Portal Hypertension Disease

DESCRIPTION

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

SIGNS AND SYMPTOMS

May be general or related to specific complications

General
- Splenomegaly
- Caput medusa
- Umbilical bruit
- Hemorrhoids
- Spider angiomata
- Gynecomastia
- Testicular atrophy
- Digital clubbing
- Palmar erythema
Gastroesophageal varices
- Hematemesis
- Melena
- Anemia
- Hypotension
- Tachycardia
Ascites
- Distended abdomen
- Fluid wave
- Shifting percussion dullness
Hepatic encephalopathy
- Confusion
- Asterixis
- Hyperreflexia
Hepatorenal syndrome
- Oliguria

CAUSES

May be intrahepatic or extrahepatic

Cirrhosis present. Accounts for > 90% of cases.
- Alcoholic
- Viral (HBV, HCV, HGV)
- Wilson's disease
- Hemochromatosis
- Primary biliary cirrhosis
- Schistosomiasis
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

LABORATORY

Non-specific changes associated with underlying disease.

Hypersplenism
- Anemia
- Leukopenia
- Thrombocytopenia
Hepatic dysfunction
- Hypoalbuminemia
- Hyperbilirubinemia
- Elevated alkaline phosphatase
- Elevated liver enzymes
- Abnormal clotting factors (PT, PTT.)
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.

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 > 12 mm./Hg.

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

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.
Gastroesophageal varices with hemorrhage
- Endoscopic variceal sclerosis
- Endoscopic variceal banding
- Portacaval shunting
- Transjugular portosystemic shunt (TIPS)
Ascites refractory to medical management
- Large volume paracentesis
- Peritoneovenous shunt

ACTIVITY

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

DIET

Restrict sodium and protein

PATIENT EDUCATION

N/A

PREVENTION/AVOIDANCE

Abstinence from alcohol. Adequate and appropriate nutrition.

POSSIBLE COMPLICATIONS

As described above

EXPECTED COURSE AND 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.

ASSOCIATED CONDITIONS

As described above

AGE-RELATED FACTORS

Pediatric: Uncommon. Generally different etiology than in adults.

Intrahepatic
- Biliary atresia
- Viral hepatitis
- Metabolic liver disease.
Extrahepatic
- Congenital anomalies of portal vein
- Neonatal omphalitis (umbilical vein catheterization, sepsis, abdominal trauma)

Geriatric: Mortality and complication rate are increased
Others: N/A

PREGNANCY

N/A

OTHER NOTES

Other treatment approaches (inadequately studied with non-control protocols)
- Transhepatic obliteration of varices
- Concomitant treatment with non-selective beta-adrenergic blockers

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.