be needed to create a large hole in the atrial septum to allow blood to mix. A surgery called an arterial switch procedure is used to permanently correct the problem within the baby's first week of life. This surgery switches the great arteries back to the normal position and keeps the coronary arteries attached to the aorta.

Outlook (Prognosis)
The child's symptoms will improve after surgery to correct the defect. Most infants who undergo arterial switch do not have symptoms after surgery and live normal lives. If corrective surgery is not performed, the life expectancy is only months.

Possible Complications
Coronary artery problems, Heart valve problems, Irregular heart rhythms (arrhythmias)

Prevention
Women who plan to become pregnant should be immunized against rubella if they are not already immune. Eating well, avoiding alcohol, and controlling diabetes both before and during pregnancy may be helpful.

7.) Tricuspid atresia

Tricuspid atresia is a type of heart disease that is present at birth (congenital heart disease), in which the tricuspid heart valve is missing or abnormally developed. The defect blocks blood flow from the right atrium to the right ventricle.

Causes
Tricuspid atresia is an uncommon form of congenital heart disease that affects about 5 in every 100,000 live births. Twenty percent of patients with this condition will also have have other heart problems. Normally, blood flows from the body into the right atrium, then through the tricuspid valve to the right ventricle and on to the lungs. If the tricuspid valve does not open, the blood cannot flow from the right atrium to the right ventricle. Blood ultimately cannot enter the lungs, where it must go to pick up oxygen (become oxygenated).

Symptoms
Bluish color to the skin, Fast breathing, Fatigue, Poor growth, Shortness of breath

Exams and Tests

This condition may be discovered during routine prenatal ultrasound imaging or when the baby is examined shortly after birth. Bluish skin is present from birth. A heart murmur is often present at birth and may increase in loudness

over several months. Tests may include the following: ECG, Echocardiogram, Chest x-ray, Cardiac catheterization, MRI of the heart

Treatment
Once the diagnosis is made, the baby will be admitted to the neonatal intensive care unit (NICU). A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to keep the blood circulating to the lungs. The condition always requires surgery. If the heart is unable to pump enough blood out to the lungs and rest of the body, the first surgery usually occurs within the first few days of life. In this procedure, an artificial shunt is inserted to keep blood flowing to the lungs. In some cases, this first surgery is not needed. Afterwards, the baby usually goes home. The child will need to take one or more daily medicines and be closely followed by a pediatric cardiologist, who will decide when the second stage of surgery should be done. Stage II of the operation is called the Glenn shunt or Hemifontan procedure. This procedure connects half of the veins carrying blue blood from the upper half of the body directly to the pulmonary artery. The surgery is usually done when the child is between 4 - 6 months old. During stage I and II, the child may still look blue (cyanotic). Stage III, the final step, is called the Fontan procedure. The rest of the veins carrying blue blood from the body are connected directly to the pulmonary artery leading to the lungs. The left ventricle now only has to pump to the body, not the lungs. This surgery is usually performed when the child is 18 months to 3 years old. After this final step, the baby is no longer blue.

Possible Complications
Irregular, fast heart rhythms (arrhythmias), Chronic diarrhea (from a disease called protein loosing enteropathy), Heart failure, Fluid in the abdomen (ascites) and in the lungs (pleural effusion), Blockage of the artificial shunt, Strokes and other nervous system complications, Sudden death

When to Contact a Medical Professional

Contact your health care provider right away if your infant has: New changes in breathing patterns, Problems eating, Skin that is turning blue

8.) Truncus arteriosus

Truncus arteriosus is a rare type of heart disease that occurs at birth (congenital heart disease), in which a single blood vessel (truncus arteriosus) comes out of the right and left ventricles, instead of the normal two vessels (pulmonary artery and aorta).

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 this condition is not treated, 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. And 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

Treatment
Surgery is needed to treat this condition. The surgery creates two separate arteries. Usually the truncal vessel is kept as the new aorta. A new pulmonary artery is created using tissue from another source. The branch pulmonary arteries are sewn to this new artery. The hole between the ventricles is closed.

Outlook (Prognosis)
Complete repair usually provides good results. Another procedure may be needed as the patient grows. Untreated cases result in death, often during the first year of life.

Possible Complications
Heart failure and High blood pressure in the lungs (pulmonary hypertension) with pulmonary obstructive lung disease

When to Contact a Medical Professional

Call your health care provider if your infant or child: Appears lethargic, Appears overly tired or mildly short of breath, Does not eat well, Does not seem to be growing or developing normally. If the skin, lips, or nail beds look blue or if the child seems to be very short of breath, take the child to the emergency room or have the child examined promptly.