This is a combination of a hole between the two ventricles, and a narrowing between the right ventricle and the lung artery. The right ventricle has extra work to do to squeeze blood through the narrowing to the lungs, and as it works at high pressure, is able to pump blood directly from it through the intraventricular hole into the aorta.
A murmur may be noted initially and then blueness and perhaps squatting will occur during the second year. If the problem is more severe, then blueness will present earlier.
Some children have ‘spells’ in which the blood flow to the lungs is reduced for a while. The child may cry abnormally as though in discomfort, breathe quickly, become bluer than usual and perhaps have glazed eyes, whimper and then become rather limp, pale and then pass off to sleep. If these episodes occur, picking the child up and cuddling and reassuring is very helpful. Put the child over your shoulder bringing the knees up onto his tummy, so that they are between his tummy and your chest. This improves the circulation and the episode will subside. It is important to let the medical team involved, know that these ‘spells’ have started to occur. Propranolol, a medicine, can help reduce these episodes. All children will require operation in due course.
In many, open heart surgical repair to patch the hole and relieve the narrowing which may require a patch across the pulmonary valve can be undertaken directly. In others, particularly if the child is small or the lung arteries are small, then a shunt operation to take extra blood into the lung arteries from an arm artery may be required. This operation is performed either through the side of the chest, or the breastbone (sternum) and a formal repair would be planned for later.
The long term results are usually very good. Sometimes, a further operation is required in those who have a significant leak of blood back from the lung artery: into the right sided chamber. This is more likely in those who required a large patch across the pulmonary valve at the time of their operation. A new biological pulmonary valve will be inserted which improves symptoms and the efficiency of the circulation.
Long-term follow-up and antibiotic prophylaxis is required.
Don't mean to step on toes, but you actually only speak of two, maybe three (it's vague, so I'm not sure) of the aspects of TOF. Tetralogy means four, so there are four parts to this defect. My daughter has TOF with PA.
and... may I ask your source? Because it worries me that it says, "sometimes" further operation is required. Very very often, it requires further surgery.
Its from some pamphlets we had given to use by Zac's cardiologist, which now that I look at them are about 15 years old. Its also a solely Australian pamphlet which might explain it too. Surgeons here tend to treat TOF in one go where possible.
Well, many children with TOF need a valve replacement during the repair, and those will not last a lifetime. They have to be replaced. I know my daughter will also have to have the wall between her two lower chambers redone-she had an absent wall. Not all are so severe, so that isn't always redone, but the valves never last a lifetime.
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