The definitive treatment for tetralogy of Fallot is surgery (i.e., Blalock-Taussig shunting procedure, aortic/pulmonary shunt, intracardiac repair, balloon pulmonary valvuloplasty and/or valve replacement). Surgical correction of this heart malformation is best accomplished during infancy. Selection of the exact surgical procedure is based on the severity of symptoms and extent of the malformation.
The surgeon will widen the pulmonary valve and the passage from the right ventricle to the pulmonary artery is enlarged. A patch covers the hole in the septum to repair the ventricular septal defect. By resolving the VSD and the pulmonary valve problems, it fixes the other two defects.
Temporary surgery may be recommended babies are too weak or small to have the full repair surgery; the full repair surgery will be performed when the baby is stronger. A tube or a “shunt” is placed between a large artery branching off the aorta and the pulmonary artery. It creates a pathway for blood to travel to the lungs to get oxygen. The tube is removed during the full repair surgery.
When early repair is not possible, other surgical measures may be taken during infancy or early childhood.
Prior to surgery, treatment to control symptoms (palliative) may include the maintenance of adequate fluid intake (hydration), monitoring of hemoglobin levels in the blood, and the avoidance of strenuous exercise. Heart medications (i.e., digitalis) may be prescribed to help control irregular heartbeats (arrhythmias), rapid heartbeats, and/or heart failure.
Episodes of severe symptoms or “blue spells” (hypoxia) may require the administration of supplemental oxygen, morphine, and/or other drugs that improve oxygen concentration. The knee-chest position may also bring some symptomatic relief. Sodium bicarbonate may be administered to lower abnormally high levels of acid in the blood (acidosis). The drug propranolol may be given to help prevent future spells and to reduce their severity. Drugs that help to remove excess fluid from the body (diuretics), dietary salt restriction, and bed rest may be effective in treating congestive heart failure.
Antibiotics may be prescribed to infants with tetralogy of Fallot to help prevent infections (prophylaxis) because children with this disorder are susceptible to bacterial infection of the heart (endocarditis). Respiratory infections must be treated vigorously and early. Children should be given antibiotics at times of predictable risk (e.g., tooth extractions and surgery). Other treatment is symptomatic and supportive.
Although the risk for tetralogy of Fallot in the siblings of infants with this disorder is thought to be very low, genetic counseling may be of benefit for parents and other family members.