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portal hypertension

Elevation of venous pressure in the portal vein. Etiology: 1) hepatic cirrhosis (most common) 2) liver metastasis 3) hepatic failure 4) alcoholic hepatitis 5) Budd-Chiari syndrome 6) right heart failure 7) portal vein thrombosis 8) myxedema 9) fatty liver of pregnancy 10) veno-occlusive disease 11) schistosomiasis (immigrants & travelers from sub-Saharan Africa) [5] Pathology: 1) increased resistance to blood flow through the cirrhotic liver & portosystemic collaterals 2) increased blood flow to the mesenteric circulation 3) splenomegaly 4) esophageal varices 5) ascites a) 50% of patients with cirrhosis within 10 years b) portends 50% 2 year survival c) in ascites due to portal hypertension, blood pressure falls resulting in reduced renal blood flow & glomerular filtration [2] Clinical manifestations: 1) esophageal varices, gastric varices 2) ascites 3) hepatic encephalopathy Laboratory: - complete blood count (CBC) may show thrombocytopenia - serum bilirubin slightly elevated - serum albumin low - serum ALT & serum AST may be normal or slightly elevated - ova & parasites in stool for schistosomiasis [5] Special laboratory: - RUQ abdominal ultrasound Complications: - hepatopulmonary syndrome - portopulmonary hypertension Management: 1) management of ascites takes precedence over prevention of esophageal varices [5] 2) non-selective beta-blocker (nadolol, propranolol) to prevent bleeding from esophageal varices - non-selective beta-blocker not indicated in the absence of esophageal varices [5] 3) upper GI bleeding - prophylactic 3rd generation cephalosporin - octreotide or a somatostatin analog + proton-pump inhibitor [6] 4) endoscopic variceal ligation (see esophageal varices) - transfuse for GI bleed to blood hemoglogin < 10 g/dL - transfusion to hemoglobin >=10 g/dL can increase portal pressure increasing risk of bleeding [2] 5) see cirrhosis & ascites - spironolactone with or without furosemide - discontinue ACE inhibitor & ARBs in the absence of hypertension - compensatory upregulation of renin-angiotensin system supports blood pressure & renal function [2] 6) see schistosomiasis [5]

Related

portal vein

Specific

portopulmonary hypertension

General

sign/symptom venous disease; venopathy liver disease

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 368
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2021.
  3. Talwalker JT & Kamath PS, Influence of recent advances in medical management on clinical outcomes of cirrhosis Mayo Clin Proc 80(11):1501, 2005
  4. American Association for the Study of Liver Diseases. Boyer TD, Haskal ZJ The Role of Transjugular Intrahepatic Portosystemic Shunt (TIPS) in the Management of Portal Hypertension: update 2009. Hepatology. 2010 Jan;51(1):306. PMID: 19902484
  5. NEJM Knowledge+ Gastroenterology
  6. Nanchal R et al. Executive Summary: Guidelines for the management of adult acute and acute-on-chronic liver failure in the ICU: Neurology, peri-transplant medicine, infectious disease, and gastroenterology considerations. Crit Care Med 2023 May; 51:653. PMID: 37052435 https://journals.lww.com/ccmjournal/Fulltext/2023/05000/Executive_Summary__Guidelines_for_the_Management.10.aspx