Before you begin reading about tetralogy of Fallot, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
Tetralogy of Fallot has four characteristics:
View Large Tetralogy of Fallot Illustration
In the animation above, doctors describe the
structure of a heart with TOF.
The symptoms of tetralogy of Fallot include:
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:
A number of children with TOF also have genetic syndromes such as DiGeorge syndrome, Trisomy 21 (Down syndrome), Alagille syndrome or 22q11 deletion syndrome. Genetic testing (a blood test) may be part of our evaluation.
Surgery is required to repair TOF.
The animation shows treatment options for tetralogy of Fallot. 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.
A child who has had surgical repair of tetralogy of Fallot will require life-long care by a cardiologist.
In the animation doctors discuss the possibility of a leaky heart valve. Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physician. Patients will need to carefully follow doctors' advice, including staying on any medications prescribed and, in some cases, limiting exercise.
Sometimes children with TOF experience heart problems later in life, including a leaky heart valve and irregular heartbeat (arrhythmia). Medicine or repeat surgery may be required.
We will help patients transition care to an adult cardiologist.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children's Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects like TOF.
Because of enormous strides in medicine and technology, today most children born with heart conditions like tetralogy of Fallot go on to lead healthy, productive lives as adults.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Elizabeth Goldmuntz, MD
Date: October 2008
For a second opinion, cardiac referral, or for more information.
To schedule an outpatient appointment.