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Rinella, M.D.1and Arun Sanyal, M.D.2 ABSTRACT A substantial number of patients with liver failure are admitted to the intensive care unit; thus a thorough understanding of the prevention

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indicator of the severity of shock Circulation 1969;39:131–

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Mechan-isms of inaccuracy in auscultatory and palpatory methods.

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thermodilution and arterial pulse contour techniques In:

Pinsky MR, Payen D, eds Functional Hemodynamic

Monitoring Update in Intensive Care and Emergency

Medicine No 42 New York: Springer-Verlag; 2005:135–152

19 Reuter DA, Goetz AE Arterial pulse contour analysis:

applicability to clinical routine In: Pinsky MR, Payen D,

eds Functional Hemodynamic Monitoring Update in

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2000–2008

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Monitoring Update in Intensive Care and Emergency

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44 Slama M Cardiac preload evaluation using

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50 Jardin F Right heart catheterization at bedside: a critical

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51 Janssen JRC, van den Berg PCM Cardiac ouput by

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Monitoring Update in Intensive Care and Emergency

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52 Boldt J Right ventricular end-diastolic volume In: Pinsky

MR, Payen D, eds Functional Hemodynamic Monitoring.

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53 Michard F, Teboul JL Using heart–lung interactions to

assess fluid responsiveness during mechanical ventilation.

Crit Care Med 2000;4:282–289

54 Pinsky MR Assessment of indices of preload and preload

responsiveness Curr Opin Crit Care 2005;11:235–239

55 Vincent JL DO 2 /VO 2 relationships In: Pinsky MR, Payen

D, eds Functional Hemodynamic Monitoring Update in

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56 Lichtenstein D, Axler O Intensive use of general ultrasound

in the intensive care unit, a prospective study of 150 patients.

Intensive Care Med 1993;19:353–355

57 Feissel M, Michard F, Mangin I, et al Respiratory changes

in aortic blood velocity as an indicator of fluid responsiveness

in ventilated patients with septic shock Chest 2001;119:

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58 Slama M, Masson H, Teboul JL, et al Respiratory variations

of aortic VTI: a new index of hypovolemia and fluid

responsiveness Am J Physiol Heart Circ Physiol 2002;

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59 Slama M Monitoring of respiratory variations of aortic

blood flow velocity, using esophageal Doppler Intensive

Care Med 2004;30:1182–1187

60 Jardin F Cyclic changes in arterial pressure during mechan-ical ventilation Physiologmechan-ical note Intensive Care Med 2004;30:1047–1050

61 Pinsky MR Using ventilation-induced aortic pressure and flow variation to diagnose preload reponsiveness [editorial].

Intensive Care Med 2004;30:1008–1010

62 Feissel M, Michard F, Faller JP, et al The respiratory cycle

in inferior vena cava diameter as a guide to fluid therapy.

Intensive Care Med 2004;30:1834–1837

63 Barbier C, Loubieres Y, Schmitt C, et al Respiratory changes in inferior vena cava diameter are helpful in predicting fluid responsiveness in ventilated patients Inten-sive Care Med 2004;30:1740–1746

64 Vieillard-Baron A, Chergui K, Rabiller A, et al Superior vena collapsibility as a gauge of volume status in ventilated septic patients Intensive Care Med 2004;30:1734–1739

65 Vignon P Evaluation of fluid responsiveness in ventilated septic patients: back to venous return [editorial] Intensive Care Med 2004;30:1699–1701

66 Pavlides GS, Hauser AM, Stewart JR, et al Contribution of transesophageal echocardiography to patient diagnosis and treatment: a prospect analysis Am Heart J 1990;120:910–

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67 Pearson AC, Castello R, Labovitz AJ, et al Safety and utility

of transesophageal echocardiography in the critically ill patient Am Heart J 1990;119:1083–1089

68 Oh JK, Seward JB, Khanderia BK, et al Transesophageal echocardiography in critically ill patients Am J Cardiol 1990;66:1492–1499

69 Reichert C, Visser C, Koolen J, et al Transesophageal echocardiography in hypotensive patients after cardiac operations: comparison with hemodynamic parameters.

J Thorac Cardiovasc Surg 1992;104:321–326

70 Hwang JJ, Shyu KG, Chen JJ, et al Usefulness of transesophageal echocardiography in the treatment of critically ill patients Chest 1993;104:861–866

71 Vignon P, Mentec H, Terre´ S, et al Diagnostic accuracy and therapeutic impact of transthoracic and transesophageal echocardiography in mechanically ventilated patients in the ICU Chest 1994;106:1829–1834

72 Poelaert J, Trouerbach J, De Buyzere M, et al Evaluation of transesophageal echocardiography as a diagnostic and therapeutic aid in a critical care setting Chest 1995;107:

774–779

73 Heidenreich PA, Stainback RF, Redberg RF, Schiller NB, Cohen NH, Foster E Transesophageal echocardiography predicts mortality in critically ill patients with unexplained hypotension J Am Coll Cardiol 1995;26:152–158

74 Sohn DW, Shin GJ, Oh JK, et al Role of transesophageal echocardiography in hemodynamically unstable patients.

Mayo Clin Proc 1995;70:925–931

75 Slama MA, Novara A, Van de Putte P, et al Diagnostic and therapeutic implications of transesophageal echocardiogra-phy in medical ICU patients with unexplained shock, hypoxemia, or suspected endocarditis [review] Intensive Care Med 1996;22:916–922

76 Megarbane B, Axler O, Mazhigi M, et al Assessment of echocardiography indications and results in an intensive care unit [abstract] Am J Respir Crit Care Med 1999;159:A520

77 Schmidlin D, Schuepbach R, Bernard E, et al Indications and impact of postoperative transesophageal echocardiogra-phy in cardiac surgical patients Crit Care Med 2001;29:

2143–2148

EVALUATION AND MANAGEMENT OFSHOCK/AXLER 239

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78 Colreavy FB, Donovan K, Lee KY, et al Transesophageal

echocardiography in critically ill patients Crit Care Med

2002;30:989–996

79 Hu¨ttemann E, Schelenz C, Kara F, et al The use and safety

of transoesophageal echocardiography in the general ICU a

minireview Acta Anaesthesiol Scand 2004;48:827–836

80 Joseph MX, Disney PJS, Da Costa R, et al Transthoracic

echocardiography to identify or exclude cardiac cause of

shock Chest 2004;126:1592–1597

81 Kumar A, Anel R, Bunnell E, et al Pulmonary artery

occlusion pressure and central venous pressure fail to predict

filling volume, cardiac performance, or the reponse to volume

infusion in normal subjects Crit Care Med 2004;32:691–699

82 Cheung AT, Savino JS, Weiss SJ, et al Echocardiography

and hemodynamic indexes of left ventricular preload in

patients with normal and abnormal ventricular function.

Anesthesiology 1994;81:376–387

83 Axler O, Tousignant C, Thompson CR, et al Small

hemodynamic effect of typical rapid volume infusions in

critically ill patients Crit Care Med 1997;25:965–970

84 Tavernier B, Makhotine O, Lebuffe G, et al Systolic pressure

variation as a guide to fluid therapy in patients with

sepsis-induced hypotension Anesthesiology 1998;89:1313–1321

85 Tousignant CP, Walsh F, Mazer CD The use of

transe-sophageal echocardiography for preload assessment in crtically

ill patients Anesth Analg 2000;90:351–355

86 Nagueh S, Kopelen HA, Zoghbi WA, et al Relation of

mean right atrial pressure to echocardiographic and Doppler

parameters of right atrial and ventricular function

Circula-tion 1996;93:1160–1169

87 Giannuzzi P, Imparato A, Temporelli PL Doppler-derived

mitral deceleration time of early filling as a strong predictor

of pulmonary capillary wedge pressure in post-infarction

patients with left ventricular systolic dysfunction J Am Coll

Cardiol 1994;23:1630–1637

88 Nagueh S, Middleton KJ, Kopelen HA, et al Doppler tissue

imaging: a non invasive technique for evaluation of of left

ventricular relaxation and estimation of filling pressures.

J Am Coll Cardiol 1997;30:1527–1533

89 Gonzalez-Vilchez F, Ares M, Ayuela J, et al Combined use

of pulsed and color M-Mode Doppler echocardiography for

the estimation of pulmonary capillary wedge pressure: an

empirical approach based on an analytical relation J Am Coll

Cardiol 1999;34:515–523

90 Ommen SR, Nishimura RA, Appleton CP, et al Clinical

Utility of Doppler Echocardiography and Tissue Doppler

Imaging in the Estimation of Left Ventricular Filling

Pressures A Comparative Simultaneous

Doppler-Catheter-ization Study Circulation 2000;102:1788–1794

91 Boussuges A, Blanc P, Molenat F, et al Evaluation in left

ventricular filling pressure by transthoracic Doppler

echo-cardiography in the intensive care unit Crit Care Med 2002;

30:362–367

92 Bouhemad B, Nicolas-Robin A, Benois A, et al

Echocardio-graphic Doppler assessment of pulmonary capillary wedge

pressure in surgical patients with postoperative circulatory shock and acute lung injury Anesthesiology 2003;98:1091– 1100

93 Combes A, Arnoult F, Trouillet JL, et al Tissue Doppler imaging estimation of pulmonary capillary wedge pressure in ICU patients Intensive Care Med 2004;30:75–81

94 Rhodes A, Sunderland R Arterial pulse power analysis: the LiDCO plus system In: Pinsky MR, Payen D, eds Functional Hemodynamic Monitoring Update in Intensive Care and Emergency Medicine No 42 New York: Springer-Verlag; 2005:183–192

95 Perel A, Preisman S, Berkenstadt H Arterial pressure variation during positive-pressure ventilation In: Pinsky

MR, Payen D, eds Functional Hemodynamic Monitoring Update in Intensive Care and Emergency Medicine No 42 New York: Springer-Verlag; 2005:313–329

96 Teboul JL, Monnet X, Richard C Arterial pulse pressure variation during positive pressure ventilation and passive leg raising In: Pinsky MR, Payen D, eds Functional Hemo-dynamic Monitoring Update in Intensive Care and Emergency Medicine No 42 New York: Springer-Verlag; 2005:332–343

97 Pinsky MR Protocolized cardiovascular management based

on ventricular-arterial coupling In: Pinsky MR, PayenD, eds Functional Hemodynamic Monitoring Update in Intensive Care and Emergency Medicine No 42 New York: Springer-Verlag; 2005:381–395

97a The National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome (ARDS) Clinical Trials Network Comparison of two fluid management strategies

in acute lung injury N Engl J Med, June 2006;10.1056/ NEJM oa062200 Available at www.nejm.org

97b Rivers EP Fluid-management strategies in acute lung injury—liberal, conservative, or both? N Engl J Med, June 2006;10.1056/NEJM e068105 Available at www.nejm.org

98 Bernard GR, Vincent JL, Laterre PF, et al Recombinant human protein C Worldwide Evaluation in Severe Sepsis (PROWESS) study group: efficacy and safety of recombi-nant human activated protein C for severe sepsis N Engl J Med 2001;344:699–709

99 van den Berghe G, Wouters P, Weekers F, et al Intensive insulin therapy in the critically ill patients N Engl J Med 2001;345:1359–1367

100 Liang DH, Thompson N A program for the use of hand-held echocardiography by intensivists to augment physical exam assessment 33rd Critical Care Medicine Congress Orlando Feb 2004 Crit Care Med 2003:33(Suppl)– A21

101 Vignon P, Frank MBJ, Lesage J, et al Handheld echocardio-graphy with Doppler capability for the assessment of critically ill patients: is it reliable? Intensive Care Med 2004;30:718– 723

102 Monnet X, Rienzo M, Osman D, et al Passive leg raising predicts fluid responsiveness in the critically ill Crit Care Med 2006;34:1402–1407

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Intensive Management of Hepatic Failure

Mary E Rinella, M.D.1and Arun Sanyal, M.D.2

ABSTRACT

A substantial number of patients with liver failure are admitted to the intensive care unit; thus a thorough understanding of the prevention and treatment of complications

in such patients is imperative The management of liver failure is demanding and often involves the combined efforts of many specialists Critically ill patients with hepatic failure encompass a broad spectrum of disease, ranging from acute liver failure in a patient with no prior history of liver disease, to acute on chronic liver failure The initial assessment and management of acute liver failure are reviewed with an emphasis on the prevention and treatment of brain edema in the pretransplant setting The current treatment of compli-cations resulting from decompensated chronic liver disease such as portal hypertensive bleeding; infection, renal failure, and hepatic encephalopathy are then discussed

KEYWORDS:Liver failure, cerebral edema, portal hypertension, management

The management of liver failure is demanding

and often involves the combined efforts of many

special-ists Critically ill patients with hepatic failure encompass

a broad spectrum of disease, ranging from acute liver

failure in a patient with no prior history of liver disease,

to end-stage decompensated cirrhosis Both sides of this

spectrum present clinical challenges that involve many

organ systems Although both sides in acute and chronic

liver failure can have a poor prognosis, careful and

comprehensive intensive care can improve outcome and

bridge eligible patients to liver transplantation Because

acute and chronic liver failure are very distinct clinical

entities, they will be discussed separately

ACUTE LIVER FAILURE

Acute liver failure (ALF) is a rapidly progressive, often

fatal syndrome characterized by jaundice,

encephalop-athy, and coagulopathy leading to multiorgan failure in a

patient with no prior history of liver disease.1,2In recent

years, advancements in supportive care have improved

survival and provided a more effective bridge to

trans-plantation Although ALF remains one of the most acute serious illnesses, thoughtful intensive management can optimize the patient’s chances for spontaneous hepatic regeneration or a successful liver transplant.3 When possible, etiology-targeted therapy should be initiated (Table 1) The goal of management should be focused

on the prevention of systemic infection, multiorgan fail-ure, hepatic encephalopathy (HE), and ultimately the development of brain edema.4–6At this time liver trans-plantation is the only definitive therapy for those who fulfill criteria for poor prognosis7–9 (Table 2) The challenge to the clinician is selection of patients for transplant that have low likelihood of spontaneous sur-vival but are not too ill to benefit from transplantation

The principles of management of ALF are reviewed here:

INITIAL EVALUATION AND MANAGEMENT Early diagnosis and identification of the subject that is unlikely to improve spontaneously constitute a critical first step in the management of ALF The initial triage

1

Division of Hepatology, Northwestern University, Chicago, Illinois;

2 Division of Gastroenterology, Department of Internal Medicine,

Virginia Commonwealth University, Richmond, Virginia.

Address for correspondence and reprint requests: Arun Sanyal,

M.D., Division of Gastroenterology, Department of Internal

Med-icine, Virginia Commonwealth University, MCV Box 980341,

Richmond, VA 23298-0341 E-mail: ajsanyal@hsc.vcu.edu.

Non-pulmonary Critical Care: Managing Multisystem Critical Ill-ness; Guest Editor, Curtis N Sessler, M.D.

Semin Respir Crit Care Med 2006;27:241–261 Copyright # 2006

by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York,

NY 10001, USA Tel: +1(212) 584-4662.

DOI 10.1055/s-2006-945528 ISSN 1069-3424.

241

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of a patient with acute liver injury to an intensive care

unit (ICU) is based on the presence of altered mental

status and the degree of coagulopathy It is imperative

to admit most subjects with acute liver injury with an

international normalized ratio (INR) > 1.5 and all

subjects with mental status changes Rapid

deteriora-tion can occur and is often irreversible in the patient

with ALF It is therefore imperative that decisions

regarding prognosis and appropriateness for liver

trans-plant be made early, and potentially suitable patients

should be referred to a liver transplant center early in

the evaluation process

The management of patients in liver failure

requires a multidisciplinary approach involving

hepatol-ogists, transplant surgeons, intensivists, and other

sub-specialists The importance of a thorough physical exam

and an accurate history cannot be overemphasized be-cause both treatment and prognosis are significantly affected by the underlying etiology A detailed account

of the psychiatric history, including suicidal ideation and family support, is essential to assess suitability for transplantation The timing of the psychiatric evalua-tion is of particular importance, given the rapid deterio-ration in mental status that occurs in such patients

DISEASE-TARGETED THERAPY

A thorough discussion of the differential diagnosis of ALF is beyond the scope of this review; however, Table 1 provides a summary of common etiologies of ALF for which potential therapies exist Only acetami-nophen will be discussed in more detail because it is the most common etiology of liver failure in the United States and has an effective antidote

Acetaminophen Idiopathic and drug-related liver injuries are the most common causes of ALF in the United States.10 Of the drug-related causes, acetaminophen overdose is the most common cause of ALF in the United Kingdom and United States Overdose can be either intentional or unintentional.11–13The patient, family, and close contacts must be questioned about regular alcohol use, dieting, diet pills, medications, or recent illness that may have resulted

in poor nutrition These factors greatly affect toxicity either through upregulating cytochrome p450 (alcohol and other drugs) promoting the formation of toxic inter-mediates, or through glutathione depletion Such details are important because as little as 2.6 to 4.0 g of acetami-nophen can lead to liver failure in this setting.14–17

It is worth noting that, at the time of presenta-tion, a patient with acetaminophen-induced liver failure may have undetectable blood levels of acetaminophen This is particularly true when the toxicity manifests itself several days after ingestion of acetaminophen for ther-apeutic purposes in a susceptible subject However, in the majority of cases, detectable acetaminophen levels are present at the time of presentation When acetami-nophen overdose is confirmed, N-acetylcysteine (NAC) must be initiated in a timely manner, ideally within

16 hours of ingestion, to have a significant impact on survival NAC decreases injury through enhancement of glutathione synthesis resulting in less formation of acetaminophen’s hepatotoxic intermediate.18,19 Even if the patient is delayed in reaching the hospital or the diagnosis is not forthcoming, there is evidence that late administration of NAC can be beneficial.20NAC may also improve outcome through its effects on micro-circulatory function A large multicenter study (the ALF study group) is currently addressing the utility of NAC in nonacetaminophen-induced ALF

Table 1 Etiology-Targeted Therapy

Etiology Potential Therapies

TOXIC

Acetaminophen N-acetyl cysteine

Amanita poisoning Penicillin and silibinin

VIRAL

Herpes simplex virus Acyclovir

Acute heptatitis B Antivirals?

METABOLIC

Wilson’s disease Transplant

Autoimmune hepatitis Corticosteroids

VASCULAR

Acute Budd-Chiari syndrome Directed thrombolysis,

transjugular intrahepatic portosystemic shunt PREGNANCY

Acute fatty liver of

pregnancy/HELLP

Urgent delivery

HELLP, hemolysis elevated liver enzymes low platelets.

Table 2 King’s College Criteria for Acute Liver Failure

Acetaminophen induced

 Arterial blood pH < 7.3 (regardless of degree of

encephalopathy)

If no acidosis then all three of the below criteria:

 Prothrombin time > 100 seconds

 Serum Creatinine > 2.5 mg/dL

 Grade 3 or 4 encephalopathy

Nonacetaminophen induced

 Prothrombin time > 100 seconds

If prothrombin time < 100 seconds, then any of the below

criteria (regardless of degree of encephalopathy):

 Drug-induced, non-A, non-B, halothane hepatitis

 Time from jaundice to encephalopathy > 7 days

 Age < 10 or > 40 years

 Prothrombin > 50 seconds

 Bilirubin > 17.5 mg/dL

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MONITORING AND GENERAL GUIDELINES

Acute hepatic dysfunction has profound effects on many

organs Therefore, one must remain cognizant of the

ramifications of specific therapies on other systems The

mental status must be documented several times daily, in

addition to frequent assessment of hepatic synthetic

function and blood glucose (Table 3) Although a liver

biopsy may be helpful if the diagnosis is in question, it can

be unreliable in predicting outcome and is risky given the

presence of underlying coagulopathy.21Low serum

phos-phate and elevateda fetoprotein can be encouraging signs

of hepatic regeneration.22,23In a retrospective analysis of

ALF patients, 74% of patients with phosphate levels

< 2.5 were alive at 1 week, in contrast to none in those

with a serum phosphate> 5.24

Coagulopathy in ALF does not usually require

correction unless an invasive procedure is planned or

overt bleeding is present because the use of fresh frozen

plasma (FFP) can mask deterioration of liver function A

common indication for the correction of coagulopathy

is placement of a central line Traditionally, FFP and

cryoprecipitates have been used for the correction of

coagulopathy in subjects with ALF This is only partially

effective in correcting coagulopathy and its effects are

short-lived It is also associated with a risk of transmitting

cytomegalovirus infection and may contribute to volume

overload and pulmonary edema, especially when renal function begins to deteriorate Alternate approaches in-clude the use of plasmapheresis where a volume of plasma equal to the amount infused is removed to prevent volume overload Recently, recombinant factor VII (40 mg/kg) has been used in conjunction with FFP to rapidly correct coagulopathy prior to either intracranial pressure monitor or central line placement in patients with ALF.25

MECHANICAL VENTILATION Mechanical ventilation should be initiated once ence-phalopathy deteriorates to  grade 3 (West Haven cri-teria) to protect the airway.26,27In addition to preventing aspiration in the patient with compromised mental status, intubation and sedatives help control agitation, which can lead to surges in intracranial pressure Patients with encephalopathy beyond grade 3 are very difficult to manage without intubation and sedation.28

Sedation is best achieved with a short-acting sedative alone or in combination with a short-acting narcotic Recent evidence supports the use of propofol for this purpose In a small study, propofol was given to seven patients with ALF and profound encephalopathy

Intracranial pressure (ICP) remained normal in six of

Table 3 Acute Liver Failure: General Management Guidelines

On Admission Daily Tid Hourly If Indicated Monitoring IV access, CVP and arterial

line, Foley catheter

Blood sugar Mental status Mechanical intubation,

ICP monitoring

Thorough history

and physical

Interview family members

Laboratories Liver panel, renal panel,

CBC, PT, Hep A,B,C serologies, HSV, CMV, EBV, ceruloplasmin, ANA, anti-sm Ab, SPEP, HIV, acetaminophen level, toxicology screen, cosyntropin stimulation test, TSH, blood type, blood cultures

Basic laboratories, AFP, arterial ammonia (or more if mental status deteriorating phosphate, factor V level

Blood gas Changes in

ICP monitor

Imaging US with Doppler Head CT for neurological

changes or suspected edema

Directed therapy where indicated Drugs, cooling for

cerebral edema AFP, alpha feta protein; ANA, antinuclear antibody; anti-sm Ab, antismooth muscle antibody; CBC, complete blood count; CMV,

cytomegalo-virus; CT, computed tomography; CVP, central venous pressure; EBV, Epstein-Barr cytomegalo-virus; HSV, herpes simplex cytomegalo-virus; HIV, human

immunode-ficiency virus; ICP, intracranial pressure; IV, intravenous; PT, prothrombin time; SPEP, serum protein electrophoresis; TSH, thyroid stimulating

hormone; US, ultrasound.

INTENSIVEMANAGEMENT OFHEPATIC FAILURE/RINELLA, SANYAL 243

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seven patients with ALF given propofol at 50 mg/kg/

min, suggesting propofol may have independent

benefi-cial effects on ICP.29 Paralytics are usually avoided

because they can mask seizure activity However, they

may be used in specific cases to facilitate management

when the subject does not respond appropriately to

sedation In such cases, it is imperative to consider the

possibility of seizure activity if the clinical picture

continues to deteriorate H2 antagonists or proton

pump inhibitors may decrease the incidence of ulcer

disease in mechanically ventilated patients30; however,

the theoretical risk of increasing the incidence of

pneumonia has not been studied in this population

PREVENTION AND MANAGEMENT

OF COMPLICATIONS

Circulatory Dysfunction

Derangements in circulatory function manifest early in

ALF and are often progressive They are characterized by

generalized vasodilation, increased cardiac output,

de-creased systemic vascular resistance, and a low mean

arterial pressure (MAP).31–33It is challenging to

distin-guish this clinical picture from the hemodynamics of

sepsis, particularly given that infection is common and

often a fatal complication.34 Factors such as adrenal

insufficiency also complicate management by making

the vasculature less responsive to vasopressive agents.35,36

In general terms, fluids and vasopressors should

be used to maintain adequate cerebral perfusion pressure

(CPP) (50 mm Hg to 65 mm Hg) while avoiding

cerebral hyperemia from hyperperfusion.37,38 Because

the circulatory disturbance in ALF is characterized by

vasodilation and increased cardiac output,

norepinephr-ine is frequently the vasopressor of choice

Infection

Patients with ALF are particularly susceptible to severe

infection due to many immunological defects such as

defective phagocytic function and decreased

comple-ment levels.39–42Bacterial or fungal sepsis is a frequent

cause of death in this population Much like other

immunocompromised hosts, their response to infection

is atypical in that signs such as fever or leukocytosis are

absent in 30% of cases.43 Thus sepsis is both frequent

and difficult to diagnose in subjects with ALF In a

prospective study of 887 patients with ALF, one or more

bacterial infections occurred in 37.8%; however, an

incidence of up to 80% has been reported.44 Of these,

gram-positive cocci were the most common organisms

isolated, although Escherichia coli and Klebsiella were also

frequent pathogens.45 Overall, pneumonias make up

50% of bacterial infections in ALF with bacteremia

and urinary tract infections occurring in 20 and 25%,

respectively These infections presented at a median of

5, 3, and 2 days after the onset of ALF.44 Given the frequency of both gram-negative and gram-positive infections in this population, broad spec-trum antibiotic coverage should be administered avoid-ing aminoglycosides due to their nephrotoxicity.34 Although no randomized controlled trials have demon-strated improved survival with prophylactic antibiotics, parenteral antibiotics are associated with a lower inci-dence of infection46 (Fig 1).47 Given these data, pro-phylactic broad-spectrum antibiotics seem justifiable given that uncontrolled infection in such patients is often catastrophic.48,49

Systemic Inflammatory Response Syndrome Even in the absence of documented infection, systemic inflammatory response syndrome (SIRS) is common in those with ALF and is likely due to a surge of cytokine release.50 In a study from King’s College, 57% of 887 patients with ALF developed SIRS The presence of SIRS on admission was independently associated with more severe illness, worsening of encephalopathy, and subsequent death In those patients that were infected (54%), mortality increased with each additional compo-nent of SIRS At this point it remains unclear how additional infection contributes or which component of the observed inflammatory response originates from humoral factors released by the necrotic liver.34,46,51

Adrenocortical Insufficiency Adrenocortical insufficiency can worsen hyperdynamic cardiovascular collapse typical of ALF or septic shock.52 This should be considered when the patient fails

Figure 1 The effect of antibiotic prophylaxis on the prevalence

of documented infection patients with acute liver failure (Adapted from Salmeron et al.47)

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to respond to volume resuscitation.35 In sepsis,

supra-physiological doses of steroids in patients with adrenal

insufficiency have been shown to reduce vasopressor

requirements and improve outcome.36,53 Adrenal

dysfunction appears to also be prevalent in patients

with ALF; 62% of patients with ALF were found to

have an abnormal response to high-dose corticotrophin

stimulation The patients with pronounced

hemody-namic instability had more marked evidence of adrenal

insufficiency, suggesting that it may contribute to the

pattern of cardiovascular collapse seen in liver failure.54

The benefit of stress-dose steroids in this population

needs to be tested in a randomized-controlled trial;

however, given these data, it is reasonable to look for

and consider treating adrenal insufficiency in patients

with ALF

Renal Failure

Renal failure is common in those with advanced liver

failure and is multifactorial in etiology Common causes

of renal failure in this population include prerenal

azotemia, renal ischemia, acute tubular necrosis, and

hepatorenal syndrome A majority of patients with

ALF complicated by profound hypotension and cerebral

edema will require renal replacement therapy.31Due to

the marked vasodilation that characterizes such patients,

continuous venovenous hemofiltration (CVVH) tends to

be better tolerated and may have more beneficial effects

on ICP.55,56 Moreover, intermittent hemodialysis has been associated with increases in ICP and decreases in CPP, whereas the opposite has been shown in patients receiving CVVH.55,57

Hepatic Encephalopathy and the Development

of Intracranial Hypertension The development of HE and subsequent cerebral edema and intracranial hypertension (ICH) define prognosis in patients with ALF.2,7,58 Treatment options for such patients are limited As a result, 30% of patients with ALF and cerebral edema succumb to cerebral herniation while awaiting an organ.7,31Without urgent transplantation, mortality can exceed 90% in those who have uncontrolled ICH

The pathogenesis of cerebral edema is complex (Fig 2) ALF leads to many hemodynamic changes, including impairment of cerebrovascular autoregulation and blood flow This impairment makes the standard assumption that CPP¼ MAP  ICP less reliable.38

Other factors such as high arterial ammonia levels contribute to brain edema through the accumulation

of glutamine and alanine in astrocytes In response to swelling, a vasodilating factor is released that leads to increased CBF and thus increased ICP.59

Arterial ammonia levels > 200 mmol/L in the setting of ALF have been shown to herald impending cerebral herniation and poor outcome.60,61 Other

Figure 2 Factors leading to the development of brain edema and potential therapeutic interventions.

INTENSIVEMANAGEMENT OFHEPATIC FAILURE/RINELLA, SANYAL 245

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markers of brain cell dysfunction and damage such as

s100-b and neuron-specific enolase (NSE) have also

been evaluated as potential predictors of impending

herniation in the setting of ALF and acute on chronic

liver failure with negative results.62Currently, no serum

markers of brain cell dysfunction reliably demonstrate

neurological injury and poor outcome

Unfortunately, it can be difficult to predict which

patients are likely to develop elevated ICP Clinical signs

such as arterial hypertension, fever, and agitation can

precede episodes of severe ICH; however, these are not

reliable predictors because elevated ICP is often

clin-ically silent.63Although a computed tomographic (CT)

scan is usually used to look for cerebral edema, a normal

scan does not exclude the presence of edema because its

appearance on imaging may be delayed

INTRACRANIAL PRESSURE MONITORING

A significant clinical challenge in the management of

ALF is the decision to place an ICP monitor There are

no strict guidelines related to the use of these monitors

and experience across institutions is highly variable

Noninvasive techniques have not proven to be

benefi-cial and direct ICP monitoring is the only reliable

modality for the measurement of ICP The benefit

that can be derived from ICP monitoring is twofold

First, it allows for the early detection and treatment of

ICH because it can be clinically silent.63Second, it can

provide invaluable information about the likelihood of

neurological recovery when deciding whether to

pro-ceed with liver transplantation, such as when CPP is

persistently low Sustained CPP< 40 mm Hg predicts

a high likelihood of ischemic brain injury that typically

results in a poor neurological outcome after

transplan-tation.64,65Figure 3 proposes an algorithm for the use

of ICP monitors in ALF

Although treatments aimed at reducing ICP can

be used without an ICP monitor, an accurate ICP reading permits targeted therapy to optimize CPP and detect abrupt surges in pressure that necessitate addi-tional therapy Concomitant measurement of jugular bulb oxygen saturation,32,66which allows measurement

of brain oxygen utilization, can also be useful in the management of these patients.32 Jugular bulb oxygen saturation > 80% or < 60% predicts elevation in ICP with good sensitivity and specificity.31Jugulovenous O2

saturations < 50% may herald an increase in anaerobic cerebral glycolysis, increased lactate:pyruvate ratio, and worsening cerebral edema.67

When and in Whom to Insert an Intracranial Pressure Monitor

To justify the risks of ICP monitor placement, the monitor needs to be placed under controlled circum-stances, when increased ICP is likely to rise but before uncontrolled ICH and herniation occur ICP monitor-ing should be considered for mechanically ventilated patients with grade 3 or 4 encephalopathy with poor prognosis (Table 2) but who are otherwise good candi-dates for liver transplant (Fig 3) Other predictors of increased ICP such as arterial ammonia> 150 mmol/L could be used to time monitor placement In those with poor prognosis without orthopedic liver transplant (OLT), ICP monitoring can guide therapy and prevent surges in ICH before and during OLT

Risks of Intracranial Pressure Monitoring

As with all interventions, the risks of ICP monitor placement need to be balanced against the accuracy and usefulness of the information to be gained No random-ized, controlled trial is available to compare different

Figure 3 Proposed algorithm for the use of an intracranial pressure monitor.

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catheters in the setting of ALF Types of catheters include

epidural, subdural, parenchymal, and intraventricular

catheters Blei et al performed a survey of transplant

centers across the United States They estimated that

20% of ICP monitoring resulted in intracranial bleeding

Epidural catheters had the lowest rate of bleeding

com-plications (3.8%) and subdural and parenchymal catheters

the highest; 20% and 22%, respectively.68A recent

multi-center study from the ALF study group showed that ICP

monitors were only used in 92/332 patients (28%) with

ALF and severe encephalopathy; however, the frequency

of monitoring differed between centers Ten percent had

intracranial bleeding as a result of the ICP monitor In

two of these patients, ICP monitoring was directly

associated with the patient’s death.69Although the risk

of complications is greater,70 subdural catheters give a

more reliable estimate of ICP than epidural catheters.68

Bleeding complications can be decreased

signifi-cantly with the use of recombinant factor rVIIa given

immediately before the procedure.25 The frequency of

factor VII dosing was variable in this study; however, as a

group all patients that received factor VII normalized

their prothrombin time (PT) and were able to have ICP

monitors placed (compared with 38% in the FFP alone

group) The ideal initial dose and subsequent doses of

factor VII necessitates further study.71 The data show

that ICP monitoring can be an effective tool for

manag-ing elevated intracranial pressure; however, ICP

mon-itors have not been shown to improve survival Currently

there is no consensus about the use of ICP monitoring or

whether the more accurate but higher-risk subdural

catheters or the less accurate but safer epidural catheter

should be used Individual centers will continue to use

what they are comfortable with; however, their decision

may be influenced by the decreased availability of

epi-dural catheters

Prevention and Treatment of Increased

Intracranial Pressure

Routine measures such as elevation of the head of the

bed to 30 degrees,55sedation, minimal stimulation, and

mechanical ventilation to minimize cerebral stimulation

should be adhered to whenever possible The

manage-ment should be focused on maintaining an adequate

CPP (> 50 mm Hg) while minimizing elevations in ICP

(< 20 mm Hg) Blood pressure should be maintained to

achieve a CPP between 50 and 65 mm Hg Prolonged

CPP below 50 mm Hg in the setting of ICH or an ICP

greater than 40 mm Hg is associated with poor

out-come.65

HYPERTONIC SALINE

The use of hypertonic saline is thought to help restore

the osmotic gradient across the astrocyte membrane A

randomized, controlled trial recently demonstrated that

induction and maintenance of hypernatremia (145 to

155 mmol/L) in patients with grade 3 or 4 encephalop-athy resulted in a decreased incidence and severity of ICH.72 Other techniques to reduce brain water accu-mulation through the reduction of arterial ammonia remain under investigation.73–75

MANNITOL

Mannitol administration leads to increased plasma os-molality in brain capillaries, resulting in movement of water out of the brain according to Starling’s law It has been shown to decrease episodes of cerebral edema and result in improved survival in a cohort of patients with ALF (47.1 and 5.9%, respectively, p ¼ 008).76 Its use, however, is limited in renal failure and can lead to a paradoxical increase in brain swelling if osmolality is not controlled If more than two doses are to be used, plasma osmolality must be checked to assure that it remains

< 320 Osm/L

HYPERVENTILATION

Hyperventilation is an effective technique to decrease cerebral blood flow (CBF) and ICP It does so through precapillary hypocapnic vasoconstriction and helps re-store CBF autoregulation.61,77–79Although prophylactic hyperventilation appears to be ineffective in preventing the development of ICH,77it can be useful in controlling acute surges in ICP

INDOMETHACIN

Indomethacin leads to cerebral vasoconstriction effects via altering cerebral temperature and extracellular pH and inhibition of the endothelial cyclooxygenase path-way.80 Its effectiveness has been proven in an animal model81and in a small cohort of patients with ALF.82 However, due to its multiple systemic side effects in patients with ALF, its routine use cannot be supported

THIOPENTAL SODIUM

In a small, uncontrolled study, thiopental sodium was effective in reducing ICP.83 Unfortunately, its use is associated with significant hemodynamic derangements that may necessitate escalation of vasopressor or inotropic support Thus thiopental use should be reserved for surges of ICH unresponsive to standard medical therapy

HYPOTHERMIA

Moderate hypothermia (32 to 33C) in animal models of ALF has been effective in improving encephalopathy and reducing brain water.84,85Clinical studies of hypo-thermia have also shown significant reduction in ICP

Jalan et al were able to demonstrate that cooling patients with refractory ICH to 32C decreased ICP to< 20 mm

Hg Subsequently they demonstrated a reciprocal in-crease in ICP with rewarming.86 Since this study, the same and other groups have also shown that moderate INTENSIVEMANAGEMENT OFHEPATIC FAILURE/RINELLA, SANYAL 247

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