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In experienced hands TIPS is a very effective method for stopping acute hemorrhage from esophageal varices but can be less effective in gastric variceal bleeding.168In the setting of var

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risks of rebleeding, although the data to support such an

approach are weak at best In those with well-preserved

liver function, a surgical shunt can also provide

long-term relief from bleeding.146

Portal Hypertensive Gastropathy

Portal hypertensive gastropathy (PHG) is a

manifes-tation of portal hypertension Endoscopically it is

characterized by a mosaic mucosal pattern with varying

degrees of submucosal hemorrhage It is often

asymp-tomatic but may lead to chronic transfusion–requiring

blood loss or acute bleeding (rarely) Although portal

gastropathy is more often seen in the setting of

gastro-esophageal varices, its severity does not correlate with

portal pressure Nevertheless, bleeding is often

amelio-rated by reduction of portal pressure Both octreotide

and nonselective b-blockade can be helpful in

decreas-ing acute bleeddecreas-ing and the degree of rebleeddecreas-ing from

PHG via reduction of portal blood flow.147–149 In

refractory cases, TIPS can be effective at reducing

transfusion requirements.150

THERAPIES TO ACHIEVE HEMOSTASIS

Drug Therapy

Drug therapy is an integral component of the

manage-ment of acute portal hypertensive hemorrhage and

should be started on presentation To optimize the

effectiveness of drug or endoscopic therapy, clotting

abnormalities must be corrected Target INR and

platelet count should be 1.5 and 75, respectively In

the setting of renal failure platelets may be

dysfunc-tional and DDAVP (Desamino-D-Arginine

Vasopres-sin) should be considered FFP alone or in combination

with rFVIIa can be given to rapidly correct

coagulop-athy A recent randomized, clinical trial showed that

when given in addition to standard therapy, 100 mg/kg

of rFVIIa may improve control of bleeding in patients

with advanced cirrhosis.151

Somatostatin or octreotide, its synthetic analogue,

stops acute bleeding from varices in 80% of cases.152It

does so through a reduction of portal pressure via effects

on vasoactive peptides or through the prevention of

postprandial hyperemia (blood meal) Octreotide has

an excellent safety profile and can be given initially as a

subcutaneous bolus of 50 to 100 mg followed by a

continuous infusion of 50 mg/h for 3 to 5 days Although

it does decrease portal pressure acutely, these effects

appear to be short lived due to rapid tachyphylaxis.153

Nevertheless, studies have shown a significant decrease

in rebleeding after endoscopic therapy in those receiving

octreotide infusion.154,155

Vasopressin reduces splanchnic blood flow in

addition to portal pressure It is effective in controlling

variceal hemorrhage; however, its use is limited due to systemic effects such as coronary and mesenteric ische-mia.156If vasopressin is used in conjunction with nitro-glycerin, these effects can be minimized.157,158

Terlipressin is a synthetic analogue of vasopressin with a longer half-life and fewer side effects It is effective in the treatment of acute variceal bleeding with or without endoscopic therapy and has been shown

to reduce mortality.125,159It appears to be as effective as vasopressin or endoscopic treatment126,160in controlling acute variceal hemorrhage, but is not yet available in the United States.

Endoscopic Therapy Endoscopic variceal band ligation (EBL) is currently the preferred endoscopic technique for the management of esophageal varices It is at least as effective as endoscopic sclerotherapy (EST) but has a superior safety profile and lower complication rate.133,161,162 EBL also decreases the incidence of bleeding, when given as primary pro-phylaxis,163,164 and death165 from variceal bleeding.

Band ligation leads to strangulation and subsequent obliteration of the banded varix.

EST involves the injection of a sclerosant (sodium morrhuate, ethanolamine, or polidocanol) into or around

a varix This leads to coagulative necrosis and obliter-ation of varices in the vicinity of the injection Compli-cations related to EST tend to occur more frequently and

be more severe than with EBL and include esophageal ulceration, stricturing, esophageal perforation, pleural effusion, and sepsis Despite a higher complication rate, a role still exists for EST It can be a useful adjunct

to EBL in the setting of massive hemorrhage with poor visibility because the location of injection need not be as precise to achieve hemostasis.

Regardless of the choice of endoscopic manage-ment for acute bleeding, follow-up endoscopy within

1 to 2 weeks for further banding is essential to decrease the risk of rebleeding EBL should then be continued in the future until variceal obliteration is achieved.

Balloon Occlusion Balloon tamponade is effective in the 5 to 10% of patients in which hemostasis cannot be achieved acutely with medical or endoscopic therapy It successfully stops bleeding from esophagogastric varices through external compression of varices, but rebleeding occurs in 50%.166 Many types of tubes exist (Sengsten-Blake Tube, Warne Surgical Products, Ltd., Armagh, Ireland, UK; and Linton Tube, Bard Manufacturing, Covington, Geor-gia, USA) with mild variations; however, in most cases;

inflation of the gastric balloon is adequate to stop variceal hemorrhage It is quite effective as a bridge to more definitive therapy (TIPS or surgery) and cannot be

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in place for more than 24 hours given the significant risk

of esophageal necrosis and rupture.167

Transjugular Intrahepatic Portosystemic

Shunt (TIPS)

TIPS involves placing a stent within the liver that

bridges a branch of the intrahepatic hepatic vein with

an intrahepatic branch of the portal vein, allowing

diversion of blood flow away from the cirrhotic liver,

decompressing portal pressures, and reducing the

im-petus to bleed In experienced hands TIPS is a very

effective method for stopping acute hemorrhage from

esophageal varices but can be less effective in gastric

variceal bleeding.168In the setting of variceal bleeding,

TIPS should be reserved for cases that are refractory to

endoscopic therapy,169or in the case of gastric varices,

used in conjunction with embolization If recurrent

bleeding occurs in a patient with a TIPS in place, the

most important intervention is interrogation of the

TIPS to document and treat thrombosis or stenosis.

Until recently, TIPS was complicated by

fre-quent stenosis and thrombosis.168

Polytetrafluoroethy-lene (PTFE)-coated stents have significantly improved

stent patency and the need for reintervention.170–172

Although they have not been directly compared with

surgical shunts, these data suggest they offer comparable

results.

Surgery

TIPS has significantly reduced the need for shunt

surgery; however, surgery remains a good option in

selected cases when TIPS is not technically feasible or

fails or in patients with significant portal hypertension in

the face of preserved hepatic synthetic function The

long-term patency rate of shunt surgery is thought to be

superior to that of TIPS; however, newer coated stents

may challenge this assumption.170–172 Surgical

alterna-tives for acute portal hypertensive hemorrhage include

total or selective shunt surgery, devascularization

proce-dures (Suguira or modification) or liver transplantation.

If surgery is necessary, it is best done at a center with extensive experience.

COMPLICATIONS ASSOCIATED WITH VARICEAL BLEEDING

Hepatic Encephalopathy The initial approach to the patient with HE should focus

on the identification and correction of any precipitant in addition to treatment of the encephalopathy Common precipitants include gastrointestinal (GI) bleeding, medications, infection, dehydration, electrolyte distur-bances, constipation, excessive protein load, portosyste-mic shunting (TIPS or spontaneous), and worsening liver function (Table 5) Potential precipitants such as these must be individually considered and subsequently excluded In the case of infection, spontaneous bacterial peritonitis (SBP) is the most common infection in this population and must be ruled out with a diagnostic paracentesis as already discussed Often, HE is the only manifestation of SBP.

TREATMENT OF HEPATIC ENCEPHALOPATHY Nonabsorbable disaccharides and antibiotics have been shown to modify gut flora and decrease blood ammonia levels, but these are not necessarily related (indicating nonbacterial sources of ammonia, which may also be decreased by these compounds) Lactulose is the first-line therapy for HE It is most effective if given orally and titrated to a dose that achieves three to four soft bowel movements a day A common mistake in the ICU

is continued lactulose despite diarrhea in the encepha-lopathic patient Not only will this not improve ence-phalopathy, it may worsen it through free water depletion If the patient is having adequate bowel move-ments on lactulose, but continues to be confused, several agents can be added Nonabsorbable antibiotics such as neomycin or rifaxamin are effective for the treatment of

HE either alone or in conjunction with lactulose Met-ronidazole is also efficacious; however, side effects limit prolonged use Zinc is a cofactor for the urea cycle and can increase the clearance of ammonia Zinc levels are decreased in patients with cirrhosis and HE Supple-mentation with zinc sulfate 600 mg/d normalizes zinc levels, decreases ammonia, and improves HE.173 Branched-chain amino acids have not convincingly shown improvement in HE; however, they may be considered in patients that are not receiving protein in any form.174,175

When HE is refractory to medical treatment other possibilities must be entertained In those with a TIPS, occlusion or narrowing of the stent lumen can improve mental status.176,177 If no TIPS or surgical shunt is present, abdominal imaging should be obtained

Table 5 Precipitants of Hepatic Encephalopathy

Infection

Gastrointestinal bleeding

Medical noncompliance

Medication—sedatives, narcotics, other

Electrolyte disturbances

Portosystemic shunting

Transjugular intrahepatic portosystemic shunt

Spontaneous

Dehydration

Excessive protein load

Constipation

Worsening liver function

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to look for a prominent portosystemic collateral that

could be radiographically embolized.178

Infection

Bacterial infections complicate 35 to 66% of cases of

gastrointestinal bleeding in patients with AoCLF.179–184

Not only is infection common after bleeding, it may also

provoke rebleeding.179Furthermore, bacterial infection

and the use of prophylactic antibiotics are independently

associated with failure to control variceal hemorrhage in

the first 5 days of admission.180 Many randomized,

controlled trials have shown that antibiotic prophylaxis

targeted at enteric organisms (such as quinolones or

third-generation cephalosporin) is effective in the

pre-vention of postbleeding infection in cirrhotic patients In

addition, a meta-analysis of randomized, controlled

trials concluded that antibiotic prophylaxis resulted in

less infections and improved short-term survival in

bleeding patients with cirrhosis.185 Given these data,

prophylactic systemic antibiotics should be given to

cirrhotic patients with gastrointestinal hemorrhage.

Spontaneous Bacterial Peritonitis

SBP is a frequent and severe complication in those with

cirrhosis and ascites.186It is associated with significant

morbidity and mortality by precipitating renal failure in

30%,187worsening HE, and causing hemodynamic

col-lapse in an already critically ill patient The deterioration

of renal function is the most sensitive predictor of

in-hospital mortality.187,188Renal failure often results from

a reduction in effective circulating blood volume,

cyto-kine surges, and activation of the renin-angiotensin

system precipitated by infection.187,188 Bacterial

trans-location from the gut is thought to be the most common mode of ascitic fluid inoculation.189,190 Predisposing factors for the development of SBP include advanced liver disease, gastrointestinal bleeding, ascitic total pro-tein fluid content  1 g/dL and a previous history of SBP.181,191–193

INTERPRETATION OF A DIAGNOSTIC PERITONEAL TAP

It is crucial to have a very low threshold to perform a diagnostic paracentesis in the patient with suspected SBP Ideally, this should be done prior to the initia-tion of antibiotics Peritoneal fluid should be sent for cell count, culture, albumin, total protein, LDH (lactate dehydrogenase) and glucose Blood culture bottles should be inoculated at the bedside to improve yield.

SBP is defined as an ascitic fluid polymorphonu-clear (PMN)  250 cells/mm3, in the setting of a positive monomicrobial ascitic fluid culture.194,195 Cul-ture positivity can fluctuate from one tap to the next;

thus culture-negative neutrocytic ascites should be treated with antibiotics as previously outlined.196 Monomicrobial non-neutrocytic bacterascites is another common variant of SBP It is defined as a fluid cell count < 250 cells/ mm3 with a positive culture.197 Runyon and Hoefs and Chu et al found that 62 to 86%

of cases of monomicrobial bacterial ascites resolve spon-taneously, and those that did not resolve were sympto-matic on presentation.197,198Thus, in a clinically stable asymptomatic patient, one could observe or consider repeat diagnostic paracentesis However, in a critically ill patient with AoCLF in the ICU, antibiotic therapy may be the best course of action.

TREATMENT OF SPONTANEOUS BACTERIAL PERITONITIS Patients should be given a non-nephrotoxic antibiotic with good enteric coverage such as a third-generation cephalosporin Cefotaxime 2 g (q8h) is the best-studied antibiotic for the treatment of SBP.199,200 Other antibiotics of comparable spectrum can be used and can be tailored if the organism is identified.

Once the patient has been on antibiotics for 48 hours,

a diagnostic tap must be repeated to assess response to treatment If there is not a significant decrement in the white blood cell (WBC) count, antibiotic coverage should be broadened Once treatment efficacy is estab-lished, antibiotics should be given for 5 days.201 Intra-venous albumin is integral to the treatment of SBP and should be used in conjunction with antibiotics It has been shown in a randomized, controlled trial to decrease the incidence of renal failure and subsequent mortality when compared with antibiotics alone.202 Based on these data, albumin should be given at a dose of 1.5 mg/kg on day 1 and 1 mg/kg on day 3 A recent study compared albumin to plasma expansion with hydroxyethyl starch Fernandez and colleagues

Table 6 Diagnostic Criteria for Hepatorenal Syndrome

(International Ascites Club)

MAJOR

Chronic or acute liver disease with advanced hepatic failure

and portal hypetension

Creatinine> 1.5mg/dLor24-hourcreatinineclearance< 40mL/min

Absence of shock, ongoing infection, use of nephrotoxic

drugs, gastrointestinal or renal fluid losses> 500 g/d or

> 1000 g/d in the setting of edema

Urine protein< 500 mg/dL

No ultrasonographic evidence of primary renal disease

No sustained improvement in renal function after hydration

MINOR

Urine sodium< 10 mEq/L

Urine osmolality> plasma osmolality

Urine red blood cells< 50 per high power field

Urine output< 500 mL/d

Serum sodium< 130 mEq/L

For the diagnosis of hepatorenal syndrome, all major criteria must be

met Minor criteria are supportive but not necessary for the diagnosis

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found that only albumin improved hemodynamics in

patients with SBP, suggesting that it may also have

direct effects on the vascular endothelium.203Lifelong

secondary antibiotic prophylaxis is mandatory in the

patient with a history of SBP Oral quinolones are

most commonly used (norfloxacin); however, many

antibiotics are effective for secondary prophylaxis of

SBP.

ASCITES

Ascites is the result of avid water and sodium retention

characteristic of the altered hemodynamics of cirrhosis

and portal hypertension It is associated with a 50%

2-year survival Uncomplicated ascites is managed

with sodium restriction (< 88 mmol/d) and diuretics;

potassium sparing (i.e., spironolactone) alone, or in

combination with a loop diuretic (i.e., furosemide).

Diuretics are advanced until therapeutic efficacy is

achieved or limited by worsening renal function or

hyponatremia In diuretic-refractory or resistant cases

repeat large-volume paracentesis (LVP) with albumin

infusion, TIPS, or peritonovenous shunts can be

effective in improving the ascites but does not improve

survival.204,205

Ascites can be a difficult problem to manage

in the ICU Copious colloid and crystalloid infusion

inevitably worsens ascites and diuretic use is often

limited by hyponatremia, hypotension, or renal failure.

Massive ascites can also alter respiratory mechanics and

make breathing more labored or mechanical ventilation

more challenging Occasionally, massive ascites can

worsen renal failure through compression of the renal

arteries LVP should be reserved for patient discomfort

and improvement of respiratory mechanics when

possi-ble to avoid large-volume shifting and activation of

vasoactive neurohumoral systems after paracentesis that

can worsen renal perfusion Such changes can be

mini-mized with the use of albumin (6 to 8 g/L removed).

Albumin administration helps maintain intravascular

volume and minimize postparacentesis circulatory

dys-function.205,206

RENAL FAILURE

Twenty percent of cirrhotic patients with tense ascites

develop renal failure characterized by the hepatorenal

syndrome (HRS).207,208 HRS is defined as functional

renal impairment in a patient with advanced liver disease

in the setting of normal tubular function and renal

histology209 (Table 6) Two types of HRS have been

described; HRS I and HRS II, based upon the rapidity

and extent of renal failure.210HRS I is characterized by a

rapid and severe deterioration of renal function with

survival measured in days to weeks, and HRS II

repre-sents a more indolent and stable renal dysfunction.

Table 6 illustrates the International Ascites Club classi-fication of HRS.

The pathophysiology of HRS is complex Splanchnic arteriolar vasodilation leads to central vaso-dilation and compensatory activation of systemic and renal vasoconstrictor systems.107,211 The resultant renal vasoconstriction leads to reduced glomerular filtration rate and increased water and sodium retention.

Treatment of Hepatorenal Syndrome Liver transplantation is the ultimate treatment for HRS After transplantation renal function returns to baseline in most cases.212,213 Combination drug ther-apy that counteracts renal and systemic vasoconstriction leading to arterial hypotension and central hypovolemia with vasoconstrictors and plasma expanders, respec-tively, is the most effective strategy Terlipressin, a long-acting vasopressin analogue that stimulates splanchnic V1a vasopressin receptors increases blood pressure, GFR (glomerular filtration rate), and urine volume in patients with HRS.214,215 Unfortunately, terlipressin is not yet available in the United States.

In a small study of patients with HRS I, the combina-tion of the a-agonist midodrine (7.5 mg tid), octreotide (100 g SQ tid), and albumin (25 g/day) was effective in improving renal function.216 In a more recent study, these findings were confirmed and insertion of a TIPS

in a subset of patients led to further improvement in renal function.217

LIVER TRANSPLANTATION—CHRONIC LIVER DISEASE

Allocation of organs in chronic liver disease changed on February 27, 2002 The model of end-stage liver disease (MELD) system was adopted to objectify the way in which livers were allocated in the United States It is a survival model based on a composite of three laboratory values: serum bilirubin, serum creatinine, and INR The model was originally used to assess short-term mortality in cirrhotic patients undergoing elective TIPS placement.218 This model was subsequently va-lidated as an independent predictor of survival in patients with cirrhosis.219,220Thus priority on the liver transplant waiting list is based on the patient’s blood group and the MELD score without emphasis on waiting time.

SUMMARY Management of the patient with acute or chronic hepatic failure remains a challenging problem, despite advances in intensive care Liver failure typically has profound effects on other organ systems and the effects

of therapeutic interventions on other organs must be

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considered A multidisciplinary approach is most

effec-tive and urgent transfer to a transplant center is

man-datory in potential transplant candidates Ideally, good,

comprehensive intensive care can support the patient

into spontaneous hepatic recovery; however, often

the goal of therapy is to bridge patients to definitive

therapy—liver transplantation.

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