Hello, everyone. Welcome back to Stay Current podcast. I'm Cecilia Jigena and I'm Ellen and Cisco, and we are research fellows at Cincinnati Children's Hospital. And along with Stay Current, we are sharing knowledge to improve child health around the globe. In this podcast, we are going to talk about portal hypertension, and for that, we have Doctor Alex Bondak, a pediatric surgeon from Cincinnati Children's Hospital. We are going to cover epidemiology, classes, clinical presentation, diagnosis, and treatment. So if you're looking for something specific, make sure to check the time stamps in the description. They'll take you to wherever you need. So let's get started. Portal hypertension as a hepatic venous portal wedge pressure of greater than 5 millimeters of mercury. Portal hypertension develops when resistant to portal blood flow increases. So remember, the portal system is the end system to the liver, and at some point the portal vein becomes a. or high resistance, so you get backfilling or back pressuring of this entire system. Great. So what are the causes of portal hypertension? For portal hypertension, we can divide the causes in prehepatic, hepatic, or posthepatic. Where we have to be more mindful in kids is prehepatic. So for the prebotic conditions, we have, the portal vein thrombosis, usually idiopathic. We always chase it with a hematology workup, and I would tell you the vast majority of those kids have no predisposition to clotting, as well as some parasitosis, drug therapy, or tumors. Within the hepatic conditions, biliary atresia is the number one cause worldwide of pediatric liver transplantation. Cystic fibrosis, liver disease just for interest. and others as chronic viral hepatitis or corollary disease. Post-obstructive, it can be anything, most commonly by charri, which is thrombosis of the hepatic venous outflow or the proximal cava, congestive heart failure, so structural heart disease usually in patients with single ventricle physiology to the point where they develop the well described entity Fontan associated liver disease. Perfect. So let's move on to clinical manifestations. The most common clinical manifestation is GI bleeding due to esophageal viruses, but it can also occur because of gastric viruses or hypertensive gastropathy. And they demonstrated that once you have that first episode of GI bleeding, your risk of complications, not Only rebleeding, but also ascites, SVP, things like associated with end stage liver disease or cirrhosis, will continue to occur. Are there some patients with higher risk for bleeding? From some reports, you have a higher incidence with a wedge pressure of greater than 12. And also there are certainly comorbid conditions, and that's a lot of what we find. Fight Against you're talking about growth failure, fat malabsorption, neurological consequences, all kinds of other things. Also, other manifestations of portal hypertension are splenomegaly, a part of pulmonary syndrome, a part of renal syndrome, spontaneous peritonitis due to bacterial translocation, and hepatic encephalopathy. Perfect. So now that we know the presentations, how can we diagnose or assess portal hypertension? So from an imaging perspective, ultrasound, as you all know, is our go to for the liver. OK, so to start, we have to do an ultrasonography with Doppler. The nice portal venous flow, it's usually the thickest thing in the hyalum. Good flow is coming at you. It's a monophasic, low resistance entities. And remember, portal vein velocity decrease in severe portal hypertension as intrahepatic resistance increase. And people have done studies to demonstrate. The pressure measurements or the density of the liver or the speed at which you have to send your radio waves through it to stratify how sick your liver is. Cross-sectional imaging is usually critical because, as you all know, if you have long-standing portal hypertension, you're also at risk for atocellular carcinoma. And then what we have often used is a portography where he actually just sticks the spleen directly and injects. And another test you can do is stringent elastography, measuring in kilopascals for clinically significant portal hypertension. And a pressure greater than 15 is highly suggestive of portal hypertension. And finally, you'll need an endoscopy to evaluate viruses. And then there are ways to grade. The varices, there's 3 separate ones. So how do they measure varices at Cincinnati Children's? They'd rate them 1 through 4, where 4 means the entire esophagus is essentially collapsed. 3 is 2/3, 2 is only 1/3, and then 1 is usually just whales, little ridges under the mucosa. So for the assessment of a patient with portal hypertension, you'll need. Labs, ultrasonography with Doppler, endoscopy, and clinical evaluation. OK, time to talk about treatment. Then the medical management is prevention of bleeding, no bleeding episodes, so that's primary prophylaxis, right? And then prevention of rebleeding is secondary prophylaxis. So those are the definitions. We do not primarily prophylax our patients. OK, so what do we have to do in an episode of acute bleeding? You stabilize them, you put them in the ICU, you do a blood product resuscitation, and then actually in the new Bovida 7. Uh, recommendations they recommend against factor 7 actually. Thevino is a place in Italy where a group of experts have their consensus conference to come up with guidelines for the management of these patients. The recommendations are made for adult patients, except for Bobino 5. We put them on IV proton pump inhibitors that we stop as soon as the bleeding stops, and then we can use octreotide. If you have oesophageal varices, it can work pretty well. Gastric varices or portal hypertensive gastropathy, not so much. And then we use non-selective beta blockade. Although propranolol was widely used, data suggests that carvedilol might be a better option. And remember to start all of these patients into IV antibiotics because of the high risk for bacteria translocation. Once the patient's stable and the bleeding stop. We have to go on secondary prophylaxis with surgical interventions. So essentially when you're trying to surgically address portal hypertension, you have to know. What the QD of need is. Now, the etiology of portal hypertension and the underlying disease, liver disease are critical, right? We don't shunt patients who have end-stage liver disease and transplant those patients. Clear, patients with end-stage liver disease go to liver transplant. Patients without it can benefit from a shunt, but what type of shunts are the most common? There are two types of spleen and renal shunts. There is the distal spleen or renal shunt or the worn shunt, which is where you take the distal splenic vein, divide it, and drop the distal end into the left renal vein. And then there's a central spleen or renal shunt where you essentially go side to side from the main body of the splenic vein to the renal vein. And again, your risk of encephalopathy goes up, your risk of complications goes up. And then you're talking about central shunts like a meso cable H type, where you take a ringed vortex, usually like 6 or 7 millimeters, and you'd have to drop it from the SMV and bring it around the mesentery and go into the cava. And of course, we have the mesorex shunt or mesopportal shunt. That involves the placement of an autologous venous graft from the mesenteric vasculature to the left intrahepatic portal vein in the rex recess. The rex shunt is physiologic flow. That is by far the number one thing we would like to do if possible, and tips. Which is a support a systemic shunt, and how do we decide which shunt to do? OK, so again, back to the original point. Where is the point of obstruction? Anatomy is crucial. So for prehepatic disease, a missorex shunt for. disease, a distal splenorenal shunt, central splenorenal shunt, or a mesocava H-type shunt, and for postoppatic H-type mesocava shunt, tips, or atrial cable shunt. OK now that we've almost covered everything we need to talk about complications and follow-up. Complications, uh, rebleeding, uh, if the shunt goes down, encephalopathy, shunt thrombosis or stenosis, chronic liver failure, or hypersplenism. And finally, how do we follow them? Long term follow up is important. Every 6 months ultrasounds, eventually every year ultrasounds, but we'll try to get platelet counts because we have found that if a platelet count starts dropping, there's probably something wrong with the shunt. And it's important to follow up with the hepatologist to assess liver failure, nutrition status and development. What a great session. Let's summarize. Portal hypertension is a hepatic portal venous wedge pressure higher than 5 millimeters of mercury. The causes can be divided into three main groups prehepatic, hepatic, or post-hepatic. As a prehepatic condition, most common is portal venous thrombosis. The primary hepatic cause is biliary atresia, and is post hepatic, one of the most common is Batchiari syndrome. Its most common clinical manifestation is GI bleeding. To assess and diagnose portal hypertension, we need a liver ultrasonography with Doppler and other tools such as cross-sectional imaging, transient allastography, and varices need to be assessed by endoscopy. The treatment is divided in three groups. Primary prophylaxis is not typically recommended. Acute bleeding treatment, and secondary prophylaxis might consist of doing a shunt to lower the portal hypertension and decrease the chances of re-bleeding. Awesome. There you have it, our podcast on portal hypertension with Doctor Alex Bundo. Thanks for listening and don't forget to follow us on social media. Subscribe to our YouTube channel and download the Stay Current app for more content. I'm Cecilia Jigena and I'm Ellen and Cisco. And we are research fellows at Cincinnati Children's Hospital. And along with Stay Current, we are sharing knowledge to improve child health around the globe.
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