31.1  Commentary

The case presented illustrates several important points:

•Prophylactic management of gastroesophageal varices, strictly speaking, is prior to the first bleeding episode. The risk of bleeding in a patient with cirrhosis is approximately 30%. Once they have had one bleeding episode, the risk of rebleeding rises to 75% without active therapy. Non-cardioselective beta-blockade with propranolol or nadolol is the preferred treatment for true prophylaxis for medium or large size varices. [Q1: C]

•Acute variceal bleeding is an emergency situation with a high mortality if not appropriately managed. Appropriate monitoring, pharmacologic therapy, and endoscopic diagnosis and treatment are the mainstays of treatment of an acute bleeding episode. It is a very small percentage of patients who do not have their bleeding controlled with the above measures and come to an emergency decompression. [Q2: A, D]

•The evaluation of the patient after an acute bleeding episode should assess the varices (endoscopy), the vascular anatomy (ultrasound and angiography) (Figs 31.1–31.4), and the liver disease (Child’s class and MELD score). [Q3: A, B, C, D, E]

•When a patient has had an acute bleeding episode, their risk of rebleeding is over 70% if they have no specific treatment. The initial approach to treatment is to reduce the portal hypertension with a non-cardioselective beta-blocker, and to deal with the bleeding varices directly with endoscopic therapy. The majority of patients do not need variceal decompression at this stage. If the patient obviously has advanced to end-stage liver disease, a transplant evaluation is in order, and appropriate candidates should move forward with that treatment [Q4: B, D]

•When patients have recurrent bleeding through first-line treatment they may need decompression of their gastroesophageal varices. Surgical therapy will do this well in 95% of patients, while the success rates of radiologic shunts in the literature are not this high. Decompression of varices does not improve the survival of patients compared to other first-line treatment options. Liver transplant provides excellent variceal decompression, but its use is dictated by end-stage disease rather than variceal bleeding. [Q5: B]

31.2 

General Considerations

The major complications of portal hypertension are variceal bleeding, ascites, and progressive hepatic dysfunction. Ascites and encephalopathy are signs of decompensation, and as a general guideline, are only effectively managed by liver transplant. Not all patients with these clinical endpoints may be suitable candidates for transplant. In contrast, variceal bleeding can occur in patients who have well-preserved liver function and therefore have a wider range of treatment options available.

The etiology of portal hypertension may be presinusoidal, as in portal vein thrombosis; sinusoidal, as in cirrhosis; and rarely, post sinusoidal, as in Budd-Chiari syndrome. Much the most common etiology in the USA and Europe is cirrhosis, with approximately 90% of patients having this etiology. The evaluation of the patient with suspected portal hypertension includes an endoscopy to assess size and extent of varices with risk factors for bleeding. Larger varices with red color signs are at increased risk of bleeding or of rebleeding. Laboratory tests should assess liver function, and overall disease status. Non-specific tests include bilirubin, prothrombin time, albumin, and liver enzymes. Recently documented is the importance of serum creatinine in assessing overall severity of disease and prognosis. ThetwostandardmethodsforassessingthisaretheChild–Pughscore(Table 31.1),andthe Model for Endstage Liver Disease (MELD score – Table 31.2). Other laboratory studies that are important relate to the etiology with hepatitis panels, alpha-fetoprotein as a marker for hepatocellular carcinoma, and specific markers for metabolic diseases such as hemochromatosis and Wilson’s disease.

Imaging studies are important in evaluation, with ultrasound used to assess the liver morphology, and Doppler evaluation for liver vasculature. Patency of the main vessels and direction of flow can be assessed well with Doppler ultrasound. Angiography is still indicated for patients being considered for surgery. Accurate assessment of the splenic, portal, and left renal veins is important for DSRS, and may further elucidate details that are not seen on ultrasound. Liver biopsy is occasionally indicated in some patients for clarification of etiology and to delineate the activity of the liver disease process.

Classification: A, 5–6 points; B, 7–9 points; C, 10–15 points

INR international normalized ratio

Table 31.2  MELD score for stratification of liver disease severity

Score = 0.957 × loge creatinine (mg/dL)

+0.378 × loge bilirubin (mg/dL)

+1.120 × loge INR

INR international normalized ratio

Management of portal hypertension falls in to three broad groups:

•Prophylactic treatment.

•Management of an acute variceal bleed.

•Prevention of recurrent variceal bleeding.

Prophylactic treatment is indicated for moderate or large size varices to reduce the risk of an initial bleed. Varices are present in 30–60% of patients with cirrhosis. Thirty percent of patients with varices will bleed from them. After an initial bleed, 20–50% will rebleed in the first week, and 75–80% will rebleed within a year. The mortality of an acute bleeding episode is approximately 25%. To reduce the risk of this initial bleed, the goal is to reduce portal pressure to <12 mm Hg or by 20% from the baseline. This is best achieved with a non-cardioselective beta-blocker (propranolol, nadolol).1 Other treatments, such as endoscopic therapy, TIPS, or surgical shunt are not indicated for prophylaxis. There are currently further ongoing trials looking at band ligation for patients with large varices where this might be an appropriate method for prophylaxis.2 [Q1: C]

Management of an acute variceal bleed involves resuscitation, pharmacologic reduction of variceal pressure, and endoscopic therapy.3 Resuscitation requires careful monitoring and enough blood volume and transfusion to maintain blood pressure, but not over-transfuse and precipitate a vicious cycle of further bleeding. Octreotide is the drug of choice for pharmacologic pressure reduction and is given as a continuous infusion of 50 mg/h. Endoscopic therapy is combined with endoscopic evaluation and it best done with banding of varices if visibility is adequate. Occasionally, direct sclerotherapy injection may be required to stop acute bleeding. In the <10% of patients who do not have their acute bleeding controlled with such measures, or in whom early significant rebleeding occurs, early decompression is occasionally required. This can best be achieved with TIPS at the current time.

Prevention of recurrent variceal bleeding has to take in to account the risk of rebleeding, and the underlying liver disease. First-line treatment to prevent rebleeding is with a course of endoscopic banding in conjunction with pharmacologic therapy to reduce portal pressure with non-cardioselective beta-blocker.3 This combination will reduce the risk of rebleeding to approximately 20%. Banding has been shown to be considerably better than sclerotherapy in terms of bleeding control and fewer complications. However, mortality is not significantly different in the randomized trials that compared banding to sclerotherapy. Concurrent with this first-line treatment, assessment and management of the underlying liver disease is important. At this time, assessment as to whether the patient is headed for transplant now or in the foreseeable future is important. If this is the case, more invasive therapies are precluded, transplant evaluation should be completed, and the patient should be appropriately listed. In this population, transplant has significantly improved the outcome of patients with Child’s class C cirrhosis who have end-stage disease, and have variceal bleeding.

For better-risk patients who have recurrent bleeding through first-line treatment, variceal decompression may be indicated. The current options are with a radiologic shunt (TIPS).4 or with a surgical shunt such as a DSRS,5 or some type of portacaval shunt. The literature data indicates that the rebleeding rate with TIPS is in the 15–20% range.

Rebleeding with surgical shunts is in the 5% range. However, TIPS can be achieved in a much less invasive fashion compared to the major surgery required for a surgical shunt. Two randomized trials have compared TIPS to surgical shunt. Rosemurgy et al.6 compared TIPS to an 8-mm H-graft interposition portacaval shunt. They showed notably lower rebleeding in the surgical shunt group, significantly less need for transplant, but no difference in mortality. They concluded that surgical shunt was preferable to TIPS. Henderson et al.7 have compared TIPS to DSRS in Child’s class A and B patients. They showed no significant difference in rebleeding between DSRS (6%) and TIPS (9%) in this trial; however, the TIPS group had an 82% reintervention rate to maintain decompression and this excellent control of bleeding. The encephalopathy rates were not significantly different in thetwogroups,andneitherwassurvival.Theconclusionfromthistrialisthatbleedingcan be equally efficaciously managed with TIPS or DSRS with no difference in survival or encephalopathy; however, significantly more reintervention is required in patients managed with TIPS. This trial is summarized in Table 31.3.

Have covered stents improved TIPS outcome? A multicenter prospective randomized trial in Europe8 has shown a significantly lower dysfunction with covered stents, with a particular advantage in control of ascites in that trial. Survival was not significantly different with covered or uncovered TIPS stents (Table 31.4).

TIPS Transjugular intrahepatic portal systemic shunt, DSRS distal splenorenal shunt

Table 31.4  Data for covered versus uncovered TIPS – European trial8

TIPS Transjugular intrahepatic portal systemic shunt