Portal hypertension

Description of Medical Condition

Increased portal venous pressure (> 10 mm Hg.) that occurs in association with splanchnic vasodilatation, portosystemic collateral formation and a hyperdynamic circulation. Course is generally progressive and may produce one or more devastating clinical disorders.

System(s) affected: Gastrointestinal, Cardiovascular, Nervous

Genetics: No known genetic patterns except those associated with specific hepatic diseases that cause portal hypertension

Incidence/Prevalence in USA: Unknown. (Incidence of bleeding from gastroesophageal varices is approximately 120 episodes per 100,000 population per year.)

Predominant age: Adult

Predominant sex: Male > Female

Medical Symptoms and Signs of Disease

May be general or related to specific complications

1. General

• Splenomegaly
• Caput medusa
• Umbilical bruit
• Hemorrhoids
• Spider angiomata
• Gynecomastia
• Testicular atrophy
• Digital clubbing
• Palmar erythema
• Gastroesophageal varices

2. Hematemesis OMelena

• Anemia
• Hypotension
• TachycardiaAscites
• Distended abdomen
• Fluid wave
• Shifting percussion dullness

3. Hepatic encephalopathy

• Confusion
• Asterixis
• Hyperreflexia

4. Hepatorenal syndrome

• Oliguria

What Causes Disease?

May be intrahepatic or extrahepatic

1. Cirrhosis present. Accounts for > 90% of cases.

•  Alcoholic
• Viral (HBV, HCV, HGV)
• Wilson disease
• Hemochromatosis
• Primary biliary cirrhosis
• Schist osomiasis

2. Cirrhosis not present.

• Portal vein thrombosis
• Hepatic vein obstruction (Budd-Chiari syndrome)
• Right ventricular failure
• Myeloproliferative disorders

Risk Factors

Many different chronic liver diseases and hepatotoxins

Diagnosis of Disease

Differential Diagnosis

Usually related to specific complications/presentations.

1. Gastroesophageal varices with hemorrhage vs:

• Portal hypertensive gastropathy
• Hemorrhagic gastritis
• Peptic ulcer disease
• Mallory-Weiss tear

2. Ascites vs:

• Spontaneous bacterial peritonitis
• Pancreatic ascites
• Peritoneal carcinomatosis
• Tuberculous peritonitis

3. Onephrotic syndrome

• Cardiac ascites

4. Hepatic encephalopathy vs:

• Delirium tremens
• Intracranial hemorrhage
• Sedative abuse
• Uremia

5. Hepatorenal syndrome vs

• Drug nephrotoxicity
• Renal tubular necrosis

Laboratory

Non-specific changes associated with underlying disease.

1. Hypersplenism

• Anemia
• Leukopenia
• Thrombocytopenia

2. Hepatic dysfunction

• Hypoalbuminemia
• Hyperbilirubinemia
• Elevated alkaline phosphatase
• Elevated liver enzymes
• Abnormal clotting factors (PT, PTT.)

2. Gastrointestinal bleeding

• Iron deficiency anemia
• Elevated serum ammonia
• Hepatorenal syndrome
• Elevated serum creatinine, BUN
• Urine Na < 20 mEq/L (< 20 mmol/L)

Drugs that may alter lab results: N/A

Disorders that may alter lab results: N/A

Pathological Findings

Specific for underlying disease

Special Tests

Specific for underlying disease

Imaging

• UGI series. May outline varices in esophagus and stomach.
• CT scan and ultrasound. May detect cirrhosis, splenomegaly, ascites and varices.
• Duplex-Doppler (ultrasound.) Can determine presence and direction of flow in portal and hepatic veins. Useful in diagnosing portal vein and/or shunt thrombosis
• Angiography. Demonstrates cork-screwing of intrahepatic vessels (cirrhosis); can identify varices and vascular anomalies.
• Contrast-enhanced MR angiography may be imaging modality of choice. Can provide three dimensional information about the patency of the portal vein and the presence of collateral pathways.

Diagnostic Procedures

• Endoscopy. Can diagnose esophageal and gastric varices and portal hypertensive gastropathy or can directly visualize other bleeding sites (peptic ulcers, gastritis, Mallory-Weiss tears.)
• Hepatic venous wedge pressure. Correlates with portal pressure; risk of variceal bleeding is increased if HVWP >12mm./Hg.

Treatment (Medical Therapy)

Appropriate Health Care

Inpatient

General Measures

• Treat underlying disease and support metabolic/nutritional needs.
• Avoid sedatives; may precipitate encephalopathy
• Transfuse packed RBCs as needed. Use caution circulation is already hyperdynamic.
• Correct coagulopathy. Administer vitamin K and/or fresh-frozen plasma.
• Limit sodium administration; cirrhotic patients avidly retain sodium

Surgical Measures

1. Liver transplantation may be recommended for selected patients with far-advanced hepatic disease. Other less aggressive approaches are available for specific complications of portal hypertension.

2. Gastroesophageal varices with hemorrhage

• Endoscopic variceal sclerosis OEndoscopic variceal banding
• Portacaval shunting
• Transjugular intrahepatic portosystemic shunt (TIPS)

3. Ascites refractory to medical management

• Large volume paracentesis
• Peritoneovenous shunt
• Transjugular intrahepatic portosystemic shunt (TIPS)

Activity

Bed rest for acute complications (bleeding, encephalopathy or hepatorenal syndrome)

Diet

Restrict sodium and protein

Medications (Drugs, Medicines)

Drug(s) of Choice

1. Therapy for variceal hemorrhage:

• For acute control: intravenous somatostatin or octreotide — synthetic analogue
• Alternative: vasopressin; but, has more complications (decreased by addition of nitroglycerin)
• For prevention of recurrence: propranolol

2. Therapy for encephalopathy:

• Lactulose. Induces diarrhea and traps intracolonic ammonia.
• Oneomycin. Reduces bacterial production of nitrogenous substances in colon.

3. Therapy for ascites:

• Furosemide (Lasix)
• Spironolactone.

Contraindications:

Vasopressin is a systemic vasodilator and may cause hypotension, bradycardia and cardiac and peripheral ischemia. Cardiac monitoring is advisable.

Precautions:

Vasopressin may cause hypertension, bradycardia, arrhythmias. Patient must be on a cardiac monitor while receiving this drug. Co-administration of nitroprusside may reduce cardiotoxicity.

Significant possible interactions:

Refer to manufacturer’s literature

Alternative Drugs

Terlipressin (Glypressin) — more selective splanchnic vasoconstrictor and may be associated with fewer complications. Studies are continuing.

Patient Monitoring

Acute complications of portal hypertension require intensive monitoring of vital signs and organ function. Long-term management includes regular follow-up of all affected organ systems.

Prevention / Avoidance

Abstinence from alcohol. Adequate and appropriate nutrition.

Possible Complications

As described above

Expected Course / Prognosis

• Variceal bleeding: 50% re-bleed, usually within 2 years unless portal pressure is reduced by shunt or TIPS procedure
• Ascites: Generally recurs. Frequency and severity can be reduced if salt restriction is observed.
• Hepatic encephalopathy. Often recurs especially if re-bleeding develops. Low protein diet advised.

Miscellaneous

Associated Conditions

As described above

Age-Related Factors

Pediatric: Uncommon. Generally different etiology than in adults.

1. Intrahepatic

• Biliary atresia
• Viral hepatitis
• Metabolic liver disease.

2. Extrahepatic

• Congenital anomalies of portal vein
• Oneonatal omphalitis (umbilical vein catheterization, sepsis, abdominal trauma)

Geriatric: Mortality and complication rate are increased

Pregnancy

N/A

International Classification of Diseases

572.3 Portal hypertension

See Also

Cirrhosis of the liver Hepatitis A Hepatitis B Hepatitis C

Other Notes

1. Other treatment approaches (inadequately studied with non-control protocols)

• Transhepatic obliteration of varices
• Concomitant treatment with non-selective beta-ad-renergic blockers