Tetralogy of Fallot (TOF) in children

Tetralogy of Fallot (TOF) is a congenital heart defect that is a combination of four heart problems. It significantly affects the heart’s structure and function, leading to poor oxygenation of blood. The four abnormalities that define TOF are:

Ventricular Septal Defect (VSD): A hole between the heart’s two lower chambers (ventricles).
Pulmonary Stenosis: Narrowing of the pulmonary valve and outflow tract, obstructing blood flow from the right ventricle to the lungs.
Right Ventricular Hypertrophy: Thickening of the muscular walls of the right ventricle.
Overriding Aorta: The aorta, which usually carries oxygen-rich blood to the body, is positioned directly over the VSD, receiving blood from both the right and left ventricles.

Symptoms of TOF

Cyanosis: A blue tint to the skin, lips, and fingernails due to low oxygen levels.
Difficulty Feeding: Babies may struggle to feed or fail to gain weight.
Failure to Thrive: Poor growth and development.
Tet Spells: Episodes of deep cyanosis, trouble breathing, or fainting.

Diagnosis

Echocardiogram: Main diagnostic tool, provides detailed images of the heart.
Chest X-ray: Can show the heart’s shape and size, which is often altered in TOF.
Electrocardiogram (ECG): Assesses the electrical activity of the heart and can detect right ventricular hypertrophy.
Cardiac Catheterization: Used to assess the severity of the defect and plan for surgery.

Treatment

Surgery: The primary treatment for TOF, usually done within the first year of life. The surgical procedure typically involves:
- Closing the VSD.
- Widening the pulmonary valve and outflow tract.
Palliative Surgery: In some cases, a temporary surgery is performed to increase blood flow to the lungs until a full repair can be done.
Long-term Care: Children with TOF require lifelong cardiac care, even after surgical repair.

Prognosis

With early surgical intervention, most children with TOF grow up to lead normal or near-normal lives.
Long-term survival rates are high, but some may require additional surgeries or interventions in adulthood.

Complications

Residual Defects: Such as leaks in the pulmonary valve.
Arrhythmias: Irregular heartbeats are common in later life.
Endocarditis: Increased risk of heart infection.

Preventive Measures

While the exact cause of TOF is unknown, good prenatal care and avoiding harmful substances during pregnancy can reduce the risk of congenital defects.
Genetic counseling may be recommended for families with a history of TOF or other congenital heart defects.

Regular follow-up with a pediatric cardiologist is crucial for children with TOF, to monitor their heart health and manage any long-term complications.