Rapid Review: Tetralogy of Fallot

Reviewed January 2024

Tetralogy of Fallot

• Most common cyanotic congenital heart disease in childhood
• History of episodes of cyanosis (tet spells) and squatting for relief (decrease R➔L shunt, increase oxygenation)
• Echo: pulmonic stenosis, right ventricular hypertrophy, overriding aorta, and VSD
• CXR: boot-shaped heart
• Mnemonic: PROVe: pulmonic stenosis, right ventricular hypertrophy, overriding aorta, VSD

Sample question:

A 1-year-old girl presents to the emergency room after her mother noticed that she became blue while crying. The patient subsequently squatted on the ground and the mother noticed a resolution in the cyanosis. On physical exam, the patient is now calm and vital signs are normal. A loud systolic ejection murmur is noted on physical exam. Chest X-ray demonstrates a “boot-shaped” heart. Which of the following is most likely the diagnosis?