Dr Supratim Sen, Senior Consultant in Paediatric Cardiology and Dr Disha Patel, Resident Paediatrician at Narayana Hospital ...

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Blue Baby Syndrome | Diagnosis &amp; Treatment | Dr Supratim Sen and Dr Disha Patel

hello everyone I'm Dr Dasha from srcc Children's Hospital managed by narana healthare and uh I have Dr supram s senior consultant pediatric cardiology with us to discuss the topic tety of f so can you tell us what is tety of f thanks disa so uh let me just explain based on this diagram so tetrology of phow is the common cytic congenital heart disease what is also known as a blue baby so there are two main defects in this disease first you have a hole in the heart you can see this hole between the two pumping Chambers which is allowing the deoxygenated or blue blood to mix with the red blood and in addition there is a block in the vessel which is taking this blue blood to the lungs this is known as the pulmonary artery so when you have these two together along with uh some other addition s this is known as tetrology of phow okay so can you tell us if it can be diagnosed during pregnancy yes so tetrology of phow can be diagnosed by fetal Echo as early as 16 to 18 weeks of gestation during pregnancy and when we are making this diagnosis we also look at some additional findings for example we see how good is the flow from the pumping chamber into the pulmonary artery what we call anti-r blood flow and we look at at the sizes of the pulmonary arteries so there is an extreme form of tetrology of phow which is known as pulmonary atreia and uh it is important for us to differentiate these two when we are doing the fetal Echo uh so what advice would you give to patients who are diagnosed with tough or during pregnancy so whenever we make this diagnosis first of all we reassure the parents that this is a treatable condition we have good surgical outcomes of this and now that it has been diagnosed at the correct time the baby after birth uh can be treated at the optimal time and they'll have great long-term results secondly based on our fetal Echo findings we also advise the parents whether the child should be delivered at a cardiac center which means that we can see them immediately after birth and then we can plan the further treatment accordingly so if not diagnosed anen then what symptoms would a child of to present at birth so the commonest symptom is what we say is desaturation which means that the oxygen saturation of these patients will be low now what is low a normal oxygen saturation is 95 and above and generally a tetrology of phow will have saturations which are less than 85% if these patients are saturating say somewhere between high 70s to even low 80s we don't need to do anything immediately we just keep them on close followup but a child is saturating in the low 70s or less than 70% then they definitely require some treatment in addition to the saturation the other thing uh these uh patients have is what is known as a CTIC spell and during a CTIC spell when these babies are crying they become extremely blue and that happens because the flow into the lungs gets really shut off and this is kind of like an emergency it needs treatment and then uh we need to plan for future surgery if the baby with tough has low oxygen levels how do we manage that so first of all if the child has a CTIC spell that is an emergency and we advise the parents to keep the baby in a knee chest position if the baby is irritable then they should pick up the baby and try to console so that the baby kind of doesn't cry as much and then they should come to the nearby hospital so that we can give some IV fluids we can give some medications to try to settle the baby a child who is stable but has low saturations we start them on a medicine known as Propranolol and this is continued till the time of surgery now as I mentioned if you have a baby on medication who has a saturation above say high 70s or 80% then we would wait till about 5 to 6 months of age to do a complete repair but often these patients will be quite blue despite the medication they might have oxygen levels which are say 70% or less and these patients are then uh they require a perative procedure so we have various options for the pallative procedure the oldest pallative procedure is the BT shunt also known as an autop pulmonary shunt and what the surgeons do here is they connect the body artery the aorta to the lung artery the pulmonary artery this in fact is one of the oldest cardiac surgeries which was done it was done way back in the 1950s the newer version of this what we do in the cardiac cath lab is known as a PDA stent so nowadays we don't need to do a open surgery for these patients we can go through this small vessel which the babies are born with and then we put in a bent into that enlarge it so that there is a good communication between the orta and the pulmonary artery and with this then there is good oxygen level till the time of the next surgery there is a third option which we have started doing over the last 5 to 10 years which is known as an rvot stent so if you see here we can pass a wire through this stenos pulmonary artery and then we can put a stent in there and once we enlarge this stent then there is going to be better flow across this pulmonary artery and that again helps to maintain the oxygen levels in these patients till their complete surgery sir at what age do we plan a complete surgical repair so Studies have shown that the best results with complete surgical repair are between 3 to 9 months of age and in India we will be very comfortable operating these patients even at 5 to 6 months of age there are however some patients who will present us late so suppose someone comes at one year or two years of age we would operate them as soon as they are diagnosed and in fact at our Center we have also done early surgical correction of tough as early as 3 to four weeks of age and these are very small babies but with the correct expertise they also have good results so is it a single surgery or do this patient require additional surgeries later so again let me come back to the initial picture off uh what the surgeons do during the surgery is they will close this defect they do a vsd closure and they will open up this block which is known as a pulmonary valvotomy now if this vessel is very small then the surgeons are not able to preserve this valve so they have to cut across and enlarge this what is known as a trans anular patch and if that is done where the surgeons cannot preserve the valve in these patients then they have a leak on the that valve and that grows as the child gets older and a leaking valve will probably need a pulmonary valve replacement sometime in the second decade of life if the surgeons are able to preserve this valve at the time of the first surgery then it's a single surgery for the child sir are any minimally invasive options available for this pulmonary Val replacement yes so over the last decade we now have percutaneous pulmonary Val implantation which is a viable and a good alternative to a surgical valve implantation and this is slowly being adopted at many centers in India also it is still relatively more expensive than the surgical option but over the next decade I think it will be become much more mainstream and much more affordable for the patients so what is a long-term outcome for these patients so techology of phow generally has a very good long-term outcome if the patient is diagnosed and treated at the correct time they go on to lead normal lives female patients can have a normal pregnancy uh and they have normal exercise tolerance they can do their daily activities they can have an active lifestyle so whenever we uh counsel the parents of a child with tough we give them a very good long-term prognosis [Music]

Dr.

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