How is tetralogy of Fallot (TOF) treated in adults?

Updated: Nov 13, 2018
  • Author: Shabir Bhimji, MD, PhD; Chief Editor: Yasmine S Ali, MD, FACC, FACP, MSCI  more...
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Answer

Most symptomatic adults with tetralogy of Fallot (TOF) require some type of surgical procedure, of which pulmonary valve replacement is the most common (usually performed under cardiopulmonary bypass).

In general, the majority of adult patients with tetralogy of Fallot are managed with elective surgery. Unlike infants with this condition, emergency surgery is very rare in the adult patient population. Once the diagnosis of pulmonary valve insufficiency is established, the patient is referred to the surgeon. Because the repair surgery is considered a "redo" surgery, albeit many years after the initial procedure, the surgeon will perform an exhaustive workup to ensure that the patient has a complication-free procedure.

In tetralogy of Fallot, the right ventricle (RV) can be dilated and hypertrophied and, hence, it may adhere to the posterior sternum. This is vital information to know before opening the chest with the mechanical saw. Thus, obtain a lateral chest x-ray to confirm this finding. If the RV is adhered to the posterior sternum, the surgeon may opt to have the patient go on bypass via the groin vessels, decompress the heart, and then open the chest with an oscillating saw. Once the chest is opened, the rest of the procedure is like that of any open heart surgery.

Risk factors for surgery in adults with tetralogy of Fallot

Factors that increase the risk for surgery in adult patients with tetralogy of Fallot include the following:

  • Cardiogenic shock

  • Poor RV function

  • Diminutive pulmonary arteries

  • Other major associated anomalies, such as tricuspid atresia or an anomalous coronary artery

  • Multiple previous surgeries

  • Advanced age

  • Severe annular hypoplasia

  • High peak RV–to–left ventricular pressure ratio

  • Multiple ventricular septal defects (VSDs)

  • Right-sided heart failure

See also the Guidelines section for the 2018 American Heart Association/American College of Cardiology (AHA/ACC) recommendations for the management of adults with tetralogy of Fallot.


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