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Tetralogy of Fallot is a congenital heart defect that is associated with four specific defects inside the heart. These defects include:

  • Ventricular septal defect – a hole in the heart between two lower chambers
  • Pulmonary stenosis – the pulmonary valve is tight and does not open properly which limits the amount of blood able to go to the lungs
  • Right ventricular hypertrophy – thickening of the wall on the right side of the heart which happens because the ventricle is pumping against the stiff pulmonary valve
  • Overriding aorta – which means the aortic is located directly over the ventricular septal defect

The majority of cases of Tetralogy of Fallot have no known cause.

Diagnosing Tetralogy of Fallot

Tactics used to diagnose tetralogy of fallot include:

  • A comprehensive physical exam
  • Echocardiogram - ultrasound of the heart
  • Chest X-ray
  • Pulse oximetry test - detects the amount of oxygen in the blood
  • Cardiac catheterization
  • Cardiac MRI

Symptoms of Tetralogy of Fallot

There are several ways a tetralogy of fallot may present itself. A common symptom is when the baby's lips and tongue turn blue or purple due to low oxygen levels.

Other symptoms include:

  • Sleepiness
  • Shortness of breath
  • Increased fussiness
  • In extreme cases, passing out

Surgery for Tetralogy of Fallot

Open heart surgery is needed to repair TOF and typically occurs during the first few months of life.

View our pediatric cardiac surgery outcomes.

In some cases, the pulmonary valve will need to be removed completely. If this is the case, your child will require another surgery or a cardiac catheterization later in life.

Some infants with severe TOF will require surgery soon after birth. In these cases, your baby will also need a “complete” TOF repair during infancy since the full repair since your baby will be too small as a newborn.

The three main parts of the TOF repair include closing the hole in your child’s heart (VSD) opening the right ventricle outflow tract, and repairing the pulmonary valve. The VSD is closed using a Gore-Tex patch and the valve is opened up.

Here at the University of Maryland, we specialize in valve-sparing TOF repair which means we try very hard to keep your child’s pulmonary valve so that he/she will not need additional surgery later in life.

After Surgery

After surgery for TOF, your child will stay in the hospital between three to 10 days. If your child is a newborn and requires surgery right away, the hospitalization will likely be longer. Your child will stay in the ICU and be monitored closely until discharge.

Once your child goes home, they are usually fully recovered by 6-8 weeks. Your surgeon and nurse practitioner will discuss your child’s particular restrictions with you following the surgery.

It is unlikely that your child will have any lifelong restrictions for sports or other activities. It is important to know that your child will be followed by a cardiology for his/her entire life following surgery.

To make an appointment with a Children's Heart Program physician, please call 410-328-4348.