How can I be sure that the patient has portal hypertension?
Portal hypertension may be clinically silent or it may present with life-threatening gastrointestinal symptoms, such as bleeding. Signs and symptoms are varied and related to the underlying cause of the portal hypertension.
– Systemic: thrombocytopenia, splenomegaly, caput medusae, hemorrhoids, spider angiomata, or palmar erythema
– Other signs of cirrhosis: jaundice, muscle wasting, Terry’s nails, gynecomastia, nodular liver, or testicular atrophy
– Varices: may be asymptomatic or present with hemorrhage
– Portal gastropathy: asymptomatic, chronic anemia, or acute upper GI bleeding
– Ascites: abdominal distention, abdominal pain, early satiety, or dyspnea
– Altered neurocognitive function: asterixis, subtly impaired neurocognitive functions, altered mental status, confusion, disorientation
– Hepatorenal syndrome: uremia, oliguria
– Hepatopulmonary syndrome: dyspnea, platypnea, orthodeoxia, digital clubbing
– Portopulmonary hypertension: dyspnea on exertion, chest pain, cor pulmonale
– Cirrhotic cardiomyopathy: lower extremity edema, dyspnea on exertion
Portal hypertension is defined as a pathologic elevation in portal venous pressures. The causes are classified as presinusoidal, sinusoidal, or postsinusoidal. In presinusoidal portal hypertension, there is obstruction or stenosis in the portal vein, its tributaries, or intrahepatic branches. Sinusoidal portal hypertension, the most common type of portal hypertension, is usually the result of cirrhosis. Postsinusoidal portal hypertension is the result of disrupted or congested hepatic venous outflow, such as that seen in Budd-Chiari syndrome.
The effects of portal hypertension depend on the area affected. Splenic vein or portal vein thrombosis may lead to the development of gastrointestinal bleeding secondary to varices while having few other signs or symptoms. Sinusoidal and postsinusoidal portal hypertension can have profound systemic effects such as varices, portal gastropathy, ascites, hepatic encephalopathy, increased infection risk, hepatorenal syndrome, hepatopulmonary syndrome, or portopulmonary hypertension.
See Table I for a listing of clinical features in portal hypertension, Table II for a listing of characteristic features, Table III less common clinical presentations, and Table IV for diseases and conditions that mimic the disease.
|Presinusoidal||Splenic vein thrombosisPortal vein thrombosisExternal compression of the portal veinSchistosomiasis||Isolated gastric varicesEsophageal varicesEpigastric pain|
|Sinusoidal||CirrhosisInfiltrative disorders (sarcoidosis, amyloidosis, hematologic malignancies)TuberculosisAlcoholic hepatitisNodular regenerative hyperplasiaPartial nodular transformationHypervitaminosis AIdiopathic||AscitesEsophageal and gastric varicesEncephalopathyPortal gastropathyPortopulmonary hypertensionHepatopulmonary syndromeHepatorenal syndromeCirrhotic cardiomyopathyThrombocytopeniaPhysical exam findings of cirrhosis|
|Post-sinusoidal||Budd-Chiari syndromeInferior vena cava stenosis or thrombosisConstrictive pericarditisRight-sided heart failureVeno-occlusive disease||AscitesAbdominal pain (particularly right upper quadrant)Hepatomegaly|
|Sequelae||Signs and symptoms||Diagnostic testing|
|Physical examination||Petechiael rash secondary to thrombocytopeniaSplenomegalyCaput medusaeHemorrhoidsSpider angiomataPalmar erythema Other signs of cirrhosisJaundiceMuscle wastingTerry’s nailsGynecomastiaNodular liver;Testicular atrophy||Complete blood countHepatic panelAbdominal ultrasound|
|Ascites||Abdominal distentionAbdominal painEarly satietyDyspnea||Serum to ascites albumin gradient (SAAG) >1.1;Total ascites protein <2 g/dLWhite blood cell count <250 pmn/L|
|Varices||AsymptomaticLife-threatening gastrointestinal bleeding||Endoscopy|
|Hepatic encephalopathy||AsymptomaticReversal of sleep-wake cycleIrritabilityConfusionPerseverationStuporComa||Clinical diagnosis+/- AsterixisPsychometric testing|
|Clinical presentation||Signs and symptoms||Diagnosis|
|Hepatorenal syndrome||OliguriaRising creatinineUrine sodium <10 meq/LUrine protein <500 mg/dayUrine RBC <50 cells per HPFLack of preceding shock ornephrotoxic drug exposure Relative lack of granularcasts||Creatinine >1.5 mg/dLNo improvement after 48 hours of diuretic withdrawal andvolume expansion with albuminType 1: doubling of initial creatinine to at least 2.5 mg/dL in <2 weeksType 2: creatinine >1.5 mg/dL with slow and progressive decline.|
|Hepatic hydrothorax||DyspneaOrthopneaHypoxiaRight-sided pleural effusion in 85%Left-sided effusion in 13%Bilateral effusion in 2%||Chest X-rayThoracentesis with SAAG >1.1 and characteristics similar to ascitic fluidRapid reaccumulation|
|Portopulmonary hypertension||Dyspnea on exertionChest painOrthopneaTricuspid regurgitant murmurEdema||EchocardiogramRight heart catherization|
|Hepatopulmonary syndrome||Dyspnea;Platypnea;Orthodeoxia;Spider angiomata;Digital clubbing||Oxygen saturation <94% on room airEchocardiogram with bubble injectionNuclear scan using technetium-labeled macroaggregated albuminArterial blood gas with calculation of shunt fraction|
|Spontaneous bacterial peritonitis (SBP)||Protean manifestations;Abdominal pain;Fever;Hepatic encephalopathy;Renal failure;Acute or chronic liver failure.||Ascitic fluid with neutrophil count <250 cells/mLPositive ascitic fluid cultureNo other intra-abdominal source|
|Cirrhotic cardiomyopathy||Asymptomatic until under stressDyspnea with exertionEdema||EchocardiogramRight and left heart catherization|
|Alternative diagnosis||Presentation||Differentiating factors|
|GI bleeding from source other than varices such as:UlcerDieulafoy’s lesionMallory Weiss tearDiverticulosisAngioectasiaNeoplasm||Upper, mid, or lower GI bleeding||Endoscopy|
|Altered mental status not due to hepatic encephalopathy:HyponatremiaStrokeDrugsAlcohol withdrawalMeningitisIntracranial bleeding||Altered mental status||Focal neurologic deficitsLack of asterixisImaging abnormalitiesLab abnormalitiesNucal rigidity|
|Congestive heart failure or pulmonary hypertension||AscitesHepatomegalyAbnormal liver function assays||Abnormal echocardiogramHigh SAAG (>1.1), high protein (>2.5 g/^ ^dL) ascitesPulsatile liverElevated jugular venous pressure|
|Malignancy such as peritoneal carcinomatosis||AscitesAbdominal pain and distention||Low SAAG (<1.1) asciteAbnormal cytopathologyAbnormal imagingLymphoma and chylous ascites|
|Pancreatitis||AscitesAbdominal pain||Low SAAG (<1.1) ascitesHigh protein (>2.5g/dl) ascitesAmylase in peritoneal fluid >1000 IU/LAbnormal imaging|
|Tuberculous peritonitis||Abdominal painFeverAscitesNight sweatsWeight loss||Low SAAG (<1.1) ascitesHigh lymphocyte countPositive PPD Elevated ascitic fluid adenosine deaminase activity|
|Severe malnutrition||AscitesAnasarcaElevated PT/INRLow albuminHigh transaminase levels, particularly AST||Administration of vitamin K will lead to reversal of coagulopathy. Lack of other evidence of underlying liver disease/ cirrhosis|
A tabular or chart listing of features and signs and symptoms
Table II. Characteristic features of portal hypertension
How can I confirm the diagnosis?
Portal hypertension is by definition the result of other pathology, and early testing should focus on determining if the patient truly has portal hypertension, evaluating for common causes of portal hypertension, and diagnosing the sequelae of portal hypertension that can lead to increased morbidity and mortality.
– Detailed history and physical to determine risk factors for cirrhosis or thrombosis
– Social history addressing alcohol use, travel, and family history
– CBC to assess for thrombocytopenia and anemia
– Hepatic panel, albumin, and prothrombin time/INR (international normalized ratio) to assess for signs of hepatic damage and function
– Evaluation for common and uncommon causes of cirrhosis: hepatitis B and C, lipid panel, glycosylated hemoglobin (A1c), iron panel, ceruloplasmin, and alpha-1 anti-trypsin
Confirmatory testing can and should be done as first-line testing if the patient has evidence of decompensated portal hypertension.
– Paracentesis if clinically detectable ascites is present:
Albumin, total protein, cell count with differential, and culture (if infection suspected) should be sent.
A serum ascites albumin gradient (SAAG) >1.1 is consistent with portal hypertension.
Further analysis such as amylase, creatinine, adenosine deaminase activity, triglycerides, and cytopathology may be sent, depending on the clinical picture and results of initial fluid analysis.
– Upper endoscopy to evaluate for varices and/or portal gastropathy
– Doppler ultrasound to evaluate for vascular patency, signs of cirrhosis, and evidence of ascites
– Echocardiogram: if severe ascites, renal dysfunction, or other evidence to suggest cardiac involvement
If the diagnosis of portal hypertension is still suspected despite negative or conflicting initial testing, then invasive testing may be warranted.
– Transjugular or percutaneous measurement of hepatic venous pressure gradient (HVPG)
HVPG <5: normal and does not represent portal hypertension
HVPG >10: will begin to form varices
HVPG >20: high risk for recurrent or severe variceal bleeding
– Liver biopsy
See Figure 1 for a diagnostic algorithm for portal hypertension and Table V.
|Test||Expected findings||Consistent findings||Inconsistent findings|
|Labs||Thrombocytopenia||Hypoalbuminemia;Elevated PT/INR;Elevated bilirubin;Macrocytic anemia;Elevated transaminases;Hyponatremia||None; any lab findings are possible.|
|Endoscopic||Esophageal varices;GOV 1 gastric varices;GOV 2 gastric varices;Isolated gastric varices;Portal gastropathy;Rectal varices||Gastric antral vascular ectasia;Nonspecific edema||There may be no endoscopic finding early in the course.|
|Radiologic||Ultrasound with Doppler is first line.CT scan with contrast and MRI are alternatives.Enlarged portal vein;Doppler evidence of stagnant or reversed portal vein flow||Portosystemic collaterals;Splenomegaly;Ascites;Portal, splenic, hepatic, or IVC thrombosis||Lack of portal vein enlargement or splenomegaly is not consistent with portal hypertension but cannot alone rule it out.|
What other diseases, conditions, or complications should I look for in patients with portal hypertension?
Portal hypertension predisposes the patient to multiple life-threatening conditions, including bleeding, infection, renal failure, encephalopathy, and death. Bleeding can occur as the result of either variceal hemorrhage, slow blood loss from portal gastropathy, or from procedure such as paracentesis.
Cirrhosis in particular leads to an immunodeficient state. Patients with portal hypertension are at increased risk of infection and even mundane infections can cause multiorgan system failure and death. Renal failure can be the direct result of portal hypertension in the form of hepatorenal syndrome or can result from over diuresis and hypotension. Encephalopathy can affect all aspects of the lives of both the patients, as well as their caretakers. Encephalopathy can be intermittent or persistent, covert or overt. The effects of encephalopathy range from reversal of the sleep-wake cycle to forgetfulness, poor driving ability, inability to work, coma, and death. Other problems such as hyponatremia, hypotension, and malnutrition also complicate portal hypertension and its management.
Portal hypertension is a sign of a larger ongoing disease process and can be broken down into presinusoidal, sinusoidal, or postsinusoidal.
Presinusoidal. This type of portal hypertension is most commonly associated with prothrombotic states, inflammation, or malignancy. Splenic vein thrombosis is frequently associated with pancreatitis or pancreatic malignancies. Portal vein thrombosis can be the result of pylephlebitis, following bouts of diverticulitis or appendicitis. Direct compression by tumors, invasion by tumors (hepatocellular carcinoma, pancreatic cancer), and sluggish portal vein flow (cirrhosis) may also trigger portal thrombosis and presinusoidal portal hypertension. Rarer disease entities, such as schistosomiasis, may also lead to this condition.
Sinusoidal portal hypertension. The most common form of portal hypertension is most strongly associated with cirrhosis. Alcoholic liver disease causes the earliest and most significant portal hypertension due to fibrosis around the central vein. Other disease states associated with sinusoidal portal hypertension include acute fatty liver of pregnancy, nodular regenerative hyperplasia, peliosis hepatis, congenital hepatic fibrosis, idiopathic portal hypertension, hypervitaminosis A, and infiltrative disorders such as tuberculosis, sarcoidosis, amyloidosis, and hematologic malignancies.
Postsinusoidal hypertension. Most commonly related to prothrombotic or cardiac conditions. Budd-Chiari syndrome is hepatic venous outflow obstruction. It is seen with oral contraceptive pill use, JAK2 kinase mutations, myeloproliferative disorders, and other prothrombotic conditions. Any malignancy can lead to a prothrombotic state causing portal hypertension. Veno-oclusive disease is an entity that is seen most commonly in the post-bone-marrow transplant setting, as well as after exposure to certain chemotherapeutic agents. Cardiac causes such as constrictive pericarditis, cor pulmonale, and chronic heart failure cause portal hypertension by increasing hepatic outflow pressures.
The complications of portal hypertension include development of varices with possibility of hemorrhage, hepatic encephalopathy, infection, and renal failure. Full discussion of complications and treatment strategies follow. (See
|Varices||2-5% of cirrhotic patients develop esophageal varices de novo per year.1 in 3 patients with varices will have a variceal bleed.Small varices have <10% chance of bleeding in 1 year.Large varices with red signs have >75% chance of bleeding within 1 year.Gastric varices develop in 20% of patients with portal hypertension and carry a 10-36% chance of bleeding||Each episode of bleeding is associated with approximately 20% chance of mortality. Rebleeding is common.|
|Hepatic encephalopathy||Minimal hepatic encephalopathy may affect up to 80% of patients with cirrhosis.Overt hepatic encephalopathy affects up to 50%.Most common cause of hospitalization in cirrhotics.||Decreases quality of life.Interferes with ability to work (especially blue collar jobs).Impairs driving ability.Overt encephalopathy can lead to chronic neurocognitive dysfunction.|
|Infection||Common cause of morbidity and mortality.32-34% of hospitalized cirrhotics are infected compared with 5-7% of the general inpatient population.At the time of decompensation, severe enough to require hospitalization:SBP most common (25%);Followed by UTI, pneumonia, skin and soft tissue infections.||30% of cirrhotics with infections will die in 1 month.Additional 30% will die within 1 year.Renal failure develops in 1/3 of cirrhotic patients who develop SBP and other severe infections.|
|Renal failure||In patients with ascites: 18% will develop hepatorenal syndrome at 1 year and 39% by 5 years.Prerenal azotemia and acute tubular necrosis are also common causes of renal failure.||1 month mortality in type 1 hepatorenal syndrome is 80%, and 90% at 6 months without liver transplant.Type 2 hepatorenal syndrome has a 20% and 60% 1- and 6-month mortality, respectively.Cirrhotic patients admitted to an ICU with renal failure have an inpatient mortality from 65-87%.|
What is the right therapy for the patient with portal hypertension?
Directly treating portal hypertension involves invasive procedures such as transjugular intrahepatic portocaval shunts and surgical portocaval shunts. These procedures carry significant morbidity and mortality. As a result, they are generally reserved for salvage. Therapy is usually targeted at the sequelae of the portal hypertension such as endoscopic banding of varices. These therapies vary significantly and will be discussed in more detail for each individual complication. In cases where there is a reversible component, such as acute portal or hepatic vein thrombosis, therapies such as anticoagulation or angioplasty can result in resolution or improvement of the portal hypertension. Liver transplantation is often the only therapeutic option that will cure portal hypertension and provide long-term survival.
Therapyfor portal hypertension is complicated and involves anticipating andpreventing serious complications from the myriad sequelae of portalhypertension. Individual treatments for the causes and complications ofportal hypertension is discussed in the following paragraphs. General principles are as follows.
– Maximize nutrition (avoid protein restrictions).
– Minimize sodium intake (goal 2 g per day).
– Maintain activity level.
– Vaccinate against preventable illnesses (influenza, pneumococcus, hepatitis A and B).
– Screen for age-appropriate malignancies (hepatocellular carcinoma, colon cancer, breast cancer, etc.).
– Anticipate and address psychological issues such as depression.
– Advocate for avoidance of harmful behavior and physical conditions (smoking, alcohol use, obesity).
– Treat underlying cause of portal hypertension when applicable.
– Regularly monitor for consequences of portal hypertension, such as ascites, encephalopathy, and bleeding.
– Follow up frequently in clinic (every 3-6 months).
What is the most effective initial therapy?
The most effective therapy for portal hypertension is removing the cause of the portal hypertension, but this is seldom possible. In cases of acute thrombosis (Budd-Chiari syndrome, acute portal vein thrombosis), therapy as simple as anticoagulation may effectively treat the portal hypertension. This is the exception rather than the rule. Table VII shows a listing of general principles in treating the causes of portal hypertension.
|Cause of portal hypertension||Initial therapy||Salvage therapy|
|Splenic vein thrombosis||Conservative management;Treatment of underlying disorder (i.e., pancreatitis);Screening for varices||If bleeding from gastric varices is persistent problem, splenectomy is recommended.Cyanoacrylate injection of gastric varices is being performed in an investigational manner in some centers.|
|Acute portal vein thrombosis||Data showing benefit of anticoagulation in cirrhotics is lacking.Screen for hypercoagulable state.Screen for varices, splenomegaly, ascites.If above are present: anticoagulate only if hypercoagulable state is discovered as thrombosis is likely to be chronic.If signs of chronic portal hypertension are negative, anticoagulate.||If patient is cirrhotic and has patent intrahepatic portal branches, there is some evidence to suggest that a TIPS plus anticoagulation is helpful and may lead to restoration of flow in the portal vein. This data is preliminary and a decision to perform a TIPS in this situation should be made in an experienced transplant center.|
|Noncirrhotic sinusoidal portal hypertension||Referral to a hepatologist or liver transplant center.|
|Cirrhosis||Treatment of underlying cause (hepatitis B/C, autoimmune);Alcohol cessation;Screening for varices;Screening for hepatocellular carcinoma||Referral to liver transplant center for evaluation as MELD score approaches 14 or if any signs of decompensation (ascites, encephalopathy, hemorrhage, hyponatremia, renal insufficiency).|
|Budd-Chiari syndrome||Anticoagulation;Screen for prothrombotic states.Angioplasty of short stenoses;Angioplasty plus anticoagulation successful in 20-30% of cases;Screening for varices;Consider liver biopsy||TIPS is a therapeutic option that can be considered first-line therapy with anticoagulation or can be reserved for those who fail angioplasty and anticoagulation. Candidates for TIPS should have well-preserved liver functions (MELD <17), no evidence of right-sided heart failure or diastolic dysfunction, and no history of severe encephalopathy. The transcaval approach is preferred. TIPS has been shown to increase survival.Surgical portocaval shunt is also an option although it should be performed by an experienced surgeon.Liver transplant referral should be made if conservative management fails or there is underlying cirrhosis.|
Listing of usual initial therapeutic options, including guidelines for use, along with expected result of therapy.
Most patients with portal hypertension will not have a readily reversible cause, and therapeutic options will be limited to treating the complications of the disease. Following is a table of first-line therapies for managing the complications of portal hypertension. For less common presentations of portal hypertension, such as portopulmonary hypertension, hepatic hydrothorax, and hepatopulmonary syndrome, referring to a liver transplant center is recommended. (See
|Ascites||Paracentesis with fluid analysis (cell count with differential, total protein and albumin);2 g sodium restriction;If sodium restriction is not effective, diuretic therapy should be initiated.Diuretic therapy with loop diuretic (furosemide) and aldosterone antagonist (spironolactone) dosed in a stepwise fashion:Step 1- Furosemide 40 mg and spironolactone 100 mg p.o. dailyStep 2- Furosemide 80 mg and spironolactone 200 mg p.o. dailyStep 3- Furosemide 120 mg and spironolactone 300 mg p.o. dailyStep 4- Furosemide 160 mg and spironolactone 400 mg p.o. dailyRenal function and electrolytes should be monitored closely after initiating diuretic therapy, during therapy, and after increasing dosages.|
|Renal insufficiency||Hold diuretics.Volume replete with albumin 1 g/kg up to 100 gm per day for 48-72 hours.Check urinalysis, urine electrolytes, urine casts, and urine eosinophils.Investigate for causes of renal failure (drugs, nephrotoxins, e.g., IV contrast, hypotension, obstruction).Investigate for hemorrhage or infection.Consider early nephrology consultation.|
|Varices||EndoscopyNo varices: repeat EGD in 3 years. Small varices with no bleeding and no increased risk of bleeding (Childs B/C, red wale signs): repeat EGD in 2 years +/- nonselective beta blocker. Small varices with no bleeding and increased risk of bleeding: nonselective beta blocker. Medium/large varices that have not bled and no increased risk of bleeding: nonselective beta blockers preferred unless issues of tolerance or compliance are anticipated, then endoscopic variceal band ligation is preferred. Medium/large varices that have not bled but are at increased risk of bleeding: nonselective beta blockers or endoscopic variceal ligation. Nonselective beta blockers (nadolol and propranolol) should be titrated to maximally tolerated doses.Evolving data suggesting carvedilol is a safe alternative and may be beneficial in patients with known cardiovascular disease.Nitrates as treatment for varices should be avoided.|
|Hepatic encephalopathy||Investigation for and removal of precipitating event (GI bleed, infection, constipation, medication noncompliance, etc.).Correct electrolytes, especially hypokalemia. Nonabsorbable disaccharides, such as lactulose and lactitol, are effective in treating both minimal and overt hepatic encephalopathy: dosed to produce 3-4 bowel movements per day. Protein should not be restricted. Some studies suggest vegetable protein less likely to cause encephalopathy than animal protein.|
|Hepatic hydrothorax||Thoracentesis with fluid sent for analysis.Treat as ascites.Avoid placing a chest tube.If diuretics fail, TIPS may be effective.Refer to transplant center.|
|Spontaneous bacterial peritonitis (SBP)||Albumin 1.5 g/kg (up to 100 g) on day 1 and 1 g/kg on day 3.Third generation cephalosporin (i.e., ceftriaxone) is first line; alternatives include amoxicillin/clavulanate or quinolones.Failure of ascitic fluid WBC count to drop to <25% of original values after 2 days may signify antibiotic failure.After infection clears, patient should remain on secondary prophylaxis with ciprofoxacin 500 mg daily or norfloxacin 400 mg daily for life. Primary prophylaxis against SBP may be reasonable in patients with very protein poor ascitic fluid and decompensated disease (Child Pugh score >9, bili >3, creat >1.2, or sodium <130).|
|Portopulmonary hypertension or hepatic hydrothorax||Referral to transplant center.|
|Cirrhotic cardiomyopathy||Referral to transplant center.|
A listing of a subset of second-line therapies, including guidelines for choosing and using these salvage therapies
Initial therapy may not be effective or the patient may not tolerate the side effects of the therapy. Table IX shows general guidelines for second-line or salvage therapies for the complications of portal hypertension.
|Refractory ascites||Repeated large-volume paracentesis associated with immediate relief, but ascites typically recurs and there is no survival benefit.TIPS: better then repeated large-volume paracentesis for control of ascites. Increased incidence of encephalopathy and no survival advantage. Candidates for TIPS should have MELD scores <17, no evidence of right-sided heart failure or diastolic dysfunction, and no history of severe hepatic encephalopathy.Surgical portocaval shunt: effective but should be performed by an experienced surgeon.|
|Type 1 hepatorenal syndrome||If not already evaluated, expedite liver transplant evaluation.Terlipressin plus albumin if available (not available in the U.S.A.);Midodrine plus octreotide plus albumin may be beneficial in absence of terlipressin.Hemodialysis only useful as a bridge to transplant.TIPS cannot be recommended as a therapy at this time.|
|Variceal bleeding||Admission to ICU with aggressive hemodynamic support and blood transfusion.Consider intubation to protect the airway and avoid aspiration.Antibiotic therapy for 5-7 days: IV ceftriaxone 1 g/day is preferred, although IV ciprofloxacin or p.o. norfloxacin are alternatives in areas with low quinolone resistance.Vasoactive pharacotherapy with:Octreotide 50 μg bolus followed by 50 μg/hr vs somatostatin 250 μg bolus followed by 250 μg/hr vs terlipressin 2 mg bolus then1 -2 mg IV every 6 hours for 3-5 days.Endoscopy with endoscopic vein ligation (banding) or sclerotherapy within 12 hours of presentation.Balloon tampanade can be used for a maximum of 24 hours as a bridge to TIPS if bleeding cannot be stopped endoscopically. Failure of treatment is determined by continued hematemesis with hemodynamic compromise and/or need for >3 unit PRBC to keep hemoglobin around 8 g/dL over a 24-hour period.TIPS or surgical shunt is indicated if bleeding cannot be stopped or if the patient has repeated bleeding despite pharmacologic and endoscopic therapy.A hepatic vein pressure gradient >20 measured within 24 hours of bleeding has been associated with treatment failure and TIPS or surgical shunting may be beneficial in this population, but further study needs to be done before this is recommended as standard of care.Addition of nonselective beta blocker after bleeding has been stopped and patient stabilized is reasonable.|
|Persistent hepatic encephalopathy||Addition of a poorly absorbed antibiotic to lactulose therapy. Rifaxamin 400 mg t.i.d. or 550 mg b.i.d. Metronidazole and neomycin have been utilized in the past but have significant side effects with long-term use.Consider radiologic evaluation (MRA) for large portosystemic shunts. Closure of the shunts can improve encephalopathy.Probiotic use may be helpful.|
Listing of these, including any guidelines for monitoring side effects.
– Check renal function and electrolytes closely after starting and changing dosages of diuretics.
– Leg cramps, elevated BUN, creatinine, and CO2 may occur.
– Hyponatremia commonly occurs and may require diuretic dose reduction or free water restriction.
– Hypo- and hyperkalemia can be an issue, depending on the underlying renal function.
– Spironolactone may have antiandrogenic side effects (gynecomastia or breast tenderness).
– Post-TIPS patients may still require diuretic therapy.
– Monitor TIPS patients for hepatic encephalopathy and TIPS dysfunction.
– Monitor heart rate in patients treated with beta blockers.
– Recent evidence suggests increased mortality in patients with refractory ascites on beta blockers.
– Bands may slip off and ulcers may form with sclerosant or band therapy, which may rebleed.
– Balloon tampanade therapy may lead to esophageal necrosis or rupture.
– Placement of NG tube after endoscopic vein ligation is not contraindicated and may be useful in determining if there is rebleeding and in administering cathartics to clear the gut of blood.
– Education of patient, patient’s family, and nursing staff necessary to ensure lactulose is taken appropriately.
– Too much lactulose may cause diarrhea that can lead to worsened encephalopathy.
– C. difficile infection has been rarely reported on rifaxamin.
How should I monitor the patient with portal hypertension?
– Monitor labs frequently (electrolytes NA, K, Mg), as well as CO2 and BUN/Cr.
– Monitor sodium intake.
– Any complaint or hospitalization in a patient with ascites warrants a diagnostic paracentesis to rule out SBP.
– Monitor for side effects that may limit compliance (breast tenderness, dry mouth, leg cramps).
– ^ ^Perform paracentesis as needed for comfort.
– Consider primary prophylaxis against SBP if patient is decompensated (Child Pugh >9, bili >3, creat >1.2, or Na <130).
– Endoscopy should be performed when patient is diagnosed with portal hypertension.
– If well compensated, disease and no varices: EGD every 3 years.
– If small varices not on treatment: EGD every 1 to 2 years.
– If varices treated with beta blocker: no further endoscopy is warranted.
– If varices treated with endoscopic vein ligation: endoscopy performed every 2 to 4 weeks with rebanding until varices have been eradicated. Then repeat EGD in 1 to 3 months followed by every 6- to 12-month screens thereafter.
– Avoid medication that will cause further confusion (narcotics, benzodiazepines).
– Monitor for signs and symptoms of hepatic encephalopathy with each visit.
– Teach proper methods of taking lactulose.
– Consider safety of driving if patient has recurrent overt encephalopathy.
– Recommend patient not live alone if patient has history of encephalopathy.
– Refer for special neurocognitive testing if persistent encephalopathy or minimal encephalopathy is suspected.
– Monitor for and avoid infections.
The patient with portal hypertension should be evaluated by a gastroenterologist/hepatologist at least every 6 months to monitor for disease progression and ensure that proper prophylactic treatment and screens are being performed. Assuming the patient has cirrhosis:
– Follow up in clinic every 3 to 6 months, depending on the severity of their disease.
– Check MELD score labs, electrolytes, renal function, CBC, and PT/INR during each visit.
– As MELD score approaches 14 or if patient has decompensation, refer patient to transplant center for evaluation.
– Vaccinate against hepatitis B/C, Influenza and pneumococcus
– Monitor weight and muscle wasting.
– Monitor for signs of ascites or lower extremity edema.
– Monitor for early signs of bleeding (occasional melena).
– Monitor for signs of infection.
– Monitor for signs of encephalopathy.
– Monitor for signs of alcohol use, tobacco use, and or illicit drug use: counsel on cessation
– Counsel against having surgery without notifying the patient’s hepatologist.
– Screen for hepatocellular cancer every 6 months with hepatic ultrasound.
Measures of progress of disease
Measures of disease progression include the MELD score and Child Pugh score. These measures should be calculated on each visit.
– Objective measure.
– Calculated using the INR, creatinine, and bilirubin.
– Scored from 6 (normal) to 40 (critically ill).
– Patients considered for transplant evaluation and listing when MELD score approaches 14.
– MELD score used to prioritize the transplant list.
– Extra MELD points can be awarded to patient on the transplant list with certain conditions (i.e., hepatocellular carcinoma, hepatopulmonary syndrome, portopulmonary hypertension, and, in some centers, hyponatremia).
– MELD score predicts 3-month mortality in patients with cirrhosis.
Child Pugh Score (Child-Turcotte-Pugh score)
– Semisubjective scoring mechanism.
– Presence/severity of ascites, presence/severity of encephalopathy, bilirubin, albumin, and PT/INR are each scored.
– Scores range from 5 (normal) to 15 (severely decompensated disease).
– Subdivided into:
Child’s class A, score 5-6: compensated disease: 1-year survival approaches 100%.
Child’s class B, score 7-9: mild decompensation: 1 year survival 81%.
Child’s class C, score >10: decompensated: 1-year survival 45%.
All patients who are possible liver transplant candidates should be referred to a liver transplant center for evaluation as their MELD score approaches 14 or if they develop Child’s B cirrhosis.
What's the evidence?
Sanyal, AJ, Bosch, J, Blei, A, Arroyo, V. “Portal hypertension and its complications”. Gastroenterology. vol. 134. 2008. pp. 1715-28. (This work is a thorough overview of the pathology, complications, and management of patients with portal hypertension.)
Bhogal, HK, Sanyal, AJ. “Using transjugular intrahepatic portosystemic shunts for complications of cirrhosis”. Clin Gastroenterol Hepatol. 2011. (Recent review that details the evidence for and against the use of TIPS for the treatment of various complications of portal hypertension, such as bleeding and ascites.)
Runyon, BA. “AASLD Practice Guidelines Committee. Management of adult patients with ascites due to cirrhosis: an update”. Hepatology. vol. 49. 2009. pp. 2087-107. (The most up-to-date guidelines from the American Association for the Study of Liver Disease detailing the treatment and monitoring of patients with ascites.)
Serste, T, Melot, C, Francoz, C. “Deleterious effects of betablockers on survival in patients with cirrhosis and refractory ascites”. Hepatology. vol. 52. 2010. pp. 1017-22. (Recent prospective study suggesting that in patients with refractory ascites, the use of beta blockers for bleeding prophylaxis may result in higher morbidity and mortality.)
Prakash, R, Mullen, KD. “Mechanisms, diagnosis and management of hepatic encephalopathy”. Nat Rev Gastroenterol Hepatol. vol. 7. 2010. pp. 515-25. (Up-to-date review of the pathogenesis, evaluation, and treatment of encephalopathy in patients with portal hypertension.)
Bass, NM, Mullen, KD, Sanyal, AJ. “Rifaximin treatment in hepatic encephalopathy”. New Engl J Med. vol. 362. 2010. pp. 1071-81. (Randomized, blinded, placebo-controlled study demonstrating the effectiveness of rifaxamin in preventing recurrent episodes of hepatic encephalopathy. Subsequent studies have demonstrated its effectiveness for the treatment of minimal hepatic encephalopathy and for improving quality of life.)
Phongsamran, PV, Kim, JW, Cupo Abbott, J, Rosenblatt, A. “Pharmacotherapy for hepatic encephalopathy”. Drugs. vol. 70. 2010. pp. 1131-48. (A detailed review of the pharmacology for hepatic encephalopathy describing the mechanisms of action, as well as evidence for use and side effects.)
Garcia-Tsao, G, Bosch, J. “Management of varices and variceal hemorrhage in cirrhosis”. N Engl J Med. vol. 362. 2010. pp. 823-32. (Utilizes up-to-date thinking on portal hypertension and its pathophysiology to provide guidelines on treatment of varices and variceal hemorrhage.)
Garcia-Tsao, G, Sanyal, AJ, Grace, ND. “Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis”. Hepatology. vol. 46. 2007. pp. 922-38. (Data-driven, up-to-date guidelines on the prevention, management, and treatment of varices and variceal hemorrhage.)
Hashizume, M, Akahoshi, T, Tomikawa, M. “Management of gastric varices”. J Gastroenterol Hepatol. vol. 26. 2011. pp. 102-8. (Up-to-date paper detailing the evolving field of treatment for gastric varices that differ in their behavior and treatment from esophageal varices.)
Arvaniti, V, D’Amico, G. “Infections in patients with cirrhosis increase mortality four-fold and should be used in determining prognosis”. Gastroenterology. vol. 139. 2010. pp. 1246-56. (Striking meta analysis that demonstrates the huge impact infection can have on the mortality and morbidity of this patient population.)
Copyright © 2017, 2013 Decision Support in Medicine, LLC. All rights reserved.
No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. The Licensed Content is the property of and copyrighted by DSM.
- How can I be sure that the patient has portal hypertension?
- A tabular or chart listing of features and signs and symptoms
- How can I confirm the diagnosis?
- What other diseases, conditions, or complications should I look for in patients with portal hypertension?
- What is the right therapy for the patient with portal hypertension?
- What is the most effective initial therapy?
- Listing of usual initial therapeutic options, including guidelines for use, along with expected result of therapy.
- A listing of a subset of second-line therapies, including guidelines for choosing and using these salvage therapies
- Listing of these, including any guidelines for monitoring side effects.
- How should I monitor the patient with portal hypertension?
Want to read more?
Please login or register first to view this content.