Portal hypertension
Encyclopedia
In medicine
, portal hypertension is hypertension
(high blood pressure) in the portal vein and its tributaries.
It is often defined as a portal pressure gradient (the difference in pressure between the portal vein and the hepatic veins) of 10 mmHg or greater.
, hemochromatosis, or congenital fibrosis). Prehepatic causes include portal vein thrombosis
or congenital atresia. Posthepatic obstruction occurs at any level between liver and right heart, including hepatic vein thrombosis, inferior vena cava thrombosis, inferior vena cava congenital malformation, and constrictive pericarditis
.
) is superior to either of them at reducing rate of rebleeding. Disadvantages of TIPS include high cost and increased risk of hepatic encephalopathy
, and it does not improve the mortality rate.
, renal failure
and death. The management includes salt restriction, diuretics (spironolactone
), paracentesis
, transjugular intrahepatic portosystemic shunt
(TIPS) and peritoneovenous shunt
.
and use of oral antibiotics.
Medicine
Medicine is the science and art of healing. It encompasses a variety of health care practices evolved to maintain and restore health by the prevention and treatment of illness....
, portal hypertension is hypertension
Hypertension
Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...
(high blood pressure) in the portal vein and its tributaries.
It is often defined as a portal pressure gradient (the difference in pressure between the portal vein and the hepatic veins) of 10 mmHg or greater.
Causes
Causes can be divided into prehepatic, intrahepatic, and posthepatic. Intrahepatic causes include liver cirrhosis, and hepatic fibrosis (e.g. due to Wilson's diseaseWilson's disease
Wilson's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues; this manifests as neurological or psychiatric symptoms and liver disease...
, hemochromatosis, or congenital fibrosis). Prehepatic causes include portal vein thrombosis
Portal vein thrombosis
Portal vein thrombosis is a form of venous thrombosis affecting the hepatic portal vein, which can lead to portal hypertension and reduction in the blood supply to the liver.-Causes:...
or congenital atresia. Posthepatic obstruction occurs at any level between liver and right heart, including hepatic vein thrombosis, inferior vena cava thrombosis, inferior vena cava congenital malformation, and constrictive pericarditis
Constrictive pericarditis
In many cases, constrictive pericarditis is a late sequela, in other words a condition that is the consequence of a previous disease, of an inflammatory condition of the pericardium...
.
Signs and symptoms
Consequences of portal hypertension are caused by blood being forced down alternate channels by the increased resistance to flow through the systemic venous system rather than the portal system. They include:- AscitesAscitesAscites is a gastroenterological term for an accumulation of fluid in the peritoneal cavity.The medical condition is also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more archaically as abdominal dropsy. Although most commonly due to cirrhosis and severe liver...
(free fluid in the peritoneal cavityPeritoneal cavityThe peritoneal cavity is a potential space between the parietal peritoneum and visceral peritoneum, that is, the two membranes that separate the organs in the abdominal cavity from the abdominal wall...
). - Hepatic encephalopathyHepatic encephalopathyHepatic encephalopathy is the occurrence of confusion, altered level of consciousness and coma as a result of liver failure. In the advanced stages it is called hepatic coma or coma hepaticum...
. - Increased risk of spontaneous bacterial peritonitisSpontaneous bacterial peritonitisSpontaneous bacterial peritonitis is the development of peritonitis despite the absence of an obvious source for the infection. It occurs almost exclusively in people with portal hypertension , usually as a result of cirrhosis of the liver...
. - Increased risk of hepatorenal syndromeHepatorenal syndromeHepatorenal syndrome is a life-threatening medical condition that consists of rapid deterioration in kidney function in individuals with cirrhosis or fulminant liver failure...
. - SplenomegalySplenomegalySplenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant of the human abdomen. It is one of the four cardinal signs of hypersplenism, some reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any...
(enlargement of the spleen) with consequent sequestration therein of red blood cells, white blood cells, and platelets, together leading to mild pancytopeniaPancytopeniaPancytopenia is a medical condition in which there is a reduction in the number of red and white blood cells, as well as platelets.If only two parameters from the full blood count are low, the term bicytopenia can be used...
. - Portacaval anastomoses: Esophageal varicesEsophageal varicesIn medicine , esophageal varices are extremely dilated sub-mucosal veins in the lower esophagus...
, gastric varicesGastric varicesGastric varices are dilated submucosal veins in the stomach, which can be a life-threatening cause of upper gastrointestinal hemorrhage. They are most commonly found in patients with portal hypertension, or elevated pressure in the portal vein system, which may be a complication of cirrhosis...
, anorectal varicesAnorectal varicesAnorectal varices refers to the dilation of collateral vessels due backflow in the veins of the rectum. Collateral veins anastomose the inferior and middle rectal veins with the portal system in the presence of portal hypertension...
(not to be confused with hemorrhoidHemorrhoidHemorrhoids or haemorrhoids , are vascular structures in the anal canal which help with stool control. They become pathological or piles when swollen or inflamed. In their physiological state they act as a cushion composed of arterio-venous channels and connective tissue that aid the passage of...
s), and caput medusaeCaput medusaeCaput medusae is the appearance of distended and engorged paraumbilical veins, which are seen radiating from the umbilicus across the abdomen to join systemic veins...
. Esophageal and gastric varices pose an ongoing risk of life-threatening hemorrhage, with hematemesisHematemesisHematemesis or haematemesis is the vomiting of blood. The source is generally the upper gastrointestinal tract. Patients can easily confuse it with hemoptysis , although the latter is more common.-Signs:...
or melenaMelenaIn medicine, melena or melaena refers to the black, "tarry" feces that are associated with gastrointestinal hemorrhage. The black color is caused by oxidation of the iron in hemoglobin during its passage through the ileum and colon.-Melena vs...
.
Prophylaxis of variceal bleeding
Both pharmacological (B-blocker like Propranolol and isosorbide mononitrate) and endoscopic (banding ligation) treatment have similar results. TIPS (transjugular intrahepatic portosystemic shuntingTransjugular intrahepatic portosystemic shunt
A Transjugular Intrahepatic Portosystemic Shunt is an artificial channel within the liver that establishes communication between the inflow portal vein and the outflow hepatic vein...
) is superior to either of them at reducing rate of rebleeding. Disadvantages of TIPS include high cost and increased risk of hepatic encephalopathy
Hepatic encephalopathy
Hepatic encephalopathy is the occurrence of confusion, altered level of consciousness and coma as a result of liver failure. In the advanced stages it is called hepatic coma or coma hepaticum...
, and it does not improve the mortality rate.
Management of active variceal bleeding
After resuscitation, the management of active variceal bleeding includes administering vasoactive drugs (somatostatin, octreotide or terlipressin), endoscopic banding ligation, balloon tamponade and TIPS.Management of ascites
This should be gradual to avoid sudden changes in systemic volume status which can precipitate hepatic encephalopathyHepatic encephalopathy
Hepatic encephalopathy is the occurrence of confusion, altered level of consciousness and coma as a result of liver failure. In the advanced stages it is called hepatic coma or coma hepaticum...
, renal failure
Renal failure
Renal failure or kidney failure describes a medical condition in which the kidneys fail to adequately filter toxins and waste products from the blood...
and death. The management includes salt restriction, diuretics (spironolactone
Spironolactone
Spironolactone , commonly referred to as simply spiro, is a diuretic and is used as an antiandrogen.It is a synthetic 17-lactone drug that is a renal competitive aldosterone antagonist in a class of pharmaceuticals called...
), paracentesis
Paracentesis
Paracentesis is a medical procedure involving needle drainage of fluid from a body cavity, most commonly the peritoneal cavity in the abdomen.A related procedure is thoracocentesis, which is needle drainage of the chest cavity...
, transjugular intrahepatic portosystemic shunt
Transjugular intrahepatic portosystemic shunt
A Transjugular Intrahepatic Portosystemic Shunt is an artificial channel within the liver that establishes communication between the inflow portal vein and the outflow hepatic vein...
(TIPS) and peritoneovenous shunt
Peritoneovenous shunt
A peritoneovenous shunt is a shunt which drains peritoneal fluid from the peritoneum into veins, usually the internal jugular vein or the superior vena cava...
.
Control of hepatic encephalopathy
This includes reduction of dietary protein, followed by lactuloseLactulose
Lactulose is a synthetic, non-digestible sugar used in the treatment of chronic constipation and hepatic encephalopathy, a complication of liver disease. It is a disaccharide formed from one molecule each of the simple sugars fructose and galactose...
and use of oral antibiotics.
External links
- VIDEO - Portal Hypertension: Shunt Surgery in the Era of Transplant and TIPS, Alysandra Lal, MD, speaks at the University of Wisconsin School of Medicine and Public Health (2007)
- Overview at Cleveland ClinicCleveland ClinicThe Cleveland Clinic is a multispecialty academic medical center located in Cleveland, Ohio, United States. The Cleveland Clinic is currently regarded as one of the top 4 hospitals in the United States as rated by U.S. News & World Report...
- Children's Liver Disease Foundation