Wonderful. Great cases. Thanks for having me in this webinar. Looking forward to the rest of the talks. I have nothing to disclose. So our case was an 11-year-old male with hypoplastic left heart syndrome, MSAS. Some noteworthy interventions. There were several biventricular recruitment surgeries, such as EFU resection, mitral valve repair, and ultimately aortic valve replacements. But ultimately ended up with a lateral tunnel Fontan palliation. In the lateral tunnel, that's going to be key to this approach. At four years of age, had a bioprosthetic aortic valve replacement. At that time, it was complicated by complete heart block. And also noteworthy, there was a stent implanted in the Fontan baffle at some point. EP history is relevant for, as the result of the complete heart block, a nippocardial biventricular dual chamber pacemaker was placed. RA, RV, and LV leads. This was at four years of age. So far, so good. Nine, five years later, just a generator change. Then this child presented as a failing Fontan. That year, had elevated thresholds on the V leads. And during another surgery, which involved a sternotomy, which was paired with some new valve work, two new anterior RV leads were placed. One year later, this child was listed for heart transplant. And later that year, those leads had elevated thresholds and so underwent a thoracotomy for two new posterior ventricular leads and a generator change. And just two months later, presented with syncope with elevated thresholds on those V leads. So three failed epicardial leads and a failing Fontan, who's listed for transplant, which made this patient a candidate for this transvenous approach. So the procedure was as follow. We accessed the right internal jugular vein, less of clavian vein, veins from below, ultimately for the hemodynamics of the case, but not necessary for the actual procedure, with the exception of arterial monitoring. Peparin was given, one time 50 units per kilogram bolus. ACTs ran between 200 and 250 for the case. The patient had a mixed venous of 75 and AO of 95 on room air and elevated Fontan pressures and wedge pressures in keeping with previous procedures, previous caths in this child. So there's probably two big learning points from this technique, and one of which is just going to be demonstrating this angio. Here, you'll see the right internal jugular vein access and less of clavian vein access. You'll see previous coils and pacer leads. Here's the Fontan baffle stent, and here you have the aortic valve, the bioprosthetic aortic valve here. And so we're going to show this wedge injection from below, and you can see the stent's well opposed, widely patent, really nothing, no right to left shuntings up here. And then in the superior baffle, it's a little bit more bulbous. And we'll see as the patch met the atrial appendage, there's just a little bit of right to left there. So it's a very, very trivial baffle leak there. But you'll see that this is part of the baffle, and this is all actually intracardiac. And so what's important here is on levophase, and I'll wait for it and then pause it. And when you see that atrium, really the distance from this stent to this bioprosthetic valve is going to be the atrium. So we'll be, in our approach, safe to enter this space here. Now let's look on the lateral. And here, again, you'll see just focusing on the superior baffle, which is going to be our target. Let's see if I can freeze this on levophase here. And now we have levophase here, and you see that the poster atrium is here, and the atrium extends all the way up to the stent, close to the anterior portion of the stent. And so you know, of course, if we're going to be in between the stent and the valve on the AP, and then we have all this distance posteriorly, but leading up to the stent here, we're going to be safely entering the atrium. So whenever I'm doing transeptals in unique anatomy, and I like to probe at first just to get a sense of how the transeptal needle may behave. I think clearly it was a nice straight shot from above, and here's a six multipurpose catheter. You know, just kind of characterize the anatomy here, the superior baffle. This is, in fact, outside the stent, not inside. And then here on the lateral again. So you know, a puncture here is going to, based on what we showed in the last slide, is clearly going to end up inside the heart. This is, in fact, just mainly a transbaffle puncture is what we're dealing with here. And so that was performed. This is the adult transeptal needle, Broca-Brown needle. We ended up aiming a little bit more medial in between those two structures on the AP, and then aiming a little bit more anteriorly. As we know, the goal was to get a ventricular lead in here, and so we're aiming more towards the atriocular valve. So that system was able to be advanced, and a wedge catheter was, I'm sorry, the needle was removed and a wire, 35 wire was advanced through the transeptal system. And then that was followed by a wedge catheter, which went to the RV, and then we looped an amplizer super stiff into the RV in exchange for a Mullen sheath. And here we're simply dilating the track with a 5 millimeter high pressure balloon. And then this is the second take home point. I fortunately didn't store this fluoro. I would have if I knew I was going to be presenting at a webinar. But what we've learned is when we need to cross the atrial septum with more than one sheath, for instance, if we need two balloons to work on a pulmonary vein, for instance, what we found is if we make a small hole in the atrial septum and leave a stiff wire, we actually can follow that track pretty easily from another access point. And so what we did is we had a JL coronary catheter, which we cut, which as it coursed across the subclavian down the IGA, and we cut it so it directly pointed at the hole that we made. And we were able to easily pass a 35 floppy tip torque wire right alongside that super stiff wire. And so made the hole and then followed it, that track, from another access. And over that torque wire, we then re-advanced the 7 French wedge catheter across that hole. And now we have access in the atrium. That was placed in the RV. And a super stiff wire was placed through that. And that was really just so we could really then advance the Medtronic 7 French sheath across that hole. And the balloon was necessary to help get that sheath across into that atrium. Here, the lead is now placed in the RV. And here, we're just documenting what kind of shunt burden we left. You can see there's a bigger, more of a blush, more of a right to left blush coming through there from the 5 millimeter hole that we made. We could see this by echo. And ultimately, that went away. So that shunt resolved. On follow up, this patient received Coumadin with an INR of 2 to 3, not 2.5 to 3.5, so perhaps we were lucky. But was already on Coumadin for the failing Fontan in the aortic valve. Two CTs were performed looking for thrombus. And none was seen on the Pacer lead or the Fontan throughout the Fontan circuits in those strokes. And then this child received a transplant 17 months later after the procedure. I believe that's it. Yes, thank you.

case study

hypoplastic left heart syndrome

surgery

pacemaker placement

transvenous approach