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Congenital and acquired heart disease in children

Therapeutic and surgical developments gave children a glimmer of hope

There are many children suffering from heart disease, some are congenital while others are acquired, meaning that the child suffers from it due to certain factors. Medical advances in pediatric cardiology have been remarkable in recent years thanks to the medical progress achieved at the level of surgery or treatments in addition to the availability of many specialized children’s hospitals given the sensitivity of dealing with this age group and the importance of receiving quality healthcare.

The heart is divided into four hollow chambers, two on the right and two on the left. To pump blood throughout the body, the heart uses its left and right sides for different tasks. The right side of the heart moves blood to the lungs through vessels called pulmonary arteries. In the lungs, blood picks up oxygen then returns to the heart’s left side through the pulmonary veins. The left side of the heart then pumps the blood through the aorta and out to the rest of the body.

A baby’s heart starts to develop at conception, but is completely formed by 8 weeks into the pregnancy. Congenital heart defects happen during this important first 8 weeks of the baby’s development. Acquired heart disease may occur after months or years. Researchers aren’t sure exactly what causes most of these defects, but they think genetics, certain medical conditions, some medications and environmental factors, such as smoking, may play a role.

One of the main causes that can lead to congenital heart disease is the advanced age of the mother at conception, or maternal diabetes, where congenital heart defects accounted for about 50% of defects and deformities that affect the fetus due to maternal diabetes because of high blood sugar levels and the consequent chemical changes.

Maternal thyroid dysfunction is associated with increased risk for congenital heart disease, causing a decrease in fetal growth and may lead to preterm birth. It also has a strong impact on the fetal heart because they cause increased heart rate.

Women with lupus are more than twice as likely to have a child with a congenital heart defect. Lupus is an autoimmune disease that can cause a number of health issues, affecting joints, organs, and/or blood vessels.

Congenital heart disease can have a range of symptoms, because the condition refers to several different types of heart defect. General signs of congenital heart disease can include a blue tinge to the skin (cyanosis), rapid breathing and rapid heartbeat, swelling in the legs, tummy and around the eyes, shortness of breath in babies during feeding and in older children and adults during exercise. 

Congenital Heart Defects in Children

In such cases, the problem is in the structure of the heart where the baby is born. The heart is formed in the first weeks of pregnancy to begin the process of pulse and the formation of large blood vessels passing to and from the heart, and may begin the development of birth defect in the heart. Most congenital heart defects result from problems early in your child’s heart development, the cause of which is unknown. However, certain environmental and genetic risk factors may play a role. 

Congenital heart defects sometimes run in families and may be associated with a genetic syndrome. Smoking during pregnancy increases the likelihood of a congenital heart defect in the baby. Certain medications taken during pregnancy may cause birth defects, including congenital heart defects. Give your doctor a complete list of medications you take before attempting to become pregnant. 

There are many different types of congenital heart defects, falling mainly into these categories:

Holes in the heart: Holes can form in the walls between heart chambers or between major blood vessels leaving the heart. In certain situations, these holes allow oxygen-poor blood to mix with oxygen-rich blood, resulting in less oxygen being carried to your child’s body. Depending on the size of the hole, this lack of sufficient oxygen can cause your child’s skin or fingernails to appear blue or possibly lead to congestive heart failure. A ventricular septal defect is a hole in the wall between the right and left chambers on the lower half of the heart (ventricles). An atrial septal defect occurs when there’s a hole between the upper heart chambers (atria).

Obstructed blood flow: When blood vessels or heart valves are narrow because of a heart defect, the heart must work harder to pump blood through them. Eventually, this leads to enlarging of the heart and thickening of the heart muscle. Examples of this type of defect are pulmonary stenosis or aortic stenosis.

Abnormal blood vessels: Several congenital heart defects happen when blood vessels going to and from the heart don’t form correctly, or they’re not positioned the way they’re supposed to be. A defect called transposition of the great arteries occurs when the pulmonary artery and the aorta are on the wrong sides of the heart.

Heart valve abnormalities: If the heart valves can’t open and close correctly, blood can’t flow smoothly. One example of this type of defect is called Ebstein’s anomaly. In Ebstein’s anomaly, the tricuspid valve — which is located between the right atrium and the right ventricle — is malformed and often leaks. Another example is pulmonary atresia, in which the pulmonary valve is missing, causing abnormal blood flow to the lungs.

An underdeveloped heart: Sometimes, a major portion of the heart fails to develop properly. For example, in hypoplastic left heart syndrome, the left side of the heart hasn’t developed enough to effectively pump enough blood to the body.

A combination of defects: Some infants are born with several heart defects. Tetralogy of Fallot is a combination of four defects: a hole in the wall between the heart’s ventricles, a narrowed passage between the right ventricle and pulmonary artery, a shift in the connection of the aorta to the heart, and thickened muscle in the right ventricle. Some congenital heart defects cause no signs or symptoms. For some people, signs or symptoms occur later in life. They can recur years after you’ve had treatment for a heart defect. Common congenital heart disease symptoms you might have as an adult include abnormal heart rhythms (arrhythmias), a bluish tint to the skin, lips and fingernails (cyanosis), shortness of breath, tiring quickly upon exertion and swelling of body tissue or organs (edema). 

Some congenital heart defects can be repaired using catheterization techniques, which allow the repair to be done without surgically opening the chest and heart. 

Some mild congenital heart defects can be treated with medications that help the heart work more efficiently. You might also need medications to prevent blood clots or to control an irregular heartbeat.

Heart Arrhythmia

Heart arrhythmia is a congenital disease in children or newborns accompanied with congenital heart defects. It can be serious and even life-threatening. If your child’s heart beats too fast or too slow, it might affect the heart’s ability to pump blood efficiently to the rest of the body. Irregular blood flow can damage organs, including the kidneys, liver, heart, and brain. Some cases may pose a risk to the health of the child, requiring immediate medical intervention through drug therapy. Some tachycardias are life-threatening or significantly interfere with a child’s normal activities. Radiofrequency catheter ablation, is done with several catheters in the heart. One is positioned right over the area that’s causing the tachycardia. Then its tip is heated and that small area of the heart is altered so electrical current won’t pass through the tissue. 

Sometimes surgery that interrupts the abnormal connection in the heart is required to permanently stop the tachycardia. A variety of rhythm disorders can be controlled with a pacemaker. Slow heart rates, such as heart block, are the most common reason to use a pacemaker. It works by sending small, painless amounts of electricity to the heart to make it beat. Some cases appear immediately after birth while others appear after the age of five years.


The pericardium is a thin sac that surrounds your heart. It protects and lubricates your heart and keeps it in place within your chest. Problems can occur when the pericardium becomes enflamed or fills with fluid. The swelling can damage your heart and affect its function. The pericardium has a few important roles: It keeps your heart fixed in place within your chest cavity. It prevents your heart from stretching too much and overfilling with blood. It lubricates your heart to prevent friction with the tissues around it as it beats.

Pericarditis is an inflammation or infection of the pericardium, the thin sac (membrane) that surrounds the heart. There is a small amount of fluid between the inner and outer layers of the pericardium. When the pericardium becomes inflamed, the amount of fluid between its two layers increases, compressing the heart and interfering with the heart’s ability to function properly.

In children, pericarditis is most likely to occur following surgery to repair congenital (present at birth) heart defects or acquired heart disease. However, other causes may include infection (viral, bacterial, fungal, parasitic), chest trauma or injury or connective tissue disorders such as systemic lupus erythematosus (lupus).

The following are the most common symptoms of pericarditis. However, each child may experience symptoms differently.  Symptoms may include chest pain that can be felt especially behind the breastbone, and sometimes beneath the clavicle (collarbone), neck, and left shoulder, chest pain that is a sharp, piercing pain over the center or left side of the chest that increases as the child takes a deep breath, a low-grade fever, irritability, fatigue, loss of appetite and irregular heartbeat.

Treatment may include medication (i.e., analgesics and anti-inflammatory drugs), aspiration (removal) of excess fluid and surgery.

Rheumatic Heart Disease 

Rheumatic fever is an inflammatory disease that can affect many connective tissues, especially in the heart. Untreated or under-treated strep infections put a person at increased risk. Children who get repeated strep throat infections are at the most risk for rheumatic fever and rheumatic heart disease. This disease can seriously cause permanent damage to the heart valves and heart muscle, by causing myocarditis, requiring immediate medical intervention to reduce the risks and complications that can affect the child. The onset of rheumatic fever usually occurs about two to four weeks after a strep throat infection. Rheumatic fever signs and symptoms, which result from inflammation in the heart, joints, skin or central nervous system, can include fever, painful and tender joints, red, hot or swollen joints, chest pain, fatigue, flat or slightly raised, painless rash with a ragged edge, jerky, uncontrollable body movements most often in the hands, feet and face and outbursts of unusual behavior, such as crying or inappropriate laughing. The goals of treatment for rheumatic fever are to destroy remaining group A streptococcal bacteria, relieve symptoms, control inflammation and prevent the condition from returning. Your child’s doctor will prescribe penicillin or another antibiotic to eliminate remaining strep bacteria. Our doctor will prescribe a pain reliever, such as aspirin or naproxen to reduce inflammation, fever and pain. If symptoms are severe or your child isn’t responding to the anti-inflammatory drugs, your doctor might prescribe a corticosteroid.

Advanced Treatments

There is no doubt that the medical development in the field of heart disease has significantly reflected on the treatments and we can say that heart disease and its treatments have benefited the most from the medical development in recent decades, whether at the level of drug treatments or surgery. Diagnosis has witnessed great development as well and pregnant women are now more aware of fetal monitoring as well as postpartum follow-up with a specialized doctor.

Your child’s healthcare provider may prescribe antibiotics to treat the acute strep infection, long-term antibiotics to prevent recurrent strep infection, steroids or nonsteroidal anti-inflammatory medicines to ease inflammation in the heart and in other parts of the body, water pills (diuretics) if heart failure develops and/or anti-inflammatory medicine for the management of fever and arthritis symptoms. Your child may also need other medicines. Some children need surgery to fix or replace damaged heart valves. Surgical treatment has witnessed remarkable developments; 30% of children used to die during open heart surgery in the 1970s, while today this rate is less than 5%.

Most often the valve leak caused by the disease is mild and does not need treatment. If the leak is severe enough to strain and enlarge the heart, surgery may be needed to eliminate the leak. This surgery may involve repair of the damaged valve. Sometimes the valve is too badly damaged to repair, in which case it must be replaced by an artificial valve. Proper nutrition is key in the treatment of rheumatic heart disease after consulting a pediatrician and a nutritionist to provide advice and guidance that aim to strengthen the child’s body. Weak bodies or malnutrition have a negative impact on the child’s health and may delay corrective surgeries.

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