Congenital Heart Diseases

Group 3: Arroyo, Alyssa Evasco, Ronell Farenas Joy Manaloto, Jeztine Cassandra Rivera, Leo Angelo Tiosen, Ronell

Congenital Heart Diseases (CHD)
y CHD results from faulty embryonic development, express

either as misplaced structures or an arrest in the progression of a normal structure from an early stage to a more advanced one (RUBINS, 5th edition)

Relative Incidence of Specific Anomalies in Patients with Congenital Heart Disease
Disease Ventricular septal defects Atrial Septal Defects Patent Ductus Arteriosus Tetralogy of Fallout Pulmonary Stenosis Coarctation of Aorta Aortic Stenosis ComplteTransposition of the Great Arteries Truncus arteriosus Tricuspid Atresia 25-30% 10-15% 10-20% 4-9% 5-7% 5-7% 4-6% 4-10% 2% 1% Percent

The cause of CHD is ascertian, but the following are considered as possible cause of CHD:
y Single- gene syndromes y Maternal rubella infection y Maternal Use of Drugs

Classification of Congenital Heart Disease Reflect Cyanosis and Shunting
y Left-to-Right Shunt

The essential pathophysiology here is that oxygenated blood recirculates in the lungs, hence cyanosis is not a usual problem in such cases unless there is heart failure. They are also called Acyanotic CongenitalHeart Diseases.
y Right-to-Left Shunt

Here, deoxygenated blood bypasses the lungs, hence the reoxygenation process in the lungs is missed, therefore such cases are associated with cyanosis. They are also called Cyanotic Congenital Heart Diseases.

Initial Left-to-Right Shunt

Ventricular Septum Defect
y Ventricular septal defect is a hole in the wall between the

right and left ventricles of the heart. This abnormality usually develops before birth and is found most often in infants. y VSD is the most common congenital heart lesion. y It is not unusual for small-to-medium sized VSD·s to eventually close spontaneously. Many, but not all that remain will require surgery.

y If a substantial size defect is not corrected, then the pressures

in the pulmonary arteries may become very high and induce changes in the arteries themselves such that even closure of the defect will no longer improve the patient. In this case, the pressures in the right side of the heart are high enough that blood may begin to flow from the right to the left side of the heart. This situation is called "Eisenmenger·s syndrome", a condition which may result from several similar abnormalites.

y Eventually, the left ventricle can work so hard that it starts to

fail. It can no longer pump blood as well as it did. Blood returning to the heart from the blood vessels backs up into the lungs, causing pulmonary congestion, and further backup into the body, causing weight gain and fluid retention. Overall, this is called congenital heart failure.

Signs and Symptoms
y Shortness of breath y Fast breathing y Hard breathing y Paleness y Failure to gain weight y Fast heart rate y Sweating while feeding y Frequent respiratory infections

y If the defect is small, no treatment is usually needed.

However, the baby should be closely monitored by a health care provider to make sure that the hole eventually closes properly and signs of heart failure do not occur. y Babies with a large VSD who have symptoms related to heart failure may need medicine to control the symptoms and surgery to close the hole. Medications may include digitalis (digoxin) and diuretics. y Surgery are sometimes needed.

Atrial Septum Defect
y This defect is characterized by incomplete closure between

the two upper chambers of the heart. Blood can therefore flow through a hole (defect) in the wall (septum) between the upper chambers of the heart (atria) from one side to the other, causing some heart chambers to have to pump extra blood. The heart can dilate, the muscle can become weak, and the pressures in the pulmonary arteries can increase (pulmonary hypertension) due to the increase in blood flow. If the defect closes on its own or with the aid of surgery, these consequences can be avoided.

Signs and Symptoms:
Small to moderate sized defects may produce no symptoms, or not until middle age or later. Symptoms that may occur can include: y Difficulty breathing (dyspnea) y Frequent respiratory infections in children y Sensation of feeling the heart beat (palpitation) in adults y Shortness of breath with activity

y ASD may not require treatment if there are few or no

symptoms, or if the defect is small. Surgical closure of the defect is recommended if the defect is large, the heart is swollen, or symptoms occur. y A procedure has been developed to close the defect without surgery. The procedure involves placing an ASD closure device into the heart through tubes called catheters. The health care provider makes a tiny surgical cut in the groin, then inserts the catheters into a blood vessel and up into the heart. The closure device is then placed across the ASD and the defect is closed.

Patent Ductus Arteriosus (PDA)
y the early embryo supposedly recapitulates an ancestral

evolutionary stage, with six aortic arches connecting the ventral and dorsal aortas as part of the branchial cleft system. y abnormal blood flow occurs between two of the major arteries connected to the heart. y is a congenital disorder in the heart wherein a neonate's ductus arteriosus fails to close after birth. y ductus arteriosus is a normal fetal structure, allowing blood to bypass circulation to the lungs.

Signs and symptoms
y tachycardia y respiratory problems y continuous machine y widened pulse pressure y poor growth y dyspnea - shortness of breath y differential cyanosis, i.e.

like heart murmur y cardiomegaly - enlarged heart y left subclavicular thrill y bounding pulse

cyanosis of the lower extremities

y PDA can be treated with both surgical and non-surgical

methods. y Doctors treat the condition with medicines, catheter-based procedures, and surgery. Most children who have PDAs live healthy, normal lives after treatment.

Truncus Anteriosus
y Persistent truncus arteriosus refers to a common trunk for

the origin of the aorta, pulmonary arteries, and coronary arteries. y the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. y 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).

The most well-known classification was the fourfold system developed by Collett and Edwards in 1949. Collett/Edwards Types I, II, and III are distinguished by the branching pattern of the pulmonary arteries:

y Type I: truncus -> one pulmonary artery -> two lateral

pulmonary arteries y Type II: truncus -> two posterior/posterolateral pulmonary arteries y Type III: truncus -> two lateral pulmonary arteries

y Bluish skin (cyanosis) y Delayed growth or growth y y y y y y


failure Fatigue Lethargy Poor feeding Rapid breathing (tachypnea) Shortness of breath (dyspnea) Widening of the finger tips

y Surgery is needed to treat this condition. Two procedures are

available. y One treatment involves banding the pulmonary arteries coming off the truncus to limit the amount of blood that can flow through them. However, this procedure is rarely used anymore. y The other procedure is called complete repair.

Anomalous Pulmonary Venous Drainage
y Total anomalous pulmonary venous return is a congenital

heart disease (present at birth) 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). y In TAPVR, oxygenated blood returns from the lungs back to the right atrium or a vein flowing into the right atrium and NOT to the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.

y If the infant is 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 rest of the body. y The severity of this condition depends on whether the pulmonary veins are obstructed. Most often in obstructed TAVPR, the pulmonary veins 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 causes symptoms early in life and can be rapidly deadly if not recognized and surgically corrected.

Signs and Symptoms
The infant may appear to be critically ill and may display the following symptoms: y Lethargy y Poor feeding y Rapid breathing y Poor growth y Frequent respiratory infections y Cyanosis (blue discoloration of the skin)

y Early complete surgical repair is needed. In surgery, the

pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.

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

Hypoplastic Left Heart Syndrome
y 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 congenital (present at birth). The problem develops before birth when there is not enough growth of the left ventricle and other structures, including the: y Aorta -- the blood vessel that carries oxygen-rich blood from the left ventricle to the entire body y Entrance and exit of the ventricle

y Mitral and aortic valves

This causes the left ventricle and aorta to be incompletely 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.

y 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.

y 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 heart catheterization.

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: y Bluish (cyanosis) or poor skin color y Cold hands and feet (extremities) y Lethargy y Poor pulse y Poor suckling and feeding y Pounding heart

y Rapid breathing y 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.

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: y Foramen ovale (a hole between the right and left atrium) y 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.

y Blockage of the artificial shunt y Chronic diarrhea (from a disease called protein losing y y y y y

enteropathy) Fluid in the abdomen (ascites) and in the lungs (pleural effusion) Heart failure Irregular, fast heart rhythms (arrhythmias) Strokes and other neurological complications Sudden death

Right- to- Left Shunt

Tetralogy of Fallot
y Tetralogy of Fallot is classified as a cyanotic heart defect

because the condition causes low oxygen levels in the blood. This leads to cyanosis (a bluish-purple color to the skin). y The classic form of tetralogy includes four related defects of the heart and its major blood vessels: y Ventricular septal defect (hole between the right and left ventricles) y Narrowing of the pulmonary outflow tract (the valve and artery that connect the heart with the lungs)

y 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 y A thickened muscular wall of the right ventricle (right ventricular hypertrophy).

y At birth, infants may not show signs of cyanosis. However,

later they may develop sudden episodes (called "Tet spells") of bluish skin from crying or feeding. y Tetralogy of Fallot is rare, but it is the most common form of cyanotic congenital heart disease. Patients with tetralogy of Fallot have a higher incidence of major non-heart congenital defects

y Clubbing of fingers (skin or bone enlargement around the y y y y y y

fingernails) Cyanosis, which becomes more pronounced when the baby is upset Difficult feeding (poor feeding habits) Failure to gain weight Passing out Poor development Squatting during episodes of cyanosis

y 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. y 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.

Tricuspid Atresia
y A congenital absence of the tricuspid valve,

results in an obligate right-to-left shunt through the patent foramen ovale. y Infants with tricuspid atresia present with cyanosis due to the atrial right-to-left shunt; and they easily become short of breath. y Surgical intervention is aimed at bypassing the atretic tricuspid valve and small right ventricle.

Tricuspid Atresia: Causes, Incidence & Risk Factors
y It affects about 5 in every 100,000 live births. 20% of

patients with this condition will also have other heart problems. y 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).

Tricuspid Atresia: Symptoms
y Bluish discoloration of the skin y Easily fatigued y Shortness of breath y Fast breathing y Poor growth

Tricuspid Atresia: Signs, Tests and Treatment
y This condition may be discovered during routine prenatal

ultrasound imaging or when the baby is examined shortly after birth. Cyanosis is present from birth. A heart murmur is often present at birth and may increase in loudness over several months. y Test may include ECG, echocardiogram, chest x-ray, cardiac catheterization and MRI of the heart. y 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 maintain blood flow to the lungs.

Tricuspid Atresia: Complications
y Irregular, fast heart rhythms y Chronic diarrhea y Heart failure y Fluid in the abdomen and in the lungs y Blockage of the artificial shunt y Strokes and other neurological complications y Sudden death

No Shunt

Transportation of the Great Arteries
y In TGA, the aorta arises from the right ventricle and the pulmonary

artery from the left ventricle. y The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle. y TGA is responsible for more than half of deaths in infant younger than 1 year with cyanotic heart disease. y Survival is possible only if there is a communication between the circuits. Virtually all infants with TGA have an atrial septal defect.

TGA: Causes, Incidence & Risk Factors
y Factors in the mother that may increase the risk of this condition

include: Age over 40, alcoholism, diabetes, poor nutrition during pregnancy, Rubella or other viral illness during pregnancy y TGA 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. Low blood oxygen leads to cyanosis and shortness of breath. y 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.

y In transposition of the great vessels, the blood goes to the lungs,

picks up oxygen, and then goes 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.

TGA: Signs, Tests and Treatments
y 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. y Tests often include the following: Cardiac catheterization, Chest xray, ECG, Echocardiogram, Pulse oximetry y The baby will immediately receive a medicine called prostaglandin through an IV (intravenous line). This medicine helps keep the ductus arteriosus open, allowing some mixing of the two blood circulations. y 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.

Transposition of the Great Arteries: Complications and Prevention
y If corrective surgery is not performed, the life expectancy is months. y Arrhythmias y Coronary artery problems y Heart valve problems y This condition can be diagnosed before birth using a fetal

echocardiogram. If not, it is usually diagnosed soon after a baby is born. y 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.

Coarctation of the Aorta
y A local constriction that almost always occurs immediately

below the origin of the left subclavian artery at the site of the ductus arteriosus. Rare coarctations can occur at any point from the aortic arch to the abdominal bifurcation. y Aortic coarctation is a narrowing of part of the aorta. It is a type of birth defect. y The condition is two to five times more frequent in males than females and is associated is associated with a bicuspid aortic valve in two-thirds of cases

Coarctation of the Aorta: Causes, Incidence and Risk Factors
y Aortic coarctation is more common in persons with certain

genetic disorders, such asTurner syndrome. However, it can also be due to birth defects of the aortic valves. y Aortic coarctation is one of the more common heart conditions that are present at birth. It is usually diagnosed in children or adults under age 40. y Coarctation of the aorta may be seen with other congenital heart defects, such as: bicuspid aortic valve, defects in which only one ventricle is present, ventricular septal defect. y About 98% of coarctations occur immediately beneath the origin of the left subclavian artery at the site of attachment of the ductus arteriosus.

Coarctation of the Aorta: Symptoms
y Symptoms depend on how much blood can flow through the artery.

Other heart defects may also play a role. y Around half of newborns with this problem will have symptoms in the first few days of life. y In milder cases, symptoms may not develop until the child has reached adolescence. Symptoms include: (1) dizziness or fainting, (2) shortness of breath ,(3)pounding headache, (4) chest pain, (5) cold feet or legs, (6) nosebleed, (7) leg cramps with exercise, (8) high blood pressure with exercise, (9) decreased ability to exercise, (10) failure to thrive, (11) poor growth. y There may be no symptoms.

Coarctation of the Aorta: Signs and Tests
y The doctor will use a stethoscope to listen to your heart and

check for murmurs. People with aortic coarctation have a harshsounding murmur that can be heard from the back. Other types of murmurs may also be present. y Coarctation is often discovered during a newborn's first examination or well-baby exam. Taking the pulses in an infant is an important part of the examination, because there may not be any other symptoms or findings until the child is older. y Tests to diagnose this condition may include: Echocardiography, chest x-ray, Heart CT, MRI or MR, sardiac catheterization and aortography

Coarctation of the Aorta: Treatment
y Most newborns with symptoms will have surgery either right after

birth or soon afterward. First they will receive medications to stabilize them. y During surgery, the narrowed part of the aorta will be removed or opened. If the problem area is small, the two free ends of the aorta may be re-connected. This is called anastomosis. If a large part of the aorta is removed, a Dacron graft (a man-made material) or one of the patient's own arteries is used to fill the gap. A tube graft connecting two parts of the aorta may also be used. y Sometimes, balloon angioplasty may be done instead of surgery, but it has a higher rate of failure.

Coarctation of the Aorta: Complications
y Without treatment, most people die before age 40. For this

reason, doctors usually recommend that the patient has surgery before age 10. Most of the time, surgery to fix the coarctation is done during infancy. y Complications that may occur before, during, or soon after surgery include: Aortic aneurysm, Aortic dissection, Aortic rupture, Bleeding in the brain, Endocarditis (infection in the heart), Heart failure, Hoarseness caused by injury to the nerve to the larynx, Impaired kidney function, Paralysis of the lower half of the body, Premature development of coronary artery disease, Severe high blood pressure and Stroke

Pulmonary Stenosis
y Pulmonary Stenosis is a congenital (present at birth) defect

that occurs due to abnormal development of the fetal heart during the first 8 weeks of pregnancy. y Pulmonary stenosis is found between the right ventricle and the pulmonary artery. It has three leaflets that function like a one-way door, allowing blood to flow forward into the pulmonary artery, but not backward into the right ventricle. y With pulmonary stenosis, problems with the pulmonary valve make it harder leaflets to open and permit blood to flow forward from the right ventricle to the lungs.

y In children, these problems can include: y * a valve that has leaflets that are partially fused together. y * a valve that has thick leaflets that do not open all the way. y * the area above or below the pulmonary valve is narrowed. y Pulmonary stenosis may be present in varying degrees,

classified according to how much obstruction to flood flow is present.

What causes Pulmonary Stenosis?
y Congenital pulmonary stenosis occurs due to improper

development of the pulmonary valve in the first 8 weeks of fetal growth. y It can be caused by a number of factors, through most of the time this heart defect occurs sporadically, with no clear reason evident for its development. y Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families

Why is pulmonary stenosis a concern?
y Mild pulmonary stenosis may not cause symptoms. Problems

can occur when pulmonary stenosis is moderate to severe, including the following. y *The right ventricle has to work harder to try to move blood through the tight pulmonary valve. Eventually, the right ventricle is no longer able to handle the extra workload, and it fails to pump forward efficiently. Pressure builds up in the right atrium, and then in the veins bringing blood back to the right side of the heart. Fluid retention and swelling may occur. y *There is a higher than average chance of developing an infection in the valves of the heart known as bacterial endocarditis.

What are symptoms of pulmonary stenosis?
y The ff are the most common symptoms of pulmonary

y y y y y y

stenosis. However, each child may experience symptoms differently. Symptoms may include: *heavy or rapid breathing *shortness of breath *fatigue *rapid heart rate *swelling in the feet, ankles, face, eyelids, and/or abdomen *fewer wet diapers or trips to the bathroom

Aortic Stenosis
y Aortic stenosis is a heart defect that may be congenital

(present at birth) or acquired (develop later in life). If the problem is congenital, then something occurred during the first 8 weeks of pregnancy to affect the development of the aortic valve. y The aortic valve is found between the left ventricle and the aorta. It has three leaflets that function like a one-way door, allowing blood to flow forward into the aorta, but not backward into the left ventricle. Aortic stenosis is the inability of the aortic valve to open completely.

y With aortic stenosis, problems with the aortic valve make it

y y y y y

harder for the leaflets to open and permit blood to flow forward from the left ventricle to the aorta. In children, these problems can include a valve that: *only has two leaflets instead of three (bicuspid aortic valve). *has leaflets that are partially fused together. *has thick leaflets that do not open all the way. *becomes damaged by rheumatic fever or bacterial endocarditis. *area above or below the valve is narrowed (supravalvar or subvalvar).

y Aortic stenosis may be present in varying degrees, classified

according to how much obstruction to blood flow is present. y A child with severe aortic stenosis will be quite ill, with major symptoms noted early in life. A child with mild aortic stenosis may have few symptoms, or perhaps none until later in adulthood. y The degree of obstruction can become worse with time.

y Congenital aortic stenosis occurs three

times more often in BOYS than in girls.

What causes Aortic stenosis?
y Congenital aortic stenosis occurs due to improper

development of the aortic valve in the first 8 weeks of fetal growth. y It can be caused by a number of factors, though, most of the time, this heart defect occurs sporadically, with no apparent reason for its development. y Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families.

Why is Aortic stenosis a concern?
y Mild aortic stenosis may not cause any symptoms. Several

problems may occur, however, when aortic stenosis is moderate to severe, including the following: y The left ventricle has to work harder to try to move blood through the tight aortic valve. Eventually, the left ventricle is no longer able to handle the extra workload, and it fails to pump blood to the body efficiently. y There is a higher than average chance that the aorta may become dilated (enlarged). This can increase the risk of an aneurysm or dissection of the aorta.

y There is a higher than average chance of developing an

infection in the lining of the heart or aorta known as bacterial endocarditis.
y The coronary arteries, which supply oxygen-rich (red) blood

to the heart muscle, may not receive enough blood to meet the demands of the heart.

What are the symptoms of Aortic stenosis?
y The following are the most common symptoms of aortic

y y y y y

stenosis. However, each child may experience symptoms differently. Symptoms may include: *fatigue *dizziness with exertion *shortness of breath *irregular heartbeats or palpitations *chest pain


y This defect happens when the main coronary artery

originates from the Pulmonary Artery instead of the Aorta. y The pulmonary artery carries less oxygen in its blood, so now the muscle of the heart which is working very hard instead of getting fresh oxygenated blood it is getting blood with less oxygen. y The muscle of the heart does not like that and starts to become weaker and weaker, the muscle walls of the heart becomes thinner and thinner, and this predisposes to a condition called Cardio Myopathy y Because the muscle of the heart becomes weak and thin the heart will not be able to contract as strongly as it should and not enough blood will be getting to the muscles, the skin, or to the body organs to give them enough blood

y The baby will be weak, tired. Usually this condition is

present in the first 2 months of life and the baby will be very tired during feeding, excessively sweating during feeding, taking long breaks before feeding, sometimes cries in severe pain as he feeds. y It is very important to diagnose this condition early because doctors may be to re-route the artery back to where it should be coming from (the aorta).

Endocardial Fibroelastosis (EFE)
y diffuse thickening of the ventricular endocardium and

presents as unexplained heart failure in infants and children. y thickening of the heart muscle lining causing heart enlargement and heart failure. y is a rare heart disorder usually associated with children two years old and younger.

y Primary endocardial fibroelastosis is not associated with any

significant structural anomaly of the heart.

y Secondary endocardial fibroelastosis is associated with other

congenital heart diseases.

y Breathing difficulty y Cough y Irritability y Anorexia y Vomiting y Weakness y Chest pain y Failure to thrive y Diaphoresis y Rapid breathing y Tachycardia y Pulmonary rales y Enlarged liver y Acyanosis y Endocardial scarring y Endocardial thickening y Enlarged left ventricle y Enlarged heart y Grunting breathing y Ventricular failure y Arrhythmia

y The cause should be identified and, where possible, the

treatment should be directed to that cause. A last resort form of treatment is heart transplant.

Ebstein·s Malformation
y 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). y 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, blood can't get out of the heart into the lungs and the person may appear blue.

y 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).

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. y Symptoms in older children may include: y Cough y Failure to grow y Fatigue y Rapid breathing y Shortness of breath y Very fast heartbeat

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

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

Complete Heart Block
y In this type of heart block, none of the electrical signals reach

the ventricles. This type also is called complete heart block or complete AV block. y When complete heart block occurs, special areas in the ventricles may create electrical signals to cause the ventricles to contract. This natural backup system is slow and isn't coordinated with the contraction of the atria. On an EKG, the normal pattern is disrupted. The P waves occur at a faster rate than the QRS waves.

y A heart that beats too fast or too slow can cause: y Lightheadedness or dizziness y Palpitations (skipping, fluttering or pounding in the chest) y Fatigue y Chest pressure or pain y Shortness of breath y Fainting spells

y Third-degree heart block patients almost always require an

artificial pacemaker to better regulate the electrical activity of the heart. If a medical crisis occurs before the pacemaker can be implanted, then a temporary pacemaker wire may be used to keep the heart beating. Most patients who are diagnosed with complete heart block will require placement of a permanent pacemaker, unless a treatable cause is identified and corrected..

y Dextrocardia is a condition in which the heart is pointed

toward the right side of the chest instead of normally pointing to the left. It is present at birth (congenital). y The simplest type of dextrocardia is one in which the heart is a mirror image of the normal heart, and no other problems exist. This condition is rare. Usually in this case, the organs of the abdomen and the lungs will also be arranged in a mirror image of their normal position. For example, the liver will be on the left side instead of the right.

y Some people with mirror-image dextrocardia have a problem

with the fine hairs (cilia) that filter the air going into their nose and air passages. This condition is called Kartagener syndrome. y In the more common types of dextrocardia, heart defects are present in addition to the abnormal location of the heart.

y The abdominal and chest organs in babies with dextrocardia

may be abnormal and may not work correctly. A very serious syndrome that appears with dextrocardia is called heterotaxy. Heterotaxy means the organs (atria of the heart and abdominal organs) are not in their usual places. y In heterotaxy, the spleen may be completely missing. Because the spleen is an extremely important part of the immune system, babies born without a spleen are in danger of severe bacterial infections and death. In another form of heterotaxy several small spleens exist, but may not work correctly.

y There are no symptoms of dextrocardia if the heart is normal. y Conditions that may include dextrocardia may cause the following y y y y y y y

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

y A complete mirror image dextrocardia with no heart defects


requires no treatment. It is important, however, to let the child's health care provider know the heart is on the right side of the chest. This information can be important in some exams and tests. y Treatment for conditions that include dextrocardia depends on whether the infant has other heart or physical problems in addition to dextrocardia.

y If heart defects are present with dextrocardia, the baby will

most likely need surgery. Critically ill babies may need treatment with medication before surgery. These medications help the baby grow larger so surgery is less difficult to perform. y The baby might also need surgery to correct problems in the organs of the abdomen.

y Children with Kartagener syndrome will need repeated

treatment with antibiotics for sinus infections. y Children with a missing or abnormal spleen need long-term antibiotics. y All children with heart defects should get antibiotics before surgeries or dental treatments.