Tetralogy of Fallot (ToF) is a complex heart defect that involves four different problems in the heart. To fully explain this complicated condition, we’ll address it in two separate posts. Today we’ll cover ToF, Part I!
What are the four problems that comprise ToF?
- Ventricular septal defect (VSD) – a hole in the wall that separates the heart’s two lower chambers (ventricles)
- Pulmonary stenosis (PS) – narrowing of the valve between heart and lungs
- Right ventricular hypertrophy (RVH) – thickening of the right ventricle wall
- Overriding aorta – misplaced or moved major blood vessel (aorta) of heart
Together, these problems lead to a mixing up of blood entering and blood leaving the heart. Blood that is low in oxygen (“blue”) mixes with blood that is oxygen-rich (“red”). Babies born with ToF sometimes are called “blue babies” because their lack of oxygenated blood can make them look blue (cyanotic).
What are the symptoms?
The symptoms of ToF may be present at birth or shortly afterward, and include:
- Blueish discoloration of skin – “blue baby”
- Clubbed fingers
- Problems with eating
- Failure to gain weight
- Developmental delays
- Episodes of passing out
What are “tet spells?”
Some babies with ToF experience “spells,” in which a sudden drop in blood oxygen level causes the baby to become very blue. During a tet spell the baby may also:
- have difficulty breathing;
- initially become very irritable, later very tired and unresponsive; and/or
- have fits/seizure attacks.
The exact cause of these episodes is not known. Tet spells can be triggered when a child is upset, has a low red blood cell count (anemia), or is dehydrated. Reducing a baby’s anxiety by anticipating what he/she needs to stop crying can help prevent tet spells.
What should an adult do during a baby’s tet spell?
- Bring the baby’s knees up tight against the baby’s chest (the knee-chest position).
- Attempt to calm the baby.
- Call your doctor and/or take the baby to the hospital if there’s no improvement.
Even if the child improves, a consultation with the treating doctor following a tet spell is mandatory.
That does it for our introduction to ToF. But for the rest of the story, including diagnosis, treatment, and post-treatment life, be sure to watch this blog for our upcoming “Have you Heard about Tetralogy of Fallot? – Part II” post!
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