Getting to the Heart of the Problem

A child can develop a heart issue at any time in his/her young life. At the Walt Disney Pavilion at Florida Hospital for Children, we know that these tiny hearts require a special brand of healthcare, one that utilizes the most advanced diagnosing tools and therapeutic procedures, administered by caring, experienced pediatric cardiology specialists who understand a child’s unique physiology.

Our hospital’s team of cardiology experts has a reputation for excellence, treating a child’s heart problems with the latest treatments available.

Common resasons for pediatric cardiology referrals:

• Hearth Murmur
• Chest Pain
• Fainting or Syncope
• Irregular heartbeat
• Palpitations

Common types of congenital heart disease:

• Aortic valvar stenosis (AS)
• Atrial septal defect (ASD)
• Biscuspid aortic valve
• Atrioventricular septal defect (or AV canal defect)
• Coarctation of the aorta
• Ebstein's anomaly of the tricuspid value
• Hypoplastic left heart syndrome (HLHS)
• Interrupted aortic arch
• Patent ductus arteriosus (PDA)
• Pulmonary stenosis (PS)
• Pulmonary atresia (PA)
• Single ventricle anomalies
• Tetralogy of Fallot (TOF)
• Total anomalous pulmonary venous return
• Transposition of the great arteries
• Tricuspid atresia
• Truncus arteriosus
• Vascular rings
• Ventricular septal defect (VSD)

Our pediatric cardiology specialists use a wide range of state-of-the-art diagnostics to assess issues quickly and accurately.

Some of these tests include:

Electrocardiogram (EKG)

This is a painless test that records the electrical activity produced by the heart. It is useful in detecting cardiac chamber enlargement and abnormal rhythms. 

Echocardiogram

This test uses ultrasound waves to help the cardiologist visualize the child’s heart anatomy and function. An echocardiogram can identify many abnormalities in the heart, such as a hole or abnormalities in the chambers or valves.

Holter Monitoring

To assess the heart’s rhythm over a 24-hour period, a monitor with five small electrodes is attached to your child’s chest to detect palpitations, irregular heartbeats and other abnormalities of rate and rhythm.

Event Monitoring

In some cases, symptoms are not present on the day you visit the physician. To identify the presence of infrequent symptoms, the pediatric cardiologist may recommend that the heart be monitored for a month using a small, portable device.

Stress Test

For older children, a stress test may be recommended. During this test, your child will walk, jog or run on a treadmill while vital signs and EKG are monitored to determine how exercise affects cardiac performance. 

Cardiac Conditions that Children Can Face Early in Life

In an ideal world, all children would be born healthy. But when they face a serious health issue, such as those affecting the heart, the specialists at the Walt Disney Pavilion at Florida Hospital for Children are ready to promptly and accurately diagnose the problem and, if necessary, refer the child to our surgical colleagues for successful repair of their condition.

Following are some common types of congenital heart conditions:

Ventricular Septal Defect

A ventricular septal defect (VSD) is a hole in the wall that separates the two pumping chambers of the heart (ventricles). This allows a certain amount of blood to flow from the left ventricle across the hole to the right ventricle and out to the pulmonary artery and the lungs, creating a short circuit between the heart and the lungs. Often times, these holes are small and tend to close spontaneously over time. About five percent (5%) of the time they will benefit from surgical closure.

VSDs  come in three sizes - small, medium and large. The small ones seldom cause any problems and do not require medical treatment. Medium size holes sometimes require medication and occasionally surgery. The large ones usually require both medication and surgery. Medium and large size VSDs can cause congestive heart failure. This does not mean that the heart stops, but rather that it is not able to handle the extra work that having a hole causes. Medication is then used to help the heart work more efficiently. 

Symptoms observed may include poor weight gain, excessive sweatiness and breathing faster than normal. Many times by giving medication early in life the need for surgery can be avoided as the hole will get smaller as the child becomes bigger. When surgery is required it is usually done between six months and three years of age. Fortunately, after surgery these children usually catch up in growth. Sometimes, surgery is necessary because the VSD can cause the pressures in the lungs to be too high and potentially damage the lungs.

Atrial Septal Defect

An atrial septal defect (ASD) is a hole in the wall that separates the two upper chambers of the heart. 

Sometimes ASDs are big enough that they need to be closed. They are usually silent during the first two to three years of life and then cause the presence of a soft heart murmur. On occasion, a large ASD will be found during the first year of life. Approximately one third of ASDs found in babies will close spontaneously in time. If they do not, the murmur will become more obvious as more and more blood will flow across the hole, causing a gradual enlargement of the right side of the heart.

Patients with an ASD have no symptoms during childhood. However, as they become teenagers and young adults most will experience decreased exercise tolerance. If a significant ASD is allowed to persist into adulthood the pressure in the lungs will increase and can sometimes cause permanent damage to the lungs and ultimately early death. Adults with ASDs are also at risk for having a stroke.

ASDs used to be closed through open-heart surgery. However, nowadays most of them can be closed at 3 or 4 years of age with a catheter procedure that delivers a device to close the defect. This technique has the advantage of avoiding surgery and usually avoiding blood transfusions. The patient can be discharged from the hospital in 24 hours. Some of these defects; however, are too big to be closed with a device or are in a location that requires surgical repair.

Patent Ductus Arteriosus (PDA)

The ductus arteriosus is a connection between the two major arteries leaving the heart, namely the aorta and the pulmonary artery. During fetal life, it allows blood to bypass the growing, nonbreathing lungs. After birth, it is no longer necessary and it normally closes spontaneously within a day or so. However, if it does not close, it will be associated with a cardiac murmur related to the blood flowing from the aorta through the ductus into the pulmonary artery.

A small ductus does not cause any symptoms and may only be discovered during a routine exam. Observation during the first year of life to see if it closes spontaneously is a common approach. A larger ductus; however, maybe associated with poor weight gain, fast breathing, sweatiness or frequent lung infections during infancy.

 When it needs to be closed, there are basically two options: The traditional way is surgical ligation or division of the PDA. Lately; however, most PDAs can be closed through a coil or device delivered through a catheterization procedure performed through a tiny puncture in the groin. Once closed, the child is able to resume his normal activities and experiences no long term effects from the PDA or its closure.     

Pulmonary Stenosis

Pulmonary stenosis is a condition where the valve between the right ventricle and the pulmonary artery does not open up completely. The pulmonary artery is the vessel that supplies blood to the lungs. When the valve does not open properly the ventricle has to work harder than normal. Pulmonary stenosis may be mild, moderate or severe. In mild cases the child will only exhibit a cardiac murmur, but will not have any symptoms and nothing needs to be done about it. In moderate cases, the patient may have some exercise intolerance or excessive fatigue with exercise. In severe cases, the child may complain of chest pain and the right ventricle will eventually enlarge and fail and may cause premature death. In the past, when treatment was necessary, this could only be achieved by open-heart surgery. However, these days we can usually use a special balloon catheter to tear the valve open while doing a cardiac catheterization, allowing the patient to go home the next day. It is not unusual for the pulmonary valve to leak a bit after being repaired. This is usually not a problem.

Most of the time pulmonary stenosis is a random event, but on occasion, it can be inherited as part of a syndrome, such as Noonan syndrome or more complex cardiac defects such as tetralogy of Fallot.

Coarctation of the Aorta

Coarctation of the aorta is a condition where there is a narrowing of the aorta, which is the large artery that distributes the blood from the heart to all parts of the body. This narrowing causes the upper half of the body to have a higher than normal blood pressure and the lower part of the body a lower than normal blood pressure. About one third of these cases are discovered in the newborn period. These babies may be quite sick with rapid breathing and poor color. They can sometimes stop breathing and sometimes die or become brain damaged if not recognized early. These babies require immediate surgery in order to allow blood to flow beyond the area of blockage. Usually in young infants we can use a medication called Prostaglandin to temporarily keep the coarctation open until the surgery is accomplished. About one third of the cases are found in babies between one and twelve months of age. The pediatrician may notice a heart murmur or a lack of pulses in the legs (femoral pulses). These children are usually repaired at the time of diagnosis. The remaining one third of coarctation patients with relatively mild coarctation will be diagnosed later in childhood because of either a murmur or high blood pressure in the arms.                 

The most accepted therapy today is surgery, which is successful in the vast majority of cases. With the recent development of balloons and stents, some cases may be treated through a cardiac catheterization procedure, without surgery, although this is limited to selective instances.

Tetralogy of Fallot

Tetralogy of Fallot (TOF) is a rare heart condition that includes a large hole between the ventricles called ventricular septal defect (VSD), an enlarged aorta straddling the hole, a narrowing of the pulmonary artery and a thickened right ventricle. About one third of babies born with TOF will turn blue during the first three months of life. The other two-thirds may show other symptoms, including a cardiac murmur, shortness of breath, fainting, abnormal rounded shaped nails on the fingers and toes, poor weight gain, irritability and fatigue.    

If tetralogy of Fallot is suspected, the doctor will conduct a series of tests, which may include electrocardiogram, chest x-ray, echocardiogram and oxygen level measurement. Babies with tetralogy of Fallot are subjected to have "TET spells", which are episodes where the baby may turn very blue, breathe very fast and may actually faint. This is a medical emergency that needs to be treated immediately.

Surgery is the only definitive treatment. In most cases, the intracardiac repair is accomplished, including patch closure of the VSD, removal of muscle within the right ventricle and repair of the pulmonary valve and/or pulmonary artery. In some cases, a temporary procedure called an aortopulmonary shunt is performed before the intracardiac repair in order to increase blood flow to the lungs. This is then removed during the intracardiac operation.

Transposition of the Great Vessels

Transposition of the great arteries (TGA) is a relatively rare form of congenital heart disease, where the two main arteries coming from the heart, namely the aorta and the pulmonary artery are transposed, meaning that the aorta comes off the right ventricle and the pulmonary artery comes off the left ventricle, which is opposite to normal. In this setting, the blue blood coming back to the heart from the body is pumped out again to the body via the aorta, whereas the red blood coming back from the lungs with the oxygen is pumped out again to the pulmonary artery and back to the lungs, resulting in two separate circuits with resulting severe cyanosis in the organs of the body and the skin. This situation, if not corrected, is lethal because the tissue of the body needs to have oxygenated blood to survive. Life is impossible unless there is mixing of the red and blue bloods either through an orifice between the two ventricles (VSD), the two atria (ASD) or through the patent ductus (PDA). Sometimes TGA can be associated with other defects of the heart, such as a ventricular septal defect or a narrowing of the pulmonary valve.

Most babies with transposition of the great vessels require open-heart surgery within the next four or five days of life. The operation is known as the "arterial switch procedure", where both great arteries are amputated from the heart and are repositioned and reconnected to the correct ventricle, namely the aorta to the left ventricle and the pulmonary artery to the right ventricle. The coronary arteries are then reattached to the aorta. Any other associated anomaly is usually repaired at the same time. Once this operation is done normal circulation is restored, the baby is pink and usually these babies can have a normal lifestyle; although, periodic evaluations by cardiologists are warranted.