Congenital Heart Surgery in Orlando

A congenital heart defect refers to when a child is born with an improperly formed heart. While we treat dozens of congenital heart defects at AdventHealth for Children, some of the most common ones include:

• Double-outlet left ventricle
• Coronary fistulas
• Valve-sparing repair of tetralogy of Fallot
• Hypoplastic left heart syndrome

What is double-outlet left ventricle?

In a normal heart, the right ventricle pumps deoxygenated blood through the pulmonary artery to the lungs. Later, the left ventricle pumps oxygenated blood through the aorta. Double-outlet left ventricle is a congenital heart defect where both the aorta and the pulmonary artery exit from the left ventricle.

What are the symptoms of double-outlet ventricle?

Some of the common symptoms include:

• A blue or purple tint to the skin, lips and nails
• Poor weight gain or appetite
• Rapid breathing or shortness of breath
• Sweating, especially during eating
• Unresponsiveness and irregular fatigue

How is double-outlet ventricle diagnosed?

To diagnose this condition, doctors use echocardiography (ECG), magnetic resonance imaging (MRI), chest X-rays and cardiac catheterization. Double-outlet left ventricle is repaired through pediatric heart surgery.

What is the Fontan procedure?

The Fontan procedure is used to treat congenital heart defects where only one side of the heart is functional. Surgeons divert venous (unoxygenated) blood to the pulmonary arteries without a pumping chamber. The single functional ventricle serves as a pump to the rest of the body to deliver oxygen and nutrients.

How is the Fontan procedure performed?

This pediatric heart surgery procedure is usually performed on young patients in two or three stages. The first stage is usually a shunt whereby a systemic artery is connected to the pulmonary arteries with a graft.

At about 6 months of age, the shunt is removed and replaced during a procedure known as the bidirectional Glenn shunt, in which blood flow is directed from the upper body directly to the lungs via pulmonary arteries after the main pulmonary artery has been detached from the heart.

The third stage takes place at about 2 and a half years of age and is known as the Fontan operation.

What is a coronary fistula?

A coronary artery fistula is an abnormal connection between one of the coronary arteries and another blood vessel or heart chamber. A coronary artery fistula can affect how well the blood flows to the heart and lead to dilation of the coronary artery.

In the womb, a baby's coronary artery may incorrectly attach to one of the chambers of the heart or a blood vessel. This abnormal connection is called a coronary artery fistula.

What are the symptoms of a coronary fistula?

While many children don’t present symptoms, others show signs of fatigue, a failure to thrive, an irregular heartbeat, or shortness of breath.

How are coronary fistulas diagnosed and treated?

The only sure way to determine the presence of the condition is by an X-ray of the heart, echocardiography, magnetic resonance imaging (MRI) and (in some rare incidents) cardiac catheterization.

While small fistulas may close spontaneously, larger ones may require pediatric heart surgery or the use of a catheter, which can effectively close the fistula.

What is a tetralogy of Fallot heart condition?

Tetralogy of Fallot (TOF) is a heart condition that includes a large hole between the ventricles, an enlarged aorta straddling the hole, a narrowing of the pulmonary artery that leads to the lung, and a thickened right ventricle.

How is tetralogy of Fallot diagnosed and treated?

For many babies with TOF, their skin will become blue within the first three months of life. Others will display symptoms such as shortness of breath, rapid breathing, fainting, abnormally round-shaped nails on the fingers and toes, irritability and fatigue.

To confirm TOF, your doctor will conduct a series of tests that may include a chest X-ray, oxygen level measurement, echocardiogram, cardiac magnetic resonance imaging (MRI) and/or cardiac catheterization. The treatment for this congenital heart disease in children is a surgical procedure called a valve-sparing repair.

What is hypoplastic left heart syndrome (HLHS)?

When the left side of the heart remains underdeveloped, it is known as hypoplastic left heart syndrome or HLHS. In HLHS, the valve that separates the heart's left atrium and left ventricle is too small or closed off entirely. In addition, the left ventricle and aortic valve may be too small or even closed in some cases.

How is HLHS diagnosed?

A newborn with HLHS may have a condition called cyanosis, which is marked by blue or purple skin, lips, or nails. These newborns may also be lethargic and have difficulty breathing or feeding. If undetected, the baby may experience circulatory collapse and shock.

To confirm the diagnosis of HLHS, your doctor will measure blood oxygen levels and perform an echocardiogram and a chest X-ray.

What is the Norwood procedure?

HLHS is repaired with the Norwood procedure, a surgery reorganizing the heart's functioning so the right ventricle can pump blood to the body instead of just pumping it to the lungs like it usually does.