08/12/2025
Understanding Tetralogy of Fallot🩺🧐👇
Tetralogy of Fallot (TOF) is a congenital (present at birth) heart defect that involves four structural abnormalities in the heart. It causes mixing of oxygen-poor and oxygen-rich blood, leading to cyanosis (bluish discoloration of skin, lips, and nails).
🛑The Four Defects in Tetralogy of Fallot
1. Ventricular Septal Defect (VSD)
• A hole between the right and left ventricles.
2. Pulmonary Stenosis (narrowing of the pulmonary valve or artery)
• Obstructs blood flow from the right ventricle to the lungs.
3. Overriding Aorta
• The aorta is positioned directly over the VSD, receiving blood from both ventricles.
4. Right Ventricular Hypertrophy (RVH)
• Thickening of the right ventricular muscle due to pumping against obstruction.
🛑Clinical Features
1. Cyanosis
• Blue lips, nails, and skin due to low oxygen levels.
2. “Tet Spells” (Hypercyanotic Episodes)
Triggered by crying, feeding, or stress. Symptoms:
• Sudden deep blue skin
• Rapid breathing
• Irritability or fainting
Cause: Increased right-to-left shunting.
3. Squatting Position
Children instinctively squat to increase systemic vascular resistance → improves oxygenation.
4. Poor feeding & failure to thrive
5. Clubbing of fingers and toes (chronic cyanosis)
🛑Diagnosis
Investigations
• Chest X-ray: “Boot-shaped heart” due to RVH
• ECG: Right ventricular hypertrophy
• Echocardiography: Confirms defects
• Pulse oximetry / ABG: Low oxygen saturation
🛑Management
1. Emergency Treatment for Tet Spell
• Knee-chest position
• O₂ therapy
• Morphine
• Beta-blockers (propranolol)
• IV fluids
• Sodium bicarbonate (if acidotic)
2.Definitive Treatment
✔ Corrective open-heart surgery
• Usually done at 3–6 months of age
• Involves closing the VSD and relieving pulmonary stenosis.
If severe cyanosis early in life:
• Temporary shunt surgery (e.g., Blalock–Taussig shunt)
🛑Prognosis
• After complete repair: Excellent survival and good quality of life
• Lifelong follow-up is necessary for:
• Pulmonary regurgitation
• Arrhythmias
• RV dysfunction