Congenital Leaky Heart Disease in Children

Leaky heart disease is a term used when a person has a leak in the heart wall due to a valve or heart septum abnormality . If the leak occurs in the upper chamber it is called ASD and if it occurs in the lower chamber it is called VSD. Generally, leaky heart disease affects older people with heart valve abnormalities that occur because one of the valves can no longer close properly. Whereas in infants and children, this condition is caused by a hole between the walls in the left and right chambers of the heart that does not close completely as a congenital abnormality or as a result of complications from an infectious disease.

Initial symptoms are "trivial" in the form of high fever accompanied by vomiting and diarrhea. However, after being examined, the doctor found that the child had a hole in the atrium of the heart, which is often called a leaky heart, one of the congenital heart diseases. Because the heart valves are leaky, some of the clean blood (which contains oxygen) mixes with dirty blood (contains toxic carbon dioxide). Mixed dirty blood is carried along with clean blood throughout the body with the general symptoms that appear are blue on the nails, tongue and mouth.

Because the symptoms are not clear, in many cases this congenital heart defect is not detected immediately, and can be overlooked. It is possible that the symptoms of coughing and high fever experienced by children are considered as common colds, because the development of their organs is not yet perfect. However, the doctor may suspect it if the child often experiences these common disorders, and has difficulty recovering, or has delayed growth, and has a strange sound heard through a stethoscope because his heartbeat is very noisy. This disorder must be treated immediately with ordinary surgery ( open heart surgery ), or more recently with heart treatment without open surgery (amplatzer).

Abnormalities that occur

The heart is a two-way pump that circulates blood throughout the body and back to the heart. This organ is mainly formed from a collection of muscles called the myocardium. Blood flows in each muscle via veins (blood vessels that return to the heart) into the atria (top of the heart), which are called the atria. It then enters the (lower) chambers, called the ventricles, which pump blood to the arteries (blood vessels from the heart to the rest of the body). The flow of blood in the chambers of the heart is controlled by unidirectional valves. The right side of the heart pumps blood to the pulmonary arteries and then to the lungs (picking up oxygen). Meanwhile, the left side of the heart pumps blood from the lungs into the aorta, and it flows around the body.

There are many types of heart disease that can affect anyone, both adults and children. However, there are two leaky heart diseases as congenital diseases experienced by children from birth as heart defects in the form of heart structure or function since the baby was in the womb. What commonly happens is: ASD ( atrial septal defect ) or perforated heart chamber partition, due to abnormalities in the heart valves and VSD ( ventricular septal defect ) or perforated heart chambers, where there is a hole between the wall in the left and right chambers of the heart because it does not close completely. Therefore, parents need to know a number of things that lead to these symptoms and conditions, so that when they appear they can be treated immediately, so that the cure rate will be higher.

  • ASD (Atrial Septal Defect), perforated heart porch

This disease is a condition that occurs when the hole between the two heart chambers is not closed by a valve. The hole that separates the left and right atrium will allow oxygen-rich blood to flow back to the lungs. If left unchecked, this condition can cause permanent damage to the blood vessels of the lungs. If the leak is large and left untreated, the blood flow can damage the heart and lungs, causing heart failure .

  • VSD (Ventricle Septal Defect), perforated heart chamber valve.

This disease is a heart disorder characterized by holes in the heart's interventricular valves. The hole causes the heart to leak in the left and right chambers of the heart, so that some of the oxygen-rich blood returns to the lungs. If the hole is small, this interference is not a significant problem. However, be careful if the resulting hole is large enough, because this disease can cause heart failure, irregular heart rhythm or pulmonary hypertension, which is a condition that occurs when the pressure in the blood vessels from the heart to the lungs is too high.

In children aged 2 years, VSD usually only triggers small holes and does not cause symptoms. The hole will usually close by itself after some time. New problems will arise in children who show signs of heart failure, which is a complication of congenital heart disease. In this case, the sufferer must immediately receive treatment.

"The severity of this leaky heart case is seen from how large the diameter of the leak is. There are several criteria, but what is often used as a benchmark is the diameter, namely: (a) Very small, if the size is 0-3 millimeters (mm), (b) 3-6 mm small, (c) Medium 6-8 mm, and ( d) Large if the size is above 8 mm," said dr. Sire.

Heart leaks in children can be identified by listening to heart sounds. Doctors can hear abnormal heart sounds such as heart sounds or additional heart sounds. Congenital heart disease is a disease that needs to be avoided, because it not only endangers the baby's health, but also causes stunted growth and development.

“Patients with congenital heart disease, especially those with large leaks, are prone to respiratory tract infections, so they are prone to infection. If you often get sick because of an infection, it can affect its growth. In order to improve his condition, the patient who has congenital heart disease must be kept away from being in an environment where there are coughs, colds or cigarette smoke, because children are easily infected," said dr. Sire. "We have to remind parents to maintain the child's immune system by completing immunizations. And, don't forget the nutritional intake, "he continued.

There are not many incidents in Indonesia, but it is quite concerning

"The incidence of congenital leaky heart disease in Indonesia is not much. But there is a trend in the range of 50-100 cases per year, said dr. Sire. “This needs attention, because the risk factors are increasing. First, from the point of view of the diet of pregnant women it increases leakage or congenital heart disease, because it is increasingly non-organic, whatever is consumed as long as it is delicious so that safety limits are not considered. Second, the detection tool is getting better, there is Eco Cardiography. Now it's easier to detect it, "he continued.

Harapan Kita National Heart Center said that every year 40,000 babies born in Indonesia have congenital heart defects. As many as 24,000 of them require surgery in the first year of life.

Symptoms and signs to watch out for

"In the case of a leaky heart, the result of damage to the heart chambers cannot function normally because dirty blood and clean blood are mixed as a result of damage to the heart chambers," said dr. Sire.

This condition can be characterized by symptoms, such as:

  • Easily feel tired and faint . Caused by a small supply of clean blood to the heart caused by a mixture of clean blood and dirty blood due to a leaky septum.
  • Heart murmur . Conditions when the heart has a blowing, whistling or hoarse sound that appears when blood moves through the heart or the blood vessels around the heart.
  • Shortness of breath and chest pain often . This happens because of the mixing of dirty blood containing carbon dioxide and clean blood containing oxygen. Dirty blood will affect the flow of clean blood which causes shortness of breath.
  • Swelling of the organs around the heart. Due to the accumulation of many dirty substances.
  • Frequent urination . The frequency is increasing.
  • Hemoglobin levels People with a leaky heart experience an increase in Hb levels in the blood until it reaches 20.0 which is normally 13.0-15.0.

The cause is not yet clearly known .

"What causes it is not known with certainty, but this disorder can occur in the first trimester of pregnancy, when the baby's heart is formed under pressure. Usually, mothers who have babies and have congenital heart defects are fine during pregnancy," said dr. Sire.

Once a baby with a leaky heart is born, if the leak is small or the illness is relatively mild, it will not be detected right away. Even those whose conditions are severe do not immediately have symptoms and are only detected after undergoing a period of growth and development. However, some are detected as soon as the baby is born.

Risk factors to avoid

"The risk factor for congenital heart disease is related to the condition of the mother during pregnancy. The first is exposure to certain infections, which are usually more prone to viral infections. Second, exposure to chemical substances, such as cigarette smoke. Then, alcohol consumption. Pregnant women sometimes have the urge to drink alcohol and drugs without a doctor's prescription," said dr. Sire.

"We also have to be wary of certain herbs, because people nowadays are very happy with herbal ones. Not bad, but we are still careful. Herbs are not definitely safe, because there are also things we have to be aware of. Risk factors occur during pregnancy which have an impact on the process of forming the fetus during pregnancy," he continued.

Examination to confirm the diagnosis

“Medical diagnosis always starts from the symptoms. If leaky heart disease is still at a mild level, most of the cases don't give symptoms. Even if there is, it's not that significant. And, if it is detected early, it is usually coincidental, for example from frequent visits to the doctor because of the immunization program, for example, the doctor accidentally discovers the symptoms of a patient's heart murmur, "said dr. Sire. Diagnosis begins with anamnesis of the patient's parents if the patient is still a child and with a physical examination, especially to detect the presence or absence of cyanosis. After that, the diagnosis can be made by chest X-ray, echocardiography and electrocardiography.

“When examining the heart with a stethoscope, usually no heart sounds are heard. Well, this is a start for further examination. Follow-up examinations can be through a stethoscope or X-rays. If the X-ray results show something abnormal, then an electrocardiography (ECG) examination is performed. From this inspection it can be detected correctly or there is no leak. And we can find out approximately how big the size of the leak is," he continued.

Anamnesis.
In the history of congenital heart disease, the symptoms that appear depend on the severity of the disorder. Symptoms of a shunt disorder ( shunt ) from right to left will be more obvious, such as the appearance of cyanosis (blue color of the skin). Parents usually report that there is tachypnea (fluid discharge from the nose or mouth that accumulates after the baby is born) in infants at rest or breastfeeding, there is cyanosis when the child is active, the child's activities are limited, the child's growth and development are stunted, there are recurrent respiratory infections (tracheal tract infections). acute respiratory failure) or extremity edema (swelling due to excessive accumulation of fluid in the cells).

Physical examination

The results of a physical examination of congenital heart disease depend on the disorder suffered. The most simple and clear assessment is the evaluation of the presence or absence of cyanosis. Cyanosis describes a shunt relationship from the right heart to the left heart. Another examination is auscultation of additional heart sounds (examination with a stethoscope to listen to the sound of a heartbeat).

Supporting investigation

Investigations for the diagnosis of congenital heart disease can be in the form of echocardiography, chest X-ray, electrocardiography and laboratory tests.

  • Echocardiography. To assess the heart chambers and measure the size of the defect that occurs. Echocardiography with Doppler can assess the direction of blood flow and reflux / turning. In addition, echocardiography can assess the size of the aortic root and other large vessels.
  • Thoracic X-Ray. View normal heart shape and size in minor congenital heart disease with small lesions. In more major abnormalities, the chest X-ray picture can vary.
  • Electrocardiography (EKG). Seeing structural abnormalities of the heart from deviations in the direction of the heart's electricity.
  • Laboratory Examination (hematology and biochemistry). Performed on patients with pulmonary hypertension due to congenital heart disease. On hematological examination, an increase in hematocrit and erythrocytosis can be found. In addition, it can also be found a decrease in glucose levels due to increased erythrocytes.
  • Other tests that are also needed are kidney function and liver function to determine whether there is organ failure due to congenital heart disease.

Doctor's help

"Handling of leaky heart cases is adjusted to the level of leakage that occurs, said dr. Sire.

  • If the leak is small .
    There are not many complaints, it can be overcome with good nutritional intake since the baby will be able to close on its own, or spontaneously, without the help of medicine.
  • In case of moderate leakage.
    If the child's growth and development conditions and nutritional intake are good, in some patients it can also close on its own. Because this is a long-term program, medication is needed for his heart to function properly.
  • If the case is in the big category .
    Depending on how big the leak is, medical intervention is needed. If you can still be helped with drugs because with a large leak, the work of the heart increases. The problem is that those with large leaks must be prepared to undergo surgery to close the leak in the heart septum.

Good nutritional intake

Good nutritional intake is important considering the child is in its infancy. Also to strengthen the body's immunity so that it does not get sick easily.

Drugs

To keep heart conditions under control, usually with heart medications, keeping the heart in a stable condition. This must be maintained by the parents because this is indeed a long term. Congenital heart conditions, especially those with large leaks, are prone to respiratory infections, which can affect their growth.

Operation

Indirect surgery is done when the patient is still a baby. There are considerations at what age is considered fit enough for this child to face surgery. Now, during this operation, it is the parents' job to be assisted by a doctor so that the patient's or child's condition remains in ideal condition. Again, you have to have good nutritional intake. If the leak is big. Drinking milk is also slow. Old impression. Her parents have to help her drink a little bit of milk," said dr. Sire.

Like surgery in general, open surgery of the patient's chest is opened, the heart is seen. The leaky part of the heart will be closed using the lining of the heart itself or by using a patch (what material is the plaster made of?) . While the closed operation is inserted into the hose and see where the leak is. The results are very good, the recovery is faster and there are no surgical scars. This operation has been performed by many specialist Thoracic Cardiac & Vascular Surgeons (BTKV) in Indonesia with a high success rate.

Self-care that can be done at home

After undergoing examinations and X-rays, the healing process for babies with leaky hearts cannot simply be carried out before the baby is three months old. Usually, the doctor will carry out further examinations before deciding on a leaky heart treatment step. Several considerations were made, including the age and health condition of the patient.

If you feel that you are old enough and have adequate health, the doctor will perform heart surgery or by catheterization. If the heart leak is located below, it will be difficult to do catheterization, for this reason this method cannot be done for all people with heart leaks.

Doctor tips

To prevent the possibility of a leaky heart, it is necessary to be prepared from early pregnancy. Pregnant women must diligently check for infection risks, starting with toxo, then if it's negative, continue with other checks.

  • During pregnancy, try to eat healthy foods. Avoid risky drinks. Avoid cigarette smoke. We must be vigilant in the first trimester of pregnancy because this is a crucial period because this is the formation of the body's organs. Maintain or prepare for a healthy pregnancy.

Article reviewed by dr. Baginda, Sp.A, M.Sc. Med (Child Specialist at EMC Cikarang Hospital).