Jonathan Ginns, MD, Austin Heart Cardiologist, Adult Congenital Heart Disease Subspecialist, presents the latest on Adult Congenital Heart Disease.
the presentations, obviously titled Adult Congenital Heart Disease. And obviously the reason for that is I just started with Austin hard a couple months ago, very excited to get the PhD program up and running and so we thought, would be a good way toe. Introduce things by going through, Ah, the area and in particular will cover a couple of cases that we've already seen here and then a two beginning also cover some of the general principles eso as you probably all aware, you've probably seen a slight ad nauseum or similar slides at various meetings. But now we all know there's an increasing population of patients with congenital heart disease now who are surviving to adulthood, with over 90% of patients with repaired defects surviving and beyond the age of 18 years. Now this has led Thio massive enlarging population eso that now when you look at the overall population of Children versus adults, what we think is that maybe about a million Children alive with congenital heart disease and 1.5 million on this was as of 2000 and 10, so the numbers have probably significantly increased from them, but there's definitely Mawr adults alive with repaired congenital heart disease than Children. At this stage, because of the enormous successes over the last half a century with surgical interventional medical on dee other kids, you may be aware that the adult congenital heart disease guidelines were recently updated in 2000 and 18. I've been a long time coming. The last update to these guidelines was in 2000 and 10, and many of my colleagues contributed to this on. And it's an important document. Um, we will allude to it at various times during the presentation on, obviously, to get a full understanding of this area. Reviewing the the guidelines is worthwhile, and they go through legion by lesion Andi. Certainly there's been a significant improvement in the amount of daughter available to contribute to Ah, really evidence based, not practice in this area Now, given the very large population of patients, um, a couple of I thought we just cover a couple of the concepts that novel in the new guidelines. Um, there's now ah, consideration of a CHD lesion. Complexity on this has been divided into low complexity, moderate complexity and high complexity lesions, and this has meant to God various things, but in particular it's meant to God, um, referral. Thio, especially clinics with low, uh, extra lesions. Maybe once or even Everett all seeing adults in general specialists. Moderate complexity lesions such as partial, almost intravenous repair. Coronary Normally, um, sub aortic stenosis. Co optation. Double chambered right ventricle. Epstein's repair tetralogy, Rastelli operations. Seeing adults in general specialist intermittently and in the more high complexity lesions such as unrepaired zoonotic in general patients Transposition Fontanne Patients on Trunk A. Eisen Mega patients interrupted, able to guards patients more frequently. Seeing sub specialists Uh, just a plug for the I. C H D program. We are aware of data this is actually comes from Canada when they introduced essentially mandatory follow up in adult congenital heart disease clinics. They did demonstrate improvement in survival in these patients. When they're refer, a referral cares, and you can see on the upper couple mile plot versus a non referrals in a care with improvements in mortality and outcomes in these patients. And, of course, an A C H D program doesn't involve just one person, as we say it takes a village with a cardiologists, surgeons, nurses, P. A's in peace, Um, the involvement of other cardiac sub specialists such as heart failure, invention, genetics, probably hypertension, and also non cardiac specialists such O, B, G, y n and hepatology. Obviously, the full suite of imaging modalities is important. Toe have available with experience readers and echo T um CTM mom and obviously, uh, excellent AP and Kath services. So I thought we just move on and just mentioning cases in particular is a couple that I've seen while so being here, which I think were very interesting and illustrative of the area. So some of you may know this this case, uh, this is a gentleman that I met around about a month ago on, but he had a history of repaired to challenge your fellow a two years of age. He had a right likely classic Bt shunt. And then, at five years of age, he had a left classic Bt shunt and then allege of 11 years, had a complete repair of the Mayor clinic. Bryant Magoon Hey had no follow up his young adult, which is obviously common with his patient. He moved to Boston in the 19 nineties hey, presented in 2000 and 10 with sustained one more figure ventricular tachycardia on underwent a jewel chamber i c d placement out in Arizona in 2018. He presented with C J Dependent Natural Photo and underwent ablation. Um, and more recently has been bothered by symptomatic practices. Well, a fib that's being medically suppressed and considering ablation. So just to cover what a BT Shante is, uh, this is the type of Shanta that this gentleman had, which is a classic BT. And that's where the subclavian artery is actually detached and attached to the pulmonary artery. And this allows additional palmeri blood flow in a patient who's got critical palm restenosis with inadequate oxygenation. Um, in the last few decades, the classic BT China's gone out of favor, mostly because of inability. It's a sort of unpredictable nature and also, obviously, that the fact that you lose blood supply to the upper limb on has been replaced by what's called a modified BT shot, which is basically pre specified size Gortex tube, which is taken from the right subclavian or left subclavian artery down to the bilateral pulmonary artery and provides a predictable primary blood flow. Uh, that operations actually less done now, and most patients will just undergo a primary tetralogy repair. But the BT China is still used in other situations where the patient can't undergo a full repair, so you'll probably recall it. This is a challenge. Your pair. You can see the oval overriding a order over what was a ventricular septal defect, which is now closed with the VSD patch and then across thes demotic Pommery valve. Most frequently, trans trans annular incision is placed with a patch to enlarge the outflow tract. And this these, uh, create some of the complications. Long term. So this patch you can obviously damage the pulmonary valve and lead to palm regurgitation. The VSD patch can lead to heart block, and both of these can lead to scar and actual. Everybody is atrial and ventricular. Everything. Use eso. Mr. J. M has experienced worsening shortness of breath on exertion for the last six months. He's now short of breath with minimal activity, gets intimate in palpitations, corresponding episodes of a fib. Hey denies any Deemer abdominal selling and has had no neurologic events. He's also overweight. We think he has hypertension, and he's anxious. He works as a radio consultant and lives in Austin with his two sons. On examination, um, importantly to note here, um, he's comfortable arrest saturation, the normal. So he doesn't have any evidence of rightto left shunting. It's blood pressure is diminished in both upper limbs because of the prior classic Beauty shuns, and he has a narrow pulse pressure on both sides. So these both these blood pressure is a completely completely unreliable. So if he turned up to the, you wouldn't know if he was in shock or not, because the beat is shut, you know, basically precludes you from measuring the blood pressure. Essentially, so he's real blood pressures most accurately represented by the blood pressures. And in his foot, which is 1 67 are, he said, bilateral poster authority economies in the midline stone on to me, his jugular venous pressure is elevated, and he has a short systolic murmur at the left up, a sternal border radiating to the back on the left, and that radiation to the back usually suggest that there may be pulmonary artery stenosis on that side, and he has a short, low pitch diastolic murmur, which is consistent with Paul Marie vegetation chest is clear. Abnormal examines normal, and he's gotten our demo. He's been subtle. Aspirin on licensure. Proehl This is easy, E C. G. And you can see he's in Sinus rhythm with a prolonged a delay that may be due to the VSD patch. And he's got the classic appearance post tetralogy with the right abroad, right bundle, branch block and this wide right bundle branch block Probably predisposes thio, ventricular tachycardia and certainly most cases that I see you MBT they have a wide curious complex, his labs essentially normal with normal synthetic function of live. Oh, so this is his echocardiogram and you can see his right ventricle significantly violated here, but the function appears relatively preserved. LV function appears pretty normal and you can see a VSD patch here on his I c D league. This is, ah, an echo looking through his outflow tract and you can see this is the blue forward flow and then he's got a very broad reversion jet upon regurgitation. Obviously, this is consistent with basically wide open P I So you're looking for this broad jet And if we had a P w w through here, very short pressure Halftime And if you see this, this is obviously clearly consistent with severe upon regurgitation. We mentioned his pressure across the tri casted valve and his obvious piers, at least 50 plus his right actual pressure. Eso is it's suggesting that he's got significant elevation of his obvious systolic pressure. Now this is abnormal. And if you've got this without any promise diagnosis, it suggests that there's some sort of obstruction at the level of the primary vascular chin on then. Last but not least, he has mild able to grow vegetation. And you can also see the VSD patch here. Um, aortic regurgitation is communist. These patients get older, do toe rehabilitation. So we went ahead and did a cardiac catheterization. This is a nice picture showing the included right subclavian artery on the right hand side with a tiny little bit of residual flow here. So this was closed off for the time of his tetralogy. This was the bt shunk, but then it was closed off at the time of the surgery. And on the left side, he's got the same thing. So his subclavian artery here is occluded. You consume system, tiny little collaterals. This is his main p a gram. You can see there's a couple of things here. Very vigorous contractions, right ventricle, Very pulse. It'll pulmonary artery here. And that's obviously consistent with his primary vegetation. Um, you can see the right pulmonary artery lighting up quite well, but interestingly, can't see the left pulmonary artery at all. Ah, and it's almost as if there's no flow going to the left lung on. When you see this, it usually means something. So we went ahead and place the catheter out in his left for Marie Artery. And lo and behold, he does have a left on the artery, but he's got a very severe L. P a stenosis, and you can actually see a little bit of retrograde filling of the old Bt shunt. So the location where the BT shunt was attached to the left coronary artery. He's developed a very severe LP, a stenosis, and because of that in the way that Pommery vasculature works, you probably is getting very minimal blood flow to the left lung. Most of his blood flow is going to the right law, and that's at least part of the reason why is P. A pressures are elevated, and you can also see the reserve the remainder of the primary. Vasculature looks quite abnormal here, with very ecstatic vessels that looked like he may have vascular disease. And this is a common appearance after someone's had a longstanding Bt Shonda. And remember, he had his BT shunt for almost 10 years. A twist. His first one he had started after three years, and then he had the other one for about six years before he underwent his final surgery. And the flow associate with Bt Shank can obviously deployment vascular disease as well. So this is his cardiac cath numbers. He can see his right actual pressures. 20 so severely elevated. Filling pressures on the right side was cardiac. Capital is normal. It's obvious. Pressure is 66 7. 19 is P a purchase 65 of the 22. So this is essentially a ventricular pressure. Tracing in the p A. And this is presentation is severe lp a cyanosis, and he's got a 30 millimeter Grady. And from the main primary artery to the left coronary artery, he's got elevated wedge pressure. Um, the cause of the elevated wedge pressure. It may be due to his long standing hypertension. It may also be a secondary effect from the CVP I eventually causing diastolic dysfunction on the left side. You can see doesn't have any evidence of a significant shunts starting at the S B C 57 going to the P 8 59 and he's able excite is a little bit lower. Uh, this is his cat scan on, and the reason we did this was done was actually to look his pulmonary arteries. But we can also use this toe. Look at the dimensions of the, uh, the memory outflow tract. You can also see his aortic root is severely dilated. At 4.9 centimeters, you can see the LPs. This is his main primary artery, and this is his left for Marie Artery. You can see this diagnosis. Also, we take measurements of the outflow tract to see if he is suitable for a trans catheter primary valuable place. And I'll show you why in a second. So the management for this gentleman involves undergoing a primary valve replacement, and he's also pride of that going to have an l. P. A stent. Um, you can see this is what a p A stand looks like, and these usually balloon expandable stands open cell that a place in the pulmonary arteries and the pH. They're actually usually pretty compliant and now dilate relatively straightforwardly, and it should be relatively low risk procedure. And the reason we elected, I'll just go back to the P a gram. The reason he elected to do this in the Catholic brothers quickly was because of how far out this LP iced and Isis is. It's almost all the way out of the Highland, and this is usually pretty difficult for the surgeons. Thio actually attack, so he's gonna get LP a stand followed by pulmonary valve replacement. It's important. Remember, pulmonary valve replacement Always gonna be a bio prosthetic valve. We don't use mechanical valves in the primary position because it's a very high rate of thrombosis. Eso the lease. You either get a tissue valve or home graph home revolve replacement. And obviously more recently, trans catheter primary valves, in particular for valve and valve situations, have become more popular. So this brings us to some of the late to securely after tetralogy repair, which I'm sure most of you are aware of but certainly for marine vegetation is extremely common, with more than two thirds having at least moderate form regurgitation. Try customer regurgitation, usually secondary to P ay ventricular tachycardia. Atrial flutter is the most common natural arrhythmia. A would have delectation this occurs in more than 50% will have an aorta greater than four centimeters over It turns out that aortic dissection is extremely rare in these patients, and then light stage congestive heart failure. CHF is actually, whilst it's present to a mall degree, it's actually forms a very small component of patients to trilogy. Patients were pretty small component, those requiring transplant with adult congenital heart disease. So what do I do for the follow up tetralogy patient? Most patients will get a yearly eco focused on the primary valve. Like we said, the custard valve you look for residual V S DS. You want to try and see the pulmonary arteries from the super sternal view and also look at the able to greet to see how dilated it is and to see if there's any associate aortic regurgitation actually doing memory every 2 to 3 years on these patients, mostly to document any change in right ventricular volumes. Obviously, if the patients not appropriate for memory because they've got a device or because they claustrophobic a C T is reasonable alternative arrhythmia, monitoring and re stratification is very important in these patients, particularly as they get older. VT seems to be more and more likely. When I first started that you know, eight or nine years ago, I didn't think this was so common. But certainly as time has gone by, we've seen more and more patients present with BT Stress testing is important to get an idea of the exercise capacity because most of them all underestimate. It will overestimate the exercise capacity, and genetic testing for 22 Q 11 is an important thing to do. Um, in terms of primary valve replacement, Um, this is the from our guidelines is obviously very busy slide talking about the if you have any of these two criteria, which is a multitude of criteria or other human dynamic abnormalities. But we're just kind of cut to the chase and the important things to take home from that slider. If the Avi volume by Emory is guarded 160 miles but made a squared with the normal volume being about 80 to 90 miles. It is great. So roughly double that or the systolic volume is greater than 80 with obvious more than double the size of the LV. If there's evidence of systolic dysfunction of the the ventricle, if there's reduction, exercise, tolerance or if they're secondary effects such as arrhythmias or try customer good station, then you should think about Palmer Valve replacement. Um, I thought I'd just bring this, uh, there is a new, uh, trans catheter valve that's become available. And actually, the trial, um, rolled out pretty quickly, and they placed a couple of valves up. But up at Columbia, where I worked before about a month ago, there first to that place about a month ago. This may become a new option for these patients. It's called Thea All Terror Device. It's, uh, from Edwards. There's a competing device from from Medtronic as well, but this device, just to give you an idea, it is under Philip that very large outflow tract, because most these patients have very wide outflow tract. And even with the 29 millimeter uh, Edwards SAPIEN valve, that's too big for that and also the outflow tract is usually too compliant. And so they've designed this basically kind of pre stent that will be placed in into the outflow tract, that self expanding and then after that's placed and has stabilized inside that and Edwards valve could be placed. And so, uh, this may be coming if the obviously if the trial is successful and they have good outcomes. Uh, in orderto for a patient like this toe, actually avoid surgery and this is how it's it's placed. You can see the priest in here. It's It's pretty faint, and then a valve valve Edwards valve is placed inside, and this is the appearance on echocardiography. Obviously, VT risk is an important thing in these patients. On the main risk factors are older age systolic dysfunction of a little ventricle. Cure s with greater than 180 milliseconds. Non sustained VT. On Holter monitors. Eso this passion to get a hold for event every one or two years. Extensive fibrosis by Emory. And when one of these is present, it's now considered reasonable, uh, to a by the recent guidelines to do an e p study to look for induce Able VT. And if they haven't usable VT then to get a different blood. Um, so the next case, um, move onto eyes. Also patient I saw just a few weeks ago. He's a gentleman who's years old, so I didn't put his agent who had a co optation repair at age four at UCSF. Unfortunately, we didn't have his surgical reports, but it was complicated by L V. Perp preparation and paraplegia. He moved to Austin about 10 years ago, has had long standing hypertension. Neil said the Device Days D closure 15 years ago, and this is just a memory. This is not his memory, but this is his own right. Just to remind us all what a calculation looks like. And this is an Emory with cattle Any, um um and you can see ascending aorta here, arch descending a water and then a very severe co optation here, just distal to the left of Cleveland. So this is a native co optation. This is my name. But just just to remind us all and then distort so that there's often act Asia of the descending aorta. These patients will frequently have a bicuspid valve and dilated aorta ascending order as Well, they're different types of computation repair. I think this gentleman had an end to end anastomosis because of the appearance. And this is the most common procedures. So they respect of the cohabitation and they bring the two ends back together like a sausage. There's an interposition graft. This is infrequently done. Um, though some adults will get this procedure because it's actually difficult to mobilize the A water a patchy angioplasty again. This is infrequently done now, but was more frequently done in the parks and this could be complicated by pseudo aneurysm formation, then So clever, so clever and turned down flat. Actually, we had a lot of these in Australia. I see this less in the U. S. But these patients were basically missing the left subclavian artery so you can't check the blood pressure in the left arm so that multiple different types of co optation repair. So he's hypertension has become more difficult to control. He's now short of breath with moderate activity. You have some atypical chest pain and certainly these patients you must think about coronary disease because of the hypertension. He's got normal renal function and no neurological symptoms past medical history otherwise, but from the paraplegia is pretty unremarkable. Is a non smoker now. Alcohol eso importantly, his examination is comfortable arrest, wheelchair bound saturation, the normal. But what's the most important thing here is to check the blood pressure and all fall in. So his blood pressure on his right arm is 1 40/80. His left arm is 1 40/85 so that makes that subclavian flap unlikely. And then in his right foot, he's got a 20 millimeter radio from on two legs. As I'm sure you're all aware, the blood person foot should be higher than the arm. So if you've got a 20 millimeter great and from arm to leg, that suggests a significant room co optation. He's got a left poster authority. Khatemi Scar. He's got a one on six systolic ejection murmur consistent with his bicuspid aortic valve, and he's got reduced local impulses. He's on em load up in and low, certain his echocardiogram demonstrates normally, size and systolic function has got moderate ableto incompetence and mean grading through his co active 20 millimeters of mercury. So it's a ME ingredient that were most interested in because this correlates well with a catheter based measurements. So this is the memory on DSO. This gentleman actually was very, very anxious because of his previous complication of his catheter procedure that he didn't wanna have any contrast. Actually, when he came to see him in the office, his blood pressure is more like 1 80 because he was so incredibly anxious because he thought it was about to go for a cat scan. So we reassured him actually, that he could get a memory without getting any contrast. Um, and this is a a study that could be done with very good resolution. And without any contrast, you could get a beautiful images of ascending ableto arch and descending aorta as Aziz for us can testify. So this is the area of the recall lactation, and it's right here. Just this doll to his left subclavian. Importantly is where you can see the ascending aorta is a little bit dilated about 3.5 centimeters, and he's arches pretty small. So this is really common in these patients. Still have a hyper plastic aorta kharj. They generally now trying deal with this at the time of surgery by sort of fill laying open the arch when they're doing repair. But this, gentlemen, looks like they basically just cut the cut the stenosis out pulled to pull the two ends back together because he's still got pretty significant now ing through his through his arch. And this can also contribute to hypertension because at about 1.5 centimeters, that's pretty small. He's co optation is about one centimeter in diameter. These descending order is about 2.5 centimeters, which is it's pretty normal. Some of these patients will get aneurysm multiplication, the descending aorta and can get can get to section there. So he's got a hyper plastic arts and a significant recall lactation. Um, this is just a scene I image through the area and you can see the jet off become up through this area. You can see Jet of Stone Isis here. That suggests he's got a significant recall Lactation. Okay, and then I actually just through this one and this. Well, this is Ah, this is not him. But you can see Ah, this is what a subclavian turned down will look like. And basically, it looks like a big aneurysm in the area. where the co optation repair was, and he's missing. And this patient is an old passion of mine. Andre missing the left subclavian artery. So big aneurysm and then a re narrowing, and then a normal descending order in this particular patient. This is what a subclavian flat turned down looks like. So each one of these repairs looks slightly different on imaging. And obviously we prefer armory in these patients because most of them young and repeated CT scans would involve a significant amount of radiation exposure. So when Dio intervene on a native or recurrent recall rotation, so obviously, um, when there's a native severe computation, it's it's pretty obvious. But the recurrent or recall lactation is the thing that we most typically seeing these repaired adults. So if there's an opera to lower extremity resting peak to peak right in Brighton 20 millimeters of mercury or remain dull places don't look right in that kind of greater than 20. Or if there's a greater and greater than 10 plus, they've got decreased Elvis historic function, significant ai or evidence of collaterals. So it's actually not that much of a great it needs to be had in order to think about re intervening in these patients, and the part of the reason is obviously there should be coupled with either a, C T and memory that demonstrates re co optation. If you haven't got a narrowing, then you need to find an alternative explanation for the Grady in. But when there's a re narrowing any other 20 millimeter Grady in or it's millimeter guardian, plus one of these other complications, you should think about fixing it. So the best evidence to proceed includes patients that that have got high blood pressure. They've got upload, extremity, blood pressure, Grady in echo, evidence of ingredient and an atomic evidence, which this gentleman all has, and then multiple factors. Thio help you decide whether or not surgery or stenting is optimal. But the patients were Rico. It was re co optation. Because I've had childhood surgery. Standing is performed in greater than 95% of situations because usually it's actually very tricky to get back in there and cooperate because there's a lot of collaterals and the surgery could be complicated by bleeding and neurologic complications, and standing is becoming the absolute preferred way. Thio fix Ricart ation. So this gentleman because of the proximity of the recall lactation to his left subclavian is actually, you know, with the vascular gonna want to go a left subclavian, too corroded bypass operation. And following that, he will undergo a stent on. This is just showing you how this is done. Basically a pigtail catheter. Take some nice pictures, figure out where the co optation is, and then place, um, stand across the republication. Um, it, uh, more frequently these days is done with either a covered stent or some sort of end a graft, mostly because of the feared complications of this procedure, which, albeit are uncommon, are devastating when they occur and could be very difficult. Thio recover from eso. Our biggest fear used to be that these patients would dissect during the procedure or, in particular rupture during the procedure. Um, it's actually pretty uncommon, but when it because it's it's a very bad situation, and so often now primarily a covered stent is used rather than an open cell stent, aunt. And as I said occasionally, sometimes. And I think Dr Zito also does this with an end of graft, that's obviously an extremely good way of doing this is. Well, um, these obviously the branch is gonna be included at the time of doing a covered stand. And so thinking about revascularization pride of that is an important thing to think about. And obviously insufficient expansion with residual qualification of the procedure used to be more of a concern when using open cells sense because off sort of using very high pressures, obviously was a risk active for rupture. And so people were a little less aggressive than they are today in terms of re expansion. But now, with covered stance, uh, peskin usually be restored to a normal caliber, a order. And so there shouldn't be any significant residual politician after the stand procedures Done. As I said, surgery is not a great option generally for these patients. Mhm. So when I see a computation patient, I am sure that I always measure both up. Elim pressures and also Lowell in Precious and see if there's a significant radiant. All of these passions should get echocardiogram to assess this. They've got a bicuspid aortic valve VSD and other associate issues such as a dilated aorta. I prefer a memory of the chest and memory, a memory or C T. If they're unsuitable for memory again, mostly for the radiation exposure we now suggest to a or two B recommendation. All of these patients that have a memory of the brain to screen for berry aneurysm, particularly the older. And if they're hypertensive, um, consider exercise testing to see the blood pressure response and obviously aggressive management of the blood pressure lipids. Another risk factors because coronary disease and cerebrovascular disease is the biggest complications. Um, s Oh, this is obviously just from the guidelines. And, you know, that was basically echoes what I said that that I do, um, that recall rotation occurs. Recall rotation occurs in about 10 to 15% of these patients. Some could get aneurysm pseudo aneurysm at the repair site. Upper body. Systemic hypertension is very common in these patients. Um, more than a third of them will have hypertension even in the presence of even in the absence of recall lactation. But if you see high blood pressure and muscle aggressively aggressively for recordation with multiple imaging modalities bond, I suggest about 10% of patients can have evidence of berry aneurysm. Um, actually, that has not been my experience I've done lots of lots of Emory brains in these patients, and I haven't seen that. But this is, um you know what other centers have seen on da Obviously hypertension, hypertension, hypertension. So in the next case, um, this is a patient of mine from New York. But it's an interesting case because it tells us a little bit about long term complications in adult congenital heart disease. So, Mr J. T. And it's not who you think it is. Um, he, uh, no one of the cardiologists at Austin High? Thankfully, um, So I first met him hey, presented with fevers and said that he had some some sort of heart surgery in childhood. Um, and he'd had ongoing swinging fevers for several months now. Otherwise he was well, but he would get fevers, feel awful for a while, and then he would get so many products and feel better, and then the fevers would come back. And he had a history of trend d t g a d s d p s and had a Rastelli operation in a redo. Avp a conduit at age five years. So this is his echocardiogram. We're looking at his right ventricle here and you can see a little bit of track customer to vegetation. And then we measure the Grady int how high his obvious P is. That's at least 79 millions of mercury, plus his alright pressure. Now, if you get this sort of pressure, this is obviously extremely abnormal, and these patients again should not have pulmonary hypertension. Eso If you've got this sort of grating, it suggests that they probably got significant, um, obstruction across the AVP conduit. And this is his short access of his R V l V. And you can see the, um, systolic and diastolic flattening of the septum here suggesting very high Avi diastolic and systolic pressures. And this is the rvp a conduit here and you can see it appears thickened, although the leaflets are moving and there is turbulence here at the level of the valve, some mild palm regurgitation and then a peak radiant through the valve of 70 millimeters of mercury and the main grading of 42. And this is obviously consistent with severe obstruction. So in this patient, now he's got a fever and severe obstruction across his palm revolve. It always makes you think that this is potentially endocarditis. And in my experience, palmeri valve into Carla's frequently presents with severe obstruction, often because they certainly can have Ebola. And you can see a plane ct chesty. You can see a couple little M bolic lesions in particular this kind of little necrotic one here in the left lower lobe. And we also got a gated C T. And this is through the outflow tract here. You can see that he's got thickening of the primary valve, and this was called Panis by the unsuspecting radiology. But this is actually all infected material. And so, see, if you could be very helpful in these patients gated cutbacks, it could be very form these patients toe look for vegetation because M R sometimes suffers a bit because of the fact that, um, there'll be a lot of artifact. You the valve S O. C t could be very helpful to see the vegetation and support the diagnosis of Nicaragua's. So he was interesting. He had inflammatory markers that were, um, not especially elevated. Ah, and his globulin, though, were extremely high. His, uh, blood cultures were all negative. A fees or negative HIV was negative But Melo was negative, Mr Plasma and luckily actually turned out. His blood was sent away for testing. Andi. I did look for PCR for Q fever and eventually with potatoes and had extremely elevated Q fever. Tito's. And so it turned out he'd be not in Afghanistan a couple of years earlier and and had a meal where that would be exposed toe goat. And so we think that he probably got it from Q. Fever obviously comes from you know butchers will get at abattoir. Workers in particular. Australia will get it. But it's, uh, it's a very low grade organism that sort of delivery early, and that's why he developed such severe obstruction. Eso Palmer involvement of Qatada's uh in the General obviously got to think about any keratitis and all these adults in general patients. Um, we think there's an increasing incidents of this, and certainly when I first started, this is not something that we've seen a lot off, but certainly now that we're doing seminary, so many primary valve replacements, both surgical and trans catheter. I think the fact that these valves are on the right side of the heart means that they're much more likely to be exposed to skiing organisms and or organisms without being filtered out through the lungs. And so, um, if you do see someone with always think about whether on the primary valve could be infected, even though, you know, I think 10 years ago the incidents of um if you ask any any of my colleagues the incidents of formative Alvin economics, we would have told you was vanishingly rare. We now see cases of this, like two or three times per year, and certainly it also became We became aware of this after the melody valve. Um, it came onto the market that with obviously very aggressive screening and follow up with these patients patients, it turned out that significant number of them were getting in the greatest. Um, there was a bit of a feeling from the surgeons. Maybe there's something wrong with the melody valve, but I think that it's turned out that just any primary valve by prosthesis can get infected and certainly see the rates seem to be equivalent in surgery and surgical Palmer valves and trans Catholic former involves wide arrange of organisms. Obviously, they frequently present with severe stenosis, anabolic phenomenon and obviously frequently need for surgical intervention. You don't wanna put a trans catheter valve inside and infected Palmeri valve. Um, this is just one paper from one group just showing the incidents of former involvement in Caracas of the 30 years follow up after surgical former valve replacement. And it's not high, but we're talking sort of. 10 to 15% of patients ended up within a Karada, so it's not that infrequent. Um, this, uh, this gentleman, um, underwent surgery. And I think the most revealing a bit about this was the fact that there's a flag monster comfortable there so comfortably struggling. It's confidentially around the most disciplined aspect of the country. But in the area of the implanted former valve and this was cultured, you had meetings on Latino apathy on the whole thing had to be removed, and it was extremely infected. And then you go to Paul Marie. I'm a graft in inside where the old the Hancock valve has been taken out. And so this was the appearance afterwards, and this is obviously a normal appearance of a surgical pulmonary valve replacement. S o r v p I. Can you It's obviously very common. Um, in this, you know, adult in general population. Um, importantly. I mean, I was guilty of this in the past as well as I kind of would tell these patients that didn't need to be on any sort of any coagulation. But now, again, since the advent of the trans catheter valves, everyone will get aspirin. And I now make sure I put all my patients on aspirin. I certainly feel personally that this decreases the incidents of, um, thrombosis on the Dalles. Obviously, very much like the aortic position. I think we're all aware of the issues with the erotica by prosthetic thrombosis, and I think the same probably occurs in the palm revolves Aled. These patients should at least be on aspirin. And I guess the other thing while we're on the subject of right sided valves is to think about when someone does a bio prosthetic bicuspid valve. I always now think about giving patients with bio prosthetic bicuspid valves 3 to 6 months of Coumadin, if not longer. Because the incidents of al from both this is extremely high and bio prosthetic. Try custom valves. I'm at least in the short term and long term, it's It's low, Andi, obviously we don't use mechanical valves on in either of these positions. Generate all these patients to get prophylactic anybody with dental procedures. And then I guess the other slide that I wanted to bring up here was thinking about endocarditis. Prophylaxis guidelines in the dark in general heart disease and it's a little bit different to everybody else. So in those with previous endocarditis should get anybody profile actress with dental work those with prosthetic valves. Patients within the six months of placement of prosthetic materials, such as even like an ASD closure device. Patients with residual inter Kati action. So usually it's like a VSD that's got a residual defect adjacent to the bio prosthetic material like the PSD patch and all those patients with uncorrected cyanotic heart disease. And I pretty much do it and all the cyanotic patients in particular. A lot of the Fontane patients. I still give antibiotic prophylaxis because they're high risk for inter components. Eso just one more case to talk about here. Um, this is a 32 year old married teacher who had a history of track hospital trees. He underwent Bt shot at age six months. Bi directional Glenn operation of three years and the lateral tunnel Fontaine H five years had a epithelial permanent pacemaker for Sinus node dysfunction, presented with one atrial flutter with one conduction and sink to pee and underwent successful radio frequency ablation, um is treated with warfarin and then transitions is there Alta which we use in a lot of these patients now because of compliance issues and now presents to establish care with recent onset of domino distension and fatigue. So I thought we just briefly touch on the different sorts of Fontana operations that have done for patients with single ventricle. So this is the classic Fontaine, where the right atrium of the right actual pendant attached directly to the primary artery. And, uh, this is the bidirectional glenn that we talked about with The SBC is attached to the right primary artery. Um, there was a classic gland with the SBC. Just went to the A p A. We see that less commonly, it's kind of very old procedure. The bidirectional Glenn. That's what everyone gets these days. Uh, the lateral tunnel. Fontane. You'll see a lot of these now in their twenties and thirties and is where a tube is attached from the inferior vena Cavor through the right atrium, up to the base, the proportion of the right primary artery. And again they get a bidirectional Glenn and the most common proceeded these days and external conduit Fontane. The reason for the for the transition from here to here is basically the thought that there's my decrease arrhythmia risk and that depend out to be to be true because of the fact that there's less operation and therefore less scar tissue on the right atrium. And these patients are much less likely to have actually really means, though they're not completely through them, and most of them are now left with what's called defenestration. So there'll be a little hole either made in the lateral tunnel or a little hole made in the conduit to allow some blue blood Thio not go through the lungs and go directly to the left side that it comes at the cost of, um, low officers and situations. But it probably decreases your, um mean Fontane pressure, which probably helps preserve you live on. So just the physiology, Um, this is, uh, this is what we look like. So if you've got a right ventricle, it's gonna be pumping from a low feeling. Pressure increasing the pressure, the P A pressure, which will go through the lungs and down into the left ventricle in these Fontane patients that don't have a right ventricle. So you need to reach that mean p a pressure through basically sheer congestion on the right side. And this is basically this is probably the precursor of a lot of the complications in these patients because they're Fontane, pressures will frequently be 15 or above, and certainly a lot of the adults you will see when you catch them. It could get up to 20 even 25 that that pressure is constantly what the liver is exposed to, leading to a lot of complications. They also have pre load failure because you know these low pressures they they can't particularly augment with exercising up to 20 or 30 or 40 with exercise, they tend to under fill the left side of the heart. So it's the other thing that's important to think about is these patients very infrequently get primary demons. So if they ever present with lung infiltrates, Paul Marie gamers on common. Thats is his SCG showing an actual paced rhythm. This is his echocardiogram, and you can see he's got a single ventricle here on this round structure in the back of the IRA. Here is the lateral tunnel and he's got try customer the trees. He hears an athletic try custard valve, but a normal, uh, normal mantra valve. So labs were Hey, we go over. It was a little elevated because of cyanosis. Platelet count was a little bit low, electrolytes were normal. Alec Foster was significantly elevated, and unfortunately, he's a f p with severely elevated eso. He underwent a C T scan of his liver, and this is one of the dreaded complications after the Fontaine operation. This is hetero cellular carcinoma, and we first started I personally first seeing this five or six years ago. And certainly the incidence of HCC is we've now realize is actually pretty significant in these patients. And obviously this is this is the worst outcome when you've got evidence of societies in a very large mass with thrombosis of the have a hepatic sort of the portal vascular show. Um, So how about cellular carcinoma appears to be associated with what's called Fontaine associated liver disease, F A L D. And if a Fontana socially liver disease is most likely due to what we just talked about in terms of the physiology, um, with congestion on the Venus side backing up, which damages theme, the basically the had a Karan coma and also the lack of forward flow. As we said, uh, these patients have reduced cardiac output due to pre load failure, and often they have systolic and diastolic function of the ventricle, so both lack of inflow. But I think most of us now think it's probably the outflow problem. That's the issue, Um, and this can lead to cirrhosis. And like any form of cirrhosis, we then see regenerative Nagy, ALS and subsequently thes. Patients could develop Habito cellular carcinoma, and this tends to occur very late. So more than 15 years after the Fontaine operation, you'll start seeing societies a self video various is. Some will eventually develop the paddock capital apathy, um, increased on our low platelets, low albumin level being. You can also see in something called protein living in our apathy and fibrosis on biopsy, and suddenly we now are extremely aware of this issue, and we pay close attention to these patients because, um, 30 years out from the Fontana operation, um, you know, at least half of these patients have evidence of advanced liver fibrosis, So this is an array of ultrasound images across the top and Emory images across the bottom. And this is going from five years, 15 years and then 25 years after the fontanel pressure. Sorry, the ultra and images are great, but that's often what you get with ultrasound. But five years after the fontanel oppression, most times the hepatic prank, my looks pretty normal. Maybe 10, 15 years out, you start seeing some coarsening and then more than 15 to 20 years out, you'll start seeing irregular profile of the liver consistent with cirrhosis. And this is the Emory appearance. All of these patients will now get armories as well as part of the screening process for HTC, and you'll see some modeling early on. And then, you know, essentially what looks like a not make liver ast time goes on with increasing um, ridiculous patent enhancement off to Catelyn IAM, and then evidence of irregularity of the Contour and these dark areas here, which hyper confused areas which could be, you know, it could be consistent with regenerative modules and sometimes happen. Oh, cellular carcinoma. And so there does seem to be this progression over time which, like, you know, most other diseases that cause cirrhosis. We can see the development of both synthetic dysfunction, portal, hypertension, and eventually, Rapido Cellular, Casa Nemo. So this this presence of this Fontana such a little disease now occupies Ah, lot of our thoughts after after the Fontana Fontana operation. Um And so, you know, post Fontane. What do I do when I see one of these patients? Um, I get comprehensive cardiac imaging with echo and M r i N C T. And in fact, our guidelines suggest that you know, Emery, every year is even reasonable, particularly if you can't get decent echo images. Um, these patients would frequently be followed with clinical examination and labs. Uh, you know, it's still a little bit variable what people do, but certainly ultrasound Remeron deliver every year. At least, um, I tend to measure alphabet of protein. Actually, some of the hepatology ists don't measure it because it can lead to false positive I get. Yeah, I get iron studies because a lot of these patients are relatively anemic. So routine liver imaging with ultrasound Marai and a lot of these patients, particularly 15 years out, we'll see a hepatology ist stress. Testing is important in these patients to get an idea of exercise capacity, consider cardiac catheterization prophylactically. Um and, um so and, uh, you know, attention to their Fontane pressures in particular. Um, so then the million dollar question. What do you do about an elevated Fontane pressure? So we certainly look for any dysfunction of the ventricle. We look for any obstruction of the Fontane pathway. So even if there is evidence of an geographic obstruction but not pressure pressure grading aggressive debilitation of P a stenosis or fixing obstructed Fontane circuits, we now think that primitive isolators are reasonable in patients who have primary vascular disease. We want these patients to stay skinny because certainly the more obese patients definitely have are worse outcomes. We definitely want their blood pressure to be well controlled because a big component of the Fontane pressure is going to be the diastolic function of the ventricle. Early aggressive arrhythmia, monitoring and management so thinking about a zio patch or Holter monitoring these patients every year or two. And if they do get actual arrhythmias, early management with with E. P. And thinking about ablation because they tend to really tolerate the actual arrhythmias very poorly ventricular arrhythmias. They're actually pretty uncommon in these patients. Um, more common scenario that we have is actually actual tachycardia with 1 to 1. And if someone presents with sink api and uh, either a narrow brawl, complex arrhythmia. I always think about a flutter with one in these patients, and that could definitely toe human Amick instability. Obviously, with a single ventricle, our heart rate of 200 it's very difficult to maintain it ease and cardiac output. Obviously, you've got to remember that risk of throwing vandalism again. In the last five years, we've certainly in our population. We started to increase in these incidents upon re embolism and stroke, and so prophylactic any coagulation if they have atrial arrhythmias, if they have evidence of residual right to left, shunning if they have a classic Fontane, so that old stall r a p a Fontane, you must definitely think about them being any coagulated with either Coumadin or no AC and transplant eligibility is frequently on, you know, forefront of our minds in particular when these patients develop, uh, cirrhosis or HTC. It does make the prospective transplant that much more difficult on. At that point, people have to think about hot liver transplant and the outcomes of heart liver transplant on particularly great, obviously depends on the Senate, but an overall average mortality around about 50% is what we tend to quote people. Some centers have had more success than that. Some centers had worse outcomes in that. But certainly the risks are higher, so definitely translating before they develop cirrhosis and before they developed HTC is important. Um and, uh, just wanna say thank you, everyone for welcoming to Austin hot and just introduce you to my family. This is my wife and my son Nathan. When Jorge Juliana and this is a new addition to the family says Okay, he was born two weeks ago. He's been eating now, so he's not gonna make it. But he may make a near parents, but my daughter talks about him every day, so he's still part of the family. From my perspective, I think we got 567 minutes. If anyone has any questions, I've got a few slides. If no one's gonna questions, I've got a few slides on hypertrophic cardiomyopathy as well. But let me know anyone got any questions? I just throw I throw this in? Um, just a question. Of course. On that guy with the distal pulmonary artery narrowing notices. Did they get pulmonary oedema once you open that stenosis. So, uh, most commonly, I've seen former oedema in severe P I with the transcript of pulmonary valve replacement. You definitely can see primary Deemer if you've had under perfusion lung aan den, you open up the p A. I've seen that occasionally, but the former oedema is more common. It's actually a really important question after you do trend. We noticed this after trans catheter upon the valve replacement. So because we've got severe p I. So I've got a huge amount of Avi, you know, additional volume, and then you get rid of the P I. And all of a sudden you get all this blood flow into the lungs and they often go in a primary DeMARE after trans gathered upon revolve replacement. But definitely. Any time you have a PST Moses and you open it up. Unilateral pulmonary oedema is a possibility. Obviously, they already had no floated that long. It functionally doesn't tend to be that much of an issue that they'll pay it off pretty quickly on Ben re profusion. Former oedema. Um, generally, unless it's extreme, you know, And they're in the I c u and its bilateral gets pretty. Gets better Pretty quickly. Jonathan. This Marc Cohn another question. Just You said that in the first case with the pulmonary artery stenosis balloon expandable stents, where the way to go, what's the I mean, why wouldn't it be a self expanding stent? Lower pressure system? Well, maybe Frank once the way in here, but it's generally because you can, uh, um, you know, just get a reliable results. I mean, you know, one of the interventions might wanna weigh in, but basically you'll expanded to the dimension that you want. That's predictable. Um, and, um, you know, that's my understanding. I mean, that's kind of what we you know what we use? Sure. No problem. I mean, I mean, you know, certain centers use more balloon expandable on a lot of different things and, um, self expanding. But just a question. Yeah, I have another question. Yeah, go ahead. Some of these patients that had, you know, prior SD repairs and stuff like that and they have RV debilitation and you never know what their function was like. You know, 10 years ago, and then you kind of got a mildly dilated RV with, you know, mildly depressed CFC. How far do you follow those by m r? I mean, do you get one every year? You know, eso You've always gotta be careful if someone's got residual of debilitation just to make sure that don't have a residual defect, particularly if it's like a sci fi Nanosys or current Sinus SD. But if they have definitely no residual defect and no residual left to right shunt, I tend to follow them mostly. With that, I'll do an m. R. I. You know, maybe every 34 years or so, but my experiences in the absence of pawning hypertension, most of them will have, like, a little bit of ivy debilitation, and that'll kind of stay like that forever. Once you get rid of the shunt that RV volume that they're left with tend to like It will shrink a bit in the first 6 to 12 months after the surgery or a device closure, and then it kind of stays the same. So it's very frequent that you see these patients five years out, 10 years out, they've got mild of debilitation. We usually go looking to make sure they don't have residual defect, particularly if they had some sort of surgical closure. Because you've all seen, you may not have all seen, but, you know, we see patients that had surgery 2030 years ago and they come back and never even close the eyes d or that close something else. So if you see residual of debilitation, always go looking to see if there's a residual defect, particularly if it was a surgical closure. Because the number of cases that I've seen, where that surgeons what they called the D, they did something else entirely different is, you know, it's not super common, but it's common enough that we have to then go back and repeat surgery or some sort of repeat trans catheter procedure. But generally, once they go back toe once they kind of find their new set point. If there's no residual defect, then they'll kind of stay the same almost forever. So I tend to tell. I reassure those patients that nothing bad is gonna happen. The only thing that you sometimes say long, long way out from that is actually arrhythmias. Andi. That's whether it's because of the surgery or because of some residual model RV dysfunction. But they don't tend to, and they won't get late forming hypertension that doesn't tend to occur. And I've never seen anyone with, like late Avi failure. So I definitely reassure these patients memory. I once in a while is reasonable, but you don't have to go. Don't have to go crazy because they do. They generally do well if they don't have for my hypertension. Well, thank you very much, everyone for tuning in. There was a lot of fun, and I think obviously we only covered a few lesions here, so I'm ready to do part two Part three part four whenever you are. So just let me know because it's a very big topic and obviously there's a lot of new information last five or 10 years, So obviously, if we want to cover federal agent, just let me know. Thank you very much. Yeah,
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