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Pediatric heart disease is a term used to describe several different heart conditions in children. The most common type of pediatric heart disease is congenital, meaning that children are born with it.

The different types of pediatric heart disease include:

  • Aortic Stenosis (AS)
  • Atrial Septal Defect (ASD)
  • Atrioventricular Septal Defect (AVSD)
  • Coarctation of the Aorta
  • Ebstein's Anomaly
  • Hypoplastic Left Heart Syndrome (HLHS)
  • Interrupted Aortic Arch (IAA)
  • Patent DuctusArteriosus (PDA)
  • Pulmonary Stenosis (PS)
  • Pulmonary Atresia (PA)
  • Single Ventricle (Functionally Univentricular Heart)
  • Tetralogy of Fallot (TOF)
  • Total Anomalous Pulmonary Venous Return (TAPVR)
  • Transposition of the Great Arteries (TGA)
  • Tricuspid Atresia
  • TruncusArteriosus
  • Vascular Rings
  • Ventricular Septal Defect (VSD)
What is tetralogy of Fallot (TOF)?

Tetralogy of Fallot has four characteristics:
Ventricular septal defect (VSD) - There is a hole between the two bottom chambers (the ventricles) of the heart that eject blood to the body and lungs.

  • Overriding aorta - The aorta, the large artery that takes blood to the body, is on top of both ventricles, instead of just the left ventricle as in a normal heart.
  • Pulmonary stenosis - There is a narrowing of the pulmonary valve, the area below the valve, or the pulmonary arteries, which carry blood from the heart to the lungs.
  • Hypertrophy - The right ventricle becomes thicker and more muscular than normal as a result of working harder to pump blood through the narrow pulmonary valve.
What are the symptoms of TOF?

The symptoms of tetralogy of Fallot include:

  • Blue or purple tint to lips, skin and nails (cyanosis)
  • Heart murmur - the heart sounds abnormal when a doctor listens with a stethoscope
  • In older children, abnormal shape of the fingertips ("clubbing")
  • Spells during which oxygen levels drop - lips and skin will become bluer, and the child will become fussy or irritable and then sleepy or unresponsive
How is tetralogy of Fallot diagnosed?

Tetralogy of Fallot may be diagnosed with fetal echocardiogram (ultrasound). Our Fetal Heart Program will prepare a plan for delivery and care immediately after birth.

Doctors might make the TOF diagnosis before the newborn leaves the hospital if they hear a murmur or see a blue tint to the skin; a primary care pediatrician might detect the same symptoms during a checkup; or a parent might notice TOF symptoms and bring the baby to a doctor or hospital.

Diagnosis of TOF may require some or all of these tests:

  • Pulse oximetry - a painless way to monitor the oxygen content of the blood
  • Electrocardiogram (ECG) - a record of the electrical activity of the heart
  • Echocardiogram (also called "echo" or ultrasound) - sound waves create an image of the heart
  • Chest X-ray
  • Cardiac MRI - a three-dimensional image shows the heart's abnormalities
  • Cardiac catheterization - a thin tube (catheter) is inserted into the heart through a large vein in the leg
What are the treatment options for tetralogy of Fallot?

Surgery is required to repair TOF.

Typically in the first few months of life we will perform open-heart surgery to patch the hole (VSD) and widen the pulmonary valve or artery. In some cases, depending on the unique needs of the patient, we will perform a temporary repair until a complete repair can be done. The temporary repair involves connecting the pulmonary arteries (which carry blood from heart to lungs) with one of the large arteries that carry blood away from the heart to the body. This increases the amount of blood that reaches the lungs, and so increases the amount of oxygen in the blood.