Commonly Treated Conditions

Texas Center for Pediatric and Congenital Heart disease treats a full spectrum of pediatric cardiac conditions. Commonly treated conditions include:

Aortic Stenosis (AS)

One of the most common heart valve diseases, aortic stenosis can affect both children and adults. In children, the characteristic narrowing of the aortic valve can be caused by a genetic condition, rheumatic fever or uncontrolled artery disease. In older adults, deposits of calcium on the valve’s leaflets can alter the valve’s function. Although aortic stenosis can become serious, early diagnosis and treatment to repair or replace the valve can help your child enjoy a full life.

Treatment plans can vary based on your child’s age, health, medical history, the level of aortic stenosis and the prognosis.

Mild to moderate aortic stenosis typically does not require treatment but should be monitored through regular visits with a cardiologist to ensure the condition doesn’t worsen or start affecting the heart. Severe to critical aortic stenosis can be treated first with medication to help improve function until valve repair or replacement can be scheduled at a pediatric heart center. Treatment options include:

  • Balloon dilation: Using the cardiac catheterization procedure, a tiny tube is again guide through the blood vessels to the heart where a balloon is inflated in the valve stretching it open.
  • Valvotomy: Your pediatric cardiologist may surgically release the scar tissue preventing the aortic valve from opening.
  • Valve replacement: The aortic valve can be surgically replaced with a biological valve made from animal tissue or mechanical valve made from carbon fibers. A portion of a pulmonary artery, either from the child or a donor, or an aortic valve from a donor can also be used to replace the valve and part of the aorta.

Atrial Septal Defect (ASD)

Known as a hole in the heart, atrial septal defect is a congenital condition that causes babies to be born with an opening in the wall that divides the two upper chambers (atria) of the heart. The wall, or atrial septum, separates oxygen-rich blood headed to the body from oxygen-depleted blood returning to the heart. A hole in the atrial septum allows the blood to mix and affects how the heart and lungs work. If the hole doesn’t close on its own, a pediatric cardiologist can repair the atrial septum to avoid the long-term health issues untreated atrial septal defects can cause.

As a child develops, small atrial septal defects may close on their own without treatment during infancy or early childhood. Your pediatric cardiologist may recommend monitoring your child’s heart, then repairing the hole if it doesn’t close on its own. Medication may be prescribed to lessen symptoms and help reduce the affects of ASD.

To repair an atrial septal defect, a pediatric cardiologist patches or plugs the hole during cardiac catheterization or open heart surgery.

  • Cardiac catheterization: A tiny tube is inserted through a blood vessel in the groin and threaded into the heart where a mesh patch or plug is placed. Heart tissue grows around the mesh and seals the hole.
  • Open heart surgery: A pediatric cardiologist places the patch through small incisions or a larger incision in the chest.

Atrioventricular (AV) Canal Defect

Present at birth, an atrioventricular canal defect is a combination of heart problems that may affect your baby and cause a hole in the center of the heart. Atrioventricular canal defect is also called endocardial cushion defect or atrioventricular septal defect. Although the cause is unknown, AV canal defects are more common in babies who have Down syndrome. An untreated defect can cause lung problems but early monitoring and treatment can help your child live a normal life.

An atrioventricular canal defect happens in the first eight weeks of pregnancy as the heart develops from a hollow tube and grows partitions that become the four chambers. If the structure doesn’t form properly, an AV defect can occur and heart function may be compromised. An atrioventricular canal defect is a complex problem that can involve more than one abnormality including:

  • Atrial septal defect: An atrial septal defect is an opening in the wall between the upper chambers (atria) of the heart.
  • Ventricular septal defect: A ventricular septal defect is an opening in the wall between the lower chambers (ventricles) of the heart.
  • Abnormal valves: Deviations in the mitral and/or tricuspid valves that separate the chambers of the heart can affect how the heart works.

A hole or improperly shaped valve allows oxygenated blood to mix with oxygen-depleted blood returning from the body or permits blood to flow or leak backward into the aorta. More blood than normal can pass through one side of the heart, causing the other side to become enlarged, and the lungs have to work harder to process more blood at one time. Your child may breathe faster than normal because of the extra blood in the lungs under higher pressure.

Treatment plans can vary based on your child’s age, health, medical history, the level of atrioventricular canal defect and the prognosis. Your pediatric cardiologist may manage your child’s condition initially with medication to help the heart and lungs function better and recommending high-calorie formula or breast milk or supplemental tube feedings to help you baby get adequate nutrition until the defect can be repaired.

To repair atrioventricular canal defect, a pediatric cardiac surgeon places a permanent patch over the hole during surgery and heart tissue grows over the patch. Surgery may also be performed to separate or replace improperly formed valves.

Cardiac Arrhythmia

Like every muscle in your body, your heart depends on electrical signals to coordinate contractions between the four chambers of heart to move blood effectively through the heart to the lungs to be oxygenated and back to the body. A cardiac arrhythmia happens when a problem occurs in the heart’s electrical conduction system. The chambers may become uncoordinated and the heart may beat too fast, too slow or develop an irregular beat that affects how blood is pumped and enough oxygenated blood may not be delivered to the body. Whether your child’s arrhythmia is harmless or requires treatment, a pediatric cardiologist can provide the best recommendation for your child’s health.

Treatment options vary depending on your child’s cardiac arrhythmia. Some arrhythmias are harmless and do not require treatment. Others may require treatment to alleviate symptoms and discomfort. Treatments your pediatric cardiologist may recommend include:

  • Medication: Your child’s arrhythmia may be able to be regulated with medication.
  • Catheter ablation: Using a tiny catheter, a pediatric cardiac surgeon may guide special wires into the heart to disrupt the electrical circuit causing your child’s arrhythmia.
  • Electrical cardioversion: A controlled electrical shock may be given that briefly halts electrical activity in the heart and resets the natural pacemaker. The heart then restarts in a normal electrical pattern.
  • Pacemaker: A pacemaker is implanted in the chest for children and the abdomen for babies. The device has wires that attach to the heart and send electrical signals for a regular heartbeat.
  • Implantable cardioverter defibrillator: Also implanted in the chest, a cardioverter defibrillator tracks the heart rate. If the heart goes into a dangerously fast rhythm, the device sends an electrical shock that may be uncomfortable for the child but can save her life by stopping the rhythm.

Coarctation of the Aorta (COA)

A congenital heart defect, coarctation of the aorta is a narrowing in the aorta, one of the large blood vessels attached to the heart that carries oxygen-rich blood to smaller vessels to be taken throughout the body. A narrower passage in the aorta, the body’s largest artery, means your heart has to work harder to pump the same amount of blood through a smaller space. Shaped like a candy cane, narrowing can happen at any point in the aorta, though it is most common just after the aortic arch.

Treatment plans to repair the narrowed vessel can vary based on your child’s age, health, medical history, the level of aortic coarctation and the prognosis. Your pediatric cardiologist may prescribe medication to control blood pressure prior to surgery and babies may be given medication to keep the blood vessel between the pulmonary artery and the lungs open.

Options to repair coarctation of the aorta include:

  • Cardiac catheterization: Using the same method as for diagnosis, a small tube can be inserted through a blood vessel in the groin and guided to the heart. A balloon can be pushed through the tube and inflated in the aorta to stretch the narrow portion. A stent may be added after the balloon is removed to ensure the aorta stays open.
  • Surgery: A pediatric cardiac surgeon may perform a surgical repair utilizing different methods, such as removing the narrow part, widening it with a patch or part of another artery or bypassing the section with a plastic tube.

Congestive Heart Failure (CHF)

Congestive heart failure affects people of all ages. When the heart doesn’t pump blood the way it should, the body doesn’t receive enough oxygenated blood and fluid builds up in the lungs or tissue causing congestion. Without treatment, a child who suffers from congestive heart failure may experience lung problems, organ failure or other serious conditions. Effective treatments for congestive heart failure are available, however, the underlying cause should be also identified and treated to best protect your child’s health.

Treatment plans for congestive heart failure can vary based on your child’s age, health, medical history, the level of failure and the prognosis. Your pediatric cardiologist may prescribe medication, including:

  • Diuretics to help the kidneys flush excess fluid and relieve fluid build up in the lungs.
  • Digoxin to help the heart pump blood more forcefully.
  • Beta blockers to lower blood pressure and slow your child’s heart rate for more efficient pumping.
  • ACE (angiotensin-converting enzyme) inhibitors to help dilate or relax blood vessels so blood flows easier from the heart.

More invasive treatments for severe congestive heart failure may include implanting a pacemaker to treat an abnormal heartbeat or coordinate contractions or transplanting a new heart from a donor.

Heart Murmur

In some cases, a heart murmur can be the first sign of a heart problem or cardiovascular disease in children. A child who looks the picture of health may not experience any symptoms of a heart condition or know anything could be wrong until a doctor listens to his heart and hears extra noise. A heart murmur means blood isn’t flowing smoothly through the heart, heart valves or blood vessels. Some heart murmurs are harmless and cause no symptoms. Other heart murmurs can be indicative of a problem in the heart and may be merely one symptom of the condition.

Innocent heart murmurs don’t require treatment because the heart is functioning properly. Your child’s doctor may treat the cause, though (such as anemia).

Treatment plans for pathologic heart murmurs may include medication or surgery, depending on what your pediatric cardiologist found to be the cause.

Hypertrophic Cardiomyopathy

Although many children don’t experience any symptoms, hypertrophic cardiomyopathy can significantly impact the how the heart pumps blood as the heart muscles thickens from the disease. Once diagnosed, hypertrophic cardiomyopathy can typically be managed to alleviate symptoms and prevent cardiac arrhythmias that can threaten your child’s life.

Treatment plans for hypertrophic cardiomyopathy can vary based on your child’s age, health, the level of disease and the prognosis. Treatments your pediatric cardiologist may recommend include:

  • Medication: Your child’s doctor may prescribe medications to lower your child’s blood pressure, help the heart muscle relax and slow a fast heartbeat. These medications can help the heart pump blood more efficiently and prevent abnormal rhythms.
  • Implantable cardioverter defibrillator: A cardioverter defibrillator can be implanted in the chest to track the heart rate. If the heart goes into a dangerously fast rhythm, the device sends an electrical shock that can be uncomfortable but may save your child’s life by stopping the rhythm.
  • Surgery: Utilized only in severe cases, a pediatric cardiac surgeon removes the portion of diseased muscle to improve the flow of blood through the heart.
  • Heart transplant: In rare cases, your child’s pediatric cardiologist may recommend a heart transplant to replace your child’s diseased heart.

Hypoplastic Left Ventricle or Hypoplastic Left Heart Syndrome (HLHS)

A rare form of congenital heart disease, hypoplastic left ventricle or hypoplastic left heart syndrome (HLHS) affects a small percentage of newborns in the U.S. Hypoplastic left heart syndrome is characterized by a combination of defects in the heart and surrounding blood vessels that happen when the left side of the heart isn’t fully formed in utero. The mitral valve, left ventricle (lower chamber), aorta and aortic valve may all be too small or absent. These parts of the heart are essential to delivering oxygenated blood to the body. Adding to the strain on the heart, a narrowing or coarctation of the aorta may also be present. With treatment and ongoing care by a pediatric cardiologist, children with hypoplastic left heart syndrome may be able to have an active life.

It’s important that hypoplastic left heart syndrome is treated immediately to protect your child’s health. Treatment can start as quickly as immediately after birth when babies with HLHS are given IV medications to stop the ductus arteriosus from closing. Your pediatric cardiologist may also prescribe medication to help dilate blood vessels. Another early treatment may involve using cardiac catheterization to widen the foramen ovale for better blood flow.

A child with HLHS will likely need three surgeries between the ages of 2 weeks and 4 years old to restore adequate function to the heart. In more severe cases, your pediatric cardiologist may recommend having a heart transplant.

Hypoplastic Right Ventricle or Tricuspid Atresia

Similar to hypoplastic left heart syndrome, a baby born with hypoplastic right ventricle or tricuspid atresia may immediately experience severe symptoms and need medical intervention quickly. In both congenital heart diseases, the ventricle (bottom chamber) is underdeveloped or nonexistent. The other side of the pumps harder to compensate but the extra stress can cause other health problems, including congestive heart failure. The tricuspid valve between the right atrium (upper chamber) and ventricle is also missing or blocked off with tricuspid atresia. After treatment, children with hypoplastic right ventricle can have active lives.

Treatment may start soon after birth with IV medications to keep the ductus arteriosus open. Your pediatric cardiologist may also prescribe medication to help dilate blood vessels. Another early treatment may involve using cardiac catheterization and a balloon to create or widen the foramen ovale and let more oxygenated blood reach the body.

A child with tricuspid atresia will likely need a series of surgeries in the first two years to restore adequate function to the heart.

Infective (Bacterial) Endocarditis (IE)

Infective endocarditis can affect any child, although it is uncommon. Children who have certain heart conditions tend to be at a higher risk for a bacterial infection in the heart valves or the inner lining (endocardium) of the heart. Infective endocarditis usually happens when bacteria from another part of the body enters the bloodstream and spreads to the heart where it attaches to damaged areas, such as a defective heart valve. Infective endocarditis can harm or destroy heart valves, causing serious damage to the heart. Children with infective endocarditis require prompt treatment from a pediatric cardiologist to protect the heart.

Pediatric cardiologists treat infective endocarditis with antibiotics. Your child may need high doses of antibiotics given through an IV line for two to six weeks. IV antibiotic treatment may be started at a medical center, then continued at home once your children begins to feel better. During treatment, your pediatric cardiologist may schedule frequent visits to ensure the antibiotics are working and periodic blood tests to see if the bacteria are still present.

Infective endocarditis that damages your child’s heart valves may require surgery to repair or replace the valve.

Kawasaki Disease

A type of vasculitis, Kawasaki disease is a rare condition that causes inflammation in arteries throughout the body, including the heart’s blood vessels, or the coronary arteries. Kawasaki disease mostly affects children who are younger than 5 years old, though children as old as 13 and as young a newborn can have the condition. What causes Kawasaki disease hasn’t been discovered. Left untreated, Kawasaki disease can increase the risk that your child’s coronary arteries and other parts of the heart may develop problems. But with treatment, most children who have Kawasaki disease fully recover without any serious or lasting effects.

Kawasaki disease is treated with medication to mitigate damage and ease symptoms as the illness runs its course. Children with acute symptoms may be hospitalized for a few days though if IV medications are used. Your pediatric cardiologist may prescribe:

  • Aspirin: Taken at high doses, aspirin helps lower the risk of blood clots and reduce inflammation. A low dose of aspirin may be taken for 6 to 8 weeks or longer to further protect the blood vessels and heart, especially if an aneurysm develops.
  • Gamma globulin or corticosteroids: Given through an IV, gamma globulin helps the immune system fight the infection and may lessen the risk of damage to the heart and coronary arteries. Children who don’t respond to gamma globulin may be given corticosteroids instead.

Patent Ductus Arteriosus (PDA)

The ductus arteriosus, a blood vessel between the pulmonary artery and the aorta, is critical to your baby’s health before birth and can be lifesaving after birth for babies who have hypoplastic left heart syndrome or hypoplastic right ventricle. In utero, the ductus arteriosus lets blood bypass the lungs because the baby is getting oxygen from his mother and isn’t breathing on his own. After birth, the baby begins breathing for himself and the ductus arteriosus closes within a few days. Patent ductus arteriosus is diagnosed when the vessel doesn’t close. An open ductus arteriosus affects how the heart works and can lead to heart problems. With treatment, your child’s symptoms should abate and the heart should function properly.

If the ductus arteriosus is small, the lungs may be able to accommodate the extra blood and treatment may not be needed. In premature babies, a PDA may close on its own after a longer period of time than normal. If your baby has a small PDA or is premature, your pediatric cardiologist may recommend monitoring your baby’s health closely.

Treatment options for a larger PDA that causes symptoms can include:

  • Medication: A pediatric cardiologist may prescribe IV medication for premature babies to help a patent ductus arteriosus close. Related to aspirin and ibuprofen, indomethacin triggers the muscles in the ductus arteriosus to constrict and close the PDA. Nonsteroidal anti-inflammatory drugs, such as ibuprofen, may also block chemicals in your baby’s body that can keep a PDA open.
  • Cardiac catheterization: A tiny catheter is inserted through a bigger blood vessel and threaded to the heart where your pediatric cardiologist can place a coil or plug in the PDA.
  • Surgery: Your pediatric cardiologist may recommend surgery to repair or close the PDA if earlier treatments were not successful.

Pulmonary Stenosis (PS)

A congenital heart defect, pulmonary stenosis affects the pulmonary valve between the right ventricle of the heart and the pulmonary artery that carries oxygen-depleted blood from the heart to the lungs. If the valve doesn’t open properly, pulmonary stenosis may be diagnosed. Pulmonary stenosis can also happen if the area on either side of the valve in the ventricle or artery is too narrow to allow enough blood to reach the lungs. Moderate to severe pulmonary stenosis can worsen over time but treatments can be very effective and your child should be able to have a healthy, active life.

If your child has mild to moderate pulmonary stenosis, treatment may not be necessary. Your pediatric cardiologist will likely recommend regular appointments to monitor your child’s heart function and ensure PS doesn’t worsen.

Child with severe to critical pulmonary stenosis require treatment to repair the valve. Treatment options include:

  • Balloon valvuloplasty: Using cardiac catheterization, a pediatric cardiologist inflates a balloon in the pulmonary valve to widen the opening and improve blood flow.
  • Surgery: A pediatric cardiac surgeon can perform several different procedures to treat pulmonary stenosis. Scar tissue may be released in the leaflets. The valve may be removed and replaced with an outflow patch. A patch may be used to enlarge a narrowed area. The valve may be replaced.

Tetralogy of Fallot (TOF)

A rare condition, tetralogy of Fallot is characterized by four related structural heart defects that are present at birth. These defects impact how the heart works and cause oxygen-poor blood to be pumped to the body. Your child’s tissues and organs don’t receive adequate oxygen and the skin may begin to look blue. Tetralogy of Fallot is often diagnosed in babies and toddler. Diagnosis may be delayed if your child has mild TOF and no symptoms. With early intervention, most children who have TOF can lead active lives.

Tetralogy of Fallot may be diagnosed soon after birth if your doctor notices a blue tinge to your baby’s skin or hears a heart murmur, the extra noise caused by turbulent blood flow. A pediatric cardiologist may confirm a TOF diagnosis using these tests:

  • Chest X-ray: Your pediatric cardiologist can see the size, shape and structure of your baby’s heart in a chest X-ray. Tetralogy of Fallot can make the right ventricle bigger than normal, giving the heart a characteristic boot shape.
  • Electrocardiogram (ECG or EKG): With each contraction of the heart, an electrocardiogram records the electrical activity. An ECG can show if the right ventricle or atrium is enlarged and if your baby has an irregular heartbeat, such as arrhythmia.
  • Echocardiogram: Using high-pitched sound waves, an echocardiogram creates a moving image of how the heart functions. An echocardiogram detects ventricular septal defects and evaluates the position of the aorta, the structure of the pulmonary valve and pulmonary artery and how the right ventricle functions.
  • Cardiac catheterization: A tiny catheter is inserted through a larger blood vessel and guided to the heart to measure blood pressure and oxygen levels in the heart and blood vessels. A dye may be injected through the tube to make the heart’s structure more visible in an X-ray.

TOF may also be diagnosed with an oxygen saturation test to measure oxygen in the bloodstream.

Transposition of the Great Arteries (TGA)

A rare congenital heart defect, transposition of the great arteries is diagnosed when the two major arteries attached to the heart are transposed or reversed. Also called transposition of the great vessels, the heart can’t send enough oxygenated blood to the body because oxygen-poor and oxygen-rich blood are separated when the pulmonary artery and aorta are transposed. Without treatment, your baby won’t have adequate oxygen in her bloodstream and may suffer serious complications, such as heart failure. Transposition of the great arteries is a serious heart condition but corrective surgery can restore heart function and help your baby live a normal life.

Until corrective surgery can be done, your pediatric cardiologist may treat your baby with IV medication to keep the ductus arteriosus open and perform balloon or atrial septostomy using cardiac catheterization to widen the foramen ovale to improve the oxygen level in the blood.

Surgery to repair transposition of the great arteries and correct other heart defects may be done in the first week or two of your baby’s life. A pediatric cardiac surgeon performs a procedure called an “arterial switch” to move the aorta to the left ventricle, the pulmonary artery to the right ventricle and connect the coronary arteries to the aorta to ensure the heart muscle receives oxygen-rich blood.

Ventricular Septal Defect (VSD)

Ventricular septal defect is a congenital heart condition that causes a hole in the ventricular septum, the wall that divides the two ventricles (lower chambers) in the heart. The defect can occur at any place in the septum and allows oxygen-poor blood returning from the body to mix with oxygen-rich blood flowing to the body. VSD affects how efficiently the heart pumps blood, causes higher blood pressure than normal in the blood vessels of the lungs and forces the right side of the heart to pump harder. Without treatment, children who have a large ventricular septal defect may develop lung disease. Treatment options can be effective and allow your child to have an active life.

Small ventricular septal defects may close on their own without treatment. Treatment options for larger VSDs include:

  • Medication: Your pediatric cardiologist may prescribe digoxin to strengthen the heart’s contractions and maintain a regular heartbeat, a diuretic to alleviate fluid retention in the lungs and body and ACE inhibitors to lower blood pressure. Beta blockers may also be used to regulate your child’s heartbeat.
  • Surgery: A pediatric cardiac surgeon can close, patch or plug a ventricular septal defect during surgery. Your child may have open-heart surgery, cardiac catheterization or a hybrid procedure using both techniques to repair the VSD.