Portal hypertension

Portal hypertension
The portal vein and its tributaries.
ICD-10	K76.6
ICD-9	572.3
DiseasesDB	10388
eMedicine	radio/570  med/1889
MeSH	D006975

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],Assistant Editor-in-Chief: Soumya Sachdeva

Overview

In medicine, portal hypertension is hypertension (high blood pressure) in the portal stem which causes an obstruction in the portal vein and its branches. It is often defined as a portal pressure gradient (the difference in pressure between the portal vein and the hepatic veins) of 12 mm Hg or greater. Many conditions can result in portal hypertension. In North America and Europe, it is usually the result of an intrahepatic block due to cirrhosis of the liver. However, in less industrialized parts of the world, climate permitting, the major cause is schistosomiasis.

Pathophysiology

Portal venous pressure is determined by-portal blood flow and portal vascular resistance.Increased portal vascular resistance is often the main factor responsible for it. The consequences of portal hypertension are due to blood being forced down alternate channels by the increased resistance to flow through the portal system. Due to formation of alternate channels initially some of the portal blood and later most of it is shunted directly to the systemic circularion bypassing the liver

Natural History and Complications

There is an increased risk of

• Spontaneous bacterial peritonitis
• Hepatorenal syndrome
• Hepatic encephalopathy
• Congestive gastropathy
• Hypersplenism
• Ascites
• Variceal bleeding
• Renal failure

Causes

Prehepatic

• Congenital abnormalities
• Idiopathic tropical splenomegaly
• Portal vein occlusion
• Splenic Vein Thrombosis
• Umbilical sepsis

Intrahepatic

• Alcoholic hepatitis
• Boeck's Diesease
• Chronic hepatitis
• Cirrhosis
• Fatty liver
• Fulminant hepatic failure
• Hodgkin's Lymphoma
• Osteomyelosclerosis
• Partial nodular transformation
• Schistosomiasis
• Wilson's Disease

Posthepatic

• Cardiomyopathy
• Congestive Heart Failure
• Constructive Pericarditis
• Inferior vena cava obstruction
• Tricuspid insufficiency

Causes by Organ System

Cardiovascular	Cardiomyopathy, Congestive heart failure, Constructive pericarditis, Inferior vena cava obstruction, Splanchnic arteriovenous fistula, Tricuspid insufficiency
Chemical / poisoning	No underlying causes
Dermatologic	NISCH syndrome
Drug Side Effect	Didanosine, Thioguanine
Ear Nose Throat	No underlying causes
Endocrine	No underlying causes
Environmental	No underlying causes
Gastroenterologic	Alcoholic hepatitis, Biliary atresia, Budd-Chiari syndrome, Cholestasis, Chronic hepatitis, Chronic liver disease, Cirrhosis, Congenital atresia or stenosis of portal vein, Congenital hepatic fibrosis, Congenital narrowing of the portal vein, Cruveilhier-Baumgarten syndrome, Fatty liver, Focal nodular hyperplasia, Fulminant hepatic failure, Hepatic amyloidosis with intrahepatic cholestasis, Hepatic arterioportal fistula, Hepatic portal vein obstruction, Hepatic vein occlusion, Hepatic vein thrombosis, Hepatic venoocclusive disease with immunodeficiency, Idiopathic liver cirrhosis, Idiopathic portal hypertension , Idiopathic tropical splenomegaly, Liver fibrosis, Mosse syndrome, Neonatal hepatitis, Nodular regenerative hyperplasia of the liver, Obliterative portal venopathy, Partial nodular transformation, Portal hypertension due to intrahepatic block, Portal vein abnormality, Portal vein compression, Portal vein occlusion, Portal vein thrombosis , Primary biliary cirrhosis, Primary sclerosing cholangitis, Splenic vein thrombosis, Alpha-1-antitrypsin deficiency, Progressive familial intrahepatic cholestasis, Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification, Hepatic metastasis Pancreatic cancer, NISCH syndrome
Genetic	Alpha-1-antitrypsin deficiency, COACH syndrome, Cystic fibrosis, Gaucher disease, Glycosylphosphatidylinositol deficiency, Hemochromatosis, Interferon gamma receptor 1 deficiency, NISCH syndrome, Progressive familial intrahepatic cholestasis, Wilson's Disease , Hepatic venoocclusive disease with immunodeficiency
Hematologic	Blackfan Diamond anemia, Congenital pure red cell aplasia , Myeloproliferative diseases, Mosse syndrome, Hemochromatosis, Osteomyelosclerosis, Hodgkin's lymphoma
Iatrogenic	No underlying causes
Infectious Disease	Schistosomiasis , Umbilical sepsis , Viral hepatitis
Musculoskeletal / Ortho	Osteomyelosclerosis
Neurologic	Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification
Nutritional / Metabolic	Gaucher disease, Glycosylphosphatidylinositol deficiency, Wilson's Disease
Obstetric/Gynecologic	No underlying causes
Oncologic	Extrinsic compression (tumors), Hepatic metastasis, Hodgkin's lymphoma, Pancreatic cancer
Opthalmologic	No underlying causes
Overdose / Toxicity	Didanosine, Thioguanine
Psychiatric	No underlying causes
Pulmonary	Cystic fibrosis, Alpha-1-antitrypsin deficiency, Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification
Renal / Electrolyte	Polycystic kidney disease
Rheum / Immune / Allergy	Hepatic venoocclusive disease with immunodeficiency, Granulomatous diseases (sarcoidosis, tuberculosis), Sarcoidosis
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Dental	No underlying causes
Miscellaneous	Granulomatous diseases (sarcoidosis, tuberculosis), Sarcoidosis, Extrinsic compression (tumors)

Causes in Alphabetical Order

Alcoholic hepatitis Alpha-1-antitrypsin deficiency Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification Biliary atresia Blackfan Diamond anemia Budd-Chiari syndrome Cardiomyopathy Cholestasis Chronic hepatitis Chronic liver disease Cirrhosis COACH syndrome Congenital atresia or stenosis of portal vein Congenital hepatic fibrosis Congenital narrowing of the portal vein Congenital pure red cell aplasia Congestive heart failure Constructive pericarditis Cruveilhier-Baumgarten syndrome Cystic fibrosis Didanosine Extrinsic compression (tumors) Fatty liver Focal nodular hyperplasia Fulminant hepatic failure Gaucher disease Glycosylphosphatidylinositol deficiency Granulomatous diseases (sarcoidosis, tuberculosis) Hemochromatosis Hepatic amyloidosis with intrahepatic cholestasis Hepatic arterioportal fistula Hepatic metastasis Hepatic portal vein obstruction Hepatic vein occlusion Hepatic vein thrombosis

Hepatic venoocclusive disease with immunodeficiency Hodgkin's lymphoma Idiopathic liver cirrhosis Idiopathic portal hypertension Idiopathic tropical splenomegaly Inferior vena cava obstruction Interferon gamma receptor 1 deficiency Liver fibrosis Mosse syndrome Myeloproliferative diseases Neonatal hepatitis NISCH syndrome Nodular regenerative hyperplasia of the liver Obliterative portal venopathy Osteomyelosclerosis Pancreatic cancer Partial nodular transformation Polycystic kidney disease Portal hypertension due to intrahepatic block Portal vein abnormality Portal vein compression Portal vein occlusion Portal vein thrombosis Primary biliary cirrhosis Primary sclerosing cholangitis Progressive familial intrahepatic cholestasis Sarcoidosis Schistosomiasis Splanchnic arteriovenous fistula Splenic vein thrombosis Thioguanine Tricuspid insufficiency Umbilical sepsis Viral hepatitis Wilson's Disease

Diagnosis

Physical Examination

Abdomen

• Ascites (free fluid in the peritoneal cavity)
• Hepatic encephalopathy
• Splenomegaly (enlargement of the spleen) with consequent sequestration therein of red blood cells, white blood cells, and platelets, together leading to mild pancytopenia
• portacaval anastomoses (Esophageal varices, hemorrhoids, caput medusae), with esophageal varices posing an ongoing risk of life-threatening hemorrhage.

Investigations

1. Barim swallow - presence of varices is seen as filling defects(bag of worms appearnace)
2. Upper gastrointestinal endoscopy - presence of cherry red spots.Very reliable
3. Ultrasonography - to note the size of liver,spleen,poratl vein,splenic vein and to look for the presence of collaterals
4. Hepatic venous pressure drainage measurement - Gold standard for measuring portal hypertension.If ore than 5 mmHg it is considered as significant
5. Portal venography - patency and the calibre of portal vein and splenic vein , presence of collaterals
6. Liver function tests for liver diseases
7. Proctoscopy - rectal varices
8. Barium enema - colonic varices

Patient #1

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Patient #2: Recanalized umbilican vein

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Treatment

Medical management

Treatment with a non-selective beta blocker is often commenced once portal hypertension has been diagnosed, and almost always if there has already been bleeding from esophageal varices. Typically, this is done with either propranolol or nadolol. The addition of a nitrate, such as isosorbide mononitrate, to the beta blocker is more effective than using beta blockers alone and may be the preferred regimen in those people with portal hypertension who have already experienced variceal bleeding. In acute or severe complications of the hypertension, such as bleeding varices, intravenous octreotide (a somatostatin analogue) or intravenous terlipressin (an antidiuretic hormone analogue) is commenced to decrease the portal pressure.

Percutaneous interventions

Transjugular intrahepatic portosystemic shunting is the creation of a connection between the portal and the venous system. As the pressure over the venous system is lower than over a hypertensive portal system, this would decrease the pressure over the portal system and a decreased risk of complications.

Surgical interventions

• Distal splenorenal shunt
• Liver transplant

The most definitive treatment of portal hypertension is a liver transplant.

External links

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