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Portal Hypertension

Portal hypertension may be defined as a portal pressure gradient of 12 mm Hg or greater . Many conditions are associated with portal hypertension, of which cirrhosis is the most common cause.

Portal Hypertension

The portal vein drains blood from the small and large intestines, stomach, spleen, pancreas, and gallbladder. The superior mesenteric vein and the splenic vein unite behind the neck of the pancreas to form the portal vein.

Portal Hypertension

The portal trunk divides into 2 lobar veins.

The right branch drains the cystic vein, and the left branch receives the umbilical and paraumbilical veins that enlarge to form umbilical varices in portal hypertension.
The coronary vein, which runs along the lesser curvature of the stomach, receives distal esophageal veins, which also enlarge in portal hypertension.

Pathophysiology:

Two important factors: vascular resistance and blood flow. Ohm law is V = IR, where V is voltage, I is current, and R is resistance. This can be applied to vascular flow, ie, P = FR, where P is the pressure gradient through the portal venous system, F is the volume of blood flowing through the system, and R is the resistance to flow. Changes in either F or R affect the pressure. In most types of portal hypertension, both the blood flow and the resistance to blood flow are altered.

Increase in vascular resistance

Poiseuille law, which can be applied to portal vascular resistance states. R = 8hL/pr4, where h is the viscosity of blood, L is the length of the blood vessel, and r is the radius of the blood vessel. The viscosity of the blood is related to the hematocrit (HCT).

Increase in vascular resistance

The lengths of the blood vessels in the portal vasculature are relatively constant. changes in portal vascular resistance are determined primarily by blood vessel radius. Because portal vascular resistance is indirectly proportional to the fourth power of the vessel radius,..................

Increase in vascular resistance

........small decreases in the vessel radius cause large increases in portal vascular resistance and, therefore, in portal blood pressure (P = F8hL/pr4, where P is portal pressure and F is portal blood flow).

Increase in vascular resistance

Liver disease is responsible for a decrease in portal vascular radius, producing a dramatic increase in portal vascular resistance.

Increase in vascular resistance

In cirrhosis, the increase occurs at the hepatic microcirculation (sinusoidal portal hypertension). A dynamic component also exists due to: contraction of myofibroblasts, activated stellate cells, and vascular smooth-muscle cells of the intrahepatic veins.

Factors that increase hepatic vascular resistance

Endothelin

Alpha-adrenergic stimulus Angiotensin II.

Factors that decrease hepatic vascular resistance

nitric oxide prostacyclin vasodilating drugs


(eg, organic nitrates, adrenolytics, calcium channel blockers).

Increase in portal blood flow

Second factor: Increase in B. F. in the portal veins, for the splanchnic arteriolar vasodilatation caused by release of endogenous vasodilators (eg, endothelial, neural, humoral). The increase in P. B. F. aggravates the increase in portal pressure and contributes to the formation of an extensive network of portosystemic collaterals that may divert as much as 80% of portal blood flow.

Increase in portal blood flow

Manifestations of splanchnic vasodilatation: Increased cardiac output Arterial hypotension Hypervolemia


This explains the rationale for treating portal hypertension with a low-sodium diet and diuretics to attenuate the hyperkinetic state.

Frequency:

In the US: Incidence is not known. Internationally: Incidence is not known

Mortality/Morbidity:

Variceal hemorrhage: most common complication. Almost 90% of patients with cirrhosis develop varices. Approximately 30% of varices bleed. First episode of variceal hemorrhage is estimated to carry a mortality rate of 3050%.

The medical history from a patient with portal hypertension

Determining the cause of portal hypertension


Presence of the complications of portal hypertension.

Determining the cause of portal hypertension

History of jaundice History of blood transfusions, intravenous drug use (hepatitis B and C) Pruritus Family history of hereditary liver disease (hemochromatosis, Wilson disease) History of alcohol abuse

Determining the presence of the complications of portal hypertension

Hematemesis or melena (gastroesophageal variceal bleeding or bleeding from portal gastropathy) Mental status changes such as lethargy, increased irritability, and altered sleep patterns (presence of portosystemic encephalopathy) Increasing abdominal girth (ascites formation) Abdominal pain and fever (spontaneous bacterial peritonitis [SBP], which also presents without symptoms) Hematochezia (bleeding from portal colopathy)

Physical exam

Signs of portosystemic collateral formation include the following: Dilated veins in the anterior abdominal wall (umbilical epigastric vein shunts) Venous pattern on the flanks (portal-parietal peritoneal shunting) Caput medusa (tortuous collaterals around the umbilicus) Rectal hemorrhoids Ascites - Shifting dullness and fluid wave (if significant amount of ascitic fluid is present) Paraumbilical hernia

Signs of liver disease

Ascites Jaundice Spider angiomas Palmar erythema Asterixis Testicular atrophy Gynecomastia Dupuytren contracture Muscle wasting Splenomegaly

Signs of hyperdynamic circulatory state

Bounding pulses Warm, well-perfused extremities


Arterial hypotension

Causes of increased resistance to flow

Prehepatic Portal vein thrombosis Splenic vein thrombosis Congenital atresia or stenosis of portal vein Extrinsic compression (tumors) Splanchnic arteriovenous fistula

Intrahepatic, predominantly presinusoidal

Schistosomiasis (early stage) Primary biliary cirrhosis (early stage) Idiopathic portal hypertension (early stage) Nodular regenerative hyperplasia Myeloproliferative diseases: direct infiltration by malignant cells. Polycystic disease Hepatic metastasis

Intrahepatic, predominantly presinusoidal

Granulomatous diseases (sarcoidosis, tuberculosis) Sarcoid granulomas frequently localize in the portal areas, resulting in injury to the portal veins.

Intrahepatic, predominantly sinusoidal and/or postsinusoidal

Hepatic cirrhosis Acute alcoholic hepatitis Schistosomiasis (advanced stage) Primary biliary cirrhosis (advanced stage) Idiopathic portal hypertension (advanced stage) Acute and fulminant hepatitis Congenital hepatic fibrosis Vitamin A toxicity: Noncirrhotic portal fibrosis Peliosis hepatitis Venoocclusive disease Budd-Chiari syndrome

Posthepatic

Inferior vena cava (IVC) obstruction Right heart failure Constrictive pericarditis Tricuspid regurgitation Budd-Chiari syndrome Arterial-portal venous fistula Increased portal blood flow Increased splenic flow

Lab studies are directed towards investigating etiologies of cirrhosis

Liver function test


PT PTT CBC Hepatitis (A,B,C)

Lab studies are directed towards investigating etiologies of cirrhosis

Antinuclear antibody, antimitochondrial antibody, antismooth muscle antibody Iron indices


Alpha1-antitrypsin deficiency Ceruloplasmin, 24-hour urinary copper - To be considered only in individuals aged 3-40 years who have unexplained hepatic, neurologic, or psychiatric disease

Imaging Studies

Duplex-Doppler ultrasonography Ultrasound (US) is a safe, economical, and effective method for screening for portal hypertension. It also can demonstrate portal flow and helps in diagnosing cavernous transformation of the portal vein, portal vein thrombosis, or splenic vein thrombosis.

Features suggestive of hepatic cirrhosis with portal hypertension include the following: Nodular liver surface is suggestive. However, this finding is not specific for cirrhosis and can be observed with congenital hepatic fibrosis and nodular regenerative hyperplasia. Splenomegaly is a suggestive finding. Patients may demonstrate the presence of collateral circulation.

Limitations of US include the following: Reproducibility of data is problematic. Many variables, such as circadian rhythm, meals, medications, and the sympathetic nervous system, affect portal hemodynamics. Significant interobserver and intraobserver variation exist in quantitative ultrasonographic measurement.

CT scan

CT scan is a useful qualitative study in cases where sonographic evaluations are inconclusive. CT scan is not affected by patients' body habitus or the presence of bowel gas. With improvement of spiral CT scan and 3dimensional angiographic reconstructive techniques, portal vasculature may be visualized more accurately.

Findings suggestive of portal hypertension include the following:

Collaterals arising from the portal system are suggestive of portal hypertension.

Limitations of CT scan include the following:

It cannot demonstrate the venous and arterial flow profile. Intravenous contrast agents cannot be used in patients with renal failure or contrast allergy.

Magnetic resonance imaging

MRI provides qualitative information similar to CT scan when Doppler findings are inconclusive. MRI angiography detects the presence of portosystemic collaterals and obstruction of portal vasculature. MRI also provides quantitative data on portal venous and azygos blood flow.

Other Tests:

Selective angiography of the superior mesenteric artery or splenic artery with venous return phase

Hemodynamic measurement of portal pressure

The most commonly used method is measurement of the hepatic venous pressure gradient (HVPG), which is an indirect measurement that closely approximates portal venous pressure. A fluid-filled balloon catheter is introduced into the femoral or internal jugular vein and advanced under fluoroscopy into a branch of the hepatic vein. Free hepatic venous pressure (FHVP) then is measured.

Hemodynamic measurement of portal pressure

Direct portal measurements usually are not performed due to the invasive nature, the risk of complications, and the interference of anesthetic agents on portal hemodynamics.

Endoscopy

screen for varices in every patient with suggestive findings of portal hypertension. all patients with cirrhosis should be considered for the presence of varices at the time of the initial diagnosis of cirrhosis. Gastroesophageal varices confirm the diagnosis of portal hypertension; however, their absence does not rule it out.

Endoscopy

At times, gastroesophageal varices are incidental findings in patients undergoing upper endoscopy for other reasons (eg, dyspepsia refractory to medications, dysphagia, weight loss). These patients should undergo further investigations for etiologies of portal hypertension.

Endoscopy

In compensated patients without varices, repeat endoscopy at 2- to 3-year intervals to evaluate for the development of varices. In compensated patients with small varices, repeat endoscopy at 1- to 2-year intervals to evaluate the progression of varices.

Histologic Findings

On liver biopsy, histologic findings are varied and depend not only on the cause of liver disease but also on the cause of portal hypertension. Zone 3 necrosis can be observed in portal hypertension secondary to congestive heart failure and Budd-Chiari syndrome. In cases of normal liver parenchyma, investigate for prehepatic causes of portal hypertension.

Medical Care:

Treatment is directed at the cause of portal hypertension. Gastroesophageal variceal hemorrhage is the most dramatic and lethal complication of portal hypertension

Emergent treatment

Bleeding from esophageal varices Each episode of variceal bleeding = 30% mortality rate . Rebleeding occurs in 40% of patients within 6 weeks.

Emergent treatment

Initial resuscitation with replacement of blood volume loss Blood should be replaced at a modest target of HCT of 25-30%. Avoid intravascular volume and variceal overexpansion to prevent rebleeding.

Emergent treatment

Prevention of complications (eg, hepatic encephalopathy, bronchial aspiration, renal failure, systemic infections, SBP) All patients with cirrhosis and upper GI bleeding are at a high risk of developing severe bacterial infections, which are associated with early rebleeding.

Emergent treatment

prophylactic antibiotics has been demonstrated to decrease the rate of bacterial infections and increase survival rates, thus prophylactic antibiotic use (norfloxacin 400 mg PO bid for 7 d; ciprofloxacin and other broad-spectrum antibiotics also could be used) in the setting of acute bleeding is recommended

Emergent treatment

Specific treatment of bleeding lesion Pharmacological therapy Somatostatin (not available in the United States) is an endogenous hormone that decreases portal blood flow by splanchnic vasoconstriction at pharmacological doses, without significant systemic adverse effects.

Endoscopic therapy

Efficacy in achieving hemostasis is higher than 80%, but its effectiveness declines to 70% at day 5 due to very early rebleeding in some patients. Failures of endoscopic treatments may be managed by a second session of endoscopic treatment, but no more than 2 sessions should be allowed before deciding to perform transjugular intrahepatic portosystemic shunt (TIPS) or surgery.

Other interventions

The Minnesota tube has 4 lumens, 1 for gastric aspiration, 2 to inflate the gastric and esophageal balloons, and 1 above the esophageal balloon to suction secretions to prevent aspiration.

Surgical Care:

Surgical care includes decompressive shunts, devascularization procedures, and liver transplantation.