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World-Class Treatment for

Pediatric Congenital Heart Disease

Approximately one in every 125 babies born in the United States has some form of pediatric congenital heart disease. Conditions can range from fairly simple to extremely complex. If a condition cannot be managed with medication, pediatric heart surgery may be required. In these matters of the heart, AdventHealth for Children, formerly Florida Hospital for Children, is here for your child and your family every step of the way, through diagnosis, treatment and beyond.

Frequently Requested Information

Pediatric Congenital Heart Disease Treatment

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Double Outlet Left Ventricle

Double outlet left ventricle (DOLV) is a very rare birth defect in children. It occurs when the aorta and pulmonary artery both exit from the left ventricle. Symptoms of the condition can include a blue or purple tint to the skin, lips and nails; poor weight gain or appetite; rapid breathing/shortness of breath; sweating, especially during feeding; unresponsiveness and irregular fatigue.

The only way to confirm the condition is by echocardiography, magnetic resonance imaging (MRI), chest X-ray, and/or cardiac catheterization. Double outlet left ventricle is repaired through pediatric heart surgery.

Fontan and Fontan Conversion

The Fontan procedure is used to treat congenital heart defects where only one side of the heart that is functional. Surgeons at AdventHealth for Children, formerly Florida Hospital for Children, use the procedure to 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.

The pediatric heart surgery procedure is normally performed on young patients in two or three stages. The first stage is usually a systemic to pulmonary artery shunt whereby a systemic artery is connected to the pulmonary arteries by way of a Goretex tube graft. At about 6 months of age, the shunt is removed and replaced during a procedure known as the bidirectional Glenn shunt in which the superior vena cava blood flow is directed to the 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.

Bidirectional Glenn Procedure

The bidirectional Glenn procedure serves as a staging operation, which prepares the patient for the eventual Fontan procedure normally performed at a later time. The surgeon creates a connection from the superior vena cava to the pulmonary artery, which establishes the shunt. It is used primarily as a treatment for a congenital heart defect in children that reduces the amount of oxygenated blood circulating through the body. The telltale sign of this is a bluish tint to the fingernails, lips, and skin.

The procedure will improve the flow of oxygen rich blood, but it is not the final step. Your child will still need to have a Fontan procedure to complete the repair. After the bidirectional Glenn, your child will likely remain in the hospital three to seven days to ensure proper healing.

Coronary Fistula

Though rare, coronary fistula can develop while a baby is in the womb, as well as after birth. In the womb during development, the coronary artery may incorrectly attach to one of the chambers of the heart or a blood vessel. After birth, your child can form a coronary artery fistula resulting from an infection that has weakened the wall between the heart and the coronary artery, or as a result of an injury to the heart itself.

A child may not show any signs of a coronary fistula. If there are signs, it may include fatigue, failure to thrive, irregular heartbeat, or shortness of breath. 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 rarely cardiac catheterization.

A small fistula may close spontaneously; however, if the fistula is large your doctor may recommend pediatric heart surgery or use of a catheter, which can effectively close the fistula.

Valve Sparing Repair of Tetralogy of Fallot

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. Many babies with TOF will become blue within the first three months of life while 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 surgery.

Norwood Procedure and Hypoplastic Left Heart Syndrome

When the left side of the heart remains under-developed, it is known as hypoplastic left heart syndrome, or HLHS. In HLHS, the mitral valve that separates the two left chambers (left atrium and left ventricle) of the heart is too small or closed off entirely. The left ventricle, which is responsible for pumping blood is also too small, as is the aortic valve, which separates the left ventricle from the aorta. It may even be closed in some cases.

A newborn with HLHS may have blue or purple skin, lips, or nails–known as cyanosis; difficulty breathing or feeding; and be lethargic. If undetected the baby will present with circulatory collapse and shock. To confirm the diagnosis of HLHS, your doctor will require echocardiogram, electrocardiogram, chest X-ray, and measurement of blood oxygen levels. HLHS can only be repaired surgically.

Frank Pigula, MD
World-Renowned Experts

In Pediatric Congenital Heart Disease Treatment

Frank Pigula, MD

Medical Director, Congenital Heart Surgery
Dr. Pigula is known worldwide for his expertise in complex congenital heart disease and extensive experience in pediatric and neonatal cardiac surgery. Dr. Pigula also conducts ongoing clinical studies.

Expert and Compassionate Care

Meet Our Team

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Advanced Surgical Techniques

For Remarkable Cardiac Outcomes

We are dedicated to providing the very latest advancements in pediatric cardiac surgery methods and technology to result in the best possible outcomes. At AdventHealth for Children, we openly share clinical volumes and outcomes of our pediatric cardiac surgical program.

Additional Conditions and

Treatments for Congenital Heart Disease in Children

  • Aortic Valvar Stenosis (AS)
  • Arrhythmias
  • Arterial Switch Operation
  • Atrial Septal Defect (ASD)
  • Atrioventricular Septal Defect (or AV canal defect)
  • Biscuspid Aortic Valve
  • Cardiac Anesthesiology
  • Coarctation of the Aorta
  • Double Outlet Right Ventricle
  • Ebstein's Anomaly of the Tricuspid Value
  • Hypoplastic Left Heart Syndrome (HLHS)
  • Interrupted Aortic Arch
  • Patent Ductus Arteriosus
  • Patent Foramen Ovale
  • Pulmonary Atresia (PA)
  • Pulmonary Stenosis (PS)
  • Single Ventricle Anomalies
  • Total Anomalous Pulmonary Venous Return
  • Transposition of the Great Arteries
  • Tricuspid Atresia
  • Truncus Arteriosus
  • Ventricular Septal Defect
  • Vascular Rings
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Intensive Care

For Our Tiniest Patients

As one of the few pediatric Cardiac Intensive Care Units in the country, our PCICU is equipped with the latest pediatric cardiology monitoring and intervention equipment and staffed by a multidisciplinary team.

We're here to provide every child with highly specialized care before and after a procedure.

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Baby Matias: A Heart Story

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It's something you can't describe because it's your baby. 

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