Article Sections

Introduction

Tetralogy of Fallot (ToF) is one of the most common congenital heart defects; it accounts for up to 10% of congenital heart disease cases and occurs with equal frequency in males and females.  ToF results from disrupted embryonic development that leads to 4 characteristic abnormalities:

  • Narrowed right ventricular outflow tract (RVOT).
  • Large ventricular septal defect (VSD).
  • Overriding aorta (ie, the aortic root is slightly shifted toward the septum and overlies the VSD).
  • Concentric right ventricular (RV) hypertrophy.

The clinical presentation of ToF depends on the severity of the RVOT obstruction, with most patients developing cyanosis during infancy or childhood due to right-to-left shunting through the large VSD.  Recognition of ToF is important because timely surgical repair is needed to reduce morbidity and prevent mortality.

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