Alerts and Notices
SynopsisPortal hypertension (portal HTN) is defined as increased pressure in the portal system due to increased resistance of portal blood flow. This most commonly affects adults and children with cirrhosis or advanced fibrosis of the liver. Noncirrhotic portal HTN is a rarer entity caused by a variety of conditions, the most common being hepatic schistosomiasis.
The most common etiologies of cirrhosis / advanced fibrosis are nonalcoholic steatohepatitis, chronic viral hepatitis (mostly B and C), alcoholic hepatitis, and hemochromatosis.
Clinical manifestations of portal HTN include porto-systemic shunting (varices), splenomegaly, and thrombocytopenia. Symptoms of underlying cirrhosis such as jaundice may present first. Symptoms of portal HTN typically do not occur until complications arise. These complications can include ascites, spontaneous bacterial peritonitis (SBP), hepatorenal syndrome (HRS), hepatic hydrothorax, hepatopulmonary syndrome, portal hypertensive gastropathy, gastroesophageal variceal bleed, and hepatic encephalopathy. If the diagnosis is in doubt, the hepatic venous pressure gradient (HVPG) can be measured for confirmation. This represents the gradient between the pressure of the portal vein compared to the inferior vena cava (IVC), which is usually between 1-5 mm Hg.
Early diagnosis of portal HTN is key, as late manifestaptions of disease can be life-threatening and can result in severe decompensation in patients with cirrhosis.
Related topic: Portopulmonary hypertension
K76.6 – Portal hypertension
34742003 – Portal hypertension
Differential Diagnosis & PitfallsThe differential diagnosis for each manifestation of portal HTN can be broad. If the diagnosis remains unclear, measurement of HVPG can confirm portal HTN.
Hematemesis from gastroesophageal varices:
- Mallory-Weiss tear
- Peptic ulcer disease
- Dieulafoy lesion
- Malignant ascites
- Cardiac ascites due to congestive heart failure
- Renal ascites due to chronic kidney disease
- Nephrotic syndrome
- Heart failure (see congestive heart failure)
- Intestinal perforation
- Postoperative anastomotic leak
- Drug-induced acute kidney injury
- Acute tubular necrosis
- Intrinsic renal diseases
- Delirium from medications
- Urea cycle defects
Drug Reaction DataBelow is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.