CriticaI Care Aspects of Chronic CriticaI Care Aspects of Chronic

Hepatic FaiIure Hepatic FaiIure

Aditya N. Dubey, MD Aditya N. Dubey, MD
Peter K. Linden, MD Peter K. Linden, MD
University of Pittsburgh Medical Center
Department Critical Care Medicine
Learning Objectives Learning Objectives
e familiar with the complications of chronic liver failure
requiring critical care support
Pathophysiology and clinical sequelae of portal
hypertension
Urgent treatments for variceal hemorrhage
Strategies to treat diuretic refractory ascites
Diagnosis, treatment, and prevention of SP
Management of hepatorenal syndrome
Causes and treatment of hepatic encephalopathy
ndications for referral for liver transplantation
Hepatic FaiIure Hepatic FaiIure
Major Reasons for ICU Admission
'ariceal hemorrhage
Encephalopathy
Refractory ascites
Spontaneous bacterial peritonitis
Hepatorenal syndrome
PortaI Hypertension PortaI Hypertension
TerminoIogy
Portal pressure = P' inflow x outflow resistance
The trans-hepatic gradient (THG) can be measured by
the difference between the free hepatic vein to a wedge
pressure in the hepatic vein (estimated P' pressure)
THG = free H'P wedged H'P
Normal gradient < 5 mm Hg
ncreased risk of bleeding > 12 mm Hg
Portal hypertension may be elevated without intrinsic
liver disease due to pre- and post-sinusoidal pathology
(see next slide).
Causes of PortaI Hypertension Causes of PortaI Hypertension
LIVER
Pre-sinusoidaI
P' thrombosis
P' extrinsic comp.
Schistosomiasis
Sarcoidosis
PC
SinusoidaI
Cirrhosis
Alcoholic hepatitis
Post SinusoidaI
udd Chiari
'eno-occlusive dis.
Severe CHF
Restrictive heart dis.
BLOOD FLOW
VariceaI Hemorrhage VariceaI Hemorrhage
Incidence and Outcome
Gastroesophageal varices in 40 - 60% cirrhotics
'ariceal hemorrhage occurs in 25 - 35% cirrhotics
30% of the initial bleeding episodes are fatal
70% have recurrent bleeding with a one-year survival
ranging from 30 - 80%
Non-variceal pathology (ulcers, gastritis, mucosal tear)
may cause bleeding in patients with known liver disease
and portal hypertension.
Sharara and Rockey. N Engl J Med. 2001;345 (9); 669.
VariceaI Hemorrhage VariceaI Hemorrhage
InitiaI evaIuation and stabiIization
Assessment of intravascular volume status
lood pressure is unreliable indicator of volume status
Hematocrit does not reflect acute blood losses
Fluid resuscitation
Place twp large bore i.v.'s and/or a central venous catheter
Colloid or crystalloid titrated to parameters of perfusion
Cross-matched or O negative blood can be used
Endotracheal intubation prior to endoscopy for:
Uncontrolled bleeding
Altered mental status, severe agitation
Respiratory distress or depression
HierarchaI Treatment for HierarchaI Treatment for
VariceaI BIeeding VariceaI BIeeding
PharmacoIogic
Endoscopic
RadioIogic shunt
TIPSS
SurgicaI Shunt
BaIIoon
Tamponade
Pharmacologic and
endoscopic therapy
are the usual 1
st
and
2
nd
interventions
Acute VariceaI Hemorrhage: Acute VariceaI Hemorrhage:
Pharmacotherapy Pharmacotherapy
Octreotide
Synthetic analogue of somatostatin
Decreases portal pressure and azygos blood flow
Stops variceal bleed in 80% of the cases
Efficacy is similar to endoscopic sclerotherapy and better than vasopressin
5-day course reduces bleeding after endoscopic therapy
Can cause mild hyperglycemia and abdominal cramping
'asopressin
Reduces portal pressure but causes myocardial and mesenteric ischemia
Terlipressin
Efficacy similar to endoscopic sclerotherapy and as effective as balloon
tamponade when used with nitroglycerin
Not approved for use in U.S.
orley DA. Gastroenterology. 2001;120(4):946-54; Harry R. :rr
Opin rit are. 2002;8:164-170; Sharara and Rockey. N Engl J
Med. 2001;345(9):669.
PossibIe Targets for Therapeutic PossibIe Targets for Therapeutic
Intervention in VariceaI Hemorrhage Intervention in VariceaI Hemorrhage
1. Reduction of cardiac output by
beta-1 blockade to prevent
bleeding (NOT for acute
bleeding!!)
2. Reduction of splanchnic blood
flow by beta-2 blockade or
vasoconstrictors such as alpha-
adrenergic agonists or
vasopressin analogues
3. Reduction of intrahepatic
resistance by vasodilators
4. Reduction of variceal or
collateral flow by beta-2
blockade, balloon tamponade, or
endoscopic therapy
EsophageaI vs. Gastric Varices EsophageaI vs. Gastric Varices
Esophageal varices
Primary approach is endoscopic
banding or sclerotherapy
TPSS, surgical shunts are
alternatives
Gastric varices
Diffuse, deep submucosal
anatomy
Endoscopic tx difficult,
dangerous
Primary approach are TPSS or
surgery
VariceaI Hemorrhage: Endoscopic Therapy VariceaI Hemorrhage: Endoscopic Therapy
Endoscopic and Ligation (see next slide)
Controls bleeding in 80 - 90% of cases
Lower complication rates than sclerotherapy
Endoscopic Sclerotherapy
ntravariceal or paravariceal injection of a sclerosing agent
Stops bleeding in 80 to 90% of the cases
Complications include perforation, ulceration and stricture
Cyanoacrylate njection
Used to control bleeding from gastric varices
Superior to EL for treatment of bleeding gastric varices
Not available in U.S.
aine . Ann Intern Med. 1995;123(4):280-7.
o GH. Hepatology. 2001;33:421-427.
Banding of EsophageaI Varix Banding of EsophageaI Varix
Post endoscopy probIems incIude. Post endoscopy probIems incIude.
AbdominaI distension: From endoscopic air insufflation, retained luminal
blood, and increased ascites from resuscitation. This can even progress to
abdominal compartment syndrome with associated respiratory compromise,
hypotension, oliguria, and acidosis. Nasogastric decompression may partially
alleviate this problem.
Worsening encephaIopathy: This may occur due to gastrointestinal
passage of blood, hepatic hypoperfusion ("shock liver), and accumulation of
sedative medication.
Recurrent bIeeding: More likely to recur in advanced cirrhosis. ncidence
can be reduced with a 5-day course of octreotide post banding and long term
use of a non-selective beta blocker (propanol, naldolol).
Infection: Spontaneous bacterial peritonitis is 3-5x higher following variceal
hemorrhage due to occult bacteremia and ascites seeding. Antimicrobial
prophylaxis (quinolone, beta-lactam) reduces the incidence of SP
significantly.
Acute VariceaI Hemorrhage Acute VariceaI Hemorrhage
BaIIoon Tamponade BaIIoon Tamponade
Effectively controls bleeding in 90% of the patients but is
only a temporizing measure in massive uncontrolled
variceal hemorrhage when initial endoscopic treatment is
delayed or unsuccessful.
Can cause aspiration, esophageal ulceration, perforation with
mediastinitis
alloon-related mortality is 3 - 5%
Gastric balloon inflation is usually sufficient
Esophageal balloon inflation should only be used when gastric balloon
is unsuccessful as it is associated with higher morbidity.
Sengstaken Sengstaken - - BIakemore Tube BIakemore Tube
Gastric balloon
Esophageal balloon
Gastric aspiration port
Minnesota Tube Minnesota Tube
Gastric balloon
Esophageal balloon
Gastric aspiration port
Esophageal aspirationport
Tube Positioning and Gastric BaIIoon InfIation Tube Positioning and Gastric BaIIoon InfIation
1. Tube inserted to 50 cm
2. Auscultate in stomach
3. nflate gastric balloon with 50 cc
4. Stat portable film
1. Re-confirm proximal position
2. nflate G 300-400 cc air
3. Pull to insure anchorage
4. Recheck film
5. 1-2 lbs of pully traction
Gastric and EsophageaI BaIIoon InfIation Gastric and EsophageaI BaIIoon InfIation
Esophageal alloon
inflated to 30 mmHg
1. Last resort
2. Deflate periodically
3. Use minimum effective
pressure
4. Complication
- ulcer
- perforation
- stricture
MaIposition of the Gastric BaIIoon of a MaIposition of the Gastric BaIIoon of a
Minnesota Tube Retroverted in the DistaI Minnesota Tube Retroverted in the DistaI
Esophagus Esophagus
TransjuguIar Intrahepatic Portosystemic TransjuguIar Intrahepatic Portosystemic
Shunt (TIPSS) Shunt (TIPSS)
Major ndications
Refractory variceal bleeding
Refractory ascites, hydrothorax
Radiologic insertion of a metallic shunt (8 -
12 mm diameter) which joins the hepatic
and portal veins
Target gradient (H'-P') < 12 mmHg
Restores hepatopedal flow
Decompression of varices
Summary of TriaIs Comparing TIPSS to Summary of TriaIs Comparing TIPSS to
Endoscopic Therapy for VariceaI BIeeding Endoscopic Therapy for VariceaI BIeeding
Stanley. ancet. 1997;350(9086):1235-1239.
Generally, higher rates of rebleeding were more common after
Endoscopy treatment, while encephalopathy rates were higher in the
TPSS groups
CompIications of TIPSS CompIications of TIPSS
Peri-procedure mortality of 1 - 2%
ntraperitoneal bleeding due to perforation of the hepatic capsule, hepatic,
or portal veins
TPSS embolization
Acute right heart failure due to increased venous return to right heart
Later complications include recurrent bleeding due to
TPSS stenosis or thrombosis, infection, and hepatic
encephalopathy.
Conditions Which May Contraindicate TIPSS Conditions Which May Contraindicate TIPSS
This venogram shows an occlusive thrombus of the
portal vein, which may make safe TPSS placement
impossible.
This abdominal CT demonstrates a large
hypodense hepatic lesion due to
hepatocellular carcinoma in a very
shrunken cirrhotic liver. Other contraindications
include hepatic vein occlusion, heart failure or
pulmonary hypertension, biliary obstruction, and
poorly controlled systemic infection.
TIPSS Thrombosis/Stenosis TIPSS Thrombosis/Stenosis
ncidence 12 - 74%
Most likely within the first month
Symptoms - recurrent bleeding, ascites
Detection - Doppler ultrasound angiography (shows
velocity gradient)
Treatment
alloon dilatation
Placement of TPS shunt
Trans Trans- -TIPSS EmboIization of Persistent Varices TIPSS EmboIization of Persistent Varices
Persistent variceal bleeding due to high flow collaterals despite a patent TPS
shunt may be coil-embolized radiologically via the TPS shunt itself.
Acute VariceaI Hemorrhage: Surgery Acute VariceaI Hemorrhage: Surgery
The distal splenorenal shunt (Warren shunt) procedure is
generally reserved for Child's A or cirrhotics.
Consider in patients with bleeding refractory to
pharmacologic, endoscopic, and radiologic treatment.
Complications include shunt thrombosis, infection, and
worsening encephalopathy.
30-day mortality is close to 80% in Child's C patients
requiring emergency shunt surgery.
ReIative Effectiveness of AvaiIabIe Therapies for ReIative Effectiveness of AvaiIabIe Therapies for
the Prevention of Recurrent VariceaI BIeeding the Prevention of Recurrent VariceaI BIeeding
eta-blockers are the single most
effective and safest strategy to
prevent the recurrence of variceal
leeding.
More aggressive strategies such as
banding, TPSS, or
shunt surgery may decrease bleeding
but are associated
with higher risks and costs.
Sharara A, et al. N Engl J Med. 2001.
Hepatic EncephaIopathy Hepatic EncephaIopathy
Hepatic encephalopathy reflects a spectrum of
neuropsychiatric abnormalities seen in patients with liver
dysfunction after exclusion of other known brain disease.
Hepatic EncephaIopathy Hepatic EncephaIopathy - - West Haven West Haven
Criteria for Grading MentaI State Criteria for Grading MentaI State
Grade 1
Trivial lack of awareness
Euphoria or anxiety
Shortened attention span
mpaired performance of addition
Grade 2
Lethargy or apathy
Minimal disorientation for time or place
Subtle personality change
nappropriate behavior
mpaired performance of subtraction
Grade 3
Somnolence to semi-stupor but responsive to verbal stimuli
Confusion
Gross disorientation
Grade 4
Coma, unresponsive to verbal or noxious stimuli
Hepatic EncephaIopathy: DifferentiaI Hepatic EncephaIopathy: DifferentiaI
Diagnosis Diagnosis
MetaboIic encephaIopathies
Hypoglycemia
Hypoxia
Uremia
Electrolyte abnormalities
Toxic encephaIopathies
Alcohol
arbiturates, other CNS depressants
Heavy metals
IntracraniaI Iesions
Subarachnoid, subdural, or intracerebral hemorrhage
Stroke
ntracranial tumor
ntracranial abscess
Epilepsy
Neuropsychiatric disorders
Hepatic EncephaIopathy: Hepatic EncephaIopathy:
Precipitating Factors Precipitating Factors
ncreased ammonia production
Gastrointestinal hemorrhage
Excess dietary protein
Azotemia
nfection including SP
lood transfusion
Hypokalemia
Systemic alkalosis
Constipation
Reduced metabolism of toxins
because of hepatic hypoxia
Dehydration
Arterial hypotension
Anemia
Portosystemic shunts
Spontaneous
TPSS
Surgical
Progressive hepatic
parenchymal damage
Hepatoma
Use of benzodiazepines or
other psychoactive drugs
Riordan. N Engl J Med. 1997; 337(7):473-479.
Why does the ammonia IeveI correIate Why does the ammonia IeveI correIate
poorIy with encephaIopathy? poorIy with encephaIopathy?
'enous ammonia levels < arterial
Time lag from NH3 and CNS
lood-brain permeability is variable
alance of NH3 / NH4
+
Processing (must be on ice, < 20 min)
Management of Hepatic EncephaIopathy Management of Hepatic EncephaIopathy
First and foremost control the underlying precipitant(s).
MedicaI therapy - optimal agent is controversial (see meta-analysis)
LactuIose - has multiple actions including cathartic, acidification of the colon to
"ion-trap ammonia as NH
4
+
, and reduces inoculum of urea-splitting bacteria.
Drawbacks include osmotic diarrhea with hypernatremia due to free water loss
and gaseous bowel distension.
Neomycin - non-absorbed aminoglycoside which reduces colon bacterial
burden. Dosed at 2-6 grams orally per day. Small incidence of ototoxicity and
nephrotoxicity with prolonged usage.
MetronidazoIe - oral dosing at 800 mg/day. No large scale reported
experience. s associated with neurotoxicity in hepatic failure due to
accumulation.
FIumazeniI - benzodiazepine receptor (GAA) antagonist.
FIumazeniI in Hepatic EncephaIopathy FIumazeniI in Hepatic EncephaIopathy
n this double-blind, placebo-controlled, randomized trial,
flumazenil showed transient benefit in higher grades of
encephalopathy. The role of flumazenil for all degrees of encephalopathy or
as a longer term agent in critically ill patients has not been determined.
FIumazeniI
N = 265
PIacebo
N = 262
Neurologic
improvement
17.5% (Gr3)
14.7% (Gr4)
3.8% (Gr3)
2.7% (Gr4)
EEG
improvement
27.8% (Gr3)
21.5% (Gr4)
5.0% (Gr3)
3.3% (Gr4)
arbaro G, et al. Hepatology. 1998
Ascites Ascites - - CriticaI Care Aspects CriticaI Care Aspects
Complicated ascites may be the principal reason for care
admission but is frequently co-associated with intensive
hemorrhage, renal failure, and/or hepatic encephalopathy.
Common complications of ascites include:
Diuretic-refractory ascites - defined as unresponsiveness to sodium
restriction and high-dose diuretics (400 mg/day spironolactone and 160
mg/day furosemide) OR rapid recurrence after therapeutic paracentesis
Tense ascites - this may result in the development of:
- Abdominal compartment syndrome with impaired venous return causing
hypotension, impaired renal perfusion causing oliguria and reduced
hepatosplanchic perfusion
- Respiratory compromise may occur due to impaired diagphagmatic
contractility and/or hydrothorax due to the passage of ascites into the pleural
space
Infection - (spontaneous bacterial peritonitis)
R:nyon A. Hepatology March 2004
Paracentesis Paracentesis
Abdominal paracentesis is the most rapid and cost-
effective technique to diagnose the cause of ascites.
An area of percussion dullness in the left lower quadrant (2 cm
cephalad and anterior to the anterior superior iliac spine) has a greater
likelihood of ascites present than the midline.
Ultrasound guidance should be utilized if ascites is difficult to localize
and to avoid venous collaterals, intestine.
Since bleeding is sufficiently uncommon, the prophylactic use of plasma
or platelets before paracentesis is not recommended.
An indwelling drainage catheter can be left for 3 - 5 days if therapeutic
drainage is required.
R:nyon A. Hepatology. 2004.
Ascites Ascites - - CIassification CIassification
High SAAG K 1.1g/dI
Cirrhosis (75% cases)
Alcoholic hepatitis
Portal vein thrombosis
udd-Chiari syndrome
Cardiac failure
'eno-occlusive disease
Low SAAG Low SAAG AA 1.1g/dI 1.1g/dI
Peritoneal carcinomatosis
Pancreatic ascites
iliary ascites
Nephrotic syndrome
Tuberculous peritonitis
rige J, et al. MJ. 2001;322.
Spontaneous BacteriaI Peritonitis Spontaneous BacteriaI Peritonitis
Spontaneous infection of ascitic fluid in the absence of a secondary
intra-abdominal source of infection
Translocation of intestinal bacteria or hematogenous seeding of
ascites
Mainly a complication of cirrhotic ascites
ncidence is 15 - 20% of cirrhotics with the highest incidence in
Child's Class C cirrhosis and following upper gastrointestinal
bleeding
E. coli, lebsiella sp., S. pne:2oniae most common
Clinical manifestations include fever, abdominal pain, unexplained
encephalopathy, although asymptomatic presentations are not
uncommon
Mortality per episode = 20 - 30%
One year follow-up mortality = 50%
Spontaneous BacteriaI Peritonitis Spontaneous BacteriaI Peritonitis
Diagnosis Diagnosis
Ascites should be processed for the following:
Total cell count and differential
acterial cultures in blood culture bottles
Other tests (protein, albumin, LDH, glucose, special cultures) may be
indicated based upon clinical judgment
A diagnosis of SP is established by any one of the
following:
> 250 polymorphonuclear cells per cubic mm of ascitic fluid and a
positive ascitic fluid culture is diagnostic.
Patients with K 250 PMN's/mm
3
but negative cultures (neutrocytic
ascites)
Positive ascites cultures and < 250 PMNs/mm
3
(monomicrobial non-
neutrocytic ascites)
R:nyon A. Hepatology. 2004.
Spontaneous BacteriaI Peritonitis Spontaneous BacteriaI Peritonitis
Treatment Treatment
ntravenous albumin 1.5g/kg at the time of diagnosis
followed by 1g/kg on day 3 helps in preventing
hepatorenal syndrome and decreases mortality
(Sort P, et al. N Engl J Med. 1999;341:403-409)
Secondary bacterial peritonitis
PMN count K 250 cells/mm3
Multiple organisms on Gram's stain and culture
Two of the following ascites criteria:
- Total protein > 1g/dl
- LDH > upper limit of normal for serum
- Glucose < 50mg/dl
Treatment Third generation cephalosporin and laparotomy
Spontaneous BacteriaI Peritonitis Spontaneous BacteriaI Peritonitis
AASLD GuideIines AASLD GuideIines
Patients with ascitic fliud PMN K 250/mm should receive empiric
antibiotic therapy e.g., cefotaxime, 2 g every 8 hours ().
Patients with ascitic fliud PMN < 250/mm with signs or symptoms
of infection should receive empiric antibiotics pending culture
results (-).
Oral ofloxacin can be considered in patients without vomiting,
shock, grade 2 hepatic encephalopathy, or serum creatinine >
3mg/dl.
Prevention of SP:
Short-term (7 days) inpatient norfloxacin or bactrim prophylaxis in
patients with gastrointestinal hemorrhage
Patients with prior SP should receive long term prophylaxis with daily
norfloxacin or bactrim (SP recurs in up to 70% of cases within one
year).
Refractory Ascites: Management Refractory Ascites: Management
Serial paracentesis every 2 to 4 weeks and/or
transjugular intrahepatic portosystemic shunts:
Post-paracentesis volume expansion is controversial but may be
considered when 5 l or more of fluid is removed. Albumin (6-8 g per l of
fluid removed), dextran 70 or hemacecel may be used.
A recent meta-analyses comparing TPS vs.
Paracentesis showed:
30-day mortality - no difference, OR 1.0 (C 0.1-10.06)
24-month mortality - no difference, OR 1.17 (C 0.52-2.66)
12-month ascitic fluid reaccumulation - less in TPS, OR 0.14 (C 0.06-
0.28)
Hepatic encephalopathy - more with TPS, OR 2.11 (C 1.22-3.66)
No difference in the incidence of G bleed, infections, or acute renal
failure.
Sheagren JN, et al. J lin Gast. 1996; Saab S. ochrane
Hepato-iliary Gro:p. 2005.
Tc LabeIed SuIfur CoIIoid Showing FIuid Tc LabeIed SuIfur CoIIoid Showing FIuid
Passage From PeritoneaI to PIeuraI Space Passage From PeritoneaI to PIeuraI Space
99 99
hattacharya, et al.
J Gastroenterol Hepatol. 2001.
Right Hydrothorax Managed with PIeruaI Right Hydrothorax Managed with PIeruaI
Catheter Drainage Catheter Drainage
Before After
HepatorenaI Syndrome HepatorenaI Syndrome
Type 1 HRS:
Acute impairment in renal function defined by doubling of
initial serum creatinine above 2.5 mg/dl or a 50%
reduction of the initial 24-hour creatinine clearance to a
level lower than 20 ml/min in less than two weeks.
Mortality is as high as 90% after 2 - 4 weeks
Type 2 HRS:
Stable or slowly progressive impairment in renal function
not meeting the above criteria. Associated with better
survival than Type 1 HRS.
HepatorenaI Syndrome HepatorenaI Syndrome
Pere Gins, et al. N Engl J Med. 2004;350:1646-1654.
HepatorenaI Syndrome HepatorenaI Syndrome
Criteria for Diagnosis of HRS:
Serum creatinine >1.5 mg/dl or 24-hr creatinine clearance < 40ml/min
Absence of shock, ongoing bacterial infection or fluid loss, and no
current treatment with nephrotoxic drugs
Absence of sustained improvement in renal function (decrease in serum
creatinine to A 1.5mg/dl) after discontinuation of diuretics and trial of
plasma expansion
Absence of proteinuria (< 500 mg/d) or hematuria (< 50 RCs per HPF)
Absence of ultrasonographic evidence of obstructive uropathy or
parenchymal renal disease
Urinary sodium concentration < 10 mmol/L
HepatorenaI Syndrome: Treatment HepatorenaI Syndrome: Treatment
Administration of one of the following drugs or drug
combinations can be considered:
Norepinephrine 0.5 - 3.0mg/h intravenously
Midodrine 7.5 mg three times daily increased to 12.5 mg three times
daily if needed in combination with octreotride 100 3g subcutaneously
three times daily, increased to 200 3g three times daily if needed
Concomitant adminstration of albumin 1 g/kg
intravenously on day one, followed by 20 - 40 g daily
This treatment is given for 5 to 15 days.
End point of the treatment is reduction of serum
creatinine to < 1.5 mg/dl
Vasoconstrictor Studies in HRS Vasoconstrictor Studies in HRS
STUDY Treatment # Pts HRS ReversaI SurvivaI Liver Tx
Guevara Or + A 8 4 5 -
Uriz Te + A 9 7 5 3
Gulberg Or, D, A 7 4 4 2
Mulkay Te + A 12 7 4 2
Ortega Te A 13 10 9 5
Angeli Mi,Oc,A 5 4 4 2
Duvoux NE + A 12 10 6 3
Moreau Te A 99 58 36 13
TOTAL 165 104 (63%) 73 (44%) 30 (18%)
Or - orIipressin NE - norepinephrine
Te - terIipressin OC - octreotide
Mi - midodrine A - aIbumin
These resuIts, aIthough encouraging, need to be
vaIidated by a Iarge, prospective randomized triaI.
HepatorenaI Syndrome: Treatment HepatorenaI Syndrome: Treatment
Hemodialysis or continuous venovenous hemofiltration
may be required as a bridge to liver transplant.
Liver transplantation offers the best survival rate of 70%
at two years.
Kidney function may return to normal post successful
liver transplant.
Other PuImonary CompIications of Chronic Other PuImonary CompIications of Chronic
Liver Disease Liver Disease
Hoeper MM, et al. ancet. 2004;363(9419):1461-8.
Hepatopulmonary syndrome
ncidence of 4 - 29%
Diagnosis requires demonstration of hypoexmia due to abnormal intrapulmonary vascular dilatations causing
shunting or severe ventilation:perfusion mismatching.
'ascular dilatations demonstrable by either agitated saline echocardiography or macro-aggregated albumin
scanning.
Orthodeoxia (desaturation with upright posture) and platypnea (dyspnea with upright posture) may be seen.
Management include supplemental oxygen to maintain SaO2.
Mortality rate of 41% at 2.5 years reported.
Severe HPS may slowly remit after successful liver transplantation although supplemental oxygen required.
Portopulmonary hypertension
ncidence of 2 - 10% (as high as 16% in those referred for liver transplant)
Mean PA pressure > 25 mmHg, P'R > 250 dyne s
-1
cm
-5
, PA occlusion (wedge) < 15 mmHg
Pathogenesis unclear but may include pulmonary arterial plexopathy in medium pulmonary arteries due to
shear stress or high output state or humoral influences.
Suspect in patients with progressive dyspnea and signs of right heart failure.
Continuous prostacyclin (PG2) infusion has shown benefit in non-randomized, open label experience but
may not improve long term survival without liver transplantation.
Severe cases (mean PA > 45) or poor right heart function contraindicates liver transplantation.
PathophysioIogy of Hypoxemia in PathophysioIogy of Hypoxemia in
HepatopuImonary Syndrome HepatopuImonary Syndrome
Hoeper MM, et al. ancet.2004;363(9419):1461-8.
Considerations for Liver TranspIantation in Considerations for Liver TranspIantation in
CriticaIIy III CriticaIIy III
Liver transplantation is the most effective treatment for
chronic liver failure with an overall, one-year, 88%
patient survival.
Patients with cirrhosis should be referred for
transplantation when evidence of hepatic dysfunction or
major complications develop.
Patients with type HRS should have an expedited
referral for liver transplantation.
The prognostic model for end stage liver disease (MELD
score) predicts liver-related mortality based upon the
serum Cr, serum bilirubin, and NR.
M:rray , arithers R. AASD Practice G:idelines: eval:ation of the
patient for liver transplantation. Hepatology. 2005.
Liver 1ransplantation