Pediatric Cardiology

In the Pediatric Cardiology Department, diagnostic and therapeutic services are offered for cardiac problems of fetus, infant, minor child, child and adolescent (until age of 18 years).Most common pediatric cardiac diseases include congenital heart diseases (hole, stenosis), acquired heart diseases (rheumatoid valve diseases, infectious diseases) and cardiac rhythm disorders.

The baseline complaints of our patients often include murmur, cyanosis, poor exercise capacity, chest pain, palpitation, dizziness, syncope or high blood pressure.The diagnosis is usually made with diagnostic methods such as electrocardiography (ECG), telecardiography and echocardiography (ECHO).Heart of fetus can be assessed with fetal ECHO during intra-uterine life.If necessary, other methods are used including but not limited to exercise test, HOLTER monitoring, cardiac catheterization and angiography.Moreover, children who are doing sports should be assessed for the risk of heart disease.Please see following titles for further details.

STRUCTURE OF HEART

The heart consists of four chambers.The upper chambers are called atrium where veins drain into, while lower chambers are called ventricle that pumps blood to the body.The wall between two atria is septum. Congenital holes on this wall are called atrial septal defect (ASD).The wall between two ventricles is ventricular septum. The holes on this wall are called ventricular septal defect (VSD).

The valve between right atrium and right ventricle is called tricuspid valve, while the valve between left atrium and left ventricle is called mitral valve.The great artery originating from the right ventricle is pulmonary artery and it pumps oxygen-poor venous blood to the lungs.The great artery originating from the left ventricle is aorta and it pumps oxygen rich arterial blood to the body.Pulmonary valve is located between right ventricle and pulmonary artery, while aortic valve is located between left ventricle and aorta.

FUNCTION OF HEART

A child’s heart usually beats 100.000 to 150.000 times a day.It depends on the child’s heart rate.Heart rate is modified according to needs of the body. Heart rate slows down when the child falls asleep, as body’s oxygen need decreases, but the heart rate accelerates when the child moves and does exercise, as the body needs more oxygen.For some cardiac rhythm disorders, heart rate may be too slow or fast.

The heart pumps blood to all organs of the body after each contraction (heart beat).This blood is distributed to all cells.Right ventricle pumps oxygen-poor venous blood to the lungs through pulmonary artery, and thus, the venous blood is oxygenated there.Meanwhile, carbon dioxide is excreted into airways to be exhaled.Oxygenated blood in the lungs flows back to left atrium.Next, it is pumped from the left atrium to the left ventricle through the mitral valve.The left ventricle pumps oxygen-rich blood to the aorta through the aortic valve.Aorta flows arterial blood to the entire body.Blood used by body organs is carried to right atrium through veins.Blood flows from right atrium into right ventricle through tricuspid valve and it is pumped to the lungs.This circulation is continuously repeated.

CONGENITAL HEART DISEASES

Congenital heart diseases are structural disorders that emerge in early pregnancy and exist at birth.Congenital heart disease is observed in about 8 of 1000 newborns.If mother, father or first degree relatives have congenital heart disease, the risk is higher for the unborn baby.Although there are a wide variety of these conditions, most of them are holes on the walls that divide cardiac chambers and stenoses in heart valves and vessels.  In some cases, congenital conditions are severe, such as an underdeveloped chamber, valve and vessel.Holes differ greatly in terms of size, number and location as well as severity (mild, moderate and severe).

WHY DO CONGENITAL HEART DISEASES DEVELOP?

Congenital heart diseases develop in very early pregnancy before formation of organs starts.The underlying cause is not known in most cases.Although it is known that some of these conditions are inherited in nature, a genetic link is demonstrated in very rare diseases.The risk for congenital heart disease is high in some genetic diseases (Down Syndrome, Turner syndrome).The use of medications that may harm the baby, maternal infections (i.e. Rubella) and exposure to radiation in the first trimester of pregnancy may lead to congenital heart diseases.When family history is investigated, no cause of congenital heart disease can be identified.Thus, it is generally accepted that congenital heart diseases are secondary to hereditary and environmental factors.For an unborn baby, heart disease can be investigated with an ultrasound test, namely “Fetal Echocardiography” (see.Fetal Echocardiography).

WHAT ARE SYMPTOMS OF CONGENITAL HEART DISEASES?

There is a large variety of congenital heart diseases.Although some of them are asymptomatic or symptoms are very mild, others follow a very serious course.Severe heart diseases manifest symptoms within the first few months or even within a few days.These symptoms are cyanosis, difficulty eating, fatigue while suckling, high respiratory rate, shortness of breath, failure to gain weight or respiratory tract infection (bronchitis, tuberculosis) at frequent intervals.Poor exercise capacity, palpitation, chest pain and syncope may be observed in older children.In some of these diseases, there is no symptom or symptoms are insignificant; only an innocent murmur is heard on the examination in the admission.

WHAT SHOULD THE CHILD WITH CONGENITAL HEART DISEASE WATCH FOR?

It should be remembered that there are various types of congenital heart diseases and each disease may require specific supervision.However, general measures are mostly similar.  The child should use antibiotic for some specific interventional conditions to prevent the heart from infection (infective endocarditis).The primary physician should inform the family above the conditions that require prevention and dose and duration of antibiotic treatment and the families should be given “Infective Endocarditis Prevention Guideline” (seeInfective Endocarditis).

Most children with congenital heart disease do not require restriction of activity.On the contrary, sports and physical activity should be encouraged in order to psychologically support the children and improve cardiac performance.For some diseases, activities requiring strenuous effort such as “contest” are forbidden.  These conditions should necessarily be determined by the pediatric cardiologist and the child should not be needlessly kept away from sports.These measures should be determined according to type and severity of the disease.Those children are encouraged to do less strenuous activities.

As is the case with healthy children, the children with congenital heart disease should be vaccinated.Some specific conditions require additional protective vaccines apart from other healthy children.There is no specific nutritional requirement for those children or general nutritional rules apply.If the weight gain of the infant is insufficient due to the heart disease, it is recommended to feed the baby with high-calorie formula or nutrients.Some conditions may require a specific diet.Children may usually continue the normal educational life.Activity restriction may be necessary for some patients.Children can continue attending school by taking certain measures; the child does not use stairs in school or have the classes on ground floor and does not attend gym classes.

Children should be regularly followed up by a pediatric cardiologist.

WHAT IS INFECTIVE ENDOCARDITIS?

Infective endocarditis implies the inflammation of endocardium, heart valves or cardiac vessels.It is a very rare condition amongst the children with no heart disease, while the risk increases for the children with heart disease.Causative microorganisms are usually the bacterial flora of the oral cavity.Those bacteria do not harm the healthy individuals under normal circumstances.On the other hand, they may cause very critical cardiac infections in children with heart disease when they reach the bloodstream.Therefore, oral hygiene and dental care are very important for people with heart disease.Children with heart disease should regularly brush their teeth and visit dentists.Protective antibiotic should be prescribed for these children, if bacterial contamination of the bloodstream is likely.High-risk conditions include oral and dental interventions that may cause bleeding, tonsil and adenoid surgeries, and urinary tract and reproductive system surgeries.The booklet that informs families about prevention of infective endocarditis addresses high-risk conditions and recommended dosage and administration times of antibiotics.

WHAT IS MURMUR?

Murmur is a sound similar to “blowing” that is caused by abnormal blood flow in the heart and vessels and can be heard by a physician in cardiac auscultation.Murmur is heard due to abnormal blood flow in almost all congenital and acquired heart diseases.Moreover, the most common murmurs in children are also heard without any failure in blood flow.They are called “innocent murmur” or “physiological murmur”.“Innocent murmur” is commonly used since it is the ideal term to specify that there is no disease in the heart.An experienced physician can mostly clarify whether the murmur is innocent or not.If the murmur is innocent, no further examination is required.If the physician is not sure about the nature of murmur, the patient should be referred to a Pediatric Cardiologist.In some cases, it may be difficult to understand whether the murmur is innocent or not. If so, echocardiography is the most effective method to establish the final diagnosis. 

WHAT IS FETAL ECHOCARDIOGRAPHY?

It is the ultrasound imaging of the baby’s heart in intra-uterine period.Ultrasound is an imaging method that uses the sound waves and it has been been safely used in the medicine for a long time.It causes no known harm for the baby or mother.Ideally, heart of a baby can be imaged starting at gestational age of 16-18 weeks.The most effective imaging is generally at Week 20 to 24.Fetal echocardiography is necessary for some pregnant women as summarized below: 

• Any suspicious sign in the heart of unborn baby on the ultrasound scanned by Gynecologist and Obstetrician, 
• History of congenital heart disease in family (mother, father, siblings or close relatives) (these conditions increase the risk for baby),
• If the future mother has diabetes or develop diabetes during pregnancy,
• In case of connective tissue disorder or phenylketonuria in mother,
• Maternal conditions that may harm baby’s organs within the first 3 months (trimester) of pregnancy (medication, alcohol, certain infections, radiation etc.),
• Actual or suspected chromosome disorder (Down syndrome etc) in baby,
• If any abnormal condition is found in other organs of baby (kidney, brain, stomach-intestine, skeleton, lip-palate),
• High nuchal translucency in baby,
• Persistently high or low heart rate or rhythm disorder in baby,
• In some cases, fetal echocardiography is recommended for pregnancies at advanced ages (above 35 years of age), multiple pregnancies (twine, triplet) and pregnancies of in vitro fertilization.​

Procedure:

Fetal echocardiography is a pain-free procedure that aims assessment of the unborn baby with an ultrasound probe involves scanning the fetus with an ultrasound transducer placed on the mother’s abdomen and it has no known harm.Image of the baby’s heart changes depending on the gestational week, body weight and position of fetus, location of the placenta, mother’s physical structure (lean-overweight) and quality of the device.

Echocardiographic study lasts approximately 15 to 30 minutes.It may prolong, if the quality of image is poor or there is suspicion of a complex anomaly.It is easy to establish diagnosis of certain congenital heart diseases in the intra-uterine life, but there also conditions that are extremely difficult to be diagnosed.In some cases, the lesion may progress throughout the pregnancy and it is reasonable to repeat the scan at certain intervals for such conditions.This method helps diagnosis of a fetal condition by 65 to 90 percent depending on the quality of image.

If fetal echocardiography points to a condition, an intervention is not required for most cases and the newborn infant is supervised by echocardiography at certain intervals.Certain heart diseases are severe and they may require early surgical procedure or invasive intervention.This group of pregnant women is recommended to give birth at a hospital, wherein the newborn infant can be managed, and thus, necessary preparations are started for the baby.For some rare heart diseases; the prognosis is even more severe and surgical or invasive technique cannot completely correct the condition.This group of families is informed about the condition and abortion of the pregnancy is recommended.For such cases, families should be informed jointly by pediatric cardiologists and cardiovascular surgeons.Depending on the family’s decision, pregnancy is either aborted or continued.

Some heart diseases allow management of fetal condition by administering certain drugs to the mother.

WHAT IS ACUTE RHEUMATIC FEVER?

Acute rheumatic fever is publicly known as either “heart rheumatism” or simply “rheumatism”.Rheumatism is caused by Group A beta hemolytical streptococci (beta) which is also the cause of pharyngitis and tonsillitis in children.Risk of rheumatism may occur in children with history of throat infection, if antibiotics are not administered at proper dose for an appropriate length of time.The risk is minimal for patients, who are duly treated.It is most common in children aged 5 to 15 years.Symptoms occur approximately 2-3 weeks after the throat infection.The condition primarily involves brain, joint, skin and brain.Usually, it is first manifested by swelling, pain, heat and mild redness in joints (knees, wrists and ankles).The major effect involves heart.It causes damage to and dysfunction in heart valves.Involuntary abnormal movements in face, hands, arms and legs (Chorea), behavioral disorders and attitude changes can be observed in children deu to involvement of brain.Dermatologic signs are rare.Patients should be started on treatment and follow-up immediately after the definitive diagnosis is established.

IS RHEUMATOID HEART DISEASE A TREATABLE CONDITION?

Mild cases of heart valve diseases may be completely cured, if treatment is started at early stage, but the damage is mostly irreversible.

DOES EVERY THROAT INFECTION CAUSE RHEUMATISM?

Only throat infections caused by Group A beta hemolytic streptococcus lead to rheumatism.Rheumatism does not develop due to other viral and bacterial throat infections.Differential diagnosis require throat culture or other examinations.Streptococcal throat infections are primarily manifested by sore throat, difficulty swallowing, fever and painful swellings in neck.Sometimes, mild symptoms may make the diagnosis difficult.

WHAT SHOULD THE CHILD WITH RHEUMATIC FEVER WATCH FOR?

The risk of recurrent infection is very high in children who has history of rheumatic fever.Therefore, certain measures should be taken to prevent recurrence of the same infection in children who have history of rheumatic fever.This prevention is based on long-term protection with regular use of antibiotics.The child can be protected against recurrence by administering penicillin once every 3 weeks through intramuscular route or antibiotic tablets every day.Preventive measures should be maintained lifelong, if the heart is involved, or it should, otherwise, be maintained until the patient is 21 years old.

As is the case with congenital heart diseases, the risk of infection is valid for the damaged heart valve in rheumatic heart diseases.Accordingly, oral and dental hygiene and health require close care (seeInfective Endocarditis).Restrictions on exercise and dietary therapy may required depending on the severity of the heart valve disease.

CHEST PAIN IN CHILDREN

Chest pain is a complaint which is more common in adults.Since it is an important symptom of heart attacks, it is a publicly known and taken into consideration.Chest pain can also occur in children or even, chest pain is the third most common type of pain in children following headache and abdominal pain.

Chest pain in children is primarily musculoskeletal pain of the chest wall.Chest pain in children can also be caused by lung diseases, asthma, diseases of esophagus and stomach, heart diseases and psychological etiology.Most important one is the pain originating from heart diseases.These diseases mostly originate from the heart muscle (myocardium) or coronary arteries that feed the heart muscle.If there is a congenital malformation or stenosis in coronary arteries of a child, cardiac muscle cannot be supplied oxygen and other nutrients and chest pain develops, as is the case with adults.

On the contrary to adults, chest pain secondary to cardiovascular disease is rare in children.If the chest pain is secondary to cardiovascular etiology, early diagnosis and treatment is a necessity, as such diseases have very serious consequences.Advanced investigation of heart diseases is strictly required for children, who complain of chest pain associated with poor exercise capacity, fainting and palpitation especially during or after exercise.

SYNCOPE IN CHILDREN

Temporary loss of consciousness as a result of sudden decrease in blood flow to the brain is called syncope.It is common in healthy children and adolescents.Half of children who reached adolescence have fainted at least once.It doesn’t indicate a serious condition although it is scary for the family.“Simple syncope” (vasovagal syncope) develops secondary to activation of autonomous nervous system and accounts for a substantial part of all syncope cases in childhood.This type of syncope is mostly provoked by sudden-onset severe pain, severe anxiety, serious excitement, standing still for extended period of time, sight of blood and exposure to heat, but they do not last long.

On the other hand, syncope can also be a sign of a very important heart disease.Children may develop syncope in certain heart muscle conditions, congenital heart diseases and cardiac rhythm disorders (heart rate below or above the normal beats of heart per minute, respectively).Cardiovascular system should be carefully reviewed for exertional dizziness and syncope, syncope associated with chest pain and palpitation, history of poor exercise capacity and family history of syncope and sudden death.

Syncope can be preceded by dizziness, fatigue, blurry vision, nausea and hot flashes.Injuries may be faced, if the patient falls down secondary to syncope.For simple syncopes with non-cardiac etiology, it is usually sufficient to elevate legs of patient.Patient usually gains consciousness a few minutes after first aid is applied.Excluding simple syncopes, treatment method varies depending on the underlying cause.

CARDIAC ASSESSMENT FOR CHILDREN WHO DO SPORTS

As it is commonly known, sports activities bear a load that is far beyond normal level on the heart.Therefore, cardiac problems may develop in kids after training heavily who had no complaints in their daily lives.Recently, parents’ awareness has been gradually increasing due to news of “child-adolescent deaths in sports events” in the press.These clinical pictures are publicly regarded as “heart attack”, but they may have very different etiologies and there is virtually no similarity with heart attacks in adults.Children with cardiac muscle diseases, certain congenital diseases, certain heart diseases that are surgically treated and rhythm disorder are not recommended to exercise.Therefore, children who exercise heavily or strenuously should be examined by a physician at least once a year with regard to cardiac health.​​