Portal Hypertension Flashcards
state the typical presentation of portal hypertension?
Butt (haemorrhoids), gut (oesophageal varices, splenomegaly and ascites) and caput (caput medusa)
Define portal hypertension?
abnormally high pressure within hepatic portal vein
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clinically significant portal hypertension is defined as a hepatic venous pressure gradient > 10 mm Hg (NORMAL is < 5mmHg)
outline the aetiology of portal hypertension?
CIRRHOSIS is most common cause
Collagen deposition and fibrosis
sodium retention and vasoactive substances such as NO ( due to accumulation of toxic metabolites) will increase plasma voluem and splanchnic vasodilation => maintains portal hypertension
how can the causes of portal hypertension be divided?
pre- hepatic- blockage of portal vein before liver
hepatic
post hepatic- blockage of hepatic veins or venules
what are the pre-hepatic caues of portal hypertension?
blockage of portal vein before liver
- congenital stenosis
- portal vein thrombosis
- splenic vein thrombosis
- extrinsic compression
what are the hepatic causes of portal hypertension?
CIRRHOSIS
Chronic hepatitis
Schistosomiasis
Granulomata
Myeloproliferative disease
what are the post hepatic causes of portal hypertension?
Budd-Chiari syndrome (hepatic vein obstruction):Sudden-onset ascites with tender hepatomegaly in the absence of jaundice
Constrictive pericarditis
Right heart failure
what is Budd-chiari syndrome?
hepatic vein obstruction
sudden onset ascites with tender hepatomegaly in the absence of jaundice
summarise the epidemiology of potal hypertension?
common consequence of cirrhosis
what are the presenting symptoms of portal hypertension?
Features of Liver Disease (likely to coexist with portal hypertension)
- Jaundice
- History of alcohol abuse
- Risk factors for viral hepatitis (e.g. tattoos, unprotected sex, IV drug use, travel abroad and blood transfusion)
- Family history (e.g. haemochromatosis)
Complications of Portal Hypertension
- Haematemesis or melaena
- Lethargy, irritability, changes in sleep (hepatic encephalopathy)
- Abdominal distension (ascites)
- Abdominal pain and fever (spontaneous bacterial peritonitis)
- Pulmonary involvement
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what are the symptoms caused by the complications of portal hypertension?
Haematemesis or melaena
Lethargy, irritability, changes in sleep (hepatic encephalopathy)
Abdominal distension (ascites)
Abdominal pain and fever (spontaneous bacterial peritonitis)
Pulmonary involvement
what are the signs of portal hypertension on physical examination?
Signs of Portal Hypertension
- Caput medusae
- Splenomegaly
- Ascites
Signs of Liver Failure
- Jaundice
- Spider naevi
- Palmar erythema
- Confusion
- Asterixis
- Fetor hepaticus
- Enlarged or small liver
- Gynaecomastia
- Testicular atrophy
what blood are required to investigate portal hypertension?
LFTs
U&Es
Blood glucose
FBC
Clotting screen (prolongation of PT is one of the earliest signs of liver failure)
what are the specific tests needed to investigate portal hypertension?
- Ferritin - haemochromatosis
- Hepatitis serology
- Autoantibodies (e.g. anti-smooth muscle antibodies in autoimmune hepatitis)
- alpha1-antitrypsin levels
- Caeruloplasmin - Wilson’s disease
what are the imaging investigations needed for portal hypertension?
- Abdominal ultrasound - check liver and spleen size and assess portal blood flow
- Doppler ultrasound - assess direction of blood flow in vessels
- CT/MRI - if other imaging methods are inconclusive
- Endoscopy - to check for oesophageal varices
what are the appropriate investigations for portal hypertension?
bloods
specific tests
imaging
measure hepatic venous pressure gradient
liver biopsy if indicated
what is the immediate treatment for portal hypertension?
terlipressin and prophylactic antibiotics
ADH agonists cause splanchnic vasoconstriction reducing portal pressure
outline a management plan for portal hypertension?
Immediate treatment: terlipressin and prophylactic antibiotics
- ADH agonist that causes splanchnic vasoconstriction reducing portal pressure
Endoscopy is done within 12h to diagnose and treat using band ligation or injection sclerotherapy
If insufficient: TIPS
Transjugular Intrahepatic Portosystemic Shunt (TIPS) - surgical shunt placed between the hepatic portal vein and the hepatic vein to ease congestion in the portal vein
Liver transplant
Beta blockers (e.g. carvedilol) used for prophylaxis of variceal bleed
what are the complications of ascites?
spontaneous bacterial peritonitis
hepatorenal syndrome
hepstic hydrothorax?
what are the possible complications of portal hypertension?
bleeding from oesophageal varices
ascites+ complications of ascites
pulmonary complications
liver failure
hepatic encephalopathy
cirrhotic cardiomyopathy
what is the defintion of hepatorenal syndrome?
a life threatening condition that consists of a rapid derioration in kindey function in individuals with cirrhosis or fulminant liver failure
what is the definition of hepatic hydrothorax?
a transudative pleural effusion in patients with portal hypertension without any cardiopulmonary cause
what are the pulmonary complications of portal hypertension?
portopulmonary hypertension
hepatopulmonary syndrome
what is hepatopulmonary syndrome a triad of?
hepatic dysfunction
hypoxaemia
extreme vasodilation ( intrapulmonary vascular dilation)
summarise the prognosis of pulmonary hypertension?
Depends on the underlying CAUSE
Variceal haemorrhages have a 1-year mortality of 40%
outline the pathophopthysiology of varices
Two venous systems drain abdominal structures – the portal venous system and the systemic venous system. The portal venous system transports venous blood to the liver for processing and the systemic system returns blood to the right atrium of the heart.
A porto-systemic anastomosis is a connection between the veins of the portal and systemic venous systems – the major sites of these include oesophageal, rectal, retroperitoneal and paraumbilical.
When blood flow through the portal system is obstructed, i.e. due to cirrhosis, the pressure rises and you get PORTAL HYPERTENSION.
This causes blood to be re-directed through the porto-systemic anastomoses as these are now under lower pressure. If a large volume of blood passes through these over a long time, the veins around the anastomosis can become abnormally dilated – known as varices. If these rupture, it can cause fatal blood loss.
Gastro-oesophageal varices develop once portal pressure is > 10mmHg.
If >12mmHg, risk of variceal bleed high.
what is the pre-endoscopic management for portal hypertension?
TERLEPRESSIN: ADH agonist that causes splanchnic vasoconstriction reducing portal pressure
prophylactic antibiotics