 Hi everyone, I'm Nelson Burbano and I split my clinical time between pediatric cardiac and adult cardiac anesthesiology at the Cleveland Clinic. Thank you very much to the organizers for the invitation to give this lecture. The title of the presentation is Segmental Approach for intraop adult congenital heart disease assessment. These are the goals for the presentation. We are going to start with this clinical case that I'll use as the base of the entire presentation. 40 year old patient with a very complex cardiac anatomy and disease that includes cytosine versus totalis, dextrocardia of course, congenitally corrected transposition of the great arteries with severe biventricular dysfunction, severe systemic AV valve regurgitation and other medical issues that you can see in the description. So this patient comes to the OR for an impella placement in that systemic right ventricle that is failing. I don't know about you but I find difficult to get a clear understanding of the cardiac anatomy of this patient based on the prior description. Then you start your T examination and this is probably one of the first images that you are going to get and anatomy looks very, very abnormal. Then you advance the multiplanes some more and get another sort of four chamber view but it's still very hard to differentiate the hard chambers. What is what is right atrium, what is left ventricle is really hard to know. Then you go deep into the stomach and get this view and then you realize that to make things even more difficult, the liver which is this one is in the left side of the patient because of the cytosine versus totalis. So the T examination for this patient with this anatomy is going to be very challenging. That's why I truly believe that the segmental anatomy approach developed by the vamprax that you see in this picture is extremely useful. It is a simple straightforward system that helped us understanding the cardiac anatomy of even the most complex cardiac defects. They describe the heart based on cytosine and you are going to hear this war a lot during the presentation. So this cytosine has two main components, location or a spatial relationship of the heart chambers to each other and their fundamental chiral characteristics. Chiral team implies that each chamber has a unique right or left nature based on its morphology regardless of where that chamber is located. The vamprax describe the heart as a series of building blocks with three main cardiac segments, the atria, the ventricles and the great arteries and two connected segments, the atrioventricular canal or AV junction and the infindivulum or conus arteriosus. Due to time constraints we will concentrate only in the three main cardiac segments. The connecting segments will be a whole different lecture. So they use braces with three letters inside to describe the visceral atrial cytos as the first letter, the ventricular looping, the second letter and the great artery cytos for the third letter. In short, atria, ventricles and great arteries are the three main cardiac segments. So we are going to use the usual anatomic orientation from the mirrors of a dual first chamber view to create this drawing. It represents the segmental anatomy system with my own adaptation to match the anatomic orientation of the heart when we see it from the fourth chamber view during the T examination. Of course, in a patient with livocardia, the heart will look like this located more in the left side of the chest and in a patient with dextrocardia will look more like this located more in the right side of the chest. However, for simplicity reasons, we will stick to the straight neutral position that does not describe the cardiac position within the chest. It does not. So now let's go over each one of the letters included in the segmental anatomy system. We'll just start with the visceral atrial cytos and we have three options here, number one here on the left, cytosolitus, which is represented with the letter S. It means that all tidally right structures are on the right side of the patient and all tidally left structures are in the left side of the patient. That includes the abdominal and thoracic organs. Second option, cytosinversus is represented with the letter I. It is a mirror image of cytosolitus. That means that all tidally right structures are on the left side of the patient and all tidally left structures are in the right side of the patient. Third option, cytosambiguous is represented with the letter A. Ambiguous means that it is hard for us to know what we are looking at. We are not sure. It can be at the atrial level that both atria look like right or left atria. This slide shows the three different types of visceral atrial cytosolitus, inversus, and ambiguous. Ambiguous is anything that is different from solitus or inversus, meaning some tidally right structures are on the left and some tidally left structures are on the right. There is a mixing of sidedness or abnormal lateralization. This is also called heterotaxis syndromes. Van Praak uses the terms asplinia to differentiate them and others use the terms of left or right isomerism. Now let's go over how to use the TE examination to figure out the visceral atrial cytosolitus. For the abdominal cytosolitus, we can use the liver. The video on the left shows the hepatic veins of a patient with a liver that is located on the right side of the patient. We can verify that very easily by just turning the probe to the right from one of the transgastric views. The video on the right from a transgastric at the base of the RV shows that the liver is located on the right side of the patient and is very closely related to that anatomically right ventricle. To determine the atrial cytosolitus, I rely heavily on two things, the atrial septum and the morphology of the atrial appendage. Let's start then with the right atrial. The image on the left shows the atrial the septum secundum from a by cable view. Remember that the septum secundum or limbus of the fossil valleys is a right sided structure and biologically it belongs to the right atrial. The clip on the right shows the image on the right that the right atrial appendage on the right side should look more triangular with a very broad base, a big opening at the base of that right atrium that is very different from the one on the left. Now let's move to the left atrium. The image on the left shows that the septum, again the septum, the atrial septum again from the by cable view. The septum primon embryologically belongs to the left atrium and it is a left sided structure. Now the image on the right shows that the normal morphology of the left atrial appendage should be elongated finger-like with a very narrow base that's very different from the from the one on the right. Now let's go back to our clinical case and apply the previous concepts to this patient. The transgastric view of the base of the right ventricle on the left shows that there is almost no no liver. Here this is the morphological right ventricle. You see a tri-leaflet valve and there is very little liver. We should be in this image see a lot of liver here close to that right ventricle. Now look at the deep transgastric view. The liver is on the right side of the display which is the left side of the patient or that means that most likely the abdominal siteus is inverses. This is the original clip that I showed earlier a four chamber view at zero degrees. We see a very large atrium here and another smaller one there on the right side of the of the display. Let's concentrate on this part on the atrial septum to see if we are able to differentiate both atria. The location of the septum primon here on this image suggests that this large cavity is the left atrium and the location of the secondum suggests that this is the right atrium. The morphologic left atrium is located on the right side of the patient so the atrial siteus seems to be inverses. Next let's look at the atrial appendages. The bicable view on the left shows the svc draining into this more anterior atrium and the appendage looks more like a right atrial appendage. In this view on the view on the right the the large atrium this one is now located on the opposite side of the display because we went from zero degrees to 130 degrees. The appendage of that very large atrium resembles the finger like more the finger like morphology of the left atrial appendage. So to conclude this part we can say that based on the location of the liver the characteristics of the atrial septum and the morphology of the atrial appendages the visceral atrial siteus is inverses. Second letter ventricular looping. I'm going to run this very short video to explain the process of ventricular looping. This is the primitive cardiac tube where the blood flows from the venous channels located at the bottom of the image all the way up to the arterial trunks located on top. So as the cardiac tube grows rapidly it loops to one side or the other as it accommodates that extra cardiac mass. So most of the time this primitive cardiac tube loops to the right side. This is right, this is left as it is shown in the video. So at the end of this whole process the morphologically the morphologic right ventricle is located on the right side of the morphologic left ventricle. When the cardiac tube loops in the opposite direction towards the left the opposite happens this right ventricle is going to be now located on the left side of the patient. We have some for the ventricular looping we have two options here. When it loops to the right we use the letter D to represent D looping and you can see here the location of the two ventricles right ventricle on the right side of the patient. When it loops to the left we use the letter L to represent L looping and it will look like this. Now the morphologic right ventricle is located on the left side of the patient. The two ventricles are switched now. After we understand the ventricular looping and that the ventricles can be switched in location we need to be able to differentiate them morphologically. We can use all these anatomic clues to identify a morphologic right ventricle and these ones on the right for a morphologic left ventricle. However, one of the easiest features to remember is what I'm showing here with the arrows. The lower or more apical implantation of this valve the tricaspin valve compared with the mitral valve. Remember that the general rule is that the AV valve tricaspin or mitral they follow the ventricle wherever the ventricle goes. So this lower valve must be the tricaspin valve and this one should be the right ventricle. Again, let's go back to our case and apply this concept. The clip on the left shows that this AV valve sits lower more towards the apex of the heart than the other. So this one must be the tricaspin valve and this is the mitral. Additionally, there seems to be some cordial attachments to the septum. Maybe you can see a little bit of the moderator band here and this most likely is the anterior popularity muscle of the tricaspin valve. All those are features of a morphologic right ventricle, which is located on the right side of the patient due to the looping. The clip on the right shows that the jet of regurgitation corresponds to tricaspin regurgitation because this is the right ventricle. Very good. Moving to the third letter, the great artery siteus. There are four options here on this session. First, solitus or normal. Again, represented with the letter S, but the following three criteria need to be present for us to be able to say that the siteus of the great artery C solitus. First, the aorta should be located posterior and rightward to the pulmonary valve. Second, there should be normal ventricular arterial alignment. That means that the right ventricle connects to the pulmonary valve and the left ventricle connects to the aorta. Number three, there should be a sub pulmonary infundivalum. Remember, the infundivalum is a structure that belongs to the right ventricle. Typically, the normal adult heart does not have a left infundivalum. Now, the second option, inversus, still normal, but inversus, is represented with the letter I. It is just a mirror image of solitus. In this case, the aorta is posterior and left ward to the pulmonary valve. The right ventricle still connects to the pulmonary valve. The LV connects to the aorta and there is a sub pulmonary infundivalum. Third letter, a little bit more complex, malposition to the left represented with the letter L to the right with the letter D or anterior with the letter A. In this case, the location of the great artery is off, but there is still ventricle arterial alignment. It's normal. The LV connects to the aorta and the RV connects to the pulmonary artery, but we don't have this normal relationship anymore. Fourth option, transposition, meaning that there is discordant ventricle arterial alignment and the letter D or L describe the great arterial cytos we are going to go into more detail later on. In this case, the right ventricle connects to the wrong vessel to the aorta and the left ventricle connects to the pulmonary artery. The vessels are transposed. We can use these two views in our normal exam, the RV inflow flow and the long axis to assess the great artery cytos using TE. The RV inflow flow on the left shows that the aorta is posterior to the pulmonary valve that is here, the pulmonary artery also shows that the right ventricle connects to the pulmonary artery that there is a sub pulmonary infundivalum. That's why there is no fibrous continuity between this AV valve, which is the tricasp valve, and the pulmonary valve because of the presence of a sub pulmonary infundivalum. Now, the long axis on the right shows that the LV connects with the aorta. So there is ventricle arterial alignment, it's normal. So when the segmental anatomy is normal or not inverted, we say that it is SDS, visceral atrial cytosolitus, D loop ventricles, and great artery cytosolitus. So if we go back to our original drawing that we used to represent that segmental anatomy, it will look like this one on the left, SDS non-inverted. Right atrium, right ventricle infundivalum or on the right side of the patient, left atrium, left ventricle on the left side of the patient with normal ventricle arterial alignment, SDS non-inverted. Of course, if there is non-inverted, there must be inverted. ILI is also considered normal. It's just a mirror image of SDS, typically seen in people with cytosine versus totalis and many of them, they don't have any other, they don't have any cardiac congenital defects. This is, I'm, this is sorry for the corky joke, but this helps illustrate the point. So Samuel L. Jackson on the left and Samuel D. Jackson on the right, still normal, right? SDS ILI, both are normal. Okay, now back to the clinical case to determine the great artery cytos, let's see how we can do that. So the clip plane on the left will be close to a RV and flow out flow where you can see that this large left atrium drains into the morphologically right ventricle, which connects to the aorta. And we know that it is the aorta because we can follow it distally. And we see that it does not be bifurcates as the pulmonary artery does. Also, if you get a short axis of this valve, you will see most likely the origin of the coronary artery is most likely the left main coronary artery, because the right is a little bit more difficult to see. We can see that there is no fibrous continuity between this valve, which is the tricuspid valve, and this which is the aortic valve, because there is a sub aortic infundibulum, which is a very, very characteristic feature in transposition of the great arteries, the presence of a sub aortic infundibulum. Now the second clip on the right shows that we can use different aids to help us navigate and understand anatomy. In this case, there is a wire for the impella that is coming from the aorta crosses the aorta valve and goes into that systemic RV. So based on these findings, we can say that the great artery siteus is transposition of the great arteries. Now let's let's try to get more data about the relationship of the great arteries to each other. The image on the left is an X plane of the previous view that we just saw. The primary image, which is this one, was obtained at about 70 degrees and shows the wire of the impella crossing the aortic valve into the systemic RV. This secondary image on the right crosses the 180 degree plane line. So that's why it is inverted. So this is the right side of the patient, and this is the left side of the patient. And then you see in this image that the aortic valve, you see the wire of the impella here, is anterior and rightward from this pulmonary valve. So we can use here the letter D to represent that. The clip on the right shows basically the same. Now we have the impella crossing the pulled the aortic valve into that systemic RV. And then you see the same, the same location of the semi lunar valve, aorta anterior and to the right of that pulmonary valve. So this is the schematic representation of the segmental anatomy for our patient. TGA, ID, the transposition of the arteries, the cytosine versus the cytosine, the visceral atrial cytosine is inverses. That's why the morphologic left atrium is to the right. D, because the ventricles are delupt, that's why the morphologic right ventricle is in the usual and expected location on the right side of the patient. And then D for the great arteries, meaning that the great arteries are transposed, but the aorta is on the right side to the pulmonary valve is rightward. It doesn't tell anything about the anterior posterior relationship between these two great arteries. So based on this TGA ID description, we can infer all of the following. There is discordant atrial ventricular alignment. There is discordant ventricular arterial alignment. We know that we mentioned before that the AV valve typically follows the ventricle, so we know that the mitral valve is here on the left side of the patient, and the tricusp valve is here on the right side of the patient. We also know that the tricusp valve is the systemic AV valve, because it follows the right ventricle, which is the systemic ventricle. And we know that any anatomic regorge of this valve that TR physiologically means mitral regurgitation for this patient. There's a lot of information that we can get only from this simple description, TGA IDD. Now remember that although the cardiac position in the chest is not part of the segmental anatomy model, after we have drawn our own model, we can simply tilt to one side or the other. So going back to our patient, his heart within the chest will probably look like this. We can see that because he has cytosine versus D loop ventricles and dextrocardia, his LV is located anterior to that morphologic RV. And this is a very interesting and infrequent finding. The more frequent combinations here are cytosoletus with D looping, levocardia, and that places the right ventricle anterior to the LV. And the other usual combination is cytosine versus with L looping and dextrocardia, and that also places the RV anterior to the LV. So this patient has a very unusual combination of cytosine versus with D looping. That's why this LV is anterior. All right. So besides all the police information that we can obtain from the segmental anatomy description, this is another important point. See how many different types of transposition we can find. So from left to right, this is the anatomy of our patient, TGA-IDD. The second one, TGA-SDD is what we call DTGA, the most common type of transposition with cyanosis that we repair during the neonatal period. The third one, TGA-SLL is what we typically call LTGA, oventricular inversion. Another type of transposition here, TGA-ILL. But the important part is, look at this, look at number one and number three. Both of these are congenitally corrected transposition, but still the description shows that the anatomy between the two of them is totally different. Remember how this patient was presented, congenitally correct transposition. These two are congenitally corrected transposition, but the anatomy is very, very different. I would like to conclude with this. The segmental anatomy approach can be integrated to the intraoperative TEE examination of adult patients with congenital heart disease. Remember that those letters, those three letters represent the main cardiac segments, the atria, the ventricles, and the great arteries. Remember also that the normal segmental anatomy is either SDS, non-inverted, or ILI inverted. Both are normal. The liver is very useful to us to help us figure out the visceral atrial cytosolitus. It's very easy to find with that during the TEE examination. Remember that there are multiple morphologic features that will help us differentiate the different cardiac chambers that includes the atrial septum, the appendages, the implantation of the AV valve that we can easily assess during the TEE examination. Thank you very much, and I would be happy to answer any questions at the end of the presentation.