commonly said factors are genetic predisposition so children with the trisomy 28 downs and all are more prone for developing congenital heart disease if you take the environmental risk factors mainly the viral infection drug toxin taken by the mothers and diabetic mothers and mothers in finland heterogeneous they have been found to have more incidence of congenital heart disease so this is the simplest classification of mental aid disease if you see this part we have the venus return from iec svc going into rb pulmonary artery so from the venous system of systemic circulation it goes to the arterial system of pollinator patient similarly venous system of pulmonary circulation goes into the arterial system of systemic circulation this is the most important thing they should have you should understand whenever you talk or you think of a congenital heart disease okay so how they are divided is they are taking this pulmonary circulation conditions in which there is increased pulmonary blood flow okay that they are taken as one part so conditions which has got an increased pulmonary blood flow so what are the conditions in which you can get increased blood flow in the palm system any communication from left side to right side could be an amd it could be a vsd could be a communication from the iota to the pulmonary id that is your pda or you can have a transmissible system called effect all those things you can have the second part is decreased poly circulation and when do you get decreased pulmonary circulation when the blood doesn't go to permanent circuit it goes to the opposite side so your right left chin so your phallus tetralogy where your esd with right election if you have a problem with the pulmonary valve it could be a pressure pulmonary atresia or it could be a tricia or egas and the third classification is abstract deletions absolutions can be in the iota like iot extensions in the pulmonary artery it could be a permanent stenosis that can be practitioner biota or it could be you can have a cardiomyopathy okay this is how this is simply they classify the tangent lattices more pulmonary circulation left right this is less permanent circulation right to left disease so before we start we'll have to see few basics okay maybe all the post judges might be knowing it but for completion sake and for some other graduates you may not know this so this is meant for them this is what i was talking to you about our systemic venus return from ivc svc enters the right atrium this is the venous side and it goes into the pulmonary artery so the amount of blood that goes from the venous side to the artery determines how much of blood is going to the pulmonary circulation similarly you have a pulmonary vein pulmonary vein becomes iota that is the arterial cell systemic circulation so this is going to determine how much is the systemic circulation so if you put a straight line cut these two things your right sides and you have a left side let's say this is systemic circulation right side is a fundamental circulation whatever you say you have a venous site you have an arterial side okay as long as the blood goes from the venous side of systemic circulation or from the permanent circulation and reaches the arterial side then there is no problem with the patient okay but we will see now so we call one term effective blood flow effective blood flow is nothing but the quantity of venous blood flow that flow from one system here it is from systemic circulation to the arterial is the venous blood going with the arterial side the kind of [Music] effective pulmonary circulation effective pulmonary blood flow or qp is nothing but the total blood that comes from sbc and ivc [Music] from there it goes to the pulmonary artery from venous site so this is what we call this effective pulmonary blood flow it is termed as qp this is very very important similarly in the systemic side we have total blood flow coming from the pulmonary system going into the iota and the further inside it goes to the systemic side because it's systemic blood flow or qs okay are you able to understand this in a normal patient if you see here the amount of blood that goes through the pulmonary artery is equal to the amount of blood that comes through the iota that means there is equal q p to q s ratio so there is pile it is going out from here there is pile it is coming out of this place so if we have a system like this nothing is going to happen to the patient okay just if not i'll go slowly also this is effective polynomial blood flow qp this is effective systemic blood flow q s in a normal baby the normal heart the qp is five years q s is five liters that is the amount of blood going out of only at least five liters amount of blood going out of iota is bilateral that means there is no communication between or there is no loss or no recirculation of blood so in this case while it is one is to one ratio next important thing that we see is shunting shunting is both physiological and pathological here the venous site from one system doesn't go to the arterial side of the other system inject the venous site from the from the systemic circulation goes to the same arterial side of the same system systemic circulation so the blood grows from the svc ivc or right atrium or right ventricle to this side left ventricle left atrium or the iota okay that means there is a td i mean asd esg or a pda okay so this is called as left to right shank so from binary pain if instead of going to iota the blood will go to the pulmonary artery it recirculates in the pulmonary system so this is called the left to right shin similarly the return venus return that comes from the right atrium right ventricle instead of going to the pulmonary artery passes through the vsd opening but there is a reverse left hand being right to left shell reaches the aorta so there is going to be cyanosis because this blood is not getting oxygenated so again there is a shunt here so in right left shunting there is a connection between the systemic vein and the systemic artery in left right there is communication between pulmonary vein and the pulmonary artery so this is the major difference between shunting and normal in normal it is only systemic being going to the pulmonary artery pulmonary vein going into systemic artery ancient it is pulmonary vein not going into systems artery but it goes to the pulmonary artery so this is where you get your kgb hmpd is systemic way going into systemic artery this is why you get a right relationship with your palate so based on that you can see the magnet production you can quantify how much of blood is being chanted if you see this is a normal heart you are having a pulmonary artery that is the output from the pulmonary circulation you have the iota that is the output from the systemic circulation you see both the blood flow and pulmonary that is five liters blood flow in the high water that is filing test so there is no shunt few peak us ratios one is to one so five liter blood is going through the pulmonary artery while trouble is going through the iota there is no shunt here in left to right shunt here we have a bsg where there is a left right shin here if you see the blood comes to the la from the binary wave goes tlb and from lv through the vsd because lb pressure is more than rb one liter of blood is getting shunted into rb so instead of five liters five liters here it becomes six liters here more blood goes to the pulmonary artery from pulmonary artery it will go to the circulation go to the lung come back to the fundamental vein come back to the left atrium okay so what is the end result there is less blood going into the iot part more blood is going to the pulmonary artery so if you see qpsq ratio six liters of blood is going through pulmonary artery when only five liters of that is going to the iota that means there's a chant of one liter per minute so if you put it into ratio it is six meters by point five meters which will be less than one so q p by q will be less than one if the shunt is going to be left to right so what happens when more blood goes into the pulmonary artery more blood comes up our left atrium there is increased venous return to the left atrium so again back to left ventricle there is volume overload to the fundamental circulation there is volume overload left ventricle so in all left directions there is increased black aluminum blood flow because of increased fundamental blood flow there is volume overload under circulation volume over to left ventricle if this shunt is going to become more more and more what is going to happen is it is going to affect the right particle also so the shunt becomes three times support times normal there is going to be volume overloading right side also this will end up in biventricular failure or cardiac failure so this is about left to right so we should know what are the signs and symptoms of any child with the heart failure it will not be like an adult sitting or leaning forward breathless and all these are the clinical symptoms disney are during feeding child cannot do a proper breastfeeding because a child is not able to breathe properly whenever the child beats they'll be sweating there'll be tachycardia tachypnea frequent respiratory tract infection because the lung is flooded left direction increased permanent circulation because of hypoxia will be irritable restless most important thing for us to diagnose is the hepatomegaly inferiority we don't get a raised jvp in pediatrics we get hepatomegaly in pediatrics these are the signs and symptoms suggest you of heart failure in children the most important thing is we don't get a discharged central lamin jugular or dependent edema which we normally see in adult with failure plus if you see the qs is 5 liters qp is 600 that means the systemic circulation is our cardiac output is going to be less in impatient with left direction one more entity in left right side is suddenly the left hand can become right left because of rise in pulmonary artery pressure the pulmonary artery pressure goes high normally in this esd you get a left right shin if the pulmonary artery resistance increases very high because of hypoxia hypercardia there is going to be raised pressure in the right ventricle so instead of normal left right chin through the vlg it will become right to left shin so this is called as ease and manga syndrome so in a patient diagnosed with left right and like asd vsg if you are going to see a central cyanosis dyspnea fatigue synchro always think of the season magnet syndrome the prognosis with the patient with decent magnet syndrome is very bad because it is because of slow changes in the pulmonary artery now we will come to right to left we are seeing the left right shoulder now instead of right to left here you see the blood is coming from htc ivc it is coming with the 5 liters per minute it comes to right atrium this is the venous return from right atrium it comes right ventricle okay from right ventricle there is a vsd and there is abstraction to pulmonary artery it could be abstraction at the arterial level at the valley level at the at the subvaller level when there is abstraction the blood cannot go here it has to go to the left and rican okay from left ventricle it will go to the iota so this is a river right left at the level of your ventricular septum so let us see what happens to the amount of blood flow okay so qp or the pulmonary blood flow is reduced because there is a pulmonary abstraction because the polynomial section the hole in the vent we have ventricle the blood pilot is a blood that comes here from r8 rv instead of going to the pulmonary artery it's getting shunted into the left ventricle so iota will have roughly five liters whereas the pulmonary artery will have only two liters okay so this is the main difference between right collection so what will happen to our qpqs ratio so qppus ratio will be less of pulmonary blood flow more of systemic blood flow so the ratio will be less than one whereas in left right chain it will be more than one one point by two so you will have to understand this q p q s ratio if the q p q s ratio is less than one then it is a right collection if the pq ratio is more than one that means more blood in the permanent circuit that can occur only when there's going to be left direction so calmness solution right right left right left should this your palo is technology this is what we are planning to discuss in today's class so what are the components the problem is due to embryological change changes or the defects this iota which normally is placed side by side comes anti-aliased to the left of the ventricular wall during development so you get a large ventricular unrestricted ventricular septal defect plus these patients will have various degrees of pulmonary obstruction the pulmonary abstractions can be subvalvular okay this is the valve this is the most common variety 50 and we call it as dynamic abstraction dynamic right particular outflow obstruction why we are saying dynamic is when the child cries when there is a pain there is a tachycardia what happened during tachycardia is the heart tries to squeeze and the heart tries to squeeze this impendibular part squeezes more when it squeezes more less amount of blood will go to pulmonary artery so when less amount of blood goes to the pulmonary artery pressure in the right particular is going to go up so more amount of blood will get shunted from the right ventricle to left ventricle producing more cyanosis okay so this is a dynamic pattern so what you do is you try to reduce this tachycardia or reduce the compression using beta blockers so this is the reason why you give beta blockers during your dead spell or during the perioperative period other places where you can get abstracted is 10 percent at the level of pulmonary valve 10 above the level of the most important component is this subvaller dynamic right flow upside right twenty cloud flow abstraction okay so one is a bsd there is a big bsd non-restrictive esg second is an abstraction right particular output obstruction at various level and third is i said that during development normally you will get here if you interventricular septum here your iotic valve should be somewhere here because of the anterior and kefla shift of this iota during the turning of the heart this iota instead of lying here it moves slightly towards the right side so that is called as overriding of iota this iota is displaced to the right and overwrites the vsg so if it overwrites the vsd means the blood from the left ventricle will go to the iota but from the right material also will go to the iota and because of the severe abstraction in the pulmonary vessel we get a right particular hypertrophy so these are the four components of fallows technology unrestricted vsd right particular outflow abstraction at the level of supervaluar we are more worried about this infanticular because the dynamic gas and because there is a low rating of iota into the right ventricle the blood which comes to the i20 deoxinated blood which comes from the rea to rb instead of going into the pulmonary artery to get oxygenated we will go into systemic circulation it produces deoxilation and cyanosis so the right left shunt is when oxinated blood returns the tissue without taking oxy the blood comes here oxygenated blood comes and comes from the palmly veins it goes to the left ventricle it should be expected to go through the iota and supply the systemic brain art everything but there is a large vsd here so the oxygenated blood instead of going in only to pulmonary artery it can get mixed here intracardiac mixing mixing with the sinus blood and because the blood is not growing into the pulmonary artery the mixed blood goes to the patient systemic circulation producing cyanosis you can clearly see here the saturation at the level of re that is the systemic venus written is 60 okay this goes to the rv again it should be but here there is a vsd there is some amount of mixing is there because of the mixing it becomes 65 it goes to the main pulmonary artery to get oxygenated and it comes to the left atrium fully oxygenated and from left atrium it will come to left ventricle it should be fully oxidative now what is the problem problem is you have a deoxygenated blood here and the amount of blood that goes to the pulmonary artery is reduced there is a large vsg so there is a mixing because there is a fresh more pressure in the right ventricle compart left ventricle the shift shunt is from right to left so there's going to be maximum mixing instead of 95 percent saturation what that comes out is only 80 or 70 or 60 so normally if we see a pivot patient that saturation will be roughly around 80. even if you give oxygen 100 oxygen it may not go more than two or three so from 80 it might go to 82 83 84 because the problem is not with oxidation the problem is only shunting okay plus as long as the hemoglobin concentration there will not be any cyanosis once the hemoglobin concentration is above 5 grams you will see the sinuses so the main problem is mixing and cyanosis this will produce lots of problems which are going to become complications of phallus tetralogy so why do you get cyanosis there is a limited permanent blood flow because of the abstraction supra pulmonary pulmonary and infra pulmonary right 20 cloud protract plus there is a large vsd so there is a intracardiac mixing so there is poor oxygenation plus there is mixed shunting right to left chanting here so this produces cyanosis so what are complications the patient will have and there is a persistent cyanosis when there is going to be decreased oxygen supply to the cells the erythropoietins start producing more rbcs so you get polycythemia when there is polycythemia autocritically go up and when there is going to be slight dehydration there is going to be sludging thrombus formation so you get thromboembolism including stroke in a patient with pallus tetralogy this is because both there is less amount of blood going into various tissues producing venous thesis plus this polycythemia and increased cementory producing thromboembolic so that is the reason the calmness to non-cardiac surgery that we do for paralysis brain abscess okay plus you will get other things like chronic hypoxia clubbing etc so i said polysaccharides associated with increase in blood viscosity because hymenotokidosis this produces red cell sludging and thrombus when the hemoglobin level goes above 65 we may have to think of doing it pre-operating phlebotomy and i use fluid management because the rheology of blood flow will get decreased so best thing will be to maintain adequate preoperative hydration in children with cyanotic heart disease so whenever you have a child with cyanotic heart disease you reduce your free of knee lowering status try to give more oral fluids if not start the patient the nine is needs otherwise you will end up in producing thromboembolic phenomena these patients with cyanotic heart disease are associated with bleeding disorder also one part is thrombus another part is bleeding disorder this is because they are associated with thrombocytopenia and what are the platelets that are available are qualitatively not good plus there are also certain plotting factor abnormalities all these things put the patient both in thrombus condition as well as in bleeding conditions so you'll have to be worried when you take up these patients for any surgery then i had said there is a right ventricular also tract abstraction at the supravaler valor are infra infundibular spasm so there is going to be increased wall tension the right ventricle you know whenever there is increased wall tension there is going to be a supply demand relationship will be affected with oxygen so these patients are known for failure they're going to be right failure when you give propofol to these patients the patients will immediately crash so you need an echocardiograph and find out how is the right ventricular contraction is very very important one more important thing is you said the blood comes from here only we are going to put a center line or whatever peripheral line the blood is going to come here so ei feeds through here it is going to come to re in t y the problem is there is a big hole here and the pressure are desired in the pressure in the lv so there is a right deletion so if you are not careful with your iv lens it will put some air in the line the bubbles will come to re-rb normally it should go to the pulmonary artery where it can get filtered out but here it's not so the air bubble from our rv will go to lv from iota the first branch of iodine species okay that is some basic things about your changes whenever you have a candida or you can manipulate the permeability the shunt is at the level of easy that is right to left shed from here it goes to left side vascular resistance and system glass resistance so things that are going to decrease the permanent vascular resistance so the decreases more blood will go to the pulmonary circuit and there will be better oxygenation the amount of sinuses in patients with right left and left shins okay so what are the methods available hyperventilate take the patient to respiratory alkalosis that you put is pulmonary vasculation you can use pulmonary visodilators like nitric oxide your volatile and anesthetics in a lower concentration they are all fundamentally muscularities one of the very important pulmonary is oxygen okay so if you give oxygen to this patient they produce the vasculature so in during textile you give oxygen only to dilate this legislature so that more blood will go from the right ventricle into the palm line circuit to get oxygenated okay so these are the methods by which you can decrease the palmarized nature what are the causes for increasing pulmonary vasculature caterpillar means your ipp positive people give a peep here this constriction here so it is going to produce resistance in the pulmonary circuit increase in pulmonary artery pressure and diabetes pressure releases up right onto the pressure is going to go up when the right-wing decrepit goes up if you are going to have a patient with left right chin the amount of shunting will get reduced now coming to a systemic circulation the methods by which you can reduce this systemic circulation reduce the systemic resistance more blood will go into the methods are you can use vasodilators you can give an epidural or final that will put a sympathetic blockage and it will open up the vascular system investigator deep general anesthesia will produce systemic vasocon massive dilatation here is a hypothermia facing cardiovascular consumption here hypothermia if you keep the patient warm the systemic expectation will open up methods by which you can increase or make this systemic condition resistance sympathetic stimulation the most important thing is vast offensive tests okay if you give a vassar constrictor this will increase the pressure the resistance here it will increase the pressure in the iota it'll increase the pressure in the left ventricle so whenever you're going to have a right to left side if you give penile left friend your patient having a psd is the right deletion patient is gone for spill so i'll give a penile airframe so that this part gets massive constricted so the pressure is transmitted back the right ventricular i mean left particular pressure goes above the right ventricle or it increases near the right particular pressure so due to this the shunting amount of shunting gets reduced the intracardiac mixing is reduced we expect more blood to go instead of through the right election go through the pulmonary artery into the permanent circulation so you get better oxygenation so this is the treatment of its pills okay so these are the various manipulations you can do you should always think of these things whenever you are doing a congenital heart disease patients you can increase the spr you can decrease the sphere you can increase the pvr and you can decrease the pvr these manipulations are the resist vascular resistance is important in managing a patient with congenital heart disease more so with chance so if you go in the in depth how are you going to control the system pass resistance so here you are having a patient with left right shunt okay so i'll give some inhalation agent or some agent that is going to decrease my systemic vascular resistance if i decrease systemic vascular resistance the blood will not go from left to right the blood will go into the iota that means i am reducing the shunt factor either in the pda level on the vhd level or in the ag level so if you have a left direction you can decrease the systemic virus resistance so you can boldly give epidurals you can take the patient into deep general anesthesia or you can use vasodilators so if you have a right to left shin at the level of vsd that is the final technology and can increase the system in gasoline resistance by being penelope so blood cannot come here so blood pressure will rise up here it will prevent or it will reduce the right election so we use spinal affine if you see in a patient with pallus tetralogy where the blood comes from ra to rb and it goes to lb because there is right i'll give phenylephrine and block this iota system resistance due to this the pressure in lv will increase so the shunt factor will get reduced the shunt factor gets reduced the only way the blood can go is it has to go through the pulmonary artery if it goes through the pulmonary artery it will go to the pulmonary cavities and oxidation will improve your saturated desaturation will settle down so this is how you treat patients with dof using fin nephron to increase system gas resistance pulmonary vascular resistance in patients with right deliverance okay if you increase the pulmonary vascular resistance here so what will happen is it will increase the right anticlock pressure there is going to be more shunting more cyanosis and patient will die so in patient with right deletion avoid increasing pulmonary vascular resistance so avoid hypoxia hypercarbia hypothermia ippv with peep and acidosis so in patient with left right hand we want the pulmonary resistance to be reduced so that there will be better output so methods of decreasing this polynomial resistance is oxygen is upon mesoderm nitric oxide is a permanent acidity you can do hyperventilation so that you reduce the carbon dioxide content that is producing alkalosis sometimes if you are systemic i mean metabolic acid these things will reduce the pulmonary particle pressure so there will be more flow into the pulmonary circuit so knowing that we'll come back to our case of tyr things to avoid in turf or there should not be a decrease in systemic vascular resistance if there's going to be a drop in systemic vascular resistance the pressure in the lv is going to go down so the right to the amount of shunting from light to left is going to increase so there's more mixing more desaturation the patient will go in for hypoxia and anaerobic metabolism and death okay so if you have a patient with joint eof don't decrease the system gas resistance so be careful during induction during the regional energy say everything and always keep by so constitute don't give vasodilators in a patient with try to lift shed avoid pulmonary vascular systems if you have a patient with hypoxia hypercardia any increase in pulmonary resistance is to come as a bad pressure the right particular pressure will go up already decide because of that this raised right to a pressure is going to increase the shunting okay and this will be increased chanting hypoxia patient is going to die so things to avoid in patient with pof or right to left center decrease in svr or increase in pvr so things to be done so if you have a patient with sinuses with the chanting maybe a phallus tetralogy this slide is your take home sign okay things to do is increase this systemic pasta resistant so keep a drug that is going to vascular constrict here phenylephrine okay then decrease the pulmonary so i want more blood to go to the lung to get oxygenated so i like to have almond vasodilators i like to hyperventilate i like to avoid acidosis and keep the patient in alkalopic state okay so take home message from this class is if you have a patient with cyanotic rbc's right to left shunt things that i'll keep work and keep penal left and ready then i try to avoid hypoxiapacabia acidosis that is going to produce vascular constriction okay that is the basics now we'll go to our finance technology so we already discussed calorie they have unrestricted non-restrictive large vsd right on the cloud protract abstraction free layers we are worried about the infertile level there is a overrating of iota into the right side then there's going to be a right hand trophy the fact is that determine the severity of tyr okay you have seen you would have heard something called pink or bloated okay so that depends on how much of this permanent vasoconstriction is there or right 20 cloud flap section is there how big is your vsd and what is the pressure difference the factor that determines the severity and cyanosis depends on the degree of right and cloud protractor abstraction more it is subtracted this blood will go to the lung so patient is going to deteriorate or become pleasant creative pressure between right ventricle left ventricle because of the substraction there is going to be arrays right now pressure during infantible spasm this pressure is going to go more okay when it becomes more the only way the blood can go is it cannot go inside it doesn't jump so there is going to be mixing when it goes to iota there is a severe severe cyanosis third is the proportion of iota over radio vsd certain condition the iota will be on the left side alone and many many serious pof the iota will be almost inside the right ventricle something like a dorb okay double outlet when two so these are the factor that determine the severity of pof so tuf is classified into pink blue and profound cyanosis when we say pink phallus means there is abstraction somewhere in supra palmy area on the palm area where there is not much of a restriction the blood is able to go into the pulmonary artery get oxygenated the patient has got some amount of pulmonary it's you know right when allowed for abstraction the patient has got a vsd there is a mixing there's a minimal rvs so that is called as pink tetralogy of followers so no or minimal right mental health obstruction little iotic overriding behaves simply like an esd with a reverse shunting and the classical blue technologies where you get the infinity glass spasm plus also a valuable abstraction so there is definitely right ventricular abstract abstraction the iota is definitely pushed well into the right ventricle so there is going to be intense mixing so you are going to have cyanosis so the babies will be sinus so blue tetralogy of follows then there is another condition called a severe or profound cyanosis phallostetology that occurs when there is going to be a hundred percent blocked that is pulmonary atresia or a trichosphere where that thing comes into the right ventricle this is a really life cycling condition baby is born blue the only method which the blood can go into the pulmonary circuit to get oxygenated is you have a communication between the iot and the pulmonary artery the pda so these babies are called as bda dependent or duct dependent cyanide or disease so after a month or so due to normal reason that the pds closes the patient will die because it was purely dependent on this pda or the survival and the pda process there is no way the blood can go into the pulmonary artery and get oxygenated the patient will die so these babies require maintenance of pda patency using your prostaglandins so clinically how do you suspect uof as cyanotic child with no features of respiratory distress and you gave oxygen sinus is not because of oxidation problem the power fingers is because of intra connection okay so they fail to respond to your oxygen therapy thing is iota is okay the return is okay so there is a good card so this is if you have a patient like this cyanotic heart disease without respiratory problem failure to respond toxin therapy with a good cardiac output suspected whenever there is a tear always see for the associated features like withdrawal syndrome down syndrome etc and there is always associated bacterial anomaly association they can have that reasonable crystal learner so once you see one anomaly always search for other nmds so that is the teaching of neonatal and it's easier other clinical features are cyanosis depending upon the right-wing cloud contract abstraction can be gradual or severe low spo2 with little response toxin therapy there will be heart murmurs which could be due to pulmonary problem not because of vsg vsg in these cases doesn't produce this murmur because it is not a small vs it's a large non-restrictive energy you get murmurs only when there is restriction okay then there will be classical steps pill episode where the child is getting agitated distress becoming sinus becoming unconscious and a bigger child will squat a smaller chain the mother will know how to flex the lower limbs so those spill episodes will be there and these children are prone for superanticlear ventricular arrhythmia because of hypoxia so these are the classical features of clinical presence of patient with palo statology the other features of dead spills because the child is hypoxic poor excess tolerance chronic hypoxia produces clubbing polycythemia the problems of policy theme is neurological developmental delays they can have failure heart failure they can have a recurrent respiratory tract infection most commonly non-cardiac surgery that we see with the patient with your cerebral abscess on physical examination the classical thing is central sinuses clubbing will be there on inspection on palpation you will get a right one tick like he paris and he will be there auscultation is very classical because you get only large single s2 you don't get a pulmonary valve closure because the pulmonary valve is almost it is it is not opening fully there's not much of blood going inside so when it is not fully opening it doesn't produce that amount of sound to produce p2 so what you get is a single load is h2 is the classical s1 is not s2 is a single load s1 okay that is the reason in classical clinical finding in a dof child you will get a systolic murmur but systolic moment is not because of vsg because it's a large vst because large bhd will not reason is any resistance or any sound the sound is because of the blood passing through the obstructed right ventricle output rack so you get a ejections historic normal so that is what you hear when the baby goes for a text pill it goes for a severe spasm no blood will go inside so you will not hear any murmur during take spelling plus the patient can have problems of science and censorship and historically so if you're taking xhs it's a classical xhs tof you get a boot shaped art the heart is upturned because there is a right ventricular hypertrophy technology because there's no blood going into the partnership you can get a concavity of the pulmonary segment okay so the ascending iota can be dilated or sometimes you can get a right iot also in these patients ecg characteristic you see a righteous hypertrophy right access deviation you get a tall pvas in v1 and you get a tall rv under slightly taller or s ways so if you add this you will get a right angle hypothetical pattern if you do equip a classic right one triple left ventricle separated by a large vsd you can see the iota is overriding the vsd okay it will classically give you the shunting plus you can find out there any other intracardiac anatomy you can find out whether how severe is rbo2 we can also find out whether the obstruction at the level of pulmonary valve or it is an infundibular abstraction all these things you can be able to find out in a patient with yours when you do a hippopotamus and we have we already discussed about stuck dependent circulation if you have severe pulmonary stenosis or trichosphere synthesis no blood will go here so this baby will be born blue the only method by which oxygen and lung can get oxygen is blood coming from iota entering to pda going into pulmonary circulation okay so after some time when the pda closes the patient will die so for these severe patients what we're supposed to do is we'll have to maintain this patents of pda you maintain using a prostaglandin like androstene or procedure okay we are seeing the various issues in twf now we should see what are the complications the most important complication both clinically asked us for an exam the second thing is the most common non-cardiac surgery that we give is get this cerebral axis because of polycythemia dehydration venous status then all these patients inaudible there's always a chance of impacting the current is so in your amateur harmonium keep antibiotics ready give it before the startups any surgical procedure and these patients are prone for try to underclap failure or sometimes both particular failure and they can go for sudden death because of sudden desaturation and if you don't treat they can go from there this is a common complication so one of the most important thing in whenever you talk about calories technology is it spin itself is nothing but acute desaturation and clinical deterioration so normally in a phallus technology the cyanotic blood from the system system becomes re goes to rb from rv it has to go to pulmonary artery get oxygenated here there is an abstraction here could be a valuer or a dynamic infanticular abstraction due to this there is a right ventricular hypertrophy the right particular pressure is more than the left ventricular pressure there is a big hole here due to this the cyanotic or deoxygenated blood gets mixed here and the iota is on the right side so the next contraction for the ox needle blood and the mixed blood goes into the iota okay this is what normally happens in the patient material but during this test spill what happens there is a severe infantive spasm when the severe inflammatory virus is passive blood will not go to the pulmonary artery if no blood goes to the pulmonary artery or very less blood goes to the pulmonary the amount of blood that is getting oxygenated in the lung gets decreased so deoxygenated blood comes through the permeability into la under lb because this high pressure whatever the deoxygenated blood that comes from systemic circulation also mixes so there's a vicious cycle going on where this desaturation becomes more and more till the patient dies okay so it is caused by a sudden increase in right deletion one is because of infinitely stressful second is because of alteration in systemic parasite systems pulmonary systems anything that produces vasodilation is going to decrease the left ventricular pressure when the left ventricular pressure decreases more blood is going to go from right to left so more right elevation anything that increases the pulmonary vascular resistance and hypoxia for caviar acetosis apart from this identical output section is going to increase the right temperature pressure and it is going to increase the right election so both those things will produce intense desaturation and clinical deterioration so various methods by which you can get severe desaturates one is infundibular muscle spasm which increases the right election we said it is a dynamic right ventricular abstraction the causes for this is trying pain anything that produces catecholamines they produce intense infanticular muscle spasm okay so you have to understand this because during perioperative period you not keep the patient calm you may have to use beta blocker to prevent this beta against action producing in particular spasm you have to give adequate pain relief that is why you give ketamine and pentane and all the procedures that are going to produce hypoxicambiasis you will have to avoid okay so that is the importance of knowing this impendible muscle spasm second is large reduction in systemic vascular insulin severe reduction system faster resistance more blood will go from right to left left ventricular so right left shank so causes for decreased system gas resistance would be bioso dilating drugs your type in tone or your nitrates all those things are vasodilator drugs your anesthetic agents your inhalation agents everything will produce vasodilation your regional analysis here epidural spinal those produces severe asthma direction sepsis it produces vasodilation the patient taking hot baths producing peripheral vasodilation everything opens up the system of gas resistance and reduces the steam gas resistance when the system invasive resistance reduces there is going to be right deletion so serious analysis so second part which produces that spin third parties this part on a vasculature previously what is always right and cloud for abstraction here this part so for it if you think of the neonatal goals hypoxia hypocarbia acidosis pain hypothermia all those things produces serious constriction when there's going to be severe pulmonary vasoconstriction it is going to be pushed back and there is going to be increased right ventricular pressure we have opening there so blood will naturally go to the right side producing more sinuses for this tachycardia anything that increases the catechol level is going to increase the tachycardia when the rate increases the amount of time available for diastolic in diastolic timing gets reduced so when the heart comes to extend the amount of blood going if the pulmonary artery is reduced so this is one method of having a producing deoxidation plus when now this catechin goes for more spasm when it goes for more spasm less blood will go into the colony so you have to avoid tachycardia using your beta problems and finally the most important part is you see there's a right ventricular hyperprotein and there is also because of cyanosis both the right ventricle left ventricle will have a implant use your traffic function that means they are not able to relax when they are not able to relax there is very rapid rise in the in diastolic pressure and to push more blood into the pulmonary artery and into the iota you may have to increase your venous done that means you will have to give some gold as a fluid so that it goes into the pulmonary artery so this is one method of treating your head so the four problems are infinity glass passion large directional system glass resistance increase in pulmonary vascular resistance increase in heart rate and decrease in diastolic usotropic function okay so these are the problem that are going to trigger so what are the triggering factors for infant blood spasm is sympathetic stimulation pain anxiety so good pain relief good pre-medication no im injections be careful with the belly punches avoid sympathetic drugs avoid exercise child crying feeding defecating all those things can produce caterpillar excess produce this abstraction of the right trade 20 cloud for crack second is reduced system gas resistant with iota if you reduce the system gas resistance the pressure in the lv is going to go down so more shunting the causes are drug induced or environment induced we'll have to be careful during index of anesthesia the third is in the peripheral part of the pulmonary vasculature pulmonary vascular constriction hypoxia hypercarbial species so what are the classical clinical features when the patient goes for spin child cries deep dedicates all those things there is some amount of hyper apnea catecholamine which stimulated the child goes in for infantile spasm becomes hypoxic restless agitated breathless if it's a bigger child child knows how to sit so they go for this starting position when they squat the chemical resistance gets compressed so there's an increase in system faster resistance the shunting factor reduces the child becomes alright the child becomes synonymous the most important thing is because there is less amount of blood going during the scale the murmur intensity gets reduced and because of severe hypoxia the child loses consciousness and there can be seizures so having known everything it is easy to treat okay so what are you going to treat give oxy to visor dilate the pulmonary system support breathing and support the circulation the problem is right collection will have to try to reduce the slight election however you're going to reduce you try to open up this part if it is going to be infinitely sparser if it's due to catecholamine use beta blockers sometimes you may have to use pulmonary vasodilators but the most common cause of pulmonary vasoconstriction are hydrogenic gas or our anesthetic causes hypoxicabilia as you know treat those conditions then most important thing is because the left ventricular pressure is lower than the right particular pressure there is more of shunting so increase the left particular pressure who are you going to increase catch here give us systemic bias of constrictor so phenylephrine is not a very good faster constrictor which acts more in the systemic circulation than in the permanent circulation so you should have phenylephrine whenever you have a patient with cyanotic heart disease more so with phallus tetralogy so in the exams both in theory and in the clinicals when they ask him how will you marry that spell the extra 14 steps okay first is remove any precipitant cause the cause could be hypoxia for cardia pain fear anything try to remove the precipitate cause second is try to calm the child provide comfort have the parents along with the child third is try to sedate the baby other parts also fourth thing will be squatting if you have a smaller child just do this niche's position so this will compress the femoral this will increase iotic resistance so systemic resistance will increase the lb pressure will go above or it will be becoming equal to rb pressure shunting will reduce next high flow oxygen or oxygen this will not do anything to the right 20 cloud crop section definitely lower the pulmonary vascular constriction pulmonary vascular resistance then boluses of slow it could be a normal saline or ring lactic what it does is it goes into the rv rb it is a volume expander it increases the pulmonary blood flow to the abstract trinomial output so this bonus is very important then you give an electron with a 5 to 10 milligram goal less after that you may have to give as an inclusion this decreases the right to election day by reversing the system class orientation that you have produced then coming to infinity glass fashion i want the heart to wait to reduce i suppose contractility reduces so what we have is a beta blocker so they reduce the rate they reduce the obstruction during spill you can use a short acting smaller the other period you can continue with the proper node if you have severe cyanosis and if you are not sure whether the patient has got a then this avoid primary beat diagonally sympathetic so there's going to be a drop in bp usually we use dopamine natural level and the patient is severely bad try to avoid giving b diagonal symptomatics to this patient even afiden also will a person because that is going to act on the heart you will incubate the patient you will ventilate the patient this is a place where you can hyperventilate the patient so that to reduce the paco2 we are already seeing acidosis is going to increase the pvr so create a respiratory alkalosis to decrease pvr reduce the right deletion if the patient is orthodontic for a very long time if your baby shows severe steroids then there is no harm in giving soda back up onto two milli equal and this will reduce the effect on system gas regime but don't give it before you see the abg if none of these 13 things are working i think that is the time going for an urgent surgical intervention it could be a palliative or a complete repair the palliative repair is nothing but there is an obstruction to the pulmonary vision so i can divert the systemic blood into the pulmonary artery distilled to the main pulmonary abstraction so that the blood will go from diode go through the duct or do it through your ponder go to the pulmonary artery get oxygenated the surgery or a temporary surgery there are various forms of systemic arterial pulmonary artery shunting one is the vatican chanting second is the pot shunting third is a classical bollock transitioning where the left subclavian is connected to pulmonary artery okay the corrective procedure for this is if you've already done a shunt you will not like it the shunt the problem is with the infinite lump the pulmonary artery so the infinite recession to open out the right anticlockwise that needs to be closed using a patch then rb tpa contact may be necessary if the patient has got a severe pulmonary efficiency okay that is about the management of text pain okay now we're coming to our final part of our lecture if this patient has come to you for a non-cardiac surgery either emergency or electric surgery and the patient is far away from surgical cardiac surgical care so how are you going to manage you have no other way to manufacture assessment then you have to take care of the casting guidelines you have to be very careful in the fasting period then you have to give a good pre-medication and then you have to think of infected endogenous propellants so if in this stratification we have classified neutral disease into minor major severe stratification and our patient who is a right to election pilots technology comes under severe form of risk in tangential heart disease so we'll have to be very careful give a high risk explain where good informed consent to the parents first thing you see is how much is it science i know this is blue or pink okay then ask for the history hyper scientific spells how frequently they are occurring okay do a spot check up sp02 okay normally for a blue values we don't expect the saturation 82 to 83 okay give oxygen it'll be only one or two will be increasing next most important thing that you should see is see for the metropolitan if the mitochondria is going to be very high that means they are prone for thrombus so you may have to do a phlebotomy or give adequate iv fluids to maintain the rheology and we already said these patients are prone for both traumas aspects for bleeding disorder so check for coagulation studies and these are the priority evaluation you should do other than proper history taking and examination coming to npur status keep these patients well hydrated this is the most important thing that you should understand cardiac patient i know fluids nothing of that sort if you have a phallus or cyanotic heart disease keep them well hydrated because the prolonged and pure period is going to produce dehydration this is going to increase the viscosity which is going to increase the endocrine producing flat g so if the hemoglobin grows more than 70 you may have to think of being a pre-operative laboratory hypo linear what it will do is it will produce catecholamine release so it will squeeze the your right ventricle output so the infinity plus spasm will become more so you should not allow the baby to go for hypolimia it has been found to a patient to be kept either you olympic or even with hyperbole okay so this will keep the right particular outflow track open so you will have to be a bit relaxed with your fluid management pre-medication is very very very very important for a divorce patient you should have a calm relaxed baby good interaction with parents inside is very very crucial to give you sedation but you'll have to take care the patient doesn't go to hypercarbia state because hypercardia is going to produce increase in pulmonary resistance so it is the right mission okay try to avoid incremental injection try it try to avoid scary things in the data because anything these things will produce anxiety categories so infinite glass fashion increase cyanosis so that you very careful carefully keep monitoring the sp water after you're given patient the patient is calm so you'll have to be very careful in this hyper caviar business when you are going to get pre-medication next important thing is the guideline says various conditions in which you will have to give infected qualities prophylaxis one of the most important thing is unrepaired cyanide congenital heart disease rtofs underpad cyanotic and genital art disease so you will have bacterial endocarditis prophylaxis you just prepare this because they'll definitely come as a short ones so there are various antibiotic regimes available commonly what we use is amoxicillin oral or amplicity in iem if this is not available or you can go for a cephalocephaltroxy they are allergic to penciling than other things so in the exams don't say whatever antibiotic name that you you know off say as for the latest available guidelines of american autism okay let us be very clear so you will have to say that adult dose periodic tape pediatric you will have to say milligram per kg so what is your primary goal in genetic management of t yf there is right to left i want to reduce it is substitution if possible i like really there right section okay so if i do this i'll have a better definition better system delivery this is not hypoxic i'll be able to maintain the ventricular function not increase the ventricular contractility because that will squeeze the right ventricle output so by doing this i should be able to maintain the cardiac output for the cardiac fluid yes because i don't between right and left forget the left hand left right nothing will happen in left right chain everything will happen in the right left hand so the tuf is right if we are going to use the inhalation induction ventricle it has to go to the brain through the iota what happens here is there is some dilution here the blood doesn't go more into the circulation the effect if you get overdosed you cannot eliminate the drug from the body because the circulation is imperfect so with induction the problems into your phase shunting here and the recovery is also going to be slow when it comes to intravenous usually gets some kind of kind of a dilution the dilution effect is not there so whatever drug you give here is going to go to the brain because it goes from right to left into the caron [Music] important thing that you should understand during perioperative management is increases there is going to be right to left shunt so there's going to be a deterioration so maintaining safari is very important so for induction and maintenance because the ktm has got increased svr capacity doesn't increase sdr interval be the ideal drug of choice for induction with uof that can be combined with pentane so during induction so i may give small to prevent the stachycardia because that will prevent that now we have this dexmeditomidine use the baby with ketamine and put it in with dexterity that is balanced by the cardiovascular reaction of vitamin combination of ketamine based melatonin very useful in reducing the right left chin ideally if you are going to use this patient with tv volatile agents can be used they can test relaxation of the rvot but they should be used only in the lower concentration if you give higher concentration that will open up the system of gas resistance so that will produce increase in right-wing levels so we can use a low concentration of volatile agent of the volatile agent halogen which we don't have now we had it that is ideal because it acts mainly in the heart less in the system gas resistance and it has got a capacity to relax the right infinite blood but it is not available so you can boldly use low concentration of co4 in avoid hypolimia avoid hypothermia because they'll increase viscosity they'll increase fundamental resistance give adequate fluids give warm iv fluid because i don't want hypothermia be very careful with the iv lengths paradoxical embolism possibilities there so free of air bubble should be there in your lines good avoid the factors that are going to increase upon mass resistance this is the goal of your united states or any anesthesia hypercarbia both metabolic and respiratory acidosis hypothermia or anything that is going to increase the sympathetic don't like the night plane etc so keep the patient really deep avoid regional energies here renal anesthetics which will produce sympathetic blockage abg will be ideal in this scenario because pulse oximeter may not be reliable because creation pre-operated of 80 when it becomes 17 is not going to show much so aabg will give you a better understanding of the oxidation status management of pain is very important but avoid regional i mean sympathetic blockage use some kind of a pain management which doesn't touch the sympathetic blockage pentel bolus and parasimal inquisition post-operatively are very good for pain relief so that is about your tof so to conclude i'd like to see things to be aware in ntof are decrease in hdr or increase in pvr things to do when you have a patient with co tyfr increase the systemic vascular resistance and decrease the pulmonary vascular resistance thank you so i'd like to once again thank you santa madam and the kim's opportunity to give a lecture on pof why i chose to uf i already said this is one of the commonest thing that you get as an emergency in pediatrics in click practice plus you will definitely get this in your exams either so long it's a short part of this incredible cases short keys afternoon session you can get a fellows uh x-ray abg you can get all the things you cannot escape from tof so that's it about what so the combination of a sledge ring almost hypoxia everything makes that particular more vulnerable to abscess formation so there is more chance of sludging hypoxic injury is always there because in these patients so the brain takes more uh chances for getting infected and more for abscess formation this incidence of you adam i can't hear it is roughly around one to one point eight percent in cyanotic or diseases this is more are you getting frequently in the cases emergencies there is no questions from the audience means you can madam i can't hear you madam there is no question from the audience now we can finish up where minimal blood flow into pulmonary tree how pulmonary vasculature as the resistance occurs this rise in pulmonary rot is totally different from pulmonary vasculature our rotis are delivered the heart pulmonary vasculature is at the level of lung the heart the block is because of infantive spasm or valve valve's noses our goals in united anesthesia so two are totally different but both will produce a rise in right ventricular pressure when now there's going to be right rise in right ventricular pressure there is going to be increase in right and left shunting so again cyanosis that vicious cycle continues so that is the difference is there any other ancestor yeah yeah so only thing i can't use it after therapeutic surgeries corrective surgery corrective surgery what a problem is mainly the act and the right ventricle they enter through the right ventricle do all those infant blood and all so most likely you will get lots of arrhythmia problem will be there that is one of the commonest problem then they can also have problems with the record laryngeal nerve and all because of the surgical surgical part nerve injuries so you can have airway problems because the bronchi is somewhere near so mainly the arithmetic problems and that also depends on how well it is corrected itself the survival is better yes previously with total correction we have one we had only around 15 to 20 survival yes we almost reached 60 to 70 survival it's a slow process because it's a everybody gets trained and the equipment's everything is improving so we are definitely seeing a better outlet output