Overall Bottom Line
- Bleeding due to portal hypertension remains a significant cause of morbidity and mortality in cirrhotic patients.
- Portal hypertension can lead to bleeding from esophageal varices, gastric varices, portal hypertensive gastropathy and ectopic varices.
- Several methods are employed to control active bleeding from portal hypertension including pharmacological, endoscopic, radiological and surgical.
- In most centers the initial approach to portal hypertensive bleeding should include adequate resuscitation, reduction of portal pressure using somatostatin analogues, and an attempt at endoscopic therapy.
- Primary and secondary prophylaxis of portal hypertensive bleeding are established treatment strategies to improve outcome.
Section 1: Background
Definition of disease
- Portal hypertensive bleeding is bleeding from porto-systemic collaterals that develop in patients with portal hypertension, most commonly in the setting of cirrhosis.
- The pressure gradient between the portal and hepatic veins needs to be above 12 mmHg for bleeding to occur from esophageal varices.
Disease classification
- There is no strict disease classification for portal hypertensive bleeding but it can be classified according to the cause of bleeding since management will differ.
Incidence/prevalence
- Variceal bleeding complicates cirrhosis in approximately one-third of cases.
- Despite improvements in management, mortality from an episode of variceal bleeding is 20–30% and the risk of rebleeding without treatment within the next year is 60–70%.
Economic impact
- The cost of managing variceal bleeding, particularly when there is failure to control the initial event and in patients with more severe liver disease, is high.
- Average inpatient costs range from $15 000 to $25 000 and up to $45 000 when more than one procedure is required to control bleeding.
Etiology
- Bleeding from portal hypertension is due to esophageal varices, gastric varices, portal hypertensive gastropathy or ectopic varices.
- Acute upper gastrointestinal bleeding in the patient with portal hypertension is usually due to esophageal or gastric varices.
- Portal hypertensive gastropathy bleeding typically presents with more insidious bleeding with melena or anemia.
- Ectopic varices can present with occult gastrointestinal bleeding depending on the location or rectal bleeding if in the colon or rectum.
Pathology/pathogenesis
- The normal portal pressure is less than 5 mmHg. The portal system has the capacity to double the flow without an increase in pressure (as occurs after eating) but obstruction to flow leads to portal hypertension. This most commonly occurs at the sinusoidal level from cirrhosis but can occur pre-sinusoidal (portal vein thrombosis or portal fibrosis) or post-sinusoidal (Budd–Chiari syndrome or sinusoidal obstruction syndrome). An increase in portal inflow is also seen in cirrhosis due to splanchnic arteriolar vasodilation (and occasionally an arterio-portal fistula can lead to portal hypertension).
- The rise in portal pressure leads to the development of porto-systemic collaterals that decompress the portal system. These can occur in several locations but typically in the distal esophagus and proximal stomach.
- A pressure gradient of >12 mmHg between the portal and hepatic veins is required for the development of bleeding from esophageal varices.
Predictive/risk factors (for esophageal variceal bleeding)
- A prognostic risk index was developed using a combination of Child’s class, variceal size and appearance that can predict the risk of initial variceal bleeding.
| Risk factor | Comment |
|---|---|
| Size of varices | Increased risk correlates with increased size |
| Endoscopic appearance of varices | Red wale signs, cherry red spots and hematocystic spots increase the risk of bleeding |
| Severity of liver disease (Child’s score) | Child’s C have higher risk of bleeding |
| Variceal pressure | Not routinely measured but directly correlates with risk of bleeding No bleeding at pressure ≤12 mmHg |
Section 2: Prevention
Clinical Pearls
- Non-selective beta-blockers such as propranolol and nadolol decrease the risk of initial variceal bleeding compared with placebo.
- Endoscopic band ligation of esophageal varices (grade 2 or larger) is as effective as non-selective beta-blockers in preventing variceal bleeding.
Screening
- All patients with cirrhosis should undergo endoscopy to assess for portal hypertension and determine the severity and risk for bleeding.
- In patients with compensated cirrhosis without varices endoscopy should be repeated every 2–3 years.
- In patients with compensated cirrhosis and small varices without stigmata of high risk for bleeding (red wale signs, etc.), non-selective beta-blockers can be used although the benefit is not clear. If pharmacotherapy is not used, endoscopy should be repeated annually.
- In patients with compensated cirrhosis and medium/large (grade 2 or higher) varices, or small varices with stigmata of high risk for bleeding, non-selective beta-blockers should be used. Repeat endoscopy is not required. In patients intolerant of beta-blockers (or contra-indicated), band ligation of esophageal varices can be performed and continued until varices are eradicated and then annual endoscopy should be performed.
- In decompensated cirrhosis the presence of varices should lead to the use of non-selective beta-blockers and repeat endoscopy is unnecessary. In patients intolerant of beta-blockers (or contra-indicated), band ligation of esophageal varices can be performed and continued until varices are eradicated and then annual endoscopy should be performed.
Primary prevention
- Most of the studies examining primary prevention deal only with esophageal varices.
- Patients with cirrhosis and other causes of portal hypertension should undergo periodic endoscopy (every 2–3 years in Child’s A patients, annually in Child’s B or C patients) to be screened for the presence of varices.
- If significant varices are present (grade 2 or higher for esophageal varices), a non-selective beta blocker (propranolol, nadolol or carvedilol) should be used if no contra-indication to decrease the portal pressure. The dose should be titrated to achieve a resting heart rate of 55–60 beats/minute.
- Non-selective beta-blockers reduce the risk of first variceal hemorrhage by up to 50% although an overall survival benefit has not been consistently demonstrated.
- Non-selective beta-blockers do not prevent the formation of varices although they may reduce the risk of smaller varices progressing to larger varices.
- Several meta-analyses have demonstrated endoscopic therapy using band ligation is at least as effective as beta-blockers for prevention of first variceal bleed and can be used as an alternative in situations where beta-blockers are contra-indicated.
Secondary prevention
- After an initial episode of variceal bleeding, one third of patients will rebleed within the next 6 weeks.
- Endoscopic therapy using band ligation at periodic intervals (typically 1–6 weeks) until esophageal varices have been eradicated is effective in preventing rebleeding.
- Non-selective beta-blockers reduce the risk of recurrent variceal bleeding by 30–40% and reduce the risk of death by 20%.
- The combination of endoscopic band ligation and non-selective beta-blockers is more effective than either modality alone in preventing recurrent variceal bleeding.
- TIPS is effective at preventing recurrent variceal bleeding (decreasing the risk down to 10–20%) and can be used as salvage therapy when bleeding cannot be controlled with endoscopy. However, it is associated with the risk of hepatic encephalopathy.
- A recent study has suggested that early TIPS (within 72 hours) in patients with cirrhosis and acute variceal bleeding who were treated with endoscopic therapy had lower rebleeding rates and decreased mortality.
- Surgery (usually distal splenorenal shunt) reduces the risk of recurrent variceal bleeding compared with endoscopic sclerotherapy but is limited to well-compensated patients, does not improve survival, and needs an experienced surgeon, severely limiting its role.
- Liver transplantation is the definitive treatment to prevent recurrent variceal bleeding in appropriate cirrhotic patients.
Section 3: Diagnosis
Clinical Pearls
- The patient presenting with portal hypertensive bleeding is typically not a diagnostic dilemma as the presentation is commonly with hematemesis or melena in a patient with underlying cirrhosis.
- Examination findings can confirm the presence of cirrhosis and portal hypertension with palmar erythema, spider nevi and splenomegaly among other stigmata of chronic liver disease.
- The initial investigations depend on the presentation. Patients with significant blood loss and hypotension need to be resuscitated, usually in an intensive care setting. Blood tests may show a low hematocrit, coagulopathy and thrombocytopenia. Emergent endoscopy can make the diagnosis and affords the opportunity to provide therapy.
- Other causes of portal hypertensive bleeding can present more insidiously with anemia in the case of portal hypertensive gastropathy, occult gastrointestinal bleeding for small bowel varices and rectal bleeding with rectal varices.
Differential diagnosis
| Differential diagnosis | Features |
|---|---|
| Any other cause of gastrointestinal bleeding | History demonstrates no risk factors for chronic liver disease but might indicate risk factors for other causes, e.g. non-steroidal anti-inflammatory drug use leading to peptic ulceration Examination does not show stigmata of chronic liver disease Endoscopy should differentiate the cause of bleeding but some other causes are also common in patients with liver disease such as peptic ulceration |
| Non-cirrhotic portal hypertension can lead to identical causes of bleeding | History may not demonstrate risk factors for cirrhosis but there may be risk factors for non-cirrhotic portal hypertension such as ethnicity (for schistosomiasis) Examination may be similar with palmar erythema, spider nevi, ascites and splenomegaly Endoscopy will show the same features |
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