 And I see Rafa has just joined us as well, so perfect time, great. So hi everyone, my name is Marcus Salvatore. I'm an anesthesiologist at Toronto General Hospital with an interest in adult congenital heart disease. I'd like to thank you all for attending today and also like to thank the organizers for giving me the opportunity to present about one of my passions. So Rafael and Fabio, they really deserve an incredible amount of recognition for creating and organizing these multi-center echo rounds, which provide a truly unique opportunity to learn from each other and compare notes between centres. So the topic of today's talk is TEE for adult congenital heart disease. I have no disclosures or competing interests, but what I do have a lot of is acknowledgements. So I'm truly fortunate to work in an amazing centre with exposure to these incredible cases, and they really make every day both fascinating and challenging. I want to thank my mentors and anesthesia listed on the left, as well as the members of the Toronto Adult Congenital Heart Disease Program, who have been so welcoming and supportive since I've joined the team. The director of the ACHD program is Dr. Rafa Alonzo, a cardiologist who specializes in heart failure and echocardiography for adults with congenital heart disease. He joins us on this call today to answer questions and to provide valuable insights. Also joining us is Dr. Ahmad Omran, consultant cardiologist and international expert in transesophageal echocardiography. I'd like to highlight some of the resources used, including the 3D models provided by Dr. Azad Mishari and the Lynn and Arnold Irwin Perioperative Imaging Lab. Also a bit of promo for two texts that have been authored here at Toronto General Hospital, Perioperative 2D TEE by Dr. Annette Vegas, as well as the cardiac anesthesia handbook by Dr. Vegas and myself. The sources for any images used are listed at the end of the presentation. So the topic of today's multi-center echo round is adult congenital heart disease. Not everyone is going to work at a center that performs elective cardiac surgery for congenital patients. However, the number of adults with congenital heart disease is growing exponentially as a result of the rapid progress made in terms of both surgical corrections and medical managing. Many centers today routinely manage congenital patients for non-cardiac surgery, and you may be called upon to perform a diagnostic or rescue TEE in these cases should an unanticipated instability occur. So it is important to have a basic understanding and practical approach so that you can interpret the TEE images for these patients. Needless to say, it is incredibly ambitious and really impossible to even scratch the surface of congenital TEE in 60 minutes. So today is very much an introduction. Interpretation of congenital TEE is dependent upon a robust understanding of normal cardiac chamber anatomy and relationships. So this is just as much a lecture on cardiac fundamentals as it is about congenital heart disease. Using the framework outlined by the Van Prague sequential segmental approach, we will learn how to identify the orientation and position of the heart within the chest, how to differentiate cardiac chambers by their unique morphological characteristics, how to determine the connections between those chambers, and then how to integrate this information for clinical interpretation. When giving a lecture, I think it's just as important to define the boundaries of what's not covered, especially as our cardiac fellows are beginning to prepare for the APT exam in five months time. So we won't be discussing guideline recommendations, although I've listed them here to direct your independent study. We also won't be discussing systems of annotation or classification, which you likely won't need for the exam. Due to time constraints, we won't be reviewing intracardiac shunts or hemodynamic calculations, but I think that these are important topics both for the exam and everyday clinical practice. Finally, we will not be discussing the evaluation of surgical corrections. Depending on your feedback, these will all be potential topics for future iterations of this lecture in years to come. So when faced with a congenital patient, the complexity at first can be overwhelming for a variety of reasons. First of all, the sheer number of variations and combinations of abnormalities can seem infinite. To add another layer of complexity, there are multiple systems of nomenclature and classification, as well as eponymous disease and procedure names completely unrelated to the underlying anatomy. When you do actually perform a TEE, you will sometimes find atypical images at unconventional angles, going to differences in structure, morphology, connections, shunts, and corrections. The key to simplifying this complexity is to disassemble the heart into its component parts or segments, and then to apply a standardized sequential approach to image interpretation. Unlike a TEE for a normal heart, the standard 28 views may not tell the whole story, but chambers and connections always do. Ultimately, the goal of this talk is to teach you a standardized approach to TEE for congenital patients. By the end of the talk, you will be able to make sense of complex images like these. However, an important disclaimer, should you need to manage a congenital patient in your practice, it is critically important to one, first understand the anatomy, including any prior surgeries, and two, involve expert congenital cardiologists who have more experience interpreting these images. We routinely call Dr. Launzer down to the OR to help us with interpretation during our most complex cases, and that integrated team-based approach is integral to our success at TGH. The sequential segmental approach to congenital heart disease was developed by the Van Proggs in the late 1970s, and it is the system of convention used throughout North America. The cardiac anatomy is assessed first by dividing the heart into three distinct segments, the atria, the ventricles, and the arterial trunks. Various sources divide the approach into different number of steps, but the principles remain the same. The approach begins by first determining the position and orientation of the heart within the chest. In step two, we identify the cytos or sidedness of the heart chambers, which is determined by the position of the right atrium. Next, we use intrinsic morphological characteristics to identify the various heart chambers regardless of their position. Once the chambers are identified, we can more easily determine the connections between them, including any shots. So step one is position and orientation. Position and orientation are independent features which can occur in various combinations. Cardiac position or displacement describes the location of the heart within the chest. Unlike all of the other cardiac features discussed today, cardiac position is not necessarily an intrinsic feature of a patient's anatomy and may be influenced by extrinsic forces. Many of you would have had the experience of trying to perform a TEE in patients with altered cardiac position due to lateral decubitus positioning or large hemothorax. Cardiac position is not often diagnosed with echocardiography, but is more easily assessed using other radiographic techniques, such as simple chest x-ray. The typical cardiac station in the left side of the chest is termed level position. Mezzo position, when the heart appears in the middle of the chest, is sometimes seen in patients with severe COPD and hyperinflation. Finally, dextral position may occur as a result of a compressive left hemothorax, severe scoliosis, or right lung collapse, but may also occur as part of a congenital cardiac condition, such as dextral cardia, which is a term that describes cardiac orientation. Orientation describes the base to apex axis of the heart as determined by the position of the cardiac apex. The three possibilities are level cardia, mesocardia, and dextral cardia, as shown here on the right side of the screen. Although orientation and position are not always interconnected, certain congenital combinations are more likely than others. For example, dextral cardia is often associated with dextral position, as we will see in the upcoming slides. So here are two clips from two different patients, each with a diagnosis of dextral cardia. I can tell you that both also have a diagnosis of dextral position, but as you can see, that doesn't really impact our ability to get reasonable midisophageal four chamber views at zero degrees with some minor adjustments in pro-rotation. Both clips clearly show a four chamber heart oriented with the apex to the patient's right, contrary to the conventional orientation. Displayed here are a series of chest X-rays and CTs, showing various combinations of cardiac position and orientation. Starting with number one, we see the heart in the left side of the chest and the apex pointing toward the patient's left. This is the nearly universal arrangement of level position and level cardia. Number two is not much different, showing a midline heart with mesoposition and mesocardia, which may represent a normal variant. Three, four and five all show dextral position, meaning that the heart is in the right side of the chest, although the specifics vary between cases. Three and four both show a heart with the apex pointed towards the patient's right, representing dextral cardia. Three shows dextral cardia with cytosolidus or normal chamber arrangement, while four shows dextral cardia with cytosinversis or inversion of heart chambers relative to their normal arrangement. An early hint to the cytosinversis in patient four is the gastric bubble, which also appears on the patient's right hand side, indicating cytosinversis totalis, affecting all thoracic and abdominal organs. Finally, number five shows the combination of dextral position and level cardia, which might occur in the context of a pneumothorax or following right pneumonectomy. These examples provide a good segue to step two of the segmental approach, which is determining the cytos or sidedness of the heart chambers. Although cytos technically translates from the Latin to site or position, it is often easier to conceptualize cytos as referring to the arrangement of various organs, chambers, and structures. As echocardiographers, you would have most often heard this term cytos in relation to the heart, but cytos is also at an eight characteristic of the lungs and abdominal organs. Cytosolidus means in the original arrangement. For the heart, this is determined by the position of the morphological right atrium, independent from cardiac position, orientation, or relationship to ventricles and blood vessels. Pulmonary cytos refers to the sidedness of the morphological right and left lungs, as defined by characteristics such as the number of lobes and the relationship of the pulmonary arteries to their bronchi. Finally, abdominal cytos refers to the position of the abdominal organs, such as the liver and stomach within the abdomen. The term cytos inverses can be best thought of as a flipped image of the natural arrangement. For example, when you look at the heart CT on this slide, you come to appreciate that rotating the heart around a single axis could never yield the inverses image. Instead, the heart appears mirrored or transposed across a sagittal plane. A several of the thoracic and abdominal organs share the same embryological origins. The cytos of the different organ systems often correlate in a phenomenon called visceral atrial concordance. This means that in the majority of cases, the cytos of the right atrium, and that's the heart, will correlate with the sidedness of the lungs and abdominal organs, which can help you when interpreting TE images. But as with all things in congenital heart disease, there are exceptions to every rule and there exist rare patients in which the cytos of the organ systems cannot be accurately determined. This arrangement is termed heterotaxi or cytos ambiguous. As we see here, there's a patient with a broad liver that spans the entire abdomen, in addition to a right-sided stomach and polysplanism. This often occurs in the context of cardiac isomerism in which the atria are mirrored, resulting in a patient with two right or left atria. All right, so you insert your TE probe and you see this patient has cleared dextrocardia. How do you go about determining the cytos of this patient? Well, this requires you to identify the various chambers according to their innate morphological characteristics. Now, this isn't always easy or straightforward, but you would be surprised with how much you can infer from subtle details. For instance, in clip one, the atrial ventricular valve here is apically displaced relative to the other side. Secondly, although the appendages cannot be identified, the atrial chamber here appears to contain a septum secundum. These are both features that you would associate with right-sided structures, although they appear on the left side of the heart in this case. Now, there are also features that are unreliable as identifiers and may serve as red herrings. For example, in this colored Doppler image, the chamber at the top of the screen appears to have at least two tributary vessels. Colored Doppler reveals the flow to be laminar, pulsatile, and directed towards the AV valve. The lateral orientation of these vessels at zero degrees suggests that they are more likely to be pulmonary veins than cava. There is no eustachian or thethobesian valves visible. Pulsed-wave Doppler reveals pulmonary vein flow. However, the pulmonary veins serve as unreliable landmarks all into phenomena such as anomalous venous drainage. But together, these findings suggest that this patient has dextrocardia with cytosine versus. These veins are the left upper and lower pulmonary veins as they originate from the morphological left lung situated in the right side of the chest. I find it interesting that despite the cytosine versus of this heart, the morphological left atrium still serves as the echocardiographic window to the heart. These next two clips are from the same patient. In the previous slides, we determined that we are looking at the heart through the morphological left atrium. We recognize the interatrial septum here, which suggests that the chamber on the bottom of the screen is the right atrium, further supported by the visible septum secundum. Regardless of cardiac position, orientation, or cytos, we know that around 90 degrees, the right side of the screen is oriented cranially, suggesting that this vessel is the SVC, further confirmed by the tip of the central line. The transgaster view shows additional findings, including inverted ventricular anatomy, as well as a left-sided liver, indicating that this patient has cytosine versus totalis, affecting all major organs. So what can we do if we suspect extracardia, but the defining chamber characteristics are not clear? For example, in this patient, the AV valves appear to originate at roughly the same level. There is no clear moderator band, and the septum secundum cannot be visualized. So what can we do? Well, an agitated saline study leaves little doubt that the left side of the heart is the chamber that receives the systemic venous return. So this highlights the next step of the algorithm, which is to identify cardiac chambers according to intrinsic morphological characteristics, regardless of their position within the heart or relative to other chambers. So let's first look at the atrium. The defining landmark of the right atrium is the broad-based triangular appendage, characterized by extensive pectin muscles throughout. The second landmark is the crystal terminalis, often seen between the SVC and the right atrial appendage, best appreciated in the bicable view. The inflow to the right atrium includes the superior and inferior vena cava, as well as the coronary sinus. However, only the IVC can be used as an identifier for the right atrium, owing to conditions such as anomalous cable venous return or unroofed coronary sinus. The intraatrial septum also has distinguishing features with the septum secundum seen on the right atrial side. In contrast, the left atrial appendage is a smooth finger-like projection with a narrow opening. Pulmonary veins are variable in congenital disease and are therefore unreliable as chamber identifiers. There is no crystal terminalis and the IAS is characterized by the septum primum. Lastly, it is important to note that the atrial ventricular valves should be conceptualized as ventricular structures and do not help on distinguishing the atria. These clips taken from a normal heart highlight these differences. Clip one shows the key right atrial identifiers, including the broad-based right atrial appendage, the crystal terminalis, and the septum secundum. Clip two shows the narrow finger-like atrial appendage. Needless to say, not all patients will have such clearly defined atria, especially amongst adults with congenital heart disease. This clip, for instance, shows a bicable view in a patient with a sinus venosis ASD. What you can see here is an atrial communication caused by a deficiency of the common wall between the superior vena cava and the right-sided pulmonary veins. On color Doppler, we can appreciate two distinct flows, blue flow through the ASD and red flow from the right middle and upper pulmonary veins into the SVC. Traditionally, these defects were repaired using the Wardham procedure, in which the SVC is detached and reconnected to the right atrial appendage. However, these days, it is much more common for surgeons to perform a double-patch repair. One patch is used to divide the SVC, creating a baffle that reroutes the anomalous pulmonary vein flow through the existing ASD into the LA. A second patch is used to enlarge the SVC to provide unimpeded drainage. Once the right atrium is identified, the cytos of the heart can be established. As mentioned, patients may rarely exhibit a mirroring of their left or right atrium in a condition called isomerism. Isomerism is associated with heterotaxi or cytosambiguous of the lungs and abdominal organs. Now that we have identified the atria, we can move on to identifying the ventricles. The right ventricle will always be associated with a tricuspid atrial ventricular valve with a more apical point of attachment. The septal leaflet of the tricuspid valve tethers to the septum, as opposed to mitral cordae, which tether to the ventricular apex. The infundibular RVOT conus is part of the characteristic shape of the RV seen in the standard vitisophageal RV inflow outflow view. Chorus trabeculations are seen throughout the RV and the moderator band will often be appreciated at the apex. In contrast, the RV is connected to a semi-lunar valve with a more basal point of attachment. There is fibrous continuity between the aortic and mitral valves, often called the aortic mitral curtain. Other distinguishing features include an absence of cordae tethering the valve to the septum and the absence of a moderator band. However, false tendons may be mistaken for a moderator band in some patients. These clips demonstrate how morphological features can be used to identify the ventricles. We see here a heart inside a solitus as indicated by the central line seen in the right atrium. However, the ventricular architecture appears grossly abnormal. The ventricle on the left side of the heart is dilated in hypertrophic with what appears to be a moderator band at the apex. There is an apically displaced atrial ventricular valve with severe regurgitation. Closer inspection of the left-sided AB valve shows that the medial leaflet tethers to the septum here. Together, these findings suggest that the ventricle on the left side of the heart is the morphological right ventricle. In the context of normal cytos, we can deduce that this patient has ventricular inversion, otherwise known as congenitally corrected transposition of the great arteries. The apical displacement of the tricuspid valve is accentuated in this patient with episthenoid valve characteristics. Correctly identifying the ventricular morphology is even more challenging when there's only a single ventricle and single atrial ventricular valve as comparisons across chambers cannot be made. Here we see a congenital patient with a large common atrium and a common inlet into a single ventricle. But is this the left or the right ventricle? Well, the moderator band and septal tethering identify this as the systemic right ventricle in a patient with a history of hypoplastic left heart. The final chambers to identify morphologically are the arterial trunks. They are simple structures without many defining features and we must rely on connections and branch vessels to discern them. The pulmonary artery bifurcates into the right and left PAs whereas the aorta has coronary arteries proximally and the head vessels that extend from the arch. In some patients, the PA and aorta are combined and form a single vessel called the truncus arteriosus. Truncus arteriosus is an uncommon congenital abnormality that occurs due to the failure of conor truncal separation during development of the fetus. It is characterized by a single arterial trunk that originates from the heart and supplies the systemic pulmonary and coronary circulations. Although there are many different manifestations and classification systems, there are two broad categories. Common trunks in which both the PA and aorta originate at the level of the truncal valve and solitary trunks in which the right and left pulmonary arteries arise independent from the valve distally along the truncus. Here is the mitisophageal long axis view of a patient with truncus arteriosus and a large VSD. Both the right and left ventricles can be seen emptying into large solitary truncus. Imaging the descending thoracic aorta reveals the branch points for the pulmonary arteries which come directly off the aorta just distilled to the left subclavian artery. Now that we have identified each of the chambers and their arrangement, we can complete the final step of the segmental approach which is defining the essential connections thereby determining the direction of blood flow. The two main connections to discern are the atrial ventricular connections and the ventricular arterial connections. Let's begin with the connection between the atria and ventricles. The three main types of atrial ventricular connections are concordant, discordant, and univentricular. As we have already learned, the cytos of the heart is determined by the position of the right atrium independent of the arrangement of all the other chambers. AV concordance is determined by which ventricle connects to the right atrium with the four possible combinations shown here. Univentricular AV connections can occur due to a number of different congenital abnormalities, such as pulmonary atresia, hypoplastic left heart syndrome, or large VSDs. These are often palliated into single ventricle or fontan physiology. Unfortunately, the various types of fontan, although fascinating, are beyond the scope of this talk. The three types of univentricular connections shown here are termed single inlet, double inlet, or common inlet. Here we have clips from a 20-year-old patient with a univentricular AV connection. We can see that he has one large common atrium that empties into a single systemic right ventricle through a common AV valve or inlet. The 3D image on the right shows the thickened tri-leaflet common AV valve from the atrial side. We can follow the infundibular conus to the truncal valve, which opens to a dilated solitary trunk. Color Doppler of the truncal valve shows significant regurgitation, which was the indication for surgery. Approximately 30% of patients with truncus arteriosus will have quadrocuspid truncal valves with successful repairs well documented in the literature. This clip shows another type of univentricular connection termed the double inlet left ventricle. Here there are two distinct atrial ventricular valves, both of which open to a common systemic left ventricle. Also near the right atrium are other features of his palliative repair, including a lateral fontan conduit and an intratrial baffle. Abnormalities may also arise at the ventricular arterial connections, also termed the ventricular outlet. The most characteristic lesion of this type is transposition of the great arteries or TGA, which exists in two main forms, LTGA and DTGA. In the schematics shown here, gray denotes native connections in chamber orientation, whereas yellow indicates abnormalities. LTGA here is also called congenitally corrected transposition. In these patients, the connections between the atria and ventricles, as well as between the ventricles and great arteries are both discordant. Compared to a normal heart, only the ventricles have changed their position. Understanding the physiology helps to clarify some of the alternative names for this condition, including ventricular inversion and double discordance. Although the connections are discordant, blood follows a normal route through the lungs, heart and systemic circulation. However, the systemic RV will eventually fail as a result of a lifetime of systemic pressures. Conversely, patients with DTGA on the right side of the screen only have a single discordant connection between the ventricles and great arteries, and it is the aorta and the PA that are inverted. Physiologically, this represents a much more abnormal configuration, as venous blood returns to the systemic circulation without passing through the lungs. This is therefore a form of ductal-dependent cyan aorta-card disease, which requires corrective surgery soon after birth, most commonly an atrial arterial switch. Here are clips from a 21-year-old patient with LTGA admitted to hospital with a first presentation of a symptomatic tachyirhythmia. This was a TEE performed to rule out thrombus before cardioversion. Although the first clip does not show the features necessary to determine cytos, the slow sweep of clip two shows that the left atrium is on the left side of the heart. Using morphological identifiers, we can see that there is a discordant AV connection as the left atrium is connected to a morphological RV. So we've identified this as left atrium with left atrial appendage, and it's connected to morphological RV with moderator band and septal tether. The ventricles are thus inverted, and the morphological RV is the systemic ventricle. Due to differences in contraction mechanics in chamber architecture, the systemic right ventricle fails early in life as evidenced here by RV hypertrophy, dilatation, and hypokinesis. Conversely, patients with DTGA will have undergone early corrective procedures, which must be clarified in order to understand and interpret images correctly. There are some key echocardiographic findings in this patients, which are easily recognized. First, as demonstrated in this clip, the left and right AV valves attach at the same level and don't show the apical septal attachment of the tricuspid valve leaflet that we previously used to identify the morphological RV. In the normal heart, the position of the pulmonic valve relative to the aortic valve can be remembered using the neuronic P-A-L-S, or PADS, as the pulmonic valve is normally anterior, lateral, and superior to the aortic valve. However, in DTGA, the aortic valve lives anterior to the pulmonic valve, as seen in these images. So in this image, this is your pulmonic valve and this is your aortic valve. Same thing here, pulmonic valve, aortic valve. And this is different than obviously what you'd expect to see in a short axis view. Due to this abnormal chamber arrangement, the aortic and pulmonic valves are coplanar and can be imaged together in either short or long axis. Here is a second patient with DTGA complicated by complex intracardiac anatomy, which precluded for repair. She also had valvular pulmonary stenosis, and so her pulmonary artery was disconnected and attached to her SBC. Here we see the coplanar aortic and pulmonary valves, the anterior position of the aortic valve and the ligated pulmonary artery. So here's aortic valve, this is her hypoplastic pulmonic valve, ligated pulmonary artery, and in the deep transgastric view, we can see the same aortic valve, hypoplastic pulmonic valve, ligated pulmonary artery. Although I won't delve too deeply into surgical repairs, understanding the corrective options possible for DTGA further highlights the physiology of this congenital disease. Surgical corrections attempt to re-approximate the normal flow of blood through the heart. Early corrective strategies accomplished this goal by switching the venous inflow to the atria using baffles and a procedure called an atrial switch or mustard procedure. Switching the atria adds a second discordant connection here which diverts venous return through the LA to the LV and out to the lungs. Oxygenated blood returns to the RAVL baffle, traveling through the RV to the systemic circulation. In this way, surgeons recreate the congenital correction in founded patients with LTGA. However, this procedure leaves the patient with a systemic right ventricle, which as we discussed will fail over time. Baffle leaks and atrial dilatation are also common complications, so this procedure is no longer performed. These days, DTGA is repaired by correcting the sole discordant connection between the ventricles and great arteries, thereby restoring conventional blood flow to the heart. This procedure is called the arterial switch or jutein procedure, as illustrated in this diagram on the right. These clips show a 43-year-old patient with DTGA status post-atrial switch procedure. Clip one shows a mitisophageal four-chamber view with a pacer wire extending through into the LA through the atrial baffle and that's right here. You can also appreciate the dilated hypokinetic and hypertrophy systemic right ventricle. The colored Doppler in clip two shows the pulmonary venous blood returning to the right atrium via an atrial baffle. And so this brings us to the end of our presentation. There are a few key takeaways I would like to reiterate. The ACHD population is rapidly growing due to advances in surgical technique and medical management. You may be required to manage these patients outside of tertiary referral centers. TEE imaging of patients with ACHD begins with a thorough understanding of the patient's anatomy and prior surgical corrections. Complex studies can be simplified using a sequential segmental approach. Identify each chamber using morphological features then define the connections between them. These are the sources for the various figures and thank you for your attention. I would like to now open the floor to any questions with the help of Dr. Rafa Alonso, medical director of the ACHD program at TGH as well as Dr. Amad Omran, consultant cardiologist. So Rafael, we'll direct you. I'm gonna stop sharing the screen and I'll let you take over as host. Is that okay? Sure, thanks. Thanks for the great talk, Marcos, and I appreciate all the effort you put to make your presentation outstanding. I don't see any questions on our chat. So I have a question, but to ask my question as I already talked to you beforehand, Marcos, I'd like to discuss a case and then hear the expert's opinion. Rafael. Yes? Someone had put the hands up. I think it's Arnold. Arnold, did you put the hands up? Yes, I do put the hands up. I have a first of all, first of all, I want to say congratulations to Marcos. Really great presentation. It's such a complicated topic and having a good approach is really helpful to assess those type of patients. My question is regarding the use of 3D printing technology in understanding the anatomy and the physiology of this patient prior to the surgery. Are you guys using this type of technology at TGH? Before the surgery to kind of program the surgery, the approach of the case? Right, so I know this is the work of an APA lab are really working making huge strides in terms of integrating the CT data into 3D printed models so that the surgeons and congenital cardiologists can both together look at it at a 3D space and plan their surgery a little bit better. Rafael, do you have any experience with when a 3D model really facilitated or helped the surgeons in terms of their approach? So we do use it in very complex patients where the anatomy is not clear or whether mainly when we cannot find exactly where the holes are when we want to close more for pre-cutaneous procedures than for surgical procedures. But we have the possibility of creating a 3D printing model that mainly use in very complex cases when the imaging alone doesn't allow us to see exactly where the problem is. But it's more common for pre-cutaneous procedures than for surgical procedures. But we have the option to use it for surgical procedures. And then Rafael, just before moving on to the next case, I think you wanted to touch briefly upon cases of salvage ECMO or rescue ECMO in congenital patients. Do you want to talk briefly about that? Yeah, that's correct. I was wondering if you experts could give us any recommendation or at least a stepwise approach in the case that we, oftentimes we are requested to do a TE for ECMO insertion and could be salvage or even more of an elective. And I was wondering if you could give us any rational when or how to consider any congenital heart disease if the ECMO is not working properly. Just in general approach for troubleshooting ECMO and when and how to consider congenital disease. Again, Rafael, do you have any experience here? I haven't personally seen ECMO in a congenital patient. I know there were two that we were discussing it for. There was a recent ebbsteins that had extremely dilated RV 480 mils and we were talking about temporary centromag support in order to support the RV, but in the end, he didn't end up needing it. And then the other conversation that we had regarding was VV ECMO in a Fontan patient that developed early pneumonia and ARDS following surgery. So those are the two that come to mind. Rafael, and any other experience that you have recently in terms of ECMO use for congenital patients? So if we exclude the patients with single ventricle and Fontan physiology, which are extremely complex and that would need a talk themselves because actually each patient is different and the cannulation depends on the configuration of the Fontan. If we talk about any vivanticular patient with vivanticular heart, regardless of the ventricular systemic RV or systemic RV, ECMO troubleshooting problems should not be different than a patient with vivanticular heart. So normally, we haven't had, as Marco said, anybody at risk, we have somebody now in the unit, but having had a case recently and we discussed those as a pre-op when we have a patient at high risk, we discussed pre-op who might need ECMO, but in terms of managing a patient with vivanticular heart, whether or not it has connective heart disease, sometimes the people, because the patient has connective heart disease acts differently, but if the patient has followed with an RV failure, it's no different than in terms of ECMO management and a patient with RV failure for another reason with a severe palmymbolism, for example. So I think that the suggestion is if the patient is vivanticular heart, which is, thank goodness, majority of the connective heart disease patients, the management would be very similar with what any vivanticular heart patient. If it's a single ventricle, I think it would be a completely different topic. Some patients, some centers might not even put ECMO on vivanticular hearts. And when you choose to put ECMO on vivanticular heart, you have to choose the cannulation very carefully and consideration of the anticoagulation and the high risk of plotting. And the only patients that might have, might need special attention are the patient that's cyanotic, because they have a high risk of plotting with ECMO. I have seen patients just having disasters on ECMO with cyanosis. We're still using ECMO in these patients if needed, but is that a group of patients that need to be more careful? Hey, I understand that. So basically, well, if the patient has univentricular physiology and it's an adult, we will most likely know beforehand. And for all other patients, you believe that we would not be surprised. So we should not include a congenital heart disease in the differentials when, for example, an ECMO is not oxygenating well or there is non-proper functioning. Is that right? The main problem that you might have is having a residual shunt. So that's when sometimes we are, you might have somebody that is not oxygenated properly to have a residual shunt. That's the first traversing that you might want to, you need to consider somebody had a previous ASD closure or ASD closure. If things are not going in the right direction and everything else is okay and you don't have any other explanation, the chance sometimes are, of course, if you have an ASD or residual ASD, you might have a desaturation. That's the main traversing. I want to clarify, based on single ventricle is not unknown. It just needs to be done in a center with expertise in congenital heart disease, but there's no unknown. So it's a patient that needs to be if possible and stable either transfer to a center with expertise of congenital heart disease or calling a center of congenital heart disease expertise to know how to cannulate. So sometimes we are on call and we get phone calls for another centers to cannulate patients with single ventricles. And if I'm on call, I don't say don't do it. And if the patient cannot come here and it's the only solution what we discuss how to cannulate and where. So I mean, I wouldn't be the first patient that is cannulated somewhere else in a center with a lot of experience on ECMO, but no experience in congenital heart disease and they managed to get the patient out of the woods and get the patient to cannulate it and transfer the patient here after. So don't say single ventricles. No, it's a single ventricle needs the idealian expertise in congenital heart disease. And the others, if you have desaturation, the main thing is to rule out a shot that you wouldn't have in a patient with no congenital heart disease in general. Perfect, thank you very much. I see there is someone else with the hand raise. I'm going to jump in and let's see. No, I think our noise too has his hands up. Like I think you can go ahead, Rachel. Yeah, so this is related to a much more simple congenital disease. It's a case that I did here in London a couple of weeks ago and I'd like to hear your opinion of you guys from experts in congenital heart disease. So this is a 56 years old male known to have a lifelong subaortic stenosis that presented with progressive shortness of breath and chest tightness on exertion. He's also an elevated BMI spoker with a gird and a sleep apnea. And he comes for a resection of a subaortic membrane. So he has symptoms of aortic stenosis due to a congenital subaortic membrane. So these are the images in the OR and I hope that everybody can appreciate good temporal resolution here. So top left, we can see that there's something here in the LVOT, which is the non subaortic membrane with flow acceleration and some AI. The 3D, which is not stellar in this case, but you can see that the subaortic membrane is here and also in a short axis and zoomed into the LVOT, it's clear that there's something here. This is just to show, this is a still image just to show that the flow acceleration in the ALIS and they start just below the OR12 compatible with the subaortic stenosis. This is a short axis of the OR12 showing some signs of calcification. Maybe a commissure fusion here, which would be compatible with bicuspid-like OR12. There's probably some features of displays here as well, but I'm not sure. Again, just a long axis dedicated here with colors to frame to show the acceleration before the LVOT and then deep turns gastric going from the apex towards the OR12, we start seeing flow acceleration at some point close, but not at the level we see maximum velocity with pulse wave Doppler and then we can only see velocities across the LVOT with continuous wave Doppler without ALIS and this is what some people would call the triple envelope sign. So we have one envelope here and second envelope here and a third envelope here. Some measurements here, but irrelevant for the case. And so we go on bump and the membrane is resected. You can see here, there are signs of resection in the area where we previously saw the membrane. We interrogate the interventory receptor and there was no signs of ESDs, Iatrogenic ESDs, but we still see that same valve which has some regurgitation. We could say based on only on this view, it's a little bit mild, maybe mild to moderate side. And here we see after the resection, this is a still frame as well. There's no acceleration anymore and everything happens. The acceleration starts at the level of the valve. So the subaortic stenosis, it's gone apparently just to compare before and after with and without color. So you see this in here and you don't see it anymore here. So we believe that there's no flow acceleration anymore at the level of the LVOT. However, this aortic valve post bump presents with a mean gradient of 21. I mean, it is a valve that we, as we saw, there is probably some fusion between the RCC and the LCC. There are some calcifications there and although this is a hyperdynamic state, mean gradient of 21, we'll be looking at mild to moderate aortic stenosis on a valve that's possibly bicuspid, which was confirmed by the surgeons under direct vision. So the surgeon, that's a question the surgeon made at that time. And this is the question I bring to you. Of course, there's no criteria to replace the valve in a patient with mild to moderate AS. However, we have a 56 years old male with the chest open over the table. And we know it's a bicuspid aortic valve with gradients on the mild to moderate. Now, should we leave the aortic valve alone or go back and replace the valve right now? So that's the question. Would you want to take it or do you want me to take it? Rafa, yeah, you can take it and we discuss it. I mean, that's a very good question. I don't think you have a right to roll answer here. I personally would not ask about it. So it's about that as mild probably AS. And you might ask, if you just change the valve, you just, first of all, if the patient had been consented for that, but even if they say the patient had been consented for that, I would probably wait until the patient, that valve gets deteriorated and I would do a valve replacement later on. You don't know if that valve can last for no 10 years. It's 10 years that the patient can get on that valve. But I can't understand why the question come. Some people might think differently and I'm quite conservative to not to change things that because we are there. So if that valve had not been, if the patient had that only that elishing with a mixed valve, this is that it's mild or mild to moderate with a normal ejection fraction, a normal ventricle, we would not offer him surgery. So I try not to change the way I think in the war. It's not because they damaged the valve or just the valve was a little mask for the suffer of the gastronosis. And now it's happened to have a mild AS. So, but I personally would not, but this could be a matter of discussion. And somebody say, yes, do it. I don't think it's understandable either. Yeah, can I say something? Yeah, I totally agree with that. If we go by the guideline, it's not moderate AS. We know that if you have a moderate AS and we are in the war, we go for that aortic valve. This is mild to moderate or even less maybe. So, and the valve, I'm not sure really is a congenital I agree is a functional by calcification, but even as a congenital, this valve might last another five, six years. So there's no point to do valve replacement now. We can do it later or we can do TAVI or something else. I would leave it. Yeah, I agree. And also like thinking about the fellows that are watching the presentation is also important to emphasize that it's always hard to make like to take decisions based on the hemodynamic hemodynamics coming off bypass. And also one thing that Raphael showed us like after they remove the submain brain like it's also important to evaluate if there is some like residual VSG or something, right? Right at the site of the resection of the submain brain. So I wouldn't do anything else. I wouldn't tell the surgeon to go back and bypass and change the valve also. Yeah, no, thank you very much. I appreciate the comments. And that's exactly what happened. I said the same. I said, you know, this adult, he's young. He's here with chest open. I would say that this is mild AS because now he's hyper dynamic in this same patient. So they didn't do anything with the valve. The patient had a very good post-op recovery and TTE three days afterwards show the gradients still in the mild to moderate range. But patients happy with the outcomes right now and we didn't do anything. We just left it alone. But thank you very much. I appreciate all the comments. I think Fabio had a question as well. Do you Fabio? Yes, actually I do also think about the fellows that are watching this presentation. Guys, I noticed at the beginning of the presentation, Marcos, you said you're not talking about gradients and hemodynamics calculation, but just briefly, could you guys just touch base and talk a little bit really briefly about like the when and how in which case use like the QPQS measurements, the relation between them. So I think it's important to just illustrate a little bit for the fellows that are watching this presentation. Thank you. It's a great question and one we talk about quite often. I'm gonna direct to Amad who teaches quite extensively on QPQS relationships. So Amad, do you mind fielding this question? When do you think it's appropriate to do QPSQS ratio to the calculation fractions and whatnot? Yeah. If we wanna go based on transistor stick, probably maybe Rafay can talk about calculation of the shunt by transistor stick because we cannot measure the pulmonary annulus very well. Maybe it's not very accurate. But in the OR, I don't think calculation of the shunt pretty up has really a value. We can diagnose the disease and the size of the ASD, ASD, what they're saying is by just the T, but it posts up is important. Because if we left a residual shunt and we wanna make a decision, in that time we can use the calculation of the shunt, either by the Doppler or maybe by the oxygen saturation. So I think the main use is post-stop to see how much is for example residual ASD or residual BSD. I don't know, maybe Rafay has some other things, yeah. I'm not a fan of calculating shunts with echo, but just because of what Amadeus said, the pulmonary annulus is extremely difficult to measure accurately. And we should, we would never make decisions clinically based on echo, on a shunt calculated by echo. It's very easy to underestimate or overestimate the shunt. So even T give you a better view of the off-road track and you really can measure better. But even though still you need to get the data that you get from echo in shunt calculation with a pinch of salt and need to mix, need to match the clinical situation you have in front of you. So in transtoracic we don't use it at all. We don't make any single decision and we don't calculate the shunt never. Even in patient with severe ASDs, it's just because of the risk of making overestimating or underestimating the shunt. So if you want to make a clinical decision on a shunt patient you have to cut the patient and get the saturation sent to the FIC to calculate your shunts properly. The echo, you can use echo for indirect science of if the patient has pulmonary hypertension or the shunt might not be plausible, but in terms of calculating the shunt, the limitation that the after tracts are difficult to measure with the transtoracic at least. Yeah, perfect. Thank you. Rafael, looks like you have someone else asking who wants to ask for a question. Hansa, thank you. Go ahead. You have a question? Yes, please. Thanks Rafael for the interesting case. I was wondering if it's another case and what do I mean by that? If it was a cabbage involving a lemal read or a lemal rema, would you do anything different? Would you suggest or discuss it further more in changing the valve? I mean, without the subaortic membrane? Yes. Just mild, no, I don't think there is any indication to replace the valve if there is mild to moderate AS. I would like to hear other people's opinion, but only if it's moderate, I don't think mild to moderate, it's an indication. Yeah, again, the guideline says moderate, yeah. If you have a moderate AS and you're going to the OR for other cardiac surgery, it's better to replace it, but this is not the moderate, yeah. Excellent. Any more questions? Okay. Rafael, there is. Yeah, there's a question which had asking, would you appreciate a device regarding the incidental ASD finding intraoperatively? It's a good question. So just to clarify, you're talking about like a seconda MSD and you don't know whether or not to tell the surgeons to repair? Yeah, how would you decide to tell the surgeons to repair or not? I don't remember, I don't recall correctly, but we had a case here a few years ago that the patient came for an elective cabbage and it was found to have an ASD. I don't remember what happened exactly, but it's a good opportunity to hear your opinion. You're going for an elective surgery and then all of a sudden you find, let's say a seconda MSD. So I think in the experience of the cases that we've had in the last couple of months, I think it depends on one, the size of the ASD, two, the direction of the shunt, and then three, what the intervention that they're going in for, or is it a cabbage or is it something intracardiac or they're doing something where they have to open the right atrium and left atrium? And does it do kind of the three main things that I can think of that will influence decision making? Ahmad, do you have any additional input? If by definition we call it ASD, it means the defect, at least more than one centimeter, I think we are in the war, we should close it. I believe this way. But if we are going to talk about less than one centimeter, something like a PFO, a slash ASD, and we are not opening the LA or RA, we are doing just a simple cabbage, we might leave it and we might decide about that percutaneously. I don't know, maybe refer as something else. Can I just add something else to this question? Let's say it's a iatrogenic ASG called, for example, patients have a mitral cleat, for example, and when they finish everything, they see that there is an iatrogenic ASG, so which would be the same approach in terms of decision making or should we just close it right away? What do you guys think? So you are in the cath lab doing mitral cleat and you create ASD yourself for crossing the catheter? Yes. No, we don't close that one because most of the time that defect by catheter the size will decrease and might totally resolve in future. So we don't do anything for that one. This is the same for mitral balloon, for example, in old time, we were doing mitral balloon, it's a tostomy, we leave them and many of them, they close their cells, we see them later, it's not there anymore. No, we don't close them. Okay, thank you. We've gotten this wrong before in that there's been patients that have gone to the OR, there was some intracrural shunt that was detected and it wasn't closed at the time of surgery and then the patient had issues with elevated pulmonary pressure, for instance, or the shunt became a right to left shunt with chronic hypoxemia that required percutaneous closure afterwards. So that's also happened. So it's always gonna be a judgment call but all the different outcomes are possible. You just try to make the decision that you think is best but as the clinical condition evolves over the subsequent days, things can change. Thank you, Grace. Excellent, I think it was a good discussion today. It's almost 10 past six. Anyone has any other questions? All right, thanks everyone. Thanks for joining today and thanks for all that participated in the discussions. Our next session is supposed to be on March 21st and Rob Chin from Ottawa will be talking about the Astrology. Thank you very much. Have a good night. Thank you guys.