(Heart Diseases from Birth in Children)

Acyanotic Heart Diseases

a. Shunt Lesions:

Shunt lesions are caused by a defect (hole) on the auricle (ASD), ventricle (VSD), or on the wall between the two auricles (AVSD), or an open sub-vein (PDA) which any neonates must have between the aortas from heart and which must close after birth. In direct proportion to size of the defect, these holes cause a small amount of blood to flow to the side with low pressure (usually to circulation in lungs) whenever the heart contracts so the balance of circulation is affected resulting in unnecessary and excess contraction of heart.

Atrial Septal Defect (ASD)

ASD means a defect on the wall between the auricles of heart. Thus, some amount of arterial blood flows to the right side of the heart. Depending on the increase in blood flowing to the lungs, it may lead to cardiac dilatation and enlargement of lung veins over the years.

Diagnosis

It does not usually present any symptoms for long years. Even there have been cases where diagnose is unable to establish until 30-60 years old for individuals who have rarely seen a doctor. This disease is suspected only when murmur and some additional sounds are auscultated during an examination for another reason, or when cardiac dilatation is viewed on the X-ray film. The final diagnosis is established after examination and echocardiography performed by a pediatric cardiologist.

Treatment

The size of the hole determines the time of the treatment. The defects failing to close spontaneously and causing raise in pressure of pulmonary artery are usually closed at the age of 3-6, in other words, before the child starts school. No problems occur during and after the operation. A surgical scar in the center of the chest remains. Different surgical options are available to ensure that this scar is on locations that are less seen. For some patients, the defect can be closed using a catheter. This practice is unable to apply on every patient and can only be performed if specific measurements seem okay.

Actions to take

In general, if no additional pathologies appear in the heart, it does not require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as operation, circumcision, tooth extraction and filling in the advice booklet of American Cardiology Association. However, protective treatment is recommended during the first six months after the operation or catheterization treatment. To protect patients against unexpected complications, a doctor check is required every one year. This applies to operated patients with less frequents.

Ventricular Septal Defect (VSD)

There is a defect (hole) on the wall between the two ventricles of the heart. Some amount of oxygenated blood in the left side of the heart flows to the right side because of this defect. Severity of patient complaint and treatment method (medication or operation) depends largely on the size of defect, in other words, on the volume of blood passing through.

Diagnosis

In small VSDs, the diagnosis is incidentally established by murmur heard during examination of a doctor seen for any reasons like fever and cough. The final diagnosis is made by examination and echocardiography by a pediatric cardiologist. In medium and large VSDs, since the volume of blood passing through the defect would be high, on one hand it causes increase in pressure of pulmonary arteries and lung diseases on the other hand the increased blood flow causes heart to work harder thus to become tired and enlarged. In medium VSDs, generally during infancy, or several weeks after birth in case of large defects, the symptoms include quick breaths, excessive sweat and tiredness particularly when nursing, and poor gain weight. The diagnosis is made by auscultation of murmurs during examination and determination of symptoms for cardiac insufficiency. Lung diseases such as pneumonia and bronchitis are also common in these patients. Because the children are taken to see a doctor in our country only when they are ill, the murmur and other symptoms are barely recognized in seriously ill and crying babies.
The symptoms of a lung disease can shadow the symptoms of a heart disease. Some of these patients partially recover through treatment for pulmonary infection but they can become recursively ill therefore making final diagnose can be delayed. For early diagnose, we need to gain the habit of taking our healthy children for doctor control. The final diagnosis is established after examination and echocardiography performed by a pediatric cardiologist.

Treatment

Generally, no treatment is needed for small defects. However, protective treatment is required against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as circumcision, tooth extraction and filling, and operation. In medium and large VSDs, increased pulmonary blood flow and whether the defect becomes small as the child grows are checked, and supportive drug therapy is applied to regulate the function of heart and to reduce the effects of symptoms of cardiac insufficiency. In patients with no recovery, this defect must be closed by a surgical operation. In VSDs failing to close spontaneously or shrink, the convenient time for a surgical operation is usually at the age of 1-2. The defect is patched to prevent blood flow. Recently, the medium size VSDs which appear to have a right diameter and location have been considered to close without an operation as in ASDs. Some part of these defects can be closed by this method but it is not as common as ASDs.

Actions to take

Although the possibility that the defect is closed spontaneously varies by location, size and structure of the defect, it highly possible with a rate of 70-80%. However, some other problems (e.g. anatomical and functional failure of valves) might arise during closure of certain defects. The patient therefore should be periodically checked. For patients who have a small defect or shrunken over time but not fully closed and who do not require a surgical operation, they require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as operation, circumcision, tooth extraction and filling. This also applies to operated patients during the first six months after the operation.

Patent Ductus Arteriosus (PDA)

Each fetus has a sub-vein between the two major arteries from heart before birth. This vein is spontaneously closed within the first 3 days after birth. In case of PDA, this vein fails closing after birth and remains open. The rate of failing to close in premature infants is higher. However, a large number of such defects in premature infants can be closed spontaneously or by medication within several months. If the vein fails to be closed in infants born as scheduled, spontaneous closure is highly rare. In such patients, a portion of oxygenated blood that is supposed to flow to the body through the open vein flows to the lungs. On one hand, this causes increase in pressure of pulmonary artery, on the other hand the increased blood flow causes heart to work harder and become tired more.

Diagnosis

Generally, no symptoms are present in young children who have a small diameter of vein. The diagnosis is incidentally made when a murmur is auscultated. If the diameter of vein is large, the symptoms include quick breaths even during infancy, excessive sweat and tiredness particularly when nursing, and poor weight gain. The diagnosis is made by auscultation of murmurs during examination and determination of symptoms for cardiac insufficiency. Lung diseases such as pneumonia and bronchitis are also common in these patients. Because the children are taken to see a doctor in our country only when they are ill, the murmur and other symptoms are barely recognized in seriously ill and crying babies. Some of these patients partially recover through treatment for pulmonary infection but they can become recursively ill therefore making final diagnose can be delayed. For early diagnose, we need to gain the habit of taking our healthy children for doctor control. The final diagnosis is established after examination and echocardiography performed by a pediatric cardiologist.

Treatment

The defect is likely to be closed spontaneously by medication in premature infants and some patients. In the cases where no spontaneous closure is not possible, the first-line therapy is that groin vein is entered by a catheter to close the defect with instruments called “coil” or “plug”. In patients with a very large defect, the open vein is tied up with a minor operation to stop blood flow if it is not possible to close with a catheter.

Actions to take

In untreated defects or defects not closed with catheter method, they require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as circumcision, tooth extraction, tooth filling, and operation. The patients need periodic checks by doctors.

b. Obstructive Lesions:

The stenosis of heart valves or main veins from the heart (may include one or more areas or valves) occurs in these patients. The myocardium becomes weak over time because it has had to work against stenosis, resulting in clinical pictures such as sudden death.

Pulmonary stenosis:

The stenosis of valve of main vein from the right side of the heart to the lungs occurs, or slightly inferior or posterior to this valve, and end of branches. The right ventricle of the heart is forced to work harder in order to pump blood from narrowed vein or valve to the lungs. However, the disease does not present any symptoms that can be recognized by parents until later stages of the disease in which the heart overworks. The stenosis can be mild, moderate and severe. Complaints of the patient, symptoms, whether any treatments are required, and treatment method totally depend on the level and location of stenosis.

Diagnosis

This disease is suspected only when murmur and some additional sounds are auscultated during an examination for another reason. The final diagnosis is established by an examination and echocardiography performed by a pediatric cardiologists.

Treatment

The mild stenosis is not intervened as it does not majorly damage the heart. However, the level of stenosis in some cases can increase over time. Therefore, the level of stenosis should be followed up by examination and echocardiography. In patients with moderate and severe stenosis of valve, the stenosis is often enlarged by a balloon catheter.
Generally, the balloon catheter is not used if the stenosis occurs inferior to the valve. If the stenosis occurs posterior to the valve, the balloon catheter is not as appropriate as stenosis of valves. If this method does not work, some patients might require an operation.

Actions to take

They require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as circumcision, tooth extraction, tooth filling, and operation. The patients need periodic checks by doctors.

Aortic Stenosis

The stenosis of valve of main vein from the left side of the heart to the lungs occurs, or slightly inferior or posterior to this valve. This causes the left ventricle of the heart to work more to pump blood to the organs. However, the disease does not present any symptoms that can be recognized by parents until later stages of the disease in which the heart overworks. The stenosis can be mild, moderate and severe. Complaints of the patient, symptoms, whether any treatments are required, and treatment method totally depend on the level and location of stenosis. The cause of this case is that this valve consists of two leaflets instead of three. This is called bicuspid aortic valve.

Diagnosis

In cases other than severe, the diagnosis is usually made by auscultation of murmur during an examination. The severe cases present with quick tiredness, fatigue, chest pain, and faint. The final diagnosis is established after an examination and echocardiography performed by a pediatric cardiologist.

Treatment

The mild stenosis is not intervened as it does not majorly damage the heart. However, the level of stenosis in some cases can increase over time. Therefore, the level of stenosis should be followed up by echocardiography. In patients with moderate and severe stenosis of valve, the stenosis is often enlarged by a balloon catheter. If this method does not work some patients might require an operation or even a valve replacement.

Actions to take

They require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as circumcision, tooth extraction, tooth filling, and operation. The patients need periodic checks by doctors. In case of complaints such as chest pain and faint particularly during effort, the immediate check is required. The patients with severe stenosis should avoid sports activities including intense exercise.

Coarctation of Aorta

Coarctation occurs most commonly in a short segment of the aorta just beyond where the arteries to the head and arms take off, as the aorta arches inferiorly toward the abdomen and legs. It causes the left ventricle of the heart work harder to pump the blood through the organs. Furthermore, the blood pressure is low inferior to stenosis while the veins to the brain and arms have a higher blood pressure. The stenosis can be mild, moderate and severe. Complaints of the patient, symptoms, whether any treatments are required, and treatment method totally depend on the level and type of stenosis.

Diagnosis

In cases other than severe, the diagnosis is usually made by auscultation of murmur during an examination. The symptoms can include high blood pressure and associated frequent nosebleed and headache. The final diagnosis is established after an examination and echocardiography performed by a pediatric cardiologist.

Treatment

The mild stenosis is not intervened as it does not damage the child. However, the level of stenosis in some cases can increase over time. Therefore, the level of stenosis should be followed up by echocardiography. In patients with moderate and severe stenosis of valve, the stenosis is often enlarged by a balloon catheter. If this method does not work, rarely an operation might be required.

Actions to take

They require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as circumcision, tooth extraction, tooth filling, and operation. The patients need periodic checks by doctors. The patients with severe stenosis should avoid sports activities including intense exercise.

c. Mitral valve Prolapse (MVP)

One of the two leaflets, or both, of the valve between the left auricle and left ventricle of the heart is thicker, looser and softer than normal. In some patients, the filaments holding the valve even can be longer than normal. The valve therefore is prolapsed toward the left ventricle when the heart contracts. And sometimes the valve is unable to close fully because of this prolapse, resulting in blood flowing back to the left auricle. The family and close relatives of the patients identified with prolapse have a possibility of 30% of having this disease, that is, it can be hereditary.

Diagnosis

It is suspected when patients complain about and see a doctor for tachycardia, chest pain and faint. Some patients are diagnosed by auscultation of murmur or a click-like sound during heart examination when they see a doctor for other reasons even though they have no complaints. The final diagnosis is made by echocardiography and color Doppler. If the patient also complains about tachycardia, twenty-four-hour Holter monitorization may be required.

Treatment

Most do not require any treatments. Periodic controls should be enough. However medication might be needed in case of some types of tachycardia. If mitral regurgitation (blood leaking) is present, protective treatment is required against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as operation, circumcision, tooth extraction, and tooth filling. The recommendations should be followed to prevent tachycardia. (See recommendations for patients with tachycardia)

Actions to take

If mitral valve does not have a serious problem, no need to restrict physical education or sports activities. Your doctor will indicate any restrictions if needed.
A control is required since the symptoms might be varied over time. The recommendations might be changed after the control if necessary.
In case an operation is needed, you must warn your doctor against the possibility of tachycardia. In case of mitral regurgitation and prior to any dental interventions and operations, infective endocarditis prophylaxis advised by your doctor should be followed.
Being dehydrated for some reason or using diuretics might result in fainting.

Cyanotic Heart Diseases:

Cyanotic heart diseases present with reduced blood flow circulating through the lungs, and are caused by mixing of blood that needs to flow to the lungs for oxygenation into the systemic circulation (namely into our body) depending on developmental disorder of different parts of the right side of the heart. Therefore, these patients have cyanosis (skin and mucosa turn bluish purple) in direct proportion to the level of mixing The most common type is the Tetralogy of Fallot (Blue/purple disease in children).

Similar clinical pictures can occur if the valve between the right auricle and ventricle of the heart, or the valve of the pulmonary artery or itself does not form or develop normally.

Tetralogy of Fallot:

Several problems may be present together in this disease.
Stenosis of pulmonary artery (PS)
Defect-hole between the ventricles (VSD)
The main artery is not where it should be and displaced to the right ventricle.

The most significant symptom of this disease is cyanosis, and it is identified at birth of some of the patients whereas some of them do not have cyanosis at birth but it appears gradually over months. In addition, thickening of finger tips towards the age of 1can be included in symptoms. Mildly ill patients might not present cyanosis at all.

Diagnosis

What conspicuous in infants is that the lips turn increasingly into blue (cyanosis) over months. Cyanosis becomes more obvious when the baby cries. The infant might start to have cyanosis and syncope attacks between the months 3 and 9. Changes in conscious from significant darkening of skin, absent state to full syncope in severe cases occur particularly when the infant wakes up from its sleep or after crying. If this starts, pediatric cardiologist must be informed immediately. The prophylactics may be required to prevent such attacks, or an operation may be necessary if prophylactics do not work. It is suspected when cyanosis is noticed and murmur is auscultated at the examination. The final diagnosis is made after examination and echocardiography performed by a pediatric cardiologist. A doctor check is required every 2 or 3 months until operation for anemia and preventing syncope. The operation schedule can be moved to an earlier time when necessary and becomes a must.

Treatment

Since no spontaneous recovery or closure of defect is impossible for this disease, a surgical correction is definitely necessary. Cardiac catheterization is usually administrated to the patients prior to the operation. Operation is often scheduled after 1 year old in our country. If pulmonary artery is not well developed in some infants, a shunt surgery is needed prior to the correction operation to improve this.

Actions to take

Before and after the correction operation the patients require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as circumcision, tooth extraction, tooth filling, and operation. The patients should be followed up for arrhythmia after the operation and periodically controlled by a doctor.

d. Mixing Lesions

The oxygenated blood gets mixed with the oxygen-poor blood and the oxygen-poor blood is distributed through the body due to abnormal development of the heart in these patients. The patients are cyanotic (skin and mucosa turn into bluish – purple). The most typical types are:

Transposition of Great Arteries:

In this disease, the pulmonary artery from the left ventricle of the heart, and the aorta from the right ventricle arise reversely. In other words, deoxygenated blood cannot be oxygenated in the lungs thus deoxygenated blood circulates through the body. To keep the patients alive after birth, a defect (hole) is required for oxygenated blood to flow to the other side.

Diagnosis

A cyanosis is present usually appearing on the first day of life. In addition, even if the heart has a defect, i.e. a hole, the disease might be noticed later because the cyanosis is mild. Murmur can be detected at the examination but not heard with every patient. The final diagnosis is established by examination and echocardiography performed by a pediatric cardiologist.

Treatment

Exact solution is to transpose the two main veins by a surgery within two weeks after birth. However, in case of insufficient space between the two sides, balloon catheterization can be used to make a space between the two auricles. Although it is not an ideal solution, other surgical methods (replacement of ventricles) are used for patients that are failed to be identified within that period thus remain not operated

Actions to take

The patients with successful arterial transposition operation require a protective treatment against endocarditis (inflammation of inner layer of the heart) prior to some interventions such as circumcision, tooth extraction, tooth filling, and operation. Some patients (especially those who have undergone a surgery to replace the ventricles), should be followed up for arrhythmia because postoperative tachycardia is common, and checked periodically by a doctor.

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