Tetralogy of Fallot

Cardiovascular · ~11% of blueprint

Pathophysiology: Most common cyanotic congenital heart disease. Four features: pulmonary stenosis, RV hypertrophy, overriding aorta, VSD. RVOT obstruction → right-to-left shunt → cyanosis.
Signs & Symptoms: Cyanosis; 'tet spells' (hypercyanotic episodes relieved by squatting); systolic ejection murmur from pulmonary stenosis; digital clubbing.
Gold Standard Diagnosis: Echocardiography. CXR: 'boot-shaped' heart (coeur en sabot).
First-line Treatment: Surgical repair. Acute tet spell: knees-to-chest/squat, oxygen, IV fluids, beta-blocker, phenylephrine.

High-Yield Pearl

Most common cyanotic congenital defect — PROVe: Pulmonary stenosis, RVH, Overriding aorta, VSD. Squatting ↑afterload → ↓right-to-left shunt, relieving tet spells. Boot-shaped heart on CXR.

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Sources

Reviewed September 2026

2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease — ACC/AHA/HRS/ISACHD/SCAI, 2025
StatPearls: Tetralogy of Fallot — NCBI Bookshelf / StatPearls, 2025

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