Differential Diagnosis Of Cardiomegaly In Children

Congenital heart diseases

              A congenital heart defect (CHD) is a defect in the structure of the heart and great vessels of a newborn. Most heart defects either obstruct blood flow in the heart or vessels near it or cause blood to flow through the heart in an abnormal pattern, although other defects affecting heart rhythm (such as long QT syndrome) can also occur. Heart defects are among the most common birth defects and are the leading cause of birth defect-related deaths. Approximately

1 in 125 babies are born each year in the US with a Congenital Heart Defect.

                                     Actuallity

 

                  Congenital heart disease can occur immediately after birth or leaking concealed. They occur with a frequency of 6-8 cases per thousand births. They take first place in infant mortality and children in the first year of life.

                   Today, thanks to the development of medicine, diagnosis of heart diseases in children has become possible even in the hospital, and under certain pathological conditions - for prenatal (before birth) stage.

Statistics:

10 children from 1000 birth have heart defects

In 1-2 children in 1000 these defects are severe, life-threatening condition (aortic coarctation, aortic valve stenosis, total anomalous pulmonary venous drainage, interruption of the aortic arch and others)

Not less than 25% of children with valvular heart disease remain undetected at discharge from the hospital.

Children are included to the risk of the availability of congenital heart disease with:

Ø     -Down syndrome and certain other genetic disorders (chromosomal abnormalities). In this case, a geneticist directs the child to a cardiologist for further examination

Ø     -Premature babies

Ø     -Children with multiple extracardiac (ie, other than the heart defect) malformations.

If a child has pronounced developmental defects, violations in the structure or function of major organs, a pediatrician should send him to the Pediatric Cardiology.

                 Predisposing factors to heart defects in the child

• genetic predisposition

• presence of the mother or relatives with congenital

        malformations

• age older than 35 years of pregnant women

• infectious diseases during pregnancy (especially in the first trimester of pregnancy)

• smoking and alcohol during pregnancy

• endocrine disorders in mother or father

• serious illness of pregnant women in the first trimester of pregnancy (severe

       toxaemia)

• the impact of environmental factors during pregnancy (eg, bad ecology, radiation,

          etc.)

• reception during pregnancy of some drugs ( drugs to treat cancer,

          a certain group of antibiotics, aspirin, etc.).

• miscarriage and stillbirth in the history of women

              Causes of congenital heart disease.

             The cause may be due to a genetic predisposition or an environmental exposure during pregnancy.

             Known genetic causes of heart disease includes chromosomal abnormalities such as trisomies 21, 13, and 18, as well as a range of newly recognised genetic point mutations, point deletions and other genetic abnormalities as seen in syndromes such as Velo-Cardio-Facial Syndrome, familial ASD with heart block, Alagille syndrome, Noonan syndrome, and many more.

               Known antenatal environmental factors include maternal infections (Rubella), drugs (alcohol, hydantoin, lithium and thalidomide) and maternal illness (diabetes mellitus, phenylketonuria, and systemic lupus erythematosus).

                               

       This is a normal fetal ultrasound showing one pattern of the fetal heartbeat. Some ultrasound machines have the ability to focus on different areas of the heart and evaluate the heartbeat. This is useful in the early diagnosis of congenital heart abnormalities. 

                           AHA classification of congenital heart disease

                 There are three common classifications of congenital heart disease (CHD) according to the American Heart Association. These are:

Ø     Septal defects.

Ø     Obstructive defects.

Ø     Cyanotic defects.

                                         Ventricular septal defect

                   Ventricular septal defect is a congenital defect of the heart, with occurs as an abnormal opening in the wall that separates the right and left ventricles. Ventricular septal defect may also be associated with other heart defects. For those defects that do not spontaneously close, the outcome is good with surgical repair.

                    Before a baby is born, the right and left ventricles of its heart are not separate. During intrauterine development of fetus ventricles are separated. 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.

                                             Causes                

           The cause of VSD is not yet known. This defect often occurs along with other congenital heart defects.

                                        Symptoms

               Patients with ventricular septal defects may not have symptoms. However, if the hole is large, symptoms, the baby often has symptoms related to heart failure.

                         The most common symptoms include:

Ø     Shortness of breath

Ø     Fast breathing

Ø     Hard breathing

Ø     Paleness

Ø     Failure to gain weight

Ø     Fast heart rate

Ø     Sweating while feeding

Ø     Frequent respiratory infections

                            Paleness

               

                       Cyanosis of rhino-lip triangle

                                Exams and Tests

          Prenatal diagnostic

                Septal defect. 12 weeks pregnancy.

                  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

   ECG-diagnostic

Chest radiograph of a patient with ventricular septal defect (direct view): the heart shadow is increased at the expense of both ventricles, marked protrusion of the arc of pulmonary trunk (indicated by arrow), pulmonary picture in the basal parts of the lungs is strengthened.

                          EchoCS

 Angiogram at VSD

                                             Treatment

            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.

           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.

            If symptoms continue despite medication, surgery to close the defect with a Gore-tex patch is needed. Some VSDs can be closed with a special device during a cardiac catheterization, although this is infrequently done.

             Surgery for a VSD with no symptoms is controversial. This should be carefully discussed with your health care provider.

                                 Outlook (Prognosis)

                Many small defects will close without therapy. For those defects that do not spontaneously close, the outcome is good with surgical repair. Complications may result if a large defect is not treated.

                                   Possible Complications

Ø     Heart failure

Ø     Infective endocarditis (bacterial infection of the heart)

Ø     Aortic insufficiency (leaking of the valve that separates the left ventricle from the aorta)

Ø     Damage to the electrical conduction system of the heart during surgery (causing arrhythmias)

Ø     Delayed growth and development (failure to thrive in infancy)

Ø     Pulmonary hypertension (high blood pressure in the lungs) leading to failure of the right side of the heart

                                                   Prevention

Except for the case of heart attack associated VSD, this condition is always present at birth.

                                 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.

  

                       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. Many problems can occur if the shunt is large, but small atrial septal defects often cause very few problems and may be found much later in life.

                   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:

ü     Atrial fibrillation (in adults)

ü     Heart failure

ü     Pulmonary overcirculation

ü     Pulmonary hypertension

ü     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)

§        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:

ü     Cardiac catheterization

ü     Chest x-ray

ü     Doppler study of the heart

ü     ECG

ü     Echocardiography

ü     Heart MRI

ü     Transesophageal echocardiography (TEE)

              Chest radiograph with atrial septal defect (direct view): increase of right ventricle, bulging of the pulmonary trunk of the arc on the left contour of the shadow of the heart (indicated by arrow); signs of hypertension of the pulmonary vessels.

                                              

                         Doppler study of the heart

              Aortogram at ASD

              Figure A shows the heart’s position in the body and the location and angle of the MRI images shown in figure C. Figure B is a MRI angiogram, which is sometimes used instead of a standard angiogram. Figure C shows MRI pictures of a normal left ventricle (left image), a left ventricle damaged from a heart attack (middle image), and a left ventricle that isn’t getting enough blood from the coronary arteries (right image).

                                                                        Treatment

                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.

                 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.

               Not all patients with atrial septal defects can have this procedure.

                   Prophylactic (preventive) antibiotics should be given prior to dental procedures to reduce the risk of developing infective endocarditis immediately after surgery for the ASD, but they are not required later on.

                           Outlook (Prognosis)

                       With a small to moderate atrial septal defect, a person may live a normal life span without symptoms. Larger defects may cause disability by middle age because of increased blood flow and shunting of blood back into the pulmonary circulation.

                           Possible Complications

ü     Arrhythmias, particularly atrial fibrillation

ü     Heart failure

ü     Pulmonary hypertension

ü     Stroke

There is no known way to prevent the defect, but some of the complications can be prevented with early detection.

                                  Patent ductus arteriosus

               

                Patent ductus arteriosus (PDA) is a heart problem that occurs soon after birth in some babies. In PDA, there is an abnormal circulation of blood between two of the major arteries near the heart. Before birth, the two major arteries—the aorta and the pulmonary artery—are normally connected by a blood vessel called the ductus arteriosus, which is an essential part of the fetal circulation. After birth, the vessel is supposed to close within a few days as part of the normal changes occurring in the baby's circulation. In some babies, however, the ductus arteriosus remains open (patent). This opening allows blood to flow directly from the aorta into the pulmonary artery, which can put a strain on the heart and increase the blood pressure in the lung arteries.                                          

                             

                                       Signs and symptoms

While some cases of PDA are asymptomatic, common symptoms include:

* tachycardia or other arrhythmia

* respiratory problems

* shortness of breath

* continuous machine-like murmur

* enlarged heart

 

                                                Diagnosis

               PDA is usually diagnosed using non-invasive techniques. The primary methods of detecting PDA are echocardiography, in which sound waves are used to capture the motion of the heart, and associated Doppler studies. Electrocardiography (ECG), in which electrodes are used to record the electrical activity of the heart, is not particularly helpful as there are no specific rhythms or ECG patterns which can be used to detect PDA.

              A chest X-ray may be taken, which reveals the overall size of infant's heart (as a reflection of the combined mass of the cardiac chambers) and the appearance of the blood flow to the lungs. A small PDA most often shows a normal sized heart and normal blood flow to the lungs. A large PDA generally shows an enlarged cardiac silhouette and increased blood flow to the lungs.

                                           Treatment

 The type and timing of surgical repair depends on the child's condition and the type and severity of heart defects.

                 In general, symptoms that indicate that surgery is needed are:

    difficulty breathing because the lungs are wet, congested, or fluid-filled (congestive heart failure)

    problems with heart rate or rhythm (arrhythmias)

    excessive work load on heart that interferes with breathing, feeding, or sleeping

                        An incision may be made through the breastbone (sternum) and between the lungs (mediastinum) while the child is deep asleep and pain-free (under general anesthesia). For some heart defect repairs, the incision is made on the side of the chest, between the ribs (thoracotomy) instead of through the breastbone. Heart-lung bypass may be needed. Tubes are used to re-route the blood through a special pump that adds oxygen to the blood and keeps it warm and moving through the rest of the body while the repair is being done.

                    Most children need to stay in the Intensive Care Unit for 3 to 7 days and stay in the hospital for 5 to 14 days. By the time the child is transferred out of the intensive care unit, most of the tubes and wires have been removed and he is encouraged to resume many of his daily activities. At the time of discharge, the parents are instructed on activity, how to care for the incision and how to give medications their child may need to take such as Digoxin, Lasix, Aldactone and Coumadin. The child needs at least several more weeks at home to recover.

                            Tetralogy of Fallot

  

             Tetralogy of Fallot is a birth defect of the heart consisting of four abnormalities that results in insufficiently oxygenated blood pumped to the body. It is classified as a cyanotic heart defect because the condition leads to cyanosis, a bluish-purple coloration to the skin, and shortness of breath due to low oxygen levels in the blood. Surgery to repair the defects in the heart is usually performed between 3 and 5 years old. In more severe forms, surgery may be indicated earlier. In most cases the heart can be surgically corrected and the outcome is good.

  Tetralogy of Fallot is rare, but it is the most common form of cyanotic congenital heart disease. Patients with tetraology of Fallot have a higher incidence of major non-heart congenital defects.

                           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).

         The classic form of tetralogy includes four related 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

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

             The cause of most congenital heart defects is unknown. Many factors seem to be involved.

 Newborn with tetralogy of Fallot

                   Factors that increase the risk for this condition during pregnancy include:

Ø     Alcoholism in the mother

Ø     Diabetes

Ø     Mother who is over 40 years old

Ø     Poor nutrition during pregnancy

Ø     Rubella or other viral illnesses during pregnancy

              There is a high incidence of chromosomal disorders in children with tetralogy of Fallot, such as Down syndrome and Di George syndrome (a condition that causes heart defects, low calcium levels, and immune deficiency).

                                                   

                                                    Clinic

                    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.

ü     Clubbing of fingers (skin or bone enlargement around the fingernails)

ü     Cyanosis, which becomes more pronounced when the baby is upset

ü     Difficult feeding (poor feeding habits)

ü     Failure to gain weight

ü     Passing out

ü     Poor development

ü     Squatting during episodes of cyanosis

 

Clubbing of fingers (skin or bone enlargement around the fingernails)

                                                 Tests may include:

Ø     Chest x-ray

Ø     Complete blood count (CBC)

Ø     Echocardiogram

Ø     Electrocardiogram (ECG)

Ø     MRI of the heart (generally after surgery)

       

Specific form of heart

Heart in the form of "boots"

                                         

                                               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. Ninety percent 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.

                                       Complications

Ø     Delayed growth and development

Ø     Irregular heart rhythms (arrhythmias)

Ø     Seizures during periods when there is not enough oxygen

Ø     Death

                                              Prevention

                          There is no known prevention.

                                   Transposition of the great vessels

               Transposition of the great vessels is a congenital heart defect in which the position of the two major vessels that carry blood away from the heart - the aorta and the pulmonary artery - is switched (transposed). This defect is classified as a cyanotic heart defect because the condition results in insufficiently oxygenated blood pumped to the body which leads to cyanosis (a bluish-purple coloration to the skin) and shortness of breath.

                                                    Causes

                    The cause of most congenital heart defects is unknown.

                   Factors in the mother that may increase the risk of this condition include:

Ø     Age over 40

Ø     Alcoholism

Ø     Diabetes

Ø     Poor nutrition during pregnancy (prenatal nutrition)

Ø     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. Low blood oxygen leads to cyanosis (a bluish-purple color to the skin) and shortness of breath.

                                     

             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, 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.

                                        Clinic

                      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

ü     Blueness of the skin

ü     Clubbing of the fingers or toes

ü     Poor feeding

ü     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)

ECG

 X-ray

 Angiogram

                                          Pulse oximetry

                                   Treatment

            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.

                  A procedure using cardiac catheterization (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 months.

                          Possible Complications

ü     Arrhythmias

ü     Coronary artery problems

ü     Heart valve problems

                                   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.

                                 Hypoplastic left heart

 

           Hypoplastic left heart syndrome is a congenital heart condition that occurs during the development of the heart in the mother's womb. During the heart's development, parts of the left side of the heart (mitral valve, left ventricle aortic valve, and aorta) do not develop completely. In patients with this condition, the left side of the heart is unable to send enough blood to the body.

           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).

                                                Causes

           Hypoplastic left heart is a rare type of congenital heart disease. It is more common in males than in females. The reason is unknown.

                                          Pathogenesis

                The problem develops before birth when there is not enough growth of the left ventricle and other structures, 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 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.

          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 vesels that carry blood to the lungs). Babies are normally born with two of these connections:

Þ   Foramen ovale (a hole between the right and left atrium)

Þ   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 heart catheterization.

                                    Clinic

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:

ü     Using the pulmonary valve and artery

ü     Connecting the hypoplastic old aorta and coronary arteries to the new aorta

ü     Removing the wall between the atria (atrial septum)

ü     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 Hemifontan 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 - 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.

              Today heart transplantation is considered a better choice than the 3-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 surgical techniques and postoperative management improve. Survival after the first stage is more than 75%.

             The size and function of the right ventricle are important in determining the child's outcome after surgery.

                                     Possible Complications

                                      Complications include:

Ø     Blockage of the artificial shunt

Ø     Chronic diarrhea (from a disease called protein losing enteropathy)

Ø     Fluid in the abdomen (ascites) and in the lungs (pleural effusion)

Ø     Heart failure

Ø     Irregular, fast heart rhythms (arrhythmias)

Ø     Strokes and other neurological complications

Ø     Sudden death

                                        Ebstein's anomaly

                    Ebstein's anomaly is a congenital heart condition which results in an abnormality of the tricuspid valve. In this condition the tricuspid valve is elongated and displaced downward towards the right ventricle. The abnormality causes the tricuspid valve to leak blood backwards into the 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, blood can't get 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.

                                                      Clinic

             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 which 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:

Ø     Chest x-ray

Ø     Magnetic resonance imaging (MRI) of the heart

Ø     Measurement of the electrical activity of the heart (ECG)

Ø     Ultrasound of the heart (echocardiogram)

Ultrasound of the heart (echocardiogram)

           PhCG with Ebstein 's anomaly in the V intercostal space on the left of the sternum: beat the average amplitude, pathological IV tone.

 

                                               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.

                                Aortic stenosis

 

       Aortic stenosis is a heart valve disorder that narrows or obstructs the aortic valve opening. Narrowing of the aortic valve prevents the valve from opening properly and obstructs the flow of blood from the left ventricle to the aorta. This can reduce the amount of blood that flows forward to the body.

         The aorta is the main artery leaving the heart. When blood leaves the heart, it flows from the lower chamber (the left ventricle), through the aortic valve, into the aorta. In aortic stenosis, the aortic valve does not open fully. This restricts blood flow.

                                                   Causes

          As the aortic valve becomes more narrow, the pressure increases inside the left heart ventricle. This causes the left heart ventricle to become thicker, which decreases blood flow and can lead to chest pain. As the pressure continues to rise, blood may back up into the lungs, and you may feel short of breath. Severe forms of aortic stenosis prevent enough blood from reaching the brain and rest of the body. This can cause lightheadedness and fainting.

                     

                                               Clinic                           

                                                  

                    People with aortic stenosis may have no symptoms at all until late in the course of the disease. The diagnosis may have been made when the healthcare provider heard a heart murmur and then performed additional tests.

                              Symptoms of aortic stenosis include:

Ø     Breathlessness with activity

Ø     Chest pain, angina-type

Ø     Crushing, squeezing, pressure, tightness

Ø     Pain increases with exercise, relieved with rest

Ø     Under the chest bone, may move to other areas

Ø     Fainting, weakness, or dizziness with activity

Ø     Sensation of feeling the heart beat (palpitations)

                          In infants and children, symptoms include:

Ø     Becoming tired or fatigued with exertion more easily than others (in mild cases)

Ø     Serious breathing problems that develop within days or weeks of birth (in severe cases)

              Children with mild or moderate aortic stenosis may get worse as they get older. They also run the risk of developing a heart infection (bacterial endocarditis).

                                          Exams and Tests

             The health care provider will be able to feel a vibration or movement when placing a hand over the person's heart. A heart murmur, click, or other abnormal sound is almost always heard through a stethoscope. There may be a faint pulse or changes in the quality of the pulse in the neck (this is called pulsus parvus et tardus).

              Infants and children with aortic stenosis may be extremely tired, sweaty, and have pale skin and fast breathing. They may also be smaller than other children their age.

               Blood pressure may be low.

                       The following tests may be performed:

ü     Chest x-ray

ü     Doppler echocardiography

ü     ECG

ü     Exercise stress testing

ü     Left cardiac catheterization

ü     MRI of the heart

ü     Transesophageal echocardiogram (TEE)

Chest radiograph with aortic stenosis: the shadow of the heart has the shape of shoe (aortic configuration) with an increase due to hypertrophy of the left ventricle (indicated by arrow), the tip is rounded.

                            Phonogram of heart at aortic stenosis: systolic murmur diamond shape (indicated by arrows).

                      EchoCG at aortic stenosis

         

                                             Treatment

                  If there are no symptoms or symptoms are mild, patients may only need to be monitored by a health care provider.

                  They are usually told not to play competitive sports, even if they don't have symptoms. If symptoms do occur, strenuous activity must be limited.

                Medications are used to treat symptoms of heart failure or abnormal heart rhythms (most commonly atrial fibrillation). These include diuretics (water pills), nitrates, and beta-blockers. High blood pressure should also be treated.

              Surgery to repair or replace the valve is the preferred treatment for adults or children who develop symptoms. Even if symptoms are not very bad, the doctor may recommend surgery. People with no symptoms but worrisome results on diagnostic tests may also require surgery.

                Some high-risk patients may be poor candidates for heart valve surgery. A less invasive procedure called balloon valvuloplasty may be done in adults or children instead. This is a procedure in which a balloon is placed into an artery in the groin, advanced to the heart, placed across the valve, and inflated. This may relieve the obstruction caused by the narrowed valve.

                Children with mild aortic stenosis may be able to participate in most activities and sports. As the illness progresses, sports such as golf and baseball may be permitted, but not more physically demanding activities.

                 Valvuloplasty is often the first choice for surgery in children. Some children may require aortic valve repair or replacement. If possible, the pulmonary valve may be used to replace the aortic valve.

 

                                          Outlook (Prognosis)

                  Without surgery, a person with aortic stenosis who has angina or signs of heart failure may do poorly.

                  Aortic stenosis can be cured with surgery. After surgery there is a risk for irregular heart rhythms, which can cause sudden death, and blood clots, which can cause a stroke. There is also a risk that the new valve will stop working and need to be replaced.

                                               Possible Complications

·        Arrhythmias

·        Endocarditis

·        Left-sided heart failure

·        Left ventricular hypertrophy (enlargement) caused by the extra work of pushing blood through the narrowed valve

                                                   Prevention

Treat strep infections promptly to prevent rheumatic fever, which can cause aortic stenosis. This condition itself often cannot be prevented, but some of the complications can be.

                                Coarctation of the aorta

               Coarctation of the aorta is a birth defect in which the aorta, the major artery from the heart, is narrowed. The narrowing results in high blood pressure before the point of coarctation and low blood pressure beyond the point of coarctation. Most commonly, coarctation is located so that there is high blood pressure in the upper body and arms and low blood pressure in the lower body and legs. Symptoms can include localized hypertension, cold feet or legs, decreased exercise performance, and heart failure.

               Aortic coarctation is a narrowing of part of the aorta (the major artery leading out of the heart). It is a type of birth defect. Coarctation means narrowing.

                                                 Causes

                  The aorta carries blood from the heart to the vessels that supply the body with blood and nutrients. If part of the aorta is narrowed, it is hard for blood to pass through the artery.

                   Aortic coarctation is more common in persons with certain genetic disorders, such as Turner syndrome. However, it can also be due to birth defects of the aortic valves.

                   Aortic coarctation is one of the more common heart conditions that are present at birth (congenital heart conditions). It is usually diagnosed in children or adults under age 40.

                                                Clinic                                         

               Symptoms depend on how much blood can flow through the artery. Other heart defects may also play a role.

               Around half of newborns with this problem will have symptoms in the first few days of life.

        In milder cases, symptoms may not develop until the child has reached adolescence.

                                   Symptoms include:

·        Dizziness or fainting

·        Shortness of breath

·        Pounding headache

·        Chest pain

·        Cold feet or legs

·        Nosebleed

·        Leg cramps with exercise

·        High blood pressure (hypertension) with exercise

·        Decreased ability to exercise

·        Failure to thrive

·        Poor growth

                                       Note: There may be no symptoms.

                                           Exams and Tests

                     The health care provider will perform a physical exam and take blood pressure and pulse in arms and legs.

                       The pulse in the femoral (groin) area or feet will be weaker than the pulse in the arms or the carotid (neck). Sometimes, the femoral pulse may not be felt at all.

                       The blood pressure in legs is usually weaker than in the arms. Blood pressure is usually higher in the arms after infancy.

                       The doctor will use a stethoscope to listen to heart and check for murmurs. People with aortic coarctation have a harsh-sounding murmur that can be heard from the back. Other types of murmurs may also be present.

                         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.

                          Tests to diagnose this condition may include:

ü     Echocardiography is the most common test to diagnose this condition, and it may also be used to monitor the patient after surgery

ü     Chest x-ray

ü     Heart CT may be needed in older children

ü     MRI or MR angiography of the chest may be needed in older children

ü     Cardiac catheterization and aortography

ü     Both Doppler ultrasound and cardiac catheterization can be used to see if there are any differences in blood pressure in different areas of the aorta.

    

The absence of the aortic arch at                    Aortogramma with contrast

Radiogramma

 Aortogramma patient with coarctation of the aorta (right oblique projection): a complete interruption of the aorta shadow in its isthmus (indicated by arrow).

                                               Treatment

                Most newborns with symptoms will have surgery either right after birth or soon afterward. First they will receive medications to stabilize them.

               Children who are diagnosed when they are older will also need surgery. Usually, the symptoms are not as severe, and more time will be taken to plan for surgery.

               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.

             Sometimes, balloon angioplasty may be done instead of surgery, but it has a higher rate of failure.

              Older children usually need medicines to treat high blood pressure after surgery. Some will need lifelong treatment for this problem.

                         

                      

                           Macroscopic remote part of the aorta in coarctation: the lumen of the aorta is narrowed sharply, which shows drawn through a needle injection.

                              

                                         Outlook (Prognosis)

             Coarctation of the aorta can be cured with surgery. Symptoms quickly get better after surgery.

          However, there is an increased risk for death due to heart problems among those who have had their aorta repaired. 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.

          Narrowing or coarctation of the artery can return after surgery. This is more likely in persons who had surgery as a newborn.

                                       Possible Complications

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 (rare complication of surgery to repair coarctation)

    Premature development of coronary artery disease (CAD)

    Severe high blood pressure

    Stroke

                          Long-term complications include:

    Continued narrowing of the aorta

    Endocarditis (infection in the heart)

    High blood pressure

                                                           Prevention

            There is no known way to prevent this disorder; however, being aware of your risk may make early diagnosis and treatment possible.

                                               Rheumatic fever

                 Rheumatic fever is a type of disease characterized by inflammation that affects the heart, joints, skin, spinal cord and brain. It is caused by Group A Streptococcal bacteria and typically affects children between the ages of 6 to 15.

                 Rheumatic fever is widespread around the globe and is considered a serious ailment, particularly in developing countries, with a mortality rate of 2 to 5%. However, the use of antibiotics has made it almost rare in the United States, with fewer eruptions of the disease in the 20th century.

                                                Causes

                 Rheumatic fever is caused by a problem in the immune system. It happens in response to group A Streptococcus pharyngitis (strep throat). In this case, the immune system not only fights the bacteria but also attacks its own tissue. It often attacks heart tissue.

                          The connection between strep throat and rheumatic fever was not realized until the late 19th century. The development and availability of antibiotics in the 20th century is credited for the diminishing frequency of rheumatic fever. Rheumatic fever is not uncommon throughout the world, especially in developing countries, but is less common in the United States since the early 20th century. According to the Merck Manual, the incidence of rheumatic fever in the United States is approximately 1/100,000.

                         

                                      

  Rheumatic fever develops about 20 days after strep throat or scarlet fever. The streptococcus infection which leads to rheumatic fever may be asymptomatic in a third of all cases.

                                                              

                    The morphological changes reflect a systemic disorganization of connective tissue, especially the cardiovascular system with specific necrotic-proliferative reactions (Aschoff's body) and nonspecific exudative manifestations. The latter are more expressive in childhood, which determines a large (compared with adults), severity and activity of the process, the severity of carditis and other manifestations of rheumatic fever. 

 

                  Myocardial Aschoff body – the cells are large with large nuclei; some are multinucleated.

                         

                         Necrotic proliferative reactions in connective tissue

                               

                                           Risk

Factors that may increase your risk of rheumatic fever include:

Ø     Age: 5 to 15 years old

Ø     Previous case of rheumatic fever

Ø     Malnutrition

Ø     Overcrowded living conditions

                                     Symptoms

                  Symptoms usually appear 2 to 4 weeks after a strep infection. They may include:

ü     Pain and swelling in large joints

ü     Fever

ü     Weakness

ü     Muscle aches

ü     Shortness of breath

ü     Chest pain

ü     Nausea and vomiting

ü     Hacking cough

ü     Circular rash

ü     Lumps under the skin

ü     Abnormal, sudden movements of arms and legs

 Diseased Heart Valve

                                                Clinical Manifestations:

                  The clinical manifestations of rheumatic fever include:

Fever

Polyarthralgia (discomfort in the joints without objective evidence of pain, redness or swelling)

Migratory polyarthritis: this asymmetrical and involves the large joints (knees, ankles, elbow and the wrist). The affected joints are painful, red, hot, and swollen for about 24 hours. After the recovery of one group of joints, the attack moves on to other groups of joints. This movement of the attack from one group of joints to the other explains the description of the arthritis as migratory. The polyarthritis lasts 1-4 weeks and subsides without leaving any residual damage in the affected joints.

Carditis: the most serious manifestation of rheumatic fever, involves all the layers of the heart wall simultaneously. The inflammation of the pericardium (outer coating of the heart) is called pericarditis. The inflammation of the myocardium (heart muscle) is called myocarditis. The inflammation of the endocardium (internal lining of the heart wall) is called endocarditis. The involvement of the heart is revealed by the occurrence of new mitral and aortic murmurs and cardiomegaly. Very severe rheumatic heart disease may lead to heart failure. The heart lesions may remain and worsen with every recurrence of the acute rheumatic fever.

Subcutaneous nodules: are several tender swellings 0.5-2cm in diameter. These nodules are found on the extensor surfaces of the bone prominences of the knees, elbows, shoulders, scapulae, the occiput and the spinal processes. The subcutaneous nodules occur in less than 15% of the cases and are indicators of a severe disease.

Sydenham chorea: is characterized by jerky, involuntary and irregular movements of the limbs and face, emotional instability, inattentiveness, clumpsiness and crying out loudly. The movements are usually bilateral but may also be unilateral. The chorea is worsened by stress and disappears when the child is asleep. Sydenham chorea is rare and affects girls more commonly than boys. After several weeks or months, spontaneous remission occurs.

Erythema marginatum: consists of non-pruritic macules or patches with central pallor and a well defined irregular margin on the trunk and the proximal parts of the limbs. Erythema marginatum occurs in 10 % of the cases of acute rheumatic fever.

The laboratory findings include acute phase reactants (leukocytosis, raised erythrocyte sedimentation rate, and elevated C-reactive protein), evidence of a preceding streptococcal infection (elevated or rising antistreptolysin titre, isolation of streptococci from throat swab culture, and positive streptozyme test) and prolonged PR interval in the Electrocardiogram (ECG).

In children aged < 2 years the clinical course of the disease tends to be mild and the correct diagnosis may often be missed in this age group.

              In 1944, the "Jones criteria" provided guidelines for the diagnosis of rheumatic fever. The guidelines, which have been revised and modified, are still used today. In addition to previous infection with streptococcus (i.e. positive throat culture, rising ASO titer), the diagnosis of rheumatic fever requires the presence of 2 major Jones criteria or 1 major plus 2 minor Jones criteria.

                                            Major Jones criteria:

§        carditis

§        migratory polyarthritis (arthritis in two or more joints)

§        Sydenham chorea

§        erythema marginatum (skin rash)  

§        subcutaneous nodules

                                          Minor Jones criteria:

§        arthralgia

§        fever

§        previous rheumatic fever or rheumatic heart disease

§        laboratory findings including elevated erythrocyte sedimentation rate, elevated C-reactive protein, elevated white blood cell count

§        prolonged PR interval on an electrocardiogram (EKG) (a pause in the electrical activity)

 

            Schematic representation of the aetiopathogenic events occurring during the     development of carditis

                

                  Symptoms of heart disease (carditis) are determined in 70-85% of cases at the beginning of the disease and more frequently in subsequent attacks, depending on the preferential localization process in the myocardium, endocardium, pericardium. Due to the complexity of feature extraction destruction of a shell in the heart of practice use the term "rheumatic heart disease.

              Aortic valve showing active valvulitis. The valve is slightly thickened and displays small vegetations – "verrucae"

                  Two-dimensional color flow Doppler image of the left ventricular inflow of a patient with mitral regurgitation in the four-chamber view (top panel) and two-dimensional parasternal long-axis view (lower panel), showing lack of apposition of the leaflets of the mitral valve during systole (arrow) 

                    

                                 X-ray except not always pronounced increase in heart determined by an impairment of tonic and myocardial contractility, mitral or aortic   configuration of the heart

                                     

                                  Doppler image of mitral valve regurgitation

                    prolonged P-Q  as criteria of RF

                                                  

    Arthritis

                              The Role of Polyarthritis in Rheumatic Fever

         Symptoms of polyarthritis include painful, tender, swollen, warm joints and usually occur early in the course of rheumatic fever. The most commonly affected joints associated with polyarthritis related to rheumatic fever are:

    ankles

    knees

    elbows

    wrists

                 Vertebral joints are not usually affected but the following joints may be affected:

    shoulders

    hips

    small joints of the hands and feet

              

         Joint pain and fever associated with rheumatic fever usually subside within 2 weeks. The laboratory value of the erythrocyte sedimentation rate usually returns to normal within 3 months if carditis does not persist.

        Arthralgia associated with rheumatic fever differs from arthralgia associated with rheumatoid arthritis by the absence of tenderness during passive movement of the affected joint (person performing examination moves patient's joint through range of motion).

                                     Sydenham’s chorea

                 Sydenham’s chorea is characterized by involuntary movements, specially on the face and limbs, muscle weakness, disturbances of speech and gait. Children usually exhibit concomitant psycologic dysfunction, especially obsessive-compulsive disorder, increased emotional lability, hyperactivity, irritablility and age-regressed behavior. It is usually a delayed manifestation, and is often the sole manifestation of ARF. However, chorea may occur in association with other major manifestations of RF, particularly in the first attack. Evidence of a recent GAS infection is often difficult to document. Most of the patients experience resolution of the symptomatology after a few months. However, a recurrence rate up to 32% has been described, despite the regular use of secondary benzathine penicillin prophylaxis. Some believe that these episodes represent exacerbations rather than distinct attacks of acute RF.

                                        

                                           

                                    Annular and nodosum erythema

             Annular erythema - pale pink rash in the form of a thin ring-shaped rim, does not rise above the skin surface and then disappears when pressing. Appears in 7-10% of children with rheumatism, mostly at an altitude of disease and usually is fragile.

   

                      

                

                                 Annular and nodosum erythema

      

                           Subcutaneous nodules

 

                        Differential diagnosis of rheumatic fever

·        Juvenile rheumatoid arthritis

·        Systemic lupus erythematosus

·        Infective endocarditis

·        Reactive arthritis

·        Sickle cell disease

·        Drug reactions

·        Other connective tissue diseases

·        Septicaemia

·        Leukaemia

·        Gonoccocal arthritis

·        Tuberculosis

·        Lyme disease

·        Serum sickness

                   

                                                      Investigations:

                As Rheumatic fever is exhibited in different forms, no exact diagnostic test has been developed to test for its presence. A careful exam by a qualified medical practitioner will involve checking the patient's skin for fever, a rash or nodules, arthritis or swelling in the joints, and listening to the heart sounds for any atypical murmurs. An electrocardiogram may also be required to do a thorough testing of the patient's heart to test for irregular rhythms. The doctor may likewise order blood tests to check for the presence of a strep infection.

         Just as there is no particular laboratory test to diagnose Rheumatic fever, there is no cure for it as well. However, the disease can be prevented by immediate treatment of a strep throat infection through antibiotic therapy, such as penicillin injections, erythromycin, or sulfadiazine.                                              

          The investigations done on suspecting acute rheumatic fever are throat swab for culture, Antistreptolysin O titre (ASOT), and blood for acute phase reactants.

                              Treatment

                              

                                    Arthritis

                Salicylates remain the first-line drugs in the treatment of arthritis. The response is usually excellent. Treatment should be started at 80 to 100 mg/kg/day (maximum, 4g daily) for 3-4 weeks. Naproxen (10-15mg/kg/day, bid) is an alternative drug, with very good response. Other nonsteroidal antiinflammatory drugs also can be used.

                                   Carditis

                 Moderate to severe carditis is usually an indication for cortiscoteroids although efficacy in reducing sequelae has not been proven so far. It seems clear that corticosteroids are superior to salicylates in rapidly resolving acute manifestations, but the advantage of the former in preventing a pathologic murmur at 1 year posttreatment was not statistically significant. Prednisone, 2mg/kg/day (maximum, 60mg/day) is used for two weeks and after that, the dose is gradually tapered, reducing 20 to 25% of the previous dose every week. Some advocate the concomitant use of salicylates to avoid rebound. In severe carditis, therapy may be initiated with intravenous methylprednisolone. Intravenous immunoglobulin seems not to alter the extent and severity of carditis or decrease chronic morbitidy.   

                Heart failure usually responds to steroids. Bed rest is always recommended and should be planned on an individual basis. Diuretics and vasodilators may be used in patients with more severe haemodynamic decompensation. Digoxin should be used with caution because of the risk of toxicity in the presence of active myocarditis.  Surgical treatment in the acute stage should be considered when clinical therapy is ineffective to control cardic failure. Valve repair, although technically more difficult, is the first choice for younger patients.  

                                        Chorea

                 Treatment with haloperidol (initial dose of 0.5 to 1mg/kg/day, maximum, 5mg/day) or valproic acid (15-20 mg/kg/day) are helpful in decreasing the severity of involuntary movements but may not improve the behavioral symptoms. Carbamazepine has also been suggested as a first-line treatment for Sydenham’s chorea. Alternatively, phenobarbital also may be used, 5-7mg/kg/day. Treatment is usually maintained for 8-12 weeks. Intravenous immunoglobulin therapy has been suggested.  

                                   Complications

               The development of rheumatic valvular heart disease is the major complication of acute rheumatic fever.

                  The acute phase of rheumatic fever lasts only 6 weeks in 75% of cases. Symptoms improve in 90% of rheumatic fever cases within 12 weeks. Symptoms of rheumatic fever continue for 6 months or more in less than 5% of patients.

The damage to an infected person's heart valves can cause long-term and serious complications. Rheumatic fever can prevent blood from flowing normally through the heart and lead to permanent scarring of the heart valves. This damage can result in an ailment also known as rheumatic heart disease, which usually escapes diagnosis until much later in a person's life.  

         Apart from damage to the heart valves, also known as Mitral stenosis or Aortic stenosis, Rhematic fever can result in complications like Endocarditis, an inflammation of both the heart valves (endocardium) and the inner lining of the heart chambers. Irregular heart rhythm or Arrhythmias may also develop, as well as Pericarditis, a condition characterized by the inflammation of the heart's sac-like lining known as the pericardium.

             In more severe cases of the disease, the inflammation brought about by Rheumatic fever causes so much damage to the heart that it results in heart failure. Surgery will be needed to correct the damage to the heart valves when this happens.

          People who have a family history of Rheumatic fever have a higher risk for contracting the disease. Living in overcrowded communities where poverty and poor nutrition is rampant is also a risk factor. So undergoing surgical procedures such as dental operations can increase the chances for infection by Streptococcal bacteria.

                                    Prevention of rheumatic fever.

               Primary prevention.

                Primary prevention means treatment of the streptococcal upper respiratory infection with antibiotics to prevent the first attack of rheumatic fever. Antibiotic therapy started up to the 9th day of the onset of symptoms of the upper respiratory infection can prevent rheumatic fever.

              Secondary prevention.

                 Secondary prevention means prevention of infection of upper respiratory tract with group A beta haemolytic streptococci in persons who have had an attack of rheumatic fever. The preferred method of secondary prevention is regular monthly intramuscular injections of benzathine penicillin G, 1.200,000 units. Patients with rheumatic carditis need a lifelong secondary prophylaxis. The individuals with no carditis continue with secondary prophylaxis until early twenties provided that at least 5 years will have passed since the last attack of rheumatic fever. Before dental or surgical procedures, patients with rheumatic carditis also need additional antibiotics to prevent infective endocarditis. The secondary prophylaxis of rheumatic fever is not enough for preventing infective endocarditis. The additional antibiotics (gentamycin, amoxycillin, cephalexin, azithromycin or erythromycin) are given within half an hour before the procedure. Remember that it can be prevented by treating a sore throat early with antibiotics. So advice parents not to ignore a child with a sore throat but to bring them for treatment as early as possible.

                             For patients who have had previous bouts of Rheumatic fever, the American Heart Association is recommending regular prophylaxis on a long-term basis to totally destroy any traces of streptococcal infection.

                  Prevention is also achieved through the school system, where children with symptoms of sore throats may be screened for Group A Streptococcal bacteria. As a whole, prompt treatment of strep throat infections through a full course of antibiotic therapy will prevent any occurrence of Rheumatic fever. Without antibiotic treatment, the disease is likely to recur within 3 to 5 years after the initial infection.

                 

                        Acquired heart anomalies

                Acquired heart diseases, as well as congenital, are violations of the heart. But, unlike the congenital, which appear in person before his birth, the development of valvular clear from its title. Acquired heart diseases - a defeat of the heart valves, most often acquired defects manifest themselves as a consequence of rheumatic fever. Often, the development of disease goes by without attacks of rheumatic fever and heart disease is detected when there are complaints from the heart.

                        The most common acquired heart diseases in children are developing because of rheumatism.  In the leaf valve develops an inflammatory process that leads to their damage, destruction and scar deformation. There is when impaired function of heart valves work with increased load. Developed hypertrophy of  heart occur. In the future, the heart cavity expands, decreases contractile force of cardiac muscle, there are signs of cardiac insufficiency.

               We can distinguish the following types of heart defects:

• Valvular insufficiency

• Stenosis

• Combined heart disease

           Insufficiency- kind of heart disease, in which, due to incomplete closure of the valve, due to its defeat, some blood flows back to those portions of the heart, from which it came. This creates an additional strain on the heart increasing its mass, and leads to exhaustion of the heart.

           Stenosis - a defeat heart valve with fusion of its wings. This leads to a narrowing of the openings between the chambers of the heart, which also prevents normal blood flow, partially blocking it.

          Combined heart defects. If the two types of acquired heart disease - valvular insufficiency and stenosis affect the cardiovascular system at the same time there is a combined heart defects.

                                   Mitral insufficiency

                   Hemodynamics in valvular mitral insufficiency is due to a constant retrograde flow of blood in the left atrium during systole of the left ventricle, which causes it to overflow and to hypertrophy. During systole the left atrium into the left ventricle sends more blood than normal, which leads to an expansion and hypertrophy. After that there is stagnation in the pulmonary veins, in the vessels of the pulmonary circulation. This increases the load on the right heart and with decompensation of right ventricular stagnation in the circulation occurs. Ascites and edema appear on the lower extremities. Long-term decompensation leads to changes in the lungs, liver, kidneys and other organs.

                       

                                   Clinic and diagnosis.

               

                     Symptoms of mitral insufficiency appear in children with acute attack of rheumatism and an enlarged heart. This condition develops after a sustained inflammatory process in cardiac muscle, which remains a persistent failure valve.

                Initially, patients complain of weakness, palpitations, pasty legs and feet. Later there are shortness of breath, swelling, pain in the heart. Seen in the heart can be seen bulging. On palpation of the chest is determined by the displacement of cardiac beat to the left.

                                    

The amplified, shifted to the left and spilled apical beat with mitral insufficiency

                   On auscultation over the apex there is blowing systolic murmur, sometimes it is accompanied by systolic tremor.

                 The ECG in patients with mitral insufficiency shows

leftcardiogram, the interval P - Q is prolonged.

                   At phonocardiogram first tone merges with systolic murmur.

                   An extension of the heart to the left in direct projection is showed on X-rays. Waist absent because of the expansion of the left atrium, enlarged left ventricle.

                   Doppler echocardiography may clarify the nature and extent of the damage.

                        Treatment of mitral insufficiency is surgical.

                 Currently there are used two types of operations: the restriction annulus valve (annuloplastics), and the replacement of the valve prosthesis.

Annuloplastics is indicated for the expansion of fibrous ring and the good condition of the valves valves.

                       Treatment depends on the type of valve lesion (you can narrow valve ring, leaf, if good, sew a special prosthesis or replace the valve completely). The prosthesis may be of human tissues (homologous) or animal tissue (heterotransplants). These transplants are treated properly, are most suitable for the growing child's body. The use of synthetic prostheses requires constant use of drugs that reduce blood clotting, so that the valve is not formed clots.

                    The first successful operation of the replacement of the mitral valve prosthesis was made in 1963 by Amosov (Ukraine surgery). Most surgeons use a ball-type prosthesis Starr - Edwards. The operation was performed under extracorporeal circulation. Mortality after mitral valve is 10-15%.

                                            Mitral stenosis

                                  

                   

                    It is the most common rheumatic heart disease, in which there is a narrowing of the left atrioventricular valve. It occurs in 75% of patients with mitral valve disease. After the attacks of rheumatic patients mitral valve is narrowing at the poles and causes can not passes the required amount of blood from the left atrium into the left ventricle. The high occurrence of sclerotic changes in the fibrous ring of the valve and papillary muscles are the reasons of stenosis. Depending on the degree of narrowing of the left atrioventricular aperture stenosis is distinguishing at:

·        severe - at a diameter of up to 0.5cm

·        large - at a diameter of 0,5 to 1cm

·        moderate - at a diameter of more than 1cm.

            

   Mitral stenosis                                                         Mitral stenosis

                                                                          Thickening of the cusps, commissures

                                                                      with calcifications and thrombus,

                                                                     thickening, compaction and

                                                                               shortening of the chord

                                             Physical examination

                   Upon auscultation of an individual with mitral stenosis, the first heart sound is unusually loud and may be palpable (tapping apex beat) because of increased force in closing the mitral valve. The first heart sound is made by the mitral and tricuspid heart valves closing. These are normally synchronous, and the sounds are termed M1 and T1 respectively. M1 becomes louder in mitral stenosis.

                If pulmonary hypertension secondary to mitral stenosis is severe, the P₂ (pulmonic) component of the second heart sound (S₂) will become loud.

               An opening snap which is a high pitched additional sound may be heard after the A₂ (aortic) component of the second heart sound (S₂), which correlates to the forceful opening of the mitral valve. The mitral valve opens when the pressure in the left atrium is greater than the pressure in the left ventricle. This happens in ventricular diastole (after closure of the aortic valve), when the pressure in the ventricle precipitously drops. In individuals with mitral stenosis, the pressure in the left atrium correlates with the severity of the mitral stenosis. As the severity of the mitral stenosis increases, the pressure in the left atrium increases, and the mitral valve opens earlier in ventricular diastole.

              A mid-diastolic rumbling murmur will be heard after the opening snap. The murmur is best heard at the apical region and is not radiated. Since it is low-pitched it should be picked up by the bell of the stethoscope. Rolling the patient towards left, as well as isometric exercise will accentuate the murmur. A thrill might be present when palpating at the apical region of the praecordium.

                              Peripheral signs include:

§        Malar flush - pulmonary hypertension is prominent in patients with mitral stenosis

§        Ankle/sacral edema (oedema) when there is right heart failure

§        Atrial fibrillation - irregular pulse and loss of 'a' wave in jugular venous pressure

§        Left parasternal heave - presence of right ventricular hypertrophy due to pulmonary hypertension

§        Tapping apex beat which is not displaced

                      

                                 

                                                  

                   

                               Periferal edema

                    Normal ECG

                      Atrial fibrillation

                                

             PhonoCG

                           EchoCG

                                           Treatment

                 Patients with mitral stenosis are operated on opened or closed techniques. The first mitral commissurotomia in our country was made by Bakulev in 1952. In 1962 Amosov performed open heart surgery of mitral commissurotomia.

            Intraventricular mitral commissurotomy with using of dubble poles dilator is currently the main method of treatment of patients with mitral stenosis. This method is most simple, effective and safe. Mortality is 2-5%. Excellent and good results are in 72-75% of cases.

                

        

                                                         

                              Insufficiency of aortic valve

                               Hemodynamic abnormalities

 

                   Hemodynamic disturbances in aortic insufficiency by the absence of complete closure of the valve opening during diastole the left ventricle, resulting in some blood from the aorta returns to the ventricle. Ventricle imposes additional workload, so hypertrophy and expansion occur. Tonogenic dilatation is replaced by myogenic and ventricle can not cope with the load and decompensation develops, initially in a small circle, then to the systemic circulation. Patients have signs of failure of the coronary circulation.

                                        Clinic

              Patients complain of weakness, retrosternal pain, dizziness and heart beat, and then short of breath. Objectively there are pale skin and pulsation of neck vessels. Pulse is fast and high. The minimum arterial pressure is dramatically reduced with high maximum.

        Auscultation: on the basis of aortic diastolic noise is auscultated (in the third intercostal space on the left at the edge of the sternum), sometimes simultaneously listening to the aorta and the systolic over the second intercostal space on the right at the edge of the sternum (associated with aortic valve sclerosis).

        The ECG - leftcardiogram and signs of deficiency of the coronary circulation.

                                        

         X-rays revealed aortic configuration of the heart due to expansion of left ventricle. Ascending aorta is extended.

                          

                             

                  Doppler image of aortic valve with regurgitation

   

                                             Treatment

              Surgical treatment consists of aortic valve replacement under extracorporeal circulation. The most frequently ball aortic valves are used. Mortality after surgery is still up to 20%. .

                                            Tricuspid insufficiency

                  

                    In its pure form is found in 3%, in combination with other defects - 25% of cases. The cause of this evil is rheumatic fever, which leads to valvulitis, sclerosis, and shortening of the valves.    

                            Circulatory dynamics

                         When tricuspid regurgitation during ventricular systole is not tight closured valve leaflets the blood from the right ventricle back into the right atrium. Stagnation of blood in the right atrium leads to increased pressure in it, blood stagnation observed in the hollow veins and the liver.

                                       Clinic

 

                         The most characteristic features in tricuspid insufficiency are pulsations of the neck veins and the liver, synchronously with the systole of the ventricles of the heart. Liver is enlarged, painful. On auscultation systolic murmur is auscultated on  right part of the sternum. X-rays indicates an increasing of the right heart, and when combined with other defects expansion of all parts of the heart. Cardiac catheterization provides an opportunity to identify high pressure in the right atrium and the portal veins.

ECG in tricuspid valve inssuficiency

                  Increased pressure and pulsation of blood flow in the portal vein in tricuspid insufficiency.

Echo symptoms of tricuspid insufficiency

          Radiography of the heart with varying degrees of tricuspid insufficiency

                                 Treatment

                    The operation on the tricuspid insufficiency is performed on the open heart surgery. It is annuloplasty or prosthetic valve ball prosthesis.

                           Tricuspid stenosis

                     This isolated defect is extremely rare disease. Most often occurs in conjunction with mitral stenosis.

                      Etiology is rheumatic endocarditis.

                       

                                        Circulatory dynamics

             In systole of the right atrium the blood dose not pass completely through the narrowed venous right atrium to ventricle. The right atrium is stretching, while the right ventricle and the left half of the heart remain the same normal load.

                               

                     View looking down on the right atrium (no tricuspid valve is present)

                                            Clinic

           Clinic in stenosis of the tricuspid valves is shortness of breath, marked cyanosis, enlargement and pulsation of neck veins and a significant increase in the liver. On auscultation over the right half of the heart at the right edge of the sternum presystolic murmur and amplified first tone may be heard. Percussion and radiological examination determine a significant expansion of the right atrium.

          At cardiac catheterization high pressure in the right atrium and decrease in the right ventricle there are determined. The pressure gradient between the right atrium and right ventricle is higher than 5mm Hg.

Echo- and Doppler sings of tricuspid stenosis

        

         PhonoCG

  

Hypertrophy of the right ventricle with stenosis of the right atrioventricular

valve

                                 Treatment

                  

           Operation on the narrowing of the tricuspid valve is commissurotomy. When mitral stenosis is combined with stenosis of the tricuspid valve operations are performed simultaneously.

                                   

                                 

                                                    Aortic stenosis

                         

                        

                                 

                                            Circulatory dynamics

         A narrow valve makes the heart work harder just to pump the blood through the valve to the body. A leaky valve lets blood back into the heart after it has been pumped out. The heart must therefore pump more blood forward to make up for the blood that is leaking backwards.

         Either way the extra work may cause symptoms of heart failure, such as shortness of breath. Early on the shortness of breath may be noticeable only with exercise. Later, with the progression of valve disease, a patient could experience shortness of breath with even light activity or at rest. Some patients will be unable to sleep flat in bed or may awaken from sleep short of breath. Another sign of heart failure that may occasionally occur is swelling of feet, particularly prominent later in the afternoon or evening although other conditions, such as varicose veins, can also cause this to occur.

        The extra work the heart has to perform may also cause chest pain or angina pectoris similar to the symptoms of a heart attack. It may be difficult to tell the difference between heart valve disease and narrowing of the blood vessels to the heart itself.

                        

     

                 Aortic valve stenosis

          A failing aortic valve may cause a variety of symptoms including:

• Shortness of breath,

• Chest pain (angina pectoris)

• Dizziness or loss of consciousness (passing out)

            Often patients complain of attacks of rheumatic fever, shortness of breath, general weakness, fatigue and pain in the heart, dizziness and fainting. Pulse is small. Systolic pressure is lowered in normal or high diastolic. Heart borders are shifted left by larger left ventricle. In the second intercostal space on the right there is systolic murmur. X-rays determined by enlargement of the heart to the left through the left ventricle of the heart Waist well defined.

                  The ECG reveales left ventricular overload, S - T intervals are shifted down, there is a negative T wave.

   

                                                  

                                   PhonoCG

                    X-ray sings of aortic stenosis

        Doppler sings of aortic stenosis

                          Treatment

                    The operation is indicated in the early stages of the defect. In the presence of Right heart decompensation surgery is contraindicated. The operation is performed on open and closed methods.

                Closed aortic commissurotomy is often performed by intraventricular access.

                Open aortic commissurotomy is performed under extracorporeal circulation with general hypothermia to 30°C and additional cooling of the heart with ice (cardioplegia).