Tetralogy of Fallot, Key Points for the Newborn

TETRALOGY OF FALLOT IS A SERIOUS AND COMPLEX TYPE OF CONGENITAL HEART DEFECT. IT CAUSES:

  • Reduced blood flow to the lungs.

  • Mixing of oxygen-rich and oxygen-poor blood inside the heart.

  • Low levels of oxygen in the blood.

TETRALOGY OF FALLOT INVOLVES FOUR SPECIFIC HEART DEFECTS:

  • Pulmonary stenosis: narrowing of the pulmonary valve and the area below the valve that causes decrease of blood flow from the right ventricle to the lungs.

  • Ventricular septal defect (VSD) a hole in the wall that separates the left and right ventricles.

  • Overriding aorta: a defect where in which the aorta sits atop of both the left and right ventricles between the left and right ventricles, over the VSD.

  • Right ventricular hypertrophy: thickening of the right ventricle.

  • Cyanosis (bluish tint of skin, lips, and nail beds) is a common sign of Tetralogy of Fallot. This happens when oxygen levels in the baby's blood are low.

  • Most babies with Tetralogy of Fallot have a heart murmur. This is an abnormal heart sound that is caused by turbulence in blood flow which may be heard with a stethoscope.

  • Doctors do not know what causes Tetralogy of Fallot.

  • Babies who have other birth defects, such as DiGeorge Syndrome or Down syndrome, are more likely to have Tetralogy of Fallot.

  • Some babies have a sudden drop in the oxygen level in their blood. This is called a Tetralogy "spell." When this happens, your baby may:

  • Have a hard time breathing.

  • Be very tired and limp.

  • Not respond to your voice or touch.

  • Be very fussy.

  • Have a seizure.

IF YOUR BABY HAS A TETRALOGY SPELL, YOU SHOULD:

  • Bring the baby's knees up tight against the bay's chest (the knee-chest position).

  • Attempt to calm the baby.

  • Call 911 if your baby's symptoms do not immediately improve.

TESTS

  • An echocardiogram is the test used most often to diagnose Tetralogy of Fallot.

  • All infants and children with Tetralogy of Fallot must have surgery to repair the defects or to help improve their symptoms.

  • Timing of surgery depends on the severity of pulmonary stenosis, cyanosis, or presence of heart failure symptoms. Most infants have surgery within first year of age.

  • With new advances in testing and treatment, most children who have Tetralogy of Fallot repaired with surgery survive to a healthy adulthood.