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Congenital heart disease (CHD) is the most common birth defect, with nearly 1% of affected newborns experiencing moderate to severe cases of CHD.

Tetralogy of Fallot is a common type of CHD that has four major features:

  • A hole between two chambers of the heart (ventricular septal defect or VSD)
  • Obstruction of blood flow to the lungs (right ventricular outflow tract obstruction or RVOTO)
  • A misplaced aorta that sits on top of the VSD (an overriding aorta)
  • Thickening of the right pumping chamber of the heart (ventricular hypertrophy)
Long-Term Survival Outcomes After Tetralogy of Fallot Surgery

Risk of Reoperation for Tetralogy of Fallot

While 30-year survival rates for Tetralogy of Fallot are quite strong, there is considerable risk that more surgeries (reoperations) will be needed later in life.

Indications for reoperation in individuals who were born with Tetralogy of Fallot include:

  • A blockage in the pathway leading from the right pumping chamber of the heart to the lungs (right ventricular outflow tract obstruction).
  • A narrowing of the blood vessel that carries blood from the right side of the heart to the lungs (pulmonary stenosis).
  • An increase in the size of the right pumping chamber of the heart (right ventricle dilation).
Chart illustration the risk of reoperation of Tetralogy of Fallot

Risk of Cardiac Catheterization for Tetralogy of Fallot

While long term survival rates for Tetralogy of Fallot are quite strong, many people who had Tetralogy of Fallot repair will still need a catheter-based procedure (re-intervention) later in life.

Indications for a catheter re-intervention in those born with Tetralogy of Fallot include:

  • Right ventricular outflow tract obstruction
  • Right ventricle dilation
  • Pulmonary stenosis
Chart illustrating the risk of cardiac catheterization for Tetralogy of Fallot

Risk of Pulmonary Valve Replacement

A key feature of Tetralogy of Fallot is the blockage of the pulmonary artery, which is the vessel that takes blood from the right side of the heart to the lungs. During the operation to repair Tetralogy of Fallot, work is often done on the pulmonary artery pathway and the valve that is between the pulmonary artery and the heart, called the pulmonary valve.

There is evidence that for people who had Tetralogy of Fallot repair, this valve will eventually degrade and leak in up to 50% of people, requiring a replacement of the pulmonary artery valve by the time they are 25 years old.

Chart illustrating the risk of pulmonary valve replacement

How to Read and Interpret These Numbers

These graphs are called Kaplan Meier curves. They are produced using our own cardiac surgery data that is stored in the CNCORe database. The goal of these graphs is to predict what may happen 10 to 15 years after a child's initial heart surgery. We collect data and information for every single patient we have operated on. Over time, however, some people stop seeing their doctor or move to different states and countries—meaning that researchers do not have complete information for every patient across the years.

The CNCORe data is used to create graphs that estimate risk for something like reoperation using the data we do have, without needing every data point for every single patient.

 

Understanding the Line Graphs

Cardiac Surgery Long-Term Outcome Graphs

For each heart condition below, you can view data about the risks of surgical procedures and the chances of a child needing additional operations over time.