Portal hypertension is the pathological elevation of portal venous pressure resulting from obstructions in portal blood flow, which can be prehepatic (e.g., portal vein thrombosis), hepatic (e.g., liver cirrhosis), or posthepatic (e.g., right-sided heart failure). The subsequent backflow of blood may lead to portosystemic anastomoses, splenomegaly, and/or ascites. A diagnosis of portal hypertension can be made based on clinical signs and knowledge of an underlying cause. In suspected cases, medical imaging and laboratory tests are used to support the diagnosis. Management involves treating the underlying condition and reducing portal pressure with nonselective beta blockers (NSBBs) and portosystemic shunts. Acute hemorrhage of esophageal varices is a potentially life-threatening complication of portal hypertension and is caused by increased blood flow via portosystemic anastomoses.
Causes of portal hypertension are described based on the anatomical location of the lesion.
- Portal vein thrombosis
- Splenic vein thrombosis
- Mesenteric vein thrombosis 
- Congenital abnormalities of the portal vein system (e.g., portal vein agenesis)
- Compression of portal or splenic veins 
- Arteriovenous fistula
Hepatic etiologies of portal hypertension are described anatomically in relation to the .
- Hepatosplenic schistosomiasis (most common cause of noncirrhotic portal hypertension worldwide) 
- Hepatoportal sclerosis
- Granulomatous diseases (e.g., sarcoidosis, tuberculosis) 
- Malignancy (e.g., lymphoma) causing portal vein occlusion
- Biliary diseases (e.g., primary biliary cholangitis, primary sclerosing cholangitis)
- Developmental or genetic abnormalities (e.g., polycystic kidney disease, arteriovenous fistula)
- Cirrhosis (most common cause of portal hypertension in the US)
- Massive hepatic metastases
- Congenital hepatic fibrosis
- Nodular regenerative hyperplasia
- Drug-induced liver injury (e.g., methotrexate-induced) or toxin-related liver injury (e.g., copper)
- Alcoholic liver disease
- Infiltrative diseases (e.g., Gaucher disease)
- Inflammatory and infectious conditions (e.g., viral hepatitis, chronic Q fever)
- Presinusoidal: obstruction of outflow in portal vein at the porta hepatis or in the portal venules within liver → ↑ hydrostatic pressure in the portal vein and its tributaries
- Sinusoidal: ↑ resistance to blood flow in the hepatic sinusoids (e.g., due to hepatic fibrosis and ↓ intrahepatic nitric oxide production in cirrhosis) → ↑ hydrostatic pressure in the hepatic sinusoids → ↑ hydrostatic pressure in the portal vein and its tributaries
- Postsinusoidal: obstruction of outflow in the central vein and/or hepatic venules → ↑ hydrostatic pressure in the hepatic sinusoids → ↑ hydrostatic pressure in the portal vein and its tributaries
The etiology determines whether portal hypertension manifests as acute or chronic.
Signs and symptoms of underlying disease, e.g.:
Signs and symptoms of complications: features of increased blood flow via portosystemic anastomoses
- Gastrointestinal bleeding 
- Caput medusae
- Signs of hypersplenism due to congestive splenomegaly (e.g., thrombocytopenia)
- Abdominal distention due to ascites
- Hepatic encephalopathy
Patients usually present with signs of their underlying disease and/or symptoms related to the complications of portal hypertension.
General principles 
- CSPH is confirmed in patients with either: 
- Once confirmed, identify the underlying
- Obtain a diagnostic workup for the most likely cause (e.g., ).
- Consult hepatology if the diagnosis remains unclear.
- Refer for EGD to screen for esophageal varices at the time of diagnosis.
Laboratory studies 
Findings are nonspecific but may further support the diagnosis and help to identify the underlying cause.
- Liver chemistries: transaminitis and/or hyperbilirubinemia suggest liver disease.
- Coagulation panel: coagulopathy is a sign of advanced liver disease
- Ascitic fluid analysis: high SAAG ≥ 1.1 g/dL 
Imaging studies 
- Indications: Obtain for all patients with nonconfirmed disease.
- Findings: Correlate with PLT levels to increase diagnostic yield.
Abdominal imaging 
Findings may help establish the diagnosis and identify the underlying cause.
Specific findings of CSPH: sufficient to establish a diagnosis of portal hypertension 
- Portosystemic collateral circulation
- Blood flow reversal in the portal system
- Nonspecific findings
Hepatic venous pressure gradient (HVPG) measurement 
- Indication: reserved for diagnostic confirmation in cases of uncertainty (gold standard test) 
- Method: Free and wedge occlusion pressures of the hepatic vein are measured via catheterization, using ultrasound or fluoroscopy. 
- Interpretation of HVPG levels 
Esophagogastroduodenoscopy (EGD) 
- Goals: screening and management of esophageal varices
- Indications for screening include: 
General principles 
Consult hepatology for specialist-guided management that includes:
- Management of the underlying causes of portal hypertension (mainstay of treatment)
- Pharmacotherapy with NSBBs
- Portosystemic shunts for refractory disease
- Management of complications of portal hypertension, e.g., treatment of ascites, treatment of hepatic encephalopathy, treatment of esophageal varices
Pharmacotherapy (off-label) 
- NSBBs: used to prevent hepatic decompensation (e.g., variceal bleeding)
- Reduce dose or discontinue in patients with complications of advanced liver disease.
- Definition: implantation of a stent between the hepatic vein and portal vein
- Refractory overt hepatic encephalopathy
- Severe circulatory diseases, i.e.:
- Ongoing infection or sepsis
- Severe, uncorrectable coagulopathy (relative contraindication) 
- Anatomical barriers (relative contraindication) 
- Procedure 
- Complications 
- Prevention of complications: Periprocedural rifaximin may be used to reduce the risk of hepatic encephalopathy. 
Surgical portosystemic shunts 
- Indications 
- Total (nonselective) portosystemic shunts: The portal vein is completely shunted to the vena cava, reducing portal pressure. 
- Selective portosystemic shunts
- Life-threatening esophageal variceal bleeding
- Portal hypertensive gastropathy
- Spontaneous bacterial peritonitis
- Hepatorenal syndrome
- Impaired liver function (see “Cirrhosis”)
- Hepatic encephalopathy
- Pulmonary complications of portal hypertension (e.g., hepatopulmonary syndrome, portopulmonary hypertension)
- Cirrhotic cardiomyopathy
- Gastric antral vascular ectasia
We list the most important complications. The selection is not exhaustive.