 Hello, my name is Marjan Giordiani. I'm an anesthesiologist at Toronto General Hospital. It's my pleasure to be here and present this case to you, non-cardiac surgery in adult congenital heart disease patients. Regarding objectives of this talk, increasing number of adult congenital heart disease patients present for non-cardiac surgery. Anesthesiologists in any hospital setting could become responsible for providing care to this population of patients. We are going to review a case and go through preoperative and intraoperative decision making with emphasis on the role of echocardiography. We will review the available guidelines on how to decide on severity of a cardiac lesion and following that what are the implications of the severity of a cardiac lesion. We are going to use this information to elaborate on what setting the procedure can be performed and what level of preoperative care is appropriate for a particular patient. Then we review the post-procedural decision making and overall at the end I'm hoping that we could form a general approach to adult congenital heart disease patient for non-cardiac surgery and decision making related to that. Our case, a 20 year old male presenting for colostectomy. He has symptoms of abdominal pain on daily basis. He was investigated and was found to have gallstones. His history is remarkable for congenital heart disease. He had oxygenation issues at birth. He was investigated and was diagnosed with epsin anomaly of tricostrid valve and also anesthesi. He underwent device closure of atrial septal defect 12 years ago with amplizer device. He is left with moderate to severe tricostrid valve regurgitation. He has normal neurodevelopment and no other comorbidities. When we saw him for preoperative assessment, he was a functional class 2. Not very active but denied any cardiorespiratory symptoms. He did not have any history of previous episodes of heart failure or hospitalization for this matter. He was at no distress at rest and did not have any abdominal pain during the clinic visit. His rumor saturation was 96%. He was a smaller person with a height of 147 cm and his weight was 52.5 kg. He had normal airway and there was no signs of right-sided heart failure in him when we saw him. Our patient was under regular follow-up by congenital cardiology clinic. He had yearly visits with clinical exam, CPS study and transtoracic echo. He had a recent visit with the clinic and overall their assessment indicated that he was in a stable condition with no need for any intervention. We are going to review some of the transtoracic images which were done preoperatively in this patient. As you see here, there is significant apical displacement of septal leaflet of tricostrid valve. It is measured about 6 cm here and marked by red arrows. There is also dilatation of atrialized portion of right ventricle, which is anatomical RV, but not the functional right ventricle. This area is marked by a green line here. Also there is dilatation of tricostrid valve analysts, which is marked by yellow line here. There are other abnormalities and they are extensively explained, but in summary there could be apical displacement of posterior leaflet and anterior leaflet as well. There is adherence of septal and posterior leaflet to the underlying myocardium. The epstein anomaly involves not only tricostrid valve, but also right ventricle as well. Here you see severe dilatation of anatomical right ventricle. A major part of this is atrialized and is not part of the functional right ventricle. Functional right ventricle is actually a smaller cavity and has some degree of dysfunction. There is significant tricostrid valve regurgitation. The assessment preoperatively was moderate to severe tricostrid valve regurgitation. This is a short axis view of the left ventricle and right ventricle. Patient had a small LV cavity size, but preserved systolic function, D-shaped interventricular septum as you see. RV was severely dilated, but this is not functional right ventricle. Functional RV is small and has mild to moderate dysfunction. There was no evidence of shunt across intracereal septum and amplizer device. Regarding decision making, we consider this case to be a semi-elective procedure, so certainly there was time to optimize the patient further if needed. Regarding severity of congenital cardiac lesion, I wanted to mention that this is a very important factor in decision making preoperatively and based on the severity of the cardiac lesion, we can decide on the level of preoperative care and the hospital setting that the procedure needs to be performed at. Also, we had to decide on performing open versus laparoscopic technique. Considering benefits of laparoscopic technique in postoperative recovery, we decided on laparoscopic cholecystectomy with low threshold to convert to open if hemodynamic instability occurred. A quick discussion on how to decide on severity of congenital cardiac lesion based on 2018 guidelines, this is done according to the anatomy of the lesion and physiological state. This is different from previous guidelines published in 2008 and in that guideline, the severity of lesion was mostly decided based on complexity of the anatomy. Regarding anatomy of congenital heart disease lesion, this could be simple, moderate or complex. Example of a simple lesion is like an isolated ASD or repaired ASD with no chamber enlargement. Examples of complex lesion are fontancell curation, isonmanger, cyanotic heart disease, and anything in between is the moderate lesion. Epstein anomaly falls into this category, but it is important to mention here that in Epstein, disease spectrum has a wide range and can be mild, moderate, or CBA. Regarding physiological stage, this is based on several factors that are proven to correlate with outcome, and they include overtake dilatation, arrhythmias, concomitant valvular heart disease, end-organ dysfunction, exercise capacity, hypoxia, cyanosis, and New York Heart Association functional class. So we have physiological stage from A to D, with A being functional class one, with no hemodynamic sequela, no arrhythmia, normal exercise capacity, no organ dysfunction, normal diameter of the aorta, and D being functional class four with refractory arrhythmias, severe hypoxemia, severe pulmonary hypertension, etc. To summarize, we have a semi-elective procedure, Loparoscopic Colesistectomy, which is a moderate risk surgery in a patient with congenital heart disease, which is Epstein anomaly. This is at least moderate complexity of anatomical classification and class C for physiological stage. Looking at 2018 guidelines regarding management of this patient, they recommend optimization before surgery and close observation and monitoring following the procedure, and also performing the procedure in hospital setting, in consultation with experts in adult congenital heart disease, if possible. Patient was accepted for the procedure. There was no indication for any optimization preoperatively. Following induction of general anesthesia and intubation of trachea, there was a significant instability with hypotension and hypoxia. Boluses of ionotrophs and pressers were needed. No chest compression was performed. Patient was resuscitated and a TEE was requested. This is a 4-chamber view, and at times you see the aortic valve here, so it may be close to 5-chamber view. We see the amplaster device here. There is apical displacement of septal leaflet of tricospid valve, and it is very significant. We see the severe dilatation of anatomical right ventricle. A major part of this is atrialized right ventricle and does not participate in RV volume or RV systolic function. There is bowing of interventricular septum toward the left ventricular outflow tract in systole, and this is secondary to dilatation of atrialized portion of right ventricle, and at times could cause LVOT gradient, but this was not the case in this patient. The initial imaging did show a very abnormal tricospid valve as was expected based on Epstein anomaly and significant amount of tricospid valve regurgitation, as you see in this image. Further imaging of tricospid valve conference presence of CBRTR, we see the RVOT portion of right ventricle here, and that quite likely has reduced contractility. This is following administration of inotropes and pressors, and there was some recovery in hemodynamics at this stage. Considering that there was hypoxia, the TE team was looking for any possible shunt, so color imaging of interatrial septum and device closure of previous ASD was done, and this was suspicious for presence of right-to-left shunt. So we performed a staining contrast study, and this was strongly positive. There was early appearance of significant amount of contrast in the left atrium, so from this image, we concluded that there must be a communication in the inferior part of amplizer device between the right atrium and the left atrium. This image shows a severely tattered posterior leaflet of tricospid valve and significant degree of tricospid valve regurgitation. Looking at short axis view of LV and RV in transgastric view, massively dilated anatomical RV. This is not functional RV, and most of very dilated anatomical right ventricle is actualized. Functional RV is small, at least moderately hypokinetic. LV cavity was small, and function was as mildly reduced. Based on these TE images, it was suggested that hypotension secondary to vasodilatation following induction of general anesthetic and also increased pulmonary vascular resistance and secondary to positive pressure ventilation and PEEP caused a relative increase in right atrial pressure compared to left atrial pressure and increase in right to left shunt through previously undiagnosed interatrial septal defect and then hypoxia as a result. Interventions such as administration of ionotrophs and pressers would improve systemic vascular resistance and would reduce the shunt and overall help the recovery of hemodynamics. The team decided on cancelling the surgery. A patient was stable on pressers. She was unevenfully extubated in ICU and was referred back to cardiology for further investigations and interventions. Following these patients had a couple of investigations including a trans thoracic echo. They performed a saline contrast study without vial salva, which was positive with early appearance of contrast in the left atrium. This showed that there was a right to left shunt. The rest of the exam was unchanged. LV function, RV function was as before and there was moderate to severe TR, which was unchanged from preoperative exam. Patient also had left and right heart cat. There was no evidence of significant left to right shunting. There was evidence of a small residual leak at the inferior aspect of the ASD device. She had normal right sided cardiac and pulmonary pressure and there was normal left sided feeling pressures as well. So there was no obvious indication of device closure of HEO septal defect. Following these investigations we accepted the patient for surgery again. We agreed to try laparoscopic technique with low intraabdominal pressure and low threshold to convert to open technique if laparoscopy is not tolerated. We thought the anesthetic care should focus on maintenance of SVR and use of oppressors as needed. Also use of inotropic support as indicated and this could be guided by TEE which could be started immediately following intubation of trachea. We also recommended maintenance of low ventilatory pressures as much as possible. Currently the patient is under observation and has not agreed to go ahead with the procedure yet. Thank you very much for your attention.