Portal HTN: The portal vein forms from the confluence of the superior mesenteric and splenic veins. Portal pressure can rise because of increased outflow resistance or increased portal inflow. Increased resistance can be due to (1) prehepatic causes (portal vein thrombosis); (2) hepatic causes (cirrhosis, inflammation with hepatocyte swelling, mass, regenerating nodules); or (3) posthepatic causes (hepatic vein obstruction; see Budd-Chiari syndrome p 267). Increased portal inflow results from peripheral vasodilatation and the hyperdynamic circulation of cirrhosis due to an imbalance of vasodilators and vasoconstrictors.

Varices and bleeding risk: In portal HTN, the increase in pressure causes the opening of collateral vessels. Most of these vessels are in the retroperitoneum. The most troublesome collaterals are veins intrinsic to the distal esophagus and proximal stomach that become dilated and tortuous (Lancet 1997;350:1235). As variceal pressure rises, wall tension and the risk of bleeding also rise (Hepatology 2000;32:842). Direct pressure measurements are notreadily available to most clinicians, and risk must be estimated by other criteria. Three clinical criteria are used to predict risk: (1) variceal size, (2) Child-Pugh grade (p 31), and (3) the presence of red wales (endoscopically identified longitudinal, dilated venules on varices that look like whip marks) (Nejm 1988;319:983). Varices are called size F1 if they are small and straight, F2 if enlarged, tortuous, and occupying less than one-third of the lumen circumference, and F3 if coiled and occupying more than one-third of the circumference of the lumen (Nejm 1988;319:983). Small varices are those ≤5 mm (Am J Gastro 2007;102:2086).

Unusual varices: Isolated gastric varices can occur with splenic vein thrombosis (Am J Gastro 1984;79:304). Ectopic varices can form in the duodenum, in adhesions to the abdominal wall, in surgical anastomoses, and at ostomies (Hepatology 1998;28:1154).

Portal hypertensive gastropathy (PHG): (Am J Gastro 2002;97:2973) PHG is a lesion seen in the stomach of pts with portal HTN. Microscopically, it represents an area of microvascular ectasia and perivascular fibrosis. Endoscopically, it is recognized as multiple small erythematous areas outlined by subtle lines giving a cracked sand or snakeskin appearance to the mucosa. In more severe disease, there is oozing and more prominent red spots, and lesions may be seen in body, fundus, and antrum. In the largest series followed endoscopically over 3 yr, acute bleeding was rare (2.5% over 3 yr), and chronic bleeding was seen in 11% (GE 2000;119:181). Disease parallels the severity of portal HTN and may worsen or improve spontaneously.

Prevention of a first variceal bleed: Pts with cirrhosis should have EGD when first diagnosed. If there are no varices in a well-compensated cirrhotic (Child’s A), the EGD is repeated in 3 yr or sooner if there is a decompensation of the cirrhosis. In decompensated cirrhotics (Child’s B/C), EGD should be done annually if the initial exam is negative for varices. Beta-blockers are notrecommended to prevent varices from developing. For small varices that have notbled: (1) Beta-blockers are used if the pt is Child’s B/C or if there are red wales on the varices; (2) if the pt is Child’s A and there are no red wales, beta-blockers can be used to prevent worsening of the varices, but the benefit is uncertain; and (3) pts nottreated should have repeat EGD in 2 yr, earlier if hepatic decompensation occurs. The nonselective beta-blockers (propranolol and nadolol) reduce the incidence of a first bleed by about 45% (for a meta-analysis, see Ann IM 1992;117:59). Ideally, pts would have the dosage adjusted on the basis of portal pressure gradients, but in practice, the dosage is adjusted until resting heart rate is reduced to 55-60. The starting dose of propranolol is 20 mg po bid, and that of nadolol is 20 mg po daily. For medium/large varices that have notbled: (1) Either beta-blockers or variceal ligation should be used if the pt is Child’s B/C or red wales are seen; (2) beta-blockers are preferred in Child’s A pts with no red wales, and ligation is reserved for those who cannot take or do nottolerate beta-blockers.

Rx of acute bleeding: Fluids are given to bring the blood pressure in the 90- to 100-mm Hg systolic range and the pulse under 100. Pts are transfused to a Hgb of about 8 gm/dL, avoiding excessive transfusion. Higher levels of transfusion increase portal pressure and the risk of rebleeding and death (Am J Gastro 2009;104:1802). Endoscopic control of bleeding is the rx of choice for acute hemorrhage. EVL is preferred over sclerotherapy because eradication occurs more quickly, with lower mortality and fewer complications (major RCT, Nejm 1992;326:1527; meta-analysis, Ann IM 1995;123:280). Octreotide, a somatostatin analogue with a longer half-life, has become the drug of choice for acute bleeding episodes and is given for 3-5 days. The addition of octreotide (50 mcg iv bolus and

50 mcg/hr as a continuous infusion for 5 days) to EVL lowers early rebleeding compared to ligation alone (Lancet 1995;346:1666) or sclerotherapy alone (Nejm 1995;333:555). Prophylaxis against infection (p 263) should be given using ceftriaxone 1 gm iv q 24 in the sickest pts. Gastric varices are difficult to treat, and injection with cyanoacrylate glue has become a widely used initial rx where available (Gastroenterol Clin North Am 2003;32:1079). TIPS is used where bleeding cannot be controlled.

Prevention of recurrent bleeding: Sclerotherapy has been well shown (in 8 trials) to be superior to placebo in the prevention of further bleeding. Ligation has been shown to be
superior to sclerotherapy regarding rebleeding rate, mortality, and complications (meta-analysis, Ann IM 1995;123:280). Beta-blockers are effective in preventing recurrent bleeding compared to placebo (meta-analysis, Lancet 1990;336:153), but in practice most pts have endoscopic obliteration with EVL. The current guideline suggests EVL in combination with beta-blockers as the best choice (Am J Gastro 2007;102:2086).

TIPS: (Gastroenterol Clin North Am 2000;29:387) A transjugular intrahepatic portosystemic shunt (TIPS) is a stent placed between a hepatic vein and an intrahepatic portion of the portal vein. It can be placed in 90% of pts, with a 10% complication rate and a 2% procedure-related death rate (Radiographics 1993;13:1185). Complications include mechanical events during placement (such as bleeding, rupture of the capsule), complications of shunting (encephalopathy in 15% [Gut 1996;39:479] and worsened liver function), and stent-related complications (hemolysis, infection, stenosis). The established indications for TIPS are active variceal bleeding despite endoscopic rx (including a second attempt) and prevention of recurrent variceal hemorrhage in pts awaiting liver transplantation (Jama 1995;273:1824). TIPS may also be useful in refractory ascites and Budd-Chiari (GE 1996;111:1700). A TIPS behaves like a surgical shunt with decompression of the portal system. A TIPS is much more prone to occlusion than a surgical shunt. Occlusion due to clot or stent kinking can occur within weeks in about 3-10% of cases. Recurrent portal HTN from stenosis due to pseudointimal hyperplasia occurs frequently (about 60%). This problem can be treated with repeated dilatations (GE 1997;112:889). Right-sided heart failure is a catastrophe if it develops in a TIPS pt, because the elevated right atrial pressure is transmitted directly to the portal system, causing recurrent varices. As long as the stent is patent, esophageal varices resolve, but fundic varices often do notbecause of splenorenal collaterals or massive splenomegaly excessively feeding the short gastric vessels (GE 1997;112:889). Monitoring with Doppler is commonly done but can miss significant stenoses. Some centers do periodic angiography and/or endoscopy.

Surgical shunts and decompression: (Gastroenterol Clin North Am 2000;29:387) A variety of surgical shunts to decompress the portal system have been described. All shunts reduce bleeding but are accompanied to varying degrees by encephalopathy and worsened liver failure. A side-to-side anastomosis of IVC to portal vein reduces bleeding and ascites, but is prone to clot and makes transplant surgery more difficult. Distal splenorenal shunt (joining a transected splenic vein to the left renal vein) lowers pressure in varices but does notreduce portal inflow, and so ascites is a problem with this technically demanding operation. Esophageal transection with an automated suturing device and other devascularization procedures are notwidely used. These procedures may be alternative salvage strategies (Hepatology 1992;15:403) for those who fail endoscopic rx and TIPS.

Balloon tamponade: Balloon tamponade is effective in controlling hemorrhage in 80-90% of pts, but complications and early rebleeding are common (Scand J Gastroenterol Suppl 1994;207:11). Aspiration pneumonia occurs in 10% of pts and orotracheal intubation is routinely used. The 4-lumen Sengstaken-Blakemore tube has esophageal and gastric balloons and an aspiration port and is most often used. The Linton-Nachlas tube has a bigger gastric balloon and is used for gastric varices. It is important to make sure that the position of the gastric balloon within the stomach is confirmed radiographically to prevent accidental inflation in the esophagus. The tube must be deflated for 30 min q 4-6 hr, and duration of use must be minimized. Generally, tubes are a temporizing
measure used in desperate circumstances when endoscopic and pharmacologic methods fail while awaiting TIPS.

Portal hypertensive gastropathy (PHG): Rx is that of the underlying portal HTN with medical rx, TIPS, or shunt (Dig Dis 1996;14:258). In a small trial, propranolol was effective in reducing bleeding (Lancet 1991;337:1431).