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Portal Hypertension

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Portal Hypertension

DIAGNOSIS

HISTORY

para>In children, portal vein thrombosis is the most common extrahepatic cause; intrahepatic causes are more likely to be biliary atresia, viral hepatitis, and metabolic liver disease.

Initial Tests (lab, imaging)

Direct calculation of HVPG (approximation of the gradient in pressure between portal vein and IVC):

Endoscopy can diagnose esophageal and gastric varices and portal hypertensive gastropathy.

Patients with portal hypertension should be managed longitudinally by both a primary care physician and a gastroenterologist.

Admission Criteria/Initial Stabilization

Use isotonic fluid for hydration.

Refrain from drinking alcohol. Resources for patients who have difficulty with not drinking alcohol can be obtained from Alcoholics Anonymous at http://www.aa.org/.

11 Llop E, Berzigotti A, Reig M, et al. Assessment of portal hypertension by transient elastography in patients with compensated cirrhosis and potentially resectable liver tumors. J Hepatol. 2012;56(1):103 " 108.22 Hayes PC, Davis JM, Lewis JA, et al. Meta-analysis of value of propranolol in prevention of variceal haemorrhage. Lancet. 1990;336(8708):153 " 156.33 Garcia-Tsao G, Sanyal AJ, Grace ND, et al. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology. 2007;46(3):922 " 938.44 De Franchis R, Baveno VI Faculty. Expanding consensus in portal hypertension: report of the Baveno VI Consensus Workshop: stratifying risk and individualizing care for portal hypertension. J Hepatol. 2015;63(3):743 " 752.

34742003 Portal hypertension (disorder)

  • Weakness/fatigue
  • Jaundice
  • Symptoms of heart failure including chest pain, shortness of breath, and/or edema
  • Hematemesis
  • Melena
  • Oliguria
  • History of chronic liver disease
  • Alcoholic hepatitis
  • Alcohol abuse

PHYSICAL EXAM

  • Exam findings may be general or related to specific complications.
  • GeneralPallorIcterusDigital clubbingPalmar erythemaSplenomegalyCaput medusaSpider angiomataUmbilical bruitHemorrhoidsGynecomastiaTesticular atrophy
  • Gastroesophageal varicesHypotensionTachycardia
  • AscitesDistended abdomenFluid waveShifting dullness with percussion
  • Hepatic encephalopathyConfusion/comaAsterixisHyperreflexia
  • Pallor
  • Icterus
  • Digital clubbing
  • Palmar erythema
  • Splenomegaly
  • Caput medusa
  • Spider angiomata
  • Umbilical bruit
  • Hemorrhoids
  • Gynecomastia
  • Testicular atrophy
  • Hypotension
  • Tachycardia
  • Distended abdomen
  • Fluid wave
  • Shifting dullness with percussion
  • Confusion/coma
  • Asterixis
  • Hyperreflexia

DIFFERENTIAL DIAGNOSIS

  • Gastroesophageal varices with hemorrhagePortal hypertensive gastropathyHemorrhagic gastritisPeptic ulcer diseaseMallory-Weiss tear
  • AscitesSpontaneous bacterial peritonitis (SBP)Pancreatic ascitesPeritoneal carcinomatosisTuberculous peritonitisNephrotic syndromeFluid overload from heart failureHepatic malignancy
  • Hepatic encephalopathyDelirium tremensIntracranial hemorrhageSedative abuseUremia
  • Hepatorenal syndromeDrug nephrotoxicityRenal tubular necrosis
  • Portal hypertensive gastropathy
  • Hemorrhagic gastritis
  • Peptic ulcer disease
  • Mallory-Weiss tear
  • Spontaneous bacterial peritonitis (SBP)
  • Pancreatic ascites
  • Peritoneal carcinomatosis
  • Tuberculous peritonitis
  • Nephrotic syndrome
  • Fluid overload from heart failure
  • Hepatic malignancy
  • Delirium tremens
  • Intracranial hemorrhage
  • Sedative abuse
  • Uremia
  • Drug nephrotoxicity
  • Renal tubular necrosis

DIAGNOSTIC TESTS & INTERPRETATION

  • HVPG = wedged hepatic venous pressure (WHVP) " “ free hepatic venous pressure (FHVP)
  • HVPG >10, esophageal varices generally develop.
  • WHVP is estimated by occlusion of the hepatic vein by a balloon catheter and measurement of the proximal static column of blood.
  • FHVP is estimated by direct measurement of the patent hepatic vein, intra-abdominal inferior vena cava, or right atrium.
  • Nonspecific changes associated with underlying disease:Hypersplenism: anemia (also may be due to malnutrition or bleeding), leukopenia, thrombocytopeniaHepatic dysfunctionHypoalbuminemiaHyperbilirubinemiaElevated alkaline phosphataseElevated liver enzymes (AST, ALT)Abnormal clotting (prothrombin time, international normalized ratio, partial thromboplastin time)GI bleedingIron deficiency anemiaElevated serum ammoniaFecal occult bloodThrombocytopeniaHepatorenal syndromeElevated serum creatinine (Cr), blood urea nitrogen (BUN)Urine Na <5 mEq/L (<20 mmol/L)US and CT scan/MRI may detect cirrhosis, splenomegaly, ascites, and varices.US/duplex DopplerCan determine presence and direction of flow in portal and hepatic veinsUseful in diagnosing portal vein thrombosis, shunt thrombosis, or the presence of ascitesCT scan/MRI: angiographic measurement of hepatic venous wedge pressure via jugular or femoral veinCorrelates with portal pressureRisk of variceal bleeding is increased if hepatic venous pressure gradient >12 mm Hg.Upper GI series may outline varices in esophagus and stomach.Transient elastography is an emerging method to determine hepatic fibrosis and to predict portal hypertension (1).
  • Hypersplenism: anemia (also may be due to malnutrition or bleeding), leukopenia, thrombocytopenia
  • Hepatic dysfunctionHypoalbuminemiaHyperbilirubinemiaElevated alkaline phosphataseElevated liver enzymes (AST, ALT)Abnormal clotting (prothrombin time, international normalized ratio, partial thromboplastin time)
  • GI bleedingIron deficiency anemiaElevated serum ammoniaFecal occult bloodThrombocytopenia
  • Hepatorenal syndromeElevated serum creatinine (Cr), blood urea nitrogen (BUN)Urine Na <5 mEq/L (<20 mmol/L)
  • US and CT scan/MRI may detect cirrhosis, splenomegaly, ascites, and varices.
  • US/duplex DopplerCan determine presence and direction of flow in portal and hepatic veinsUseful in diagnosing portal vein thrombosis, shunt thrombosis, or the presence of ascites
  • CT scan/MRI: angiographic measurement of hepatic venous wedge pressure via jugular or femoral veinCorrelates with portal pressureRisk of variceal bleeding is increased if hepatic venous pressure gradient >12 mm Hg.
  • Upper GI series may outline varices in esophagus and stomach.
  • Transient elastography is an emerging method to determine hepatic fibrosis and to predict portal hypertension (1).
  • Hypoalbuminemia
  • Hyperbilirubinemia
  • Elevated alkaline phosphatase
  • Elevated liver enzymes (AST, ALT)
  • Abnormal clotting (prothrombin time, international normalized ratio, partial thromboplastin time)
  • Iron deficiency anemia
  • Elevated serum ammonia
  • Fecal occult blood
  • Thrombocytopenia
  • Elevated serum creatinine (Cr), blood urea nitrogen (BUN)
  • Urine Na <5 mEq/L (<20 mmol/L)
  • Can determine presence and direction of flow in portal and hepatic veins
  • Useful in diagnosing portal vein thrombosis, shunt thrombosis, or the presence of ascites
  • Correlates with portal pressure
  • Risk of variceal bleeding is increased if hepatic venous pressure gradient >12 mm Hg.

TREATMENT

GENERAL MEASURES

  • Avoid sedatives that may precipitate encephalopathy.
  • Limit sodium intake because cirrhotic patients avidly retain sodium.

MEDICATION

  • Prophylaxis against variceal bleeding (2,3)[A]:Nonselective Ž ²-blockadePropranolol: start with 10 to 20 mg/day PO BID to TIDNadolol: 40 to 80 mg/day PO once-daily dosingDoses may be titrated up as tolerated to maximum recommended doses; goal resting HR of 55 to 60 bpm
  • Therapy for acute variceal hemorrhage:Vasopressin: start with 0.2 to 0.4 U/min IV; increase to maximum dose 1 U/min as needed; pediatric dose: 0.002 to 0.005 U/kg/min; do not exceed 0.01 U/kg/min. After bleeding stops, continue at same dose for 12 hours and then taper off over 24 to 48 hours.Octreotide: 50 Ž Όg IV bolus, followed by 25 to 50 Ž Όg/hr continuous infusion; pediatric dose: 1 Ž Όg/kg bolus followed by 1 Ž Όg/kg/hr is used traditionally; treat for up to 5 days.
  • For prevention of recurrence and for overall reduction in mortality:Propranolol: 10 to 60 mg/day PO BID to QID; pediatric dose: 0.5 to 1 mg/kg/day PO divided q6 " “8hNadolol: 40 to 80 mg/day PO reduces portal venous blood inflow by blocking the adrenergic dilatation of the mesenteric arterioles.Tetrandrine, a calcium channel blocker, also has been found to reduce the rate of rebleeding with fewer side effects.
  • Initial treatment for ascites (along with salt and fluid restriction):Furosemide: 20 to 40 mg/day PO; pediatric dose: 1 to 2 mg/kg/dose PO ‚ ± IV albumin infusionSpironolactone: 50 to 100 mg/day PO; pediatric dose: 1 to 3 mg/kg/day PO
  • Nonselective Ž ²-blockadePropranolol: start with 10 to 20 mg/day PO BID to TIDNadolol: 40 to 80 mg/day PO once-daily dosing
  • Doses may be titrated up as tolerated to maximum recommended doses; goal resting HR of 55 to 60 bpm
  • Propranolol: start with 10 to 20 mg/day PO BID to TID
  • Nadolol: 40 to 80 mg/day PO once-daily dosing
  • Vasopressin: start with 0.2 to 0.4 U/min IV; increase to maximum dose 1 U/min as needed; pediatric dose: 0.002 to 0.005 U/kg/min; do not exceed 0.01 U/kg/min. After bleeding stops, continue at same dose for 12 hours and then taper off over 24 to 48 hours.
  • Octreotide: 50 Ž Όg IV bolus, followed by 25 to 50 Ž Όg/hr continuous infusion; pediatric dose: 1 Ž Όg/kg bolus followed by 1 Ž Όg/kg/hr is used traditionally; treat for up to 5 days.
  • Propranolol: 10 to 60 mg/day PO BID to QID; pediatric dose: 0.5 to 1 mg/kg/day PO divided q6 " “8h
  • Nadolol: 40 to 80 mg/day PO reduces portal venous blood inflow by blocking the adrenergic dilatation of the mesenteric arterioles.
  • Tetrandrine, a calcium channel blocker, also has been found to reduce the rate of rebleeding with fewer side effects.
  • Furosemide: 20 to 40 mg/day PO; pediatric dose: 1 to 2 mg/kg/dose PO ‚ ± IV albumin infusion
  • Spironolactone: 50 to 100 mg/day PO; pediatric dose: 1 to 3 mg/kg/day PO
  • Terlipressin (2 mg IV q4h; titrate down to 1 mg IV q4h once hemorrhage is controlled; may be used for up to 48 hours) is a more selective splanchnic vasoconstrictor and may be associated with fewer complications. It is currently used when standard therapy with somatostatin or octreotide fails.
  • Addition of nitrates, such as nitroglycerin or isosorbide mononitrate, reduces portal pressures and bleeding rates and has been shown to reduce mortality. Because the risk " “benefit ratio is not clear, nitrates are not considered first-line treatment.
  • Studies are ongoing for possible benefits of other agents including simvastatin, clonidine, verapamil, and losartan.

ISSUES FOR REFERRAL

SURGERY/OTHER PROCEDURES

  • Treatments available for specific complications of portal hypertension (in addition to or if refractory to medications):Gastroesophageal varices with hemorrhageEndoscopic variceal banding or sclerosis (the first-line treatment in many cases for acute hemorrhage) within 12 hours of presentation (4)[A]Balloon tamponade (not used commonly when endoscopic treatment is available)Transjugular intrahepatic portosystemic shunt (TIPS)Portocaval shuntingAscites refractory to medical managementLarge-volume paracentesisPeritoneovenous shuntTIPS
  • Liver transplantation should be considered for patients with advanced disease.
  • Gastroesophageal varices with hemorrhageEndoscopic variceal banding or sclerosis (the first-line treatment in many cases for acute hemorrhage) within 12 hours of presentation (4)[A]Balloon tamponade (not used commonly when endoscopic treatment is available)Transjugular intrahepatic portosystemic shunt (TIPS)Portocaval shunting
  • Ascites refractory to medical managementLarge-volume paracentesisPeritoneovenous shuntTIPS
  • Endoscopic variceal banding or sclerosis (the first-line treatment in many cases for acute hemorrhage) within 12 hours of presentation (4)[A]
  • Balloon tamponade (not used commonly when endoscopic treatment is available)
  • Transjugular intrahepatic portosystemic shunt (TIPS)
  • Portocaval shunting
  • Large-volume paracentesis
  • Peritoneovenous shunt
  • TIPS

INPATIENT CONSIDERATIONS

  • Acute GI bleeding should be managed in the inpatient setting, either on the regular medical floor if the patient is hemodynamically stable or occasionally in the ICU if the patient is unstable.
  • Patients with mental status changes from encephalopathy need to be evaluated in the inpatient setting.
  • Acute bleeding from the intestinal tract, either vomiting or per rectum
  • Acute confusional state/mental status changes
  • If acute variceal bleeding:Type and cross patient 's blood.Initial resuscitation with isotonic fluid until packed RBCs are availableCorrect coagulopathy with vitamin K and fresh frozen plasma (FFP).Endoscopy as soon as the patient is stabilized (for diagnosis and treatment)
  • Avoid sedatives that may precipitate encephalopathy.
  • Limit sodium administration because cirrhotic patients avidly retain sodium.
  • Restrict protein only if encephalopathic.
  • Type and cross patient 's blood.
  • Initial resuscitation with isotonic fluid until packed RBCs are available
  • Correct coagulopathy with vitamin K and fresh frozen plasma (FFP).
  • Endoscopy as soon as the patient is stabilized (for diagnosis and treatment)

If the patient is an active alcohol drinker, watch for signs and symptoms of withdrawal. Follow inpatient protocols for alcohol withdrawal management.

  • For GI bleeding:No active bleeding in 24 hoursStable hemoglobin and hematocritHemodynamically stable (especially heart rate)
  • For encephalopathy: improvement in or resolution of mental status changes to baseline
  • No active bleeding in 24 hours
  • Stable hemoglobin and hematocrit
  • Hemodynamically stable (especially heart rate)

ONGOING CARE

DIET

PATIENT EDUCATION

PROGNOSIS

  • Hepatic reserve defined by Child-Pugh classification: rating based on encephalopathy, ascites, bilirubin, albumin, prothrombin
  • Variceal bleeding1/3 of patients with known varices will bleed eventually.50% rebleed, usually within 2 years, unless portal pressure is reduced by surgical or TIPS procedure.15 " “20% mortality rate
  • Ascites and encephalopathy often recur.
  • Prognosis of patients with ascites is poor: 50% 1-year survival without liver transplant (compared with 90% for patients with cirrhosis and no ascites)
  • 1/3 of patients with known varices will bleed eventually.
  • 50% rebleed, usually within 2 years, unless portal pressure is reduced by surgical or TIPS procedure.
  • 15 " “20% mortality rate

REFERENCES

ADDITIONAL READING

  • Abraldes ‚ JG, Angermayr ‚ B, Bosch ‚ J. The management of portal hypertension. Clin Liver Dis. 2005;9(4):685 " “713.
  • Bloom ‚ S, Kemp ‚ W, Lubel ‚ J. Portal hypertension: pathophysiology, diagnosis and management. Intern Med J. 2015;45(1):16 " “26. doi:10.1111/imj.12590.
  • Bosch ‚ J, Berzigotti ‚ A, Garcia-Pagan ‚ JC, et al. The management of portal hypertension: rational basis, available treatments and future options. J Hepatol. 2008;48(Suppl 1):S68 " “S92.
  • Sanyal ‚ AJ, Bosch ‚ J, Blei ‚ A, et al. Portal hypertension and its complications. Gastroenterology. 2008;134(6):1715 " “1728.

CODES

ICD10

ICD9

SNOMED

CLINICAL PEARLS

  • Portal hypertension can be diagnosed based on physical examination in the setting of known risk factors, specifically cirrhosis.
  • Endoscopic treatment is successful for acute variceal hemorrhage 85% of the time.
  • Prognosis of patients with ascites is poor: 50% 1-year survival without liver transplant (compared with 90% for patients with cirrhosis and no ascites).
  • Advantages and disadvantages of balloon tamponade for acute variceal bleed:Advantages include rapid and often effective control of bleeding (30 " “90%) and common availability of device.Disadvantages include recurrence of bleeding when balloon is deflated, patient discomfort, and risk of esophageal perforation.
  • Advantages include rapid and often effective control of bleeding (30 " “90%) and common availability of device.
  • Disadvantages include recurrence of bleeding when balloon is deflated, patient discomfort, and risk of esophageal perforation.