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TOF (Total Correction; four abnormality correction)

Definition

Tetralogy of Fallot refers to four types of heart defects present at birth (congenital).

Causes, incidence, and risk factors

Tetralogy of Fallot is classified as a cyanotic heart defect because the condition causes too little oxygen levels in the blood, which leads to cyanosis (a bluish-purple coloration to the skin).

The classic form of Tetralogy includes 4 defects within the heart structures:

At birth, infants may not show the signs of the cyanosis, but later may develop sudden frightening episodes (called "Tet spells") of bluish skin from crying or feeding.Tetralogy of Fallot occurs in approximately 5 out of 10,000 infants.

The cause of most congenital heart defects is unknown. Multiple factors seem to be involved. Prenatal factors associated with higher than normal risk for this condition include maternal rubella or other viral illnesses during pregnancy, poor prenatal nutrition, maternal alcoholism, mother over 40 years old, and diabetes.

There is a high incidence of chromosomal disorders in children with tetralogy of Fallot, such as Down syndrome and Di George's syndrome (a partial gene deletion that results in heart defects, low calcium levels, and immune deficiency.)

Symptoms

Signs and tests

A physical examination with a stethoscope almost always reveals a heart murmur.

Tests may include:

Treatment

Surgery to repair heart defects is always done when the infant is very young. Sometimes more than one surgery is needed. The first surgery may be done to help increase blood flow to the lungs, and a surgery to correct the problem is done at a later time. Corrective surgery is done to widen part of the narrowed pulmonary tract and close the ventricular septal defect.

Tips for parents of children with tetralogy of Fallot:

Expectations (prognosis)

Most cases can be corrected with surgery. Babies that have surgery usually do well. Without surgery, death usually occurs when the person reaches age 20.

Patients who have continued, severe leakiness of the pulmonary valve may need the valve replaced. Regular follow up with a cardiologist to monitor for life-threatening arrhythmias (irregular heart rhythms) is recommended.

Complications

Prevention

There is no known prevention.


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