If your child turns deep blue, becomes limp or unresponsive during a spell, go to hospital immediately. While waiting for help, hold the child with knees drawn tightly up to the chest — this position is known to help during a blue spell. Keep them calm.
The commonest cause of a child being born blue. Four features occur together: a hole between the pumping chambers, narrowing of the route to the lungs, an aorta sitting over the hole, and thickening of the right ventricle.
Tetralogy of Fallot sounds alarming and is, in fact, one of the great successes of cardiac surgery. Children who have complete repair in good time usually grow up active and well. What matters is not delaying, and knowing what to do during a blue spell.
Four abnormalities occur together, and they are linked. A ventricular septal defect connects the two pumping chambers. The route from the right ventricle to the lungs is narrowed. The aorta sits over the defect rather than solely over the left ventricle. And the right ventricle thickens from working against the narrowing.
The consequence is that some blood which should go to the lungs to collect oxygen instead crosses the hole and goes out to the body. The child receives blood that has not been fully oxygenated, and this is why the lips, tongue and fingertips look blue.
How blue a child is depends mainly on how tight the narrowing to the lungs is. A mild narrowing may produce a child who looks almost normal — sometimes called pink Fallot. A tight narrowing produces a deeply blue baby who needs early intervention.
Older children often learn instinctively to squat during a spell, drawing their knees to their chest. It is not a habit; it is an effective manoeuvre that raises the pressure in the body's circulation and pushes more blood towards the lungs.
Stay calm, because distress worsens the spell. Hold your child with their knees pressed firmly up against their chest, or help an older child to squat. Keep them comforted and quiet, and get medical help. If the child becomes limp, unresponsive, or does not improve within a few minutes, treat it as an emergency and go to hospital. Frequent or severe spells mean the repair should not be postponed — tell us straight away if your child is having them.
Echocardiography establishes the diagnosis; further imaging is used to plan the operation.
Together these two tell us most of what we need: whether the anatomy is suitable for complete repair in one operation, and how urgent it is. If a CT or MRI of the pulmonary arteries has been done, send that too — the size of those vessels is often what determines whether a staged approach is needed first.
Most children have a single complete repair. A minority need a preparatory operation first.
One operation closes the ventricular septal defect with a patch and relieves the narrowing to the lungs, sometimes with a patch across the pulmonary valve. Most children are repaired between three and twelve months of age. The result is usually excellent, with normal oxygen levels afterwards and a child who grows and plays like any other.
Where the pulmonary arteries are too small or the child is too unwell or too small for complete repair, a shunt is created to increase blood flow to the lungs. This buys time and allows the pulmonary arteries to grow. Complete repair follows some months later.
Beta blockers can reduce the frequency of blue spells while surgery is arranged, and iron deficiency is corrected because it makes spells worse. This is preparation for surgery rather than an alternative to it.
Where the original repair involved patching across the pulmonary valve, the valve often leaks over subsequent years. Many patients repaired in childhood eventually need the pulmonary valve replaced, sometimes in their teens or twenties, and this can often be done by catheter rather than surgery.
This is what decides between single-stage and staged repair. It is measured on echocardiography and, where unclear, on CT or catheterisation.
Frequent spells or very low oxygen saturations push the timing earlier. A stable, mildly blue child can be planned around calmly.
Fallot repair needs a paediatric cardiac surgeon who performs it regularly, a paediatric perfusion team and a dedicated paediatric cardiac intensive care unit. We verify all three before recommending anywhere.
Late presentation is common among the families who contact us, and it is usually still treatable. Adults with unrepaired tetralogy of Fallot have been repaired successfully — send the reports rather than assuming the opportunity has passed.
Blue spells are the sign that changes urgency in this condition. If your child is having them, tell us in the first message and see your local paediatrician without waiting for our reply.
Photographs taken on your phone are fine. Reports in Arabic, Russian or Bengali are fine — we translate them ourselves.
Complete repair is most commonly performed between three and twelve months of age. If your child is having blue spells or has very low oxygen levels, it may be brought forward or a shunt placed first. If your child is older and unrepaired, repair is usually still possible — the echocardiogram determines what is feasible.
Very probably not. Children and adults are repaired well beyond infancy, and outcomes in late repair are generally good. What we need to check is the size of the pulmonary arteries and whether any other complications have developed. Send the echocardiogram and we will give you a straight answer.
Possibly, in two situations. Some children need a shunt first if their pulmonary arteries are small, with complete repair following months later. And many patients repaired in childhood eventually need the pulmonary valve replaced years afterwards, because the valve leaks after being patched. That later procedure can often be done by catheter.
Stay calm and comfort the child, because crying makes it worse. Hold their knees firmly against their chest, or help an older child to squat. Get medical help. If the child goes limp, becomes unresponsive, or does not improve within a few minutes, treat it as an emergency.
Most children who have a good complete repair lead normal, active lives, including school sport. The specific advice depends on how well the repair went and on later follow-up, particularly the state of the pulmonary valve. Your cardiologist will give you written guidance.
Plan on four to six weeks for a complete repair, allowing for assessment, around ten days to two weeks in hospital including intensive care, and recovery before your child is fit to fly. One parent travels on a medical attendant visa, and accommodation near the hospital is part of what we arrange.
Send your child's echocardiogram report and their oxygen saturation reading. Tell us if they are having blue spells — that changes how quickly we move.
Your reports go directly to our medical team. We do not share your records with hospitals until you tell us to.
WhatsApp +91 83035 86344 · Phone +91 83035 86344 · Email tibhind@gmail.com
Coordinators available 9:00–20:00 IST. We speak Arabic, English, Russian and Bengali.