Tetralogy of Fallot

Tetralogy of Fallot (TOF) is an abnormality of the heart that predominantly affects the right side of the heart and results in inadequate blood flow to the lungs for oxygenation.  

As an international referral center for children with complex congenital heart disease, the University of Michigan C.S. Mott Children's Hospital Congenital Heart Center is one of the largest and best pediatric heart programs in the United States. University of Michigan pediatric heart specialists offer state-of-the-art treatment and management of congenital heart conditions, including a comprehensive range of support services and long-term follow up care for children and their families.  Our team has deep expertise caring for children with tetralogy of Fallot from diagnosis and treatment through life-long follow up care.

What is Tetralogy of Fallot?

Tetralogy of Fallot represents approximately 10% of all cases of congenital heart disease (CHD), occurs in 3-6 infants for every 10,000 births, and is the most common form of cyanotic (or blue) CHD.  The cause of tetralogy of Fallot is unknown and likely a result of many factors, but it is more common with certain genetic abnormalities (Down syndrome, DiGeorge syndrome, Goldenhar syndrome) and maternal risk factors (diabetes, phenylketonuria, rubella, etc).  

The four cardinal features of tetralogy of Fallot include right ventricular outflow tract obstruction, ventricular septal defect, right ventricular hypertrophy, and aortic override of the septal defect.

Symptoms of tetralogy of Fallot

The symptoms of tetralogy of Fallot are primarily related to the severity of right ventricular outflow tract obstruction, and may include: 

  • Bluish skin discoloration (cyanosis) particularly around the lips, eyes, and nail beds, or episodes of bluish, pale skin during crying or feeding (e.g. “Tet” or hypercyanotic spells)
  • Difficulty with feeding
  • Failure to thrive (poor weight gain) 

Diagnosing tetralogy of Fallot

Tetralogy of Fallot is often suspected by a combination of history, physical exam findings, pulse oximetry, chest radiograph, and electrocardiography.  Ultimately, echocardiography (cardiac ultrasound) confirms the diagnosis, and helps physicians understand each specific patient’s anatomy and plan the appropriate surgical approach for repair.

In rare cases, cardiac catheterization with angiography and/or cardiac magnetic resonance imaging (MRI) is necessary to further determine the anatomy of each child’s heart.

Treatment for tetralogy of Fallot

Because tetralogy of Fallot is a progressive disorder, most children require some type of surgery.  The ideal operation is primary correction, and this procedure is often performed electively between 4-8 months of age, or when the diagnosis is made in older infants and children. 

The goals of the operation are to close the ventricular septal defect with a patch, and enlarge the right ventricular outflow tract to relieve the obstruction (ideally with preservation of the pulmonary valve). 

In certain infants, primary repair may not be recommended (for example for infants with very small pulmonary arteries, low birth weight, or major associated anomalies), and an initial operation may consist of an aorta-to-pulmonary artery shunt to increase pulmonary blood flow and oxygenation.  These infants may be considered for later surgical correction when they are older.

Life with tetralogy of Fallot

The majority of patients with tetralogy of Fallot who have undergone surgical correction will survive into adulthood with a generally good quality of life and few restrictions related to their heart.  However, these patients do have increased risk for pulmonary valve regurgitation, with progressive right-sided heart failure and the development of heart rhythm abnormalities.  Ultimately, many of patients who struggle with regurgitation will require pulmonary valve replacement.

For this reason, patients with tetralogy of Fallot should remain under the care of a pediatric cardiologist for ongoing monitoring throughout childhood.  As the child transitions into adulthood, he or she should be monitored by an adult congenital heart specialist for early identification of any complications that may arise.

Advancing understanding of tetralogy of Fallot

The congenital heart specialists at University of Michigan have vast experience and tremendous success with primary repair and management of tetralogy of Fallot.  We have pioneered newer surgical techniques to preserve pulmonary valve function (decreasing the incidence of late pulmonary valve regurgitation) and minimize right ventricular dysfunction (via a trans-atrial approach to ventricular septal defect closure) whenever possible.  We have also been a leading center for advancements in non-operative, minimally invasive, trans-catheter approaches to pulmonary valve replacement in adults with tetralogy of Fallot, pulmonary valve regurgitation, and right-sided heart failure.

Why choose C.S. Mott Children’s Hospital for tetralogy of Fallot care

The Congenital Heart Center at C.S. Mott Children’s Hospital has been a national and international leader in surgical and catheter-based interventions for tetralogy of Fallot for more than four decades. 

Our specialists design personalized treatment plans for each patient, created through tight collaboration between our pediatric cardiac surgeons and our interventional cardiology team.

We have one of the largest pediatric cardiology and cardiac surgical programs in the country, and we are uniquely positioned to care for these patients from infancy through adulthood as a children’s hospital directly attached to an adjacent adult cardiovascular center.  In addition, through our innovative research and participation in multi-institutional consortia and registries, we are on the forefront of clinical quality improvement initiatives and novel approaches to management for patients with tetralogy of Fallot.

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