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    Tetralogy of Fallot

    Exams and Tests

    Even if the bluish color and other symptoms have resolved by the time the child gets medical attention, the health care provider will immediately suspect a heart problem. Medical tests will focus on identifying the cause of the cyanosis.

    • Lab tests: The red blood cell count and hemoglobin may be elevated as the body attempts to compensate for the lack of oxygen to the tissues.

    • Electrocardiogram (ECG): This painless, quick test measures and records the electrical activity of the heart. Structural abnormalities of the heart usually produce abnormal recordings on ECG. In tetralogy of Fallot, right ventricular hypertrophy is almost always present.

    • Chest x-ray imaging: This image may demonstrate the classic "boot-shaped heart." This occurs because the right ventricle is enlarged. It also may show an abnormal aorta.

    • Echocardiography: This imaging test is key. It will demonstrate the ventricular septal defect or large hole between the left and right ventricles, the degree of pulmonary stenosis, and it will reveal other unanticipated defects. Many patients do not need cardiac catheterization if the clinical, ECG, and echocardiogram findings are routine and as expected.

    • Cardiac catheterization: This is an invasive procedure accomplished by a cardiologist in a special laboratory with the patient under local or general anesthesia. This procedure was done on all patients with suspected tetralogy prior to echocardiography because it was the only procedure that could be used to confirm the diagnosis. If needed, a small tube (catheter) is inserted through the skin into a blood vessel (usually in the groin) and advanced up the inferior vena cava into the heart. An x-ray image is taken while a small amount of dye is infused. The dye helps highlight the ventricular septal defect, pulmonary stenosis, overriding aorta, and the size of the pulmonary arteries.

    Tetralogy of Fallot Treatment

    Self-Care at Home

    If your child starts to turn blue, place the child on his or her back in the knee-to-chest position and call 911 or your local emergency number.

    Medical Treatment

    Surgery is the primary way to correct the heart problem. Your child may be prescribed medication for tet spells. You will also be given information for dealing with future tet spells.


    • The child will be placed on his or her back in the knee-to-chest position to increase aortic resistance. The increased aortic and left ventricular pressure reduces the rush of blood through the septal hole from the right ventricle and improves blood circulation to the lungs, so more red blood reaches the tissues.

    • The child may be given oxygen through a face mask to increase the amount of oxygen in the blood.

    • The child may be given morphine, propranolol (or metoprolol), or, in extreme cases, phenylephrine (Alconefrin, Vicks Sinex). These medications decrease the frequency and severity of tet spells.


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