eMedicine Specialties > Gastroenterology > Liver

Portal Hypertension: Differential Diagnoses & Workup

Author: Jesus Carale, MD, Consulting Gastroenterologist, Arkansas Valley Regional Medical Center, La Junta, Colorado
Coauthor(s): Sandeep Mukherjee, MB, BCh, MPH, FRCPC, Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Consulting Staff, Section of Gastroenterology and Hepatology, Veteran Affairs Medical Center
Contributor Information and Disclosures

Updated: Aug 24, 2009

Differential Diagnoses

Budd-Chiari Syndrome
Schistosomiasis
Cirrhosis
Tricuspid Regurgitation
Myeloproliferative Disease
Tuberculosis
Pericarditis, Constrictive
Vitamin A Toxicity
Polycystic Kidney Disease
Sarcoidosis

Other Problems to Be Considered

Many conditions are associated with portal hypertension (see Causes).

Workup

Laboratory Studies

  • Lab studies are directed towards investigating etiologies of cirrhosis, which is the most common cause of portal hypertension. Lab studies include the following:
    • Liver function tests
    • Prothrombin time
    • Albumin
    • Viral hepatitis serologies
    • Platelet count
    • Antinuclear antibody, antimitochondrial antibody, antismooth muscle antibody
    • Iron indices
    • Alpha1-antitrypsin deficiency
    • Ceruloplasmin, 24-hour urinary copper - To be considered only in individuals aged 3-40 years who have unexplained hepatic, neurologic, or psychiatric disease

Imaging Studies

  • Duplex-Doppler ultrasonography
    • Ultrasound (US) is a safe, economical, and effective method for screening for portal hypertension. It also can demonstrate portal flow and helps in diagnosing cavernous transformation of the portal vein, portal vein thrombosis, or splenic vein thrombosis.6
    • Features suggestive of hepatic cirrhosis with portal hypertension include the following:
      • Nodular liver surface is suggestive. However, this finding is not specific for cirrhosis and can be observed with congenital hepatic fibrosis and nodular regenerative hyperplasia.
      • Splenomegaly is a suggestive finding.
      • Patients may demonstrate the presence of collateral circulation.
    • Limitations of US include the following:
      • Reproducibility of data is problematic.
      • Many variables, such as circadian rhythm, meals, medications, and the sympathetic nervous system, affect portal hemodynamics.
      • Significant interobserver and intraobserver variation exist in quantitative ultrasonographic measurement.
  • CT scan
    • CT scan is a useful qualitative study in cases where sonographic evaluations are inconclusive.
    • CT scan is not affected by patients' body habitus or the presence of bowel gas.
    • With improvement of spiral CT scan and 3-dimensional angiographic reconstructive techniques, portal vasculature may be visualized more accurately.
    • Findings suggestive of portal hypertension include the following:
      • Collaterals arising from the portal system are suggestive of portal hypertension.
      • Dilatation of the IVC also is suggestive of portal hypertension.
    • Limitations of CT scan include the following:
      • It cannot demonstrate the venous and arterial flow profile.
      • Intravenous contrast agents cannot be used in patients with renal failure or contrast allergy.
  • Magnetic resonance imaging
    • MRI provides qualitative information similar to CT scan when Doppler findings are inconclusive.
    • MRI angiography detects the presence of portosystemic collaterals and obstruction of portal vasculature.
    • MRI also provides quantitative data on portal venous and azygos blood flow.
  • Liver-spleen scan
    • This is described for historical interest only.
    • Liver-spleen scan uses technetium sulfur colloid taken up by cells in the reticuloendothelial system.
    • A colloidal shift from the liver to the spleen or bone marrow is suggestive of increased portal pressure.
    • Limitations include the following:
      • Portal hypertension cannot be ruled out in the absence of this shift.
      • Liver-spleen scan images lack spatial resolution.
      • It has been superseded by US and CT scan.

Other Tests

  • Selective angiography of the superior mesenteric artery or splenic artery with venous return phase

Procedures

  • Hemodynamic measurement of portal pressure
    • Direct portal measurements usually are not performed due to the invasive nature, the risk of complications, and the interference of anesthetic agents on portal hemodynamics. The most commonly used method is measurement of the hepatic venous pressure gradient (HVPG), which is an indirect measurement that closely approximates portal venous pressure.
    • A fluid-filled balloon catheter is introduced into the femoral or internal jugular vein and advanced under fluoroscopy into a branch of the hepatic vein. Free hepatic venous pressure (FHVP) then is measured.
    • The balloon is inflated until it is wedged inside the hepatic vein, occluding it completely, thus equalizing the pressure throughout the static column of blood. The occluded hepatic venous pressure (ie, wedged hepatic venous pressure) minus the unoccluded, or free, portal venous pressure (ie, FHVP) is the HVPG.
  • Endoscopy
    • Perform upper endoscopy, as appropriate, to screen for varices in every patient with suggestive findings of portal hypertension. Additionally, all patients with cirrhosis should be considered for the presence of varices at the time of the initial diagnosis of cirrhosis. Gastroesophageal varices confirm the diagnosis of portal hypertension; however, their absence does not rule it out. At times, gastroesophageal varices are incidental findings in patients undergoing upper endoscopy for other reasons (eg, dyspepsia refractory to medications, dysphagia, weight loss). These patients should undergo further investigations for etiologies of portal hypertension.
    • Various indirect indices, such as platelet count, spleen size, albumin, and Child-Pugh score, have been studied to help diagnose varices without endoscopy. A recent case review study revealed some of these predictors as unreliable. For the time being, endoscopy remains the criterion standard for screening patients with cirrhosis for varices.
    • In compensated patients without varices, repeat endoscopy at 2- to 3-year intervals to evaluate for the development of varices.
    • In compensated patients with small varices, repeat endoscopy at 1- to 2-year intervals to evaluate the progression of varices.

Histologic Findings

On liver biopsy, histologic findings are varied and depend not only on the cause of liver disease but also on the cause of portal hypertension. Zone 3 necrosis can be observed in portal hypertension secondary to congestive heart failure and Budd-Chiari syndrome. In cases of normal liver parenchyma, investigate for prehepatic causes of portal hypertension.

More on Portal Hypertension

Overview: Portal Hypertension
Differential Diagnoses & Workup: Portal Hypertension
Treatment & Medication: Portal Hypertension
Follow-up: Portal Hypertension
Multimedia: Portal Hypertension
References
Further Reading
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References

  1. Theodorakis NG, Wang YN, Wu JM, Maluccio MA, Sitzmann JV, Skill NJ. Role of endothelial nitric oxide synthase in the development of portal hypertension in the carbon tetrachloride induced liver fibrosis model. Am J Physiol Gastrointest Liver Physiol. Jul 23 2009;[Medline].

  2. Sanyal AJ, Bosch J, Blei A, Arroyo V. Portal hypertension and its complications. Gastroenterology. May 2008;134(6):1715-28. [Medline].

  3. W. Ray KimRobert S. Brown Jr.Norah A. TerraultHashem El-Serag. Burden of liver disease in the United States: Summary of a workshop conducted by the AASLD. May 2001.

  4. Hou W, Sanyal AJ. Ascites: diagnosis and management. Med Clin North Am. Jul 2009;93(4):801-17, vii. [Medline].

  5. Chawla Y, Duseja A, Dhiman RK. Review article: Modern management of portal vein thrombosis. Aliment Pharmacol Ther. Aug 12 2009;[Medline].

  6. Singal AK, Ahmad M, Soloway RD. Duplex Doppler Ultrasound Examination of the Portal Venous System: An Emerging Novel Technique for the Estimation of Portal Vein Pressure. Dig Dis Sci. Jul 23 2009;[Medline].

  7. Sass DA, Chopra KB. Portal hypertension and variceal hemorrhage. Med Clin North Am. Jul 2009;93(4):837-53, vii-viii. [Medline].

  8. Rimola A, Garcia-Tsao G, Navasa M. Diagnosis, treatment and prophylaxis of spontaneous bacterial peritonitis: a consensus document. International Ascites Club. J Hepatol. Jan 2000;32(1):142-53. [Medline].

  9. Garcia-Tsao G, Sanyal AJ, Grace ND, Carey W. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology. Sep 2007;46(3):922-38. [Medline].

  10. Ferreira FG, Ribeiro MA, de Fátima Santos M, Assef JC, Szutan LA. Doppler Ultrasound Could Predict Varices Progression and Rebleeding After Portal Hypertension Surgery: Lessons from 146 EGDS and 10 Years of Follow-Up. World J Surg. Aug 13 2009;[Medline].

  11. Bonnet S, Sauvanet A, Bruno O, Sommacale D, Francoz C, Dondero F, et al. Long-term survival after portal vein arterialization for portal vein thrombosis in orthotopic liver transplantation. Gastroenterol Clin Biol. Jul 28 2009;[Medline].

  12. Abraczinskas DR, Ookubo R, Grace ND. Propranolol for the prevention of first esophageal variceal hemorrhage: a lifetime commitment?. Hepatology. Dec 2001;34(6):1096-102. [Medline].

  13. Baik SK, Jeong PH, Ji SW. Acute hemodynamic effects of octreotide and terlipressin in patients with cirrhosis: a randomized comparison. Am J Gastroenterol. Mar 2005;100(3):631-5. [Medline].

  14. Bhasin DK, Siyad I. Variceal bleeding and portal hypertension: new lights on old horizon. Endoscopy. Feb 2004;36(2):120-9. [Medline].

  15. Chalasani N, Imperiale TF, Ismail A. Predictors of large esophageal varices in patients with cirrhosis. Am J Gastroenterol. Nov 1999;94(11):3285-91. [Medline].

  16. Conn HO. Portal hypertension, varices, and transjugular intrahepatic portosystemic shunts. Clin Liver Dis. Feb 2000;4(1):133-50, vii. [Medline].

  17. de Franchis R. Updating consensus in portal hypertension: report of the Baveno III Consensus Workshop on definitions, methodology and therapeutic strategies in portal hypertension. J Hepatol. Nov 2000;33(5):846-52. [Medline].

  18. Feldman M, Scharschmidt B, Zorab R, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. Philadelphia, Pa:. WB Saunders;1998.

  19. Garcia-Pagan JC, Bosch J. Medical treatment of portal hypertension. Baillieres Best Pract Res Clin Gastroenterol. Dec 2000;14(6):895-909. [Medline].

  20. Garcia-Tsao G. Current management of the complications of cirrhosis and portal hypertension: variceal hemorrhage, ascites, and spontaneous bacterial peritonitis. Gastroenterology. Feb 2001;120(3):726-48. [Medline].

  21. Goh SH, Tan WP, Lee SW. Clinical predictors of bleeding esophageal varices in the ED. Am J Emerg Med. Jul 2005;23(4):531-5. [Medline].

  22. Henderson JM. Surgical treatment of portal hypertension. Baillieres Best Pract Res Clin Gastroenterol. Dec 2000;14(6):911-25. [Medline].

  23. Krige JE, Beckingham IJ. ABC of diseases of liver, pancreas, and biliary system. Portal hypertension-1: varices. BMJ. Feb 10 2001;322(7282):348-51. [Medline].

  24. Krige JE, Shaw JM, Bornman PC. The evolving role of endoscopic treatment for bleeding esophageal varices. World J Surg. 2005;29:966-73. [Medline].

  25. Lowe RC, Grace ND. Pharmacologic therapy for portal hypertension. Curr Gastroenterol Rep. Feb 2001;3(1):24-9. [Medline].

  26. Merkel C, Zoli M, Siringo S. Prognostic indicators of risk for first variceal bleeding in cirrhosis: a multicenter study in 711 patients to validate and improve the North Italian Endoscopic Club (NIEC) index. Am J Gastroenterol. Oct 2000;95(10):2915-20. [Medline].

  27. Pruvot FR, Quandalle P, Paris JC. [What's left for surgical treatment of portal hypertension in cirrhosis patients?]. Gastroenterol Clin Biol. Nov 2003;27(11):1013-20. [Medline].

  28. Russo MW, Brown RS Jr. Endoscopic treatment of patients with portal hypertension. Gastrointest Endosc Clin N Am. Jan 2001;11(1):1-14. [Medline].

  29. Samonakis DN, Triantos CK, Thalheimer U. Management of portal hypertension. Postgrad Med J. Nov 2004;80(949):634-41. [Medline].

  30. Schiff ER, Sorrell MF, Maddrey WC, eds. Schiff's Diseases of the Liver. Philadelphia, Pa:. Lippincott-Raven;1999.

  31. Sherlock S, Dooley J, eds. Diseases of the Liver and Biliary System. Oxford, UK:. Blackwell Science;1997.

  32. Soares-Weiser K, Brezis M, Tur-Kaspa R. Antibiotic prophylaxis of bacterial infections in cirrhotic inpatients: a meta-analysis of randomized controlled trials. Scand J Gastroenterol. Feb 2003;38(2):193-200. [Medline].

  33. Sterling RK, Sanyal AJ. Are TIPS tops in the treatment of portal hypertension? A review on the use and misuse of transjugular intrahepatic portosystemic shunts. Can J Gastroenterol. Nov 2000;14 Suppl D:122D-128D. [Medline].

  34. Wongcharatrawee S, Groszmann RJ. Diagnosing portal hypertension. Baillieres Best Pract Res Clin Gastroenterol. Dec 2000;14(6):881-94. [Medline].

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Keywords

portal hypertension, hypertension, liver transplant, ascites, esophageal varices, liver diseases, cirrhosis, portal vein, variceal hemorrhage

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Contributor Information and Disclosures

Author

Jesus Carale, MD, Consulting Gastroenterologist, Arkansas Valley Regional Medical Center, La Junta, Colorado
Jesus Carale, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association
Disclosure: Nothing to disclose.

Coauthor(s)

Sandeep Mukherjee, MB, BCh, MPH, FRCPC, Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Consulting Staff, Section of Gastroenterology and Hepatology, Veteran Affairs Medical Center
Sandeep Mukherjee, MB, BCh, MPH, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Ann Ouyang, MBBS, Professor, Department of Internal Medicine, Pennsylvania State University College of Medicine; Attending Physician, Division of Gastroenterology and Hepatology, Milton S Hershey Medical Center
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Noel Williams, MD, Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada
Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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