portal hypertension

Prehepatic
Portal Vein Thrombosis
Intrahepatic
Presinusoidal: schistosomiasis
Sinusoidal*: Cirrhosis, infiltrative disorders
Postsinusoidal: Veno-Occlusive Disease
Posthepatic
Budd-Chiari Syndrome, Right Heart Failure, Constrictive Pericarditis

Jaundice
Hepatomegaly
Splenomegaly*
Ascites*
Edema
Spider angiomata
Palmar erythema
Gynecomastia
Asterixis

Thrombocytopenia**
Hypoalbuminemia*
Prolonged PT*
Hyperbilirubinemia

Nodular liver*
Splenomegaly*
Ascites*
Large collaterals*

Portal P = blood flow x R
incr blood flow, splanchnic dilation, incr CO all incr splanchnic blood flow which increases portal P.
intrahepatic R, collateral R also incr Portal P

GI bleeding
Ascites
Spontaneous Bacterial Peritonitis
Hepatorenal Syndrome
Encephalopathy

esophageal varices
gastric varices
portal hypertensive gastropathy

much less common:
rectal
stomal
anastomotic

5-10 mmHg =normal
>12mmHg for bleed

prevent bleeding from esoph varicies

Screening EGD for the diagnosis of esophageal and gastric varices is recommended when the dx of cirrhosis is made.*
In patients with compensated cirrhosis and no varices, repeat screening EGD in 3 years.
In patients with decompensated cirrhosis and no varices, repeat screening EGD annually.
If varices are large, nonselective B-blockers or EVL are recommended for prevention of EVH.*
If small varices are present with red wale marks, or in the setting of decompensated cirrhosis, nonselective B-blockers are recommended for prevention of EVH.
If EVL is chosen, repeat EGD every 6-12 months to survey for variceal recurrence.

Octreotide
Endoscopic therapy
Minnesota tube
TIPS- portocaval shunt done non surgically

Octreotide 50 mcg IVP followed by 50 mcg/hr IV should be given in all cases of suspected EVH and continued for 3-5 days if EVH is confirmed by EGD.
Antibiotics should be given for 7 days.
norfloxacin 400 mg PO bid, or
ciprofloxacin 400 mg IV q 12 hours
EGD should be performed within 12 hours, and if EVH confirmed, EVL or EVS should be performed.
TIPS is indicated when octreotide and EVL fail to control EVH.

Patients who survive acute variceal hemorrhage should receive therapy to prevent recurrent EVH. 50-70% will have recurrence
Combinations of nonselective B-blockers plus EVL is the best option for secondary prophylaxis of EVH.

Accumulation of fluid in the peritoneal space
500 cc before clinically evident
Differential diagnosis:
Portal Hypertension
Malignant ascites, nephrotic syndrome, TB
SAAG = Serum albumin – Ascites albumin
> 1.1 in portal hypertension

cirrhosis- incr R to portal Q - Portal HTN - (Splanchnic vasodil, systemic arterial underfiling, activation of ADH, renal Na and H2O retention)- ascites

and Portal HTN- incr hydrostatic P and decr albumin (decr oncotic P) then ascites

flank dullness
shifting dullness
fluid wave
These correlate best with the gold standard (ultrasound), but 1500 cc needs to be present.
Ultrasound can detect 100 cc of ascites.
ascites is unlikely in the absence of LE edema
Always remember to check neck veins in any patient with new-onset ascites. (neck veins up -cardiogenic)

2 gram Na diet
1000 cc fluid restriction only if Na<130
Avoid NSAID
Lasix 40 mg/d (160 mg/d max)
Spironolactone 100 mg/d (400 mg/d max)
Large Volume Therapeutic Paracentesis
Consider IV albumin if removing > 5 liters
TIPS

sodium restriction of 2000 mg per day
diuretic therapy
spironolactone 100 mg PO q AM, and
furosemide 40 mg PO q AM

Ascitic fluid infection without an evident intraabdominal surgically treatable source
Bacterial translocation:
Bacteria traverse colon wall and colonize lymphatics
Small lymphatic vessels rupture into ascites
Bacteria move from lymphatics into systemic circulation
Unique to cirrhotic ascites
Very rarely seen in malignant or cardiac ascites
Diagnosis based on ascites fluid culture and ascites fluid PMN > 250 cells/mm3

e.coli, kleisibella pneumo, strp pneumo, a hemolytic strep, group D strep, other strep, enterbact, misc

Patients with ascites PMN>250 should receive cefotaxime 2 gm IV q 8 hours
when PMN>250, check LDH, TP, glucose, and Gram’s stain to evaluate for secondary peritonitis
Patients with ascites PMN<250 and fever or abdominal pain should receive IV cefotaxime 2 gm IV q 8 hours while awaiting cultures.
Oral ofloxacin 400 mg PO bid can be used in place of IV cefotaxime in patients with no vomiting, shock, PSE, or Cr>3 mg/dl.
All patients with suspected SBP should also receive IV albumin 1.5 g/kg within 6 hrs and 1 g/kg on day 3

All cirrhotic patients with GI bleeding should be given antibiotics for 7 days.
norfloxacin 400 mg PO bid, or
ciprofloxacin 400 mg IV q 12 hours
Patients who have survived an episode of SBP should receive long-term prophylaxis with daily norfloxacin or trimethoprim/sulfamethoxazole.
Patients with cirrhotic ascites and no prior episode of SBP should receive daily antibiotic prophylaxis if ascites TP < 1 gm or T.bili > 2.5

Advanced chronic or acute liver failure with portal hypertension
Serum Cr > 1.5 or 24 hour CrCl < 40
Absence of shock, infection, massive GIB, massive renal fluid loss, nephrotoxic drugs
No sustained improvement in renal function with 1 mg/kg of IV albumin for 2 days
Less than 500 mg/dl proteinuria and no u/s evidence of obstruction or parenchymal dz

Hepatopulmonary syndrome
Increased A-a gradient
Liver disease
Evidence of IPVDs
(intrapulmonary vascular dilations)-- pts feel better lying down
Portopulmonary syndrome
Pulmonary Hypertension in the setting of Portal Hypertension
Patients present with DOE, syncope, chest pain
Hepatic Hydrothorax

Pleural effusion of >500 cc in patients with cirrhosis and no underlying cardiac or pulmonary disease
Likely cause is passage of ascites fluid through small defects in the diaphragm
Because of negative intrathoracic pressure, most patients have only mild ascites
Usually unilateral right-sided effusion (85%)
Typical symptoms: dyspnea, cough

2 gram Na diet
1000 cc fluid restriction only if Na<130
Avoid NSAID
Lasix 40 mg/d (160 mg/d max)
Spironolactone 100 mg/d (400 mg/d max)
Large Volume Therapeutic Thoracentesis
Consider IV albumin if removing > 5 liters
TIPS

A syndrome of reversible brain dysfunction in the setting of significant liver disease
Pathogenesis likely multifactorial
Endogenous toxins: ammonia
Inhibitory neurotransmitters: GABA
Decreased hepatic clearance of ammonia due to liver dysfunction, portosystemic shunts

History
sleep disturbance (insomnia/hypersomnia)
lethargy, somnolence, confusion
Exam
asterixis, hyperreflexia, fetor hepaticus
Laboratory
BMP, drug screen, ammonia
CT head

Correct precipitating factors
Lactulose
titrate to achieve 2-3 soft BM/day
can be used in enema form
Antibiotics
rifaximin, metronidazole, or neomycin
Consider safety issues: driving

Lactulose is the treatment of choice, dosed to achieve 2-3 soft bowel movements daily.

Patients at high risk for HCC should be entered into a surveillance program.*
All patients on the liver transplant waiting list should be screened for HCC.
Surveillance for HCC should be performed using ultrasonography.*
AFP alone should NOT be used for screening, unless ultrasound is not available.
Patients should be screened at 6 - 12 month intervals*

EARLY with fulminant hepatic failure
occurrence of poor prognostic event
Gastrointestinal hemorrhage
Ascites - especially if refractory or SBP
Hepatic encephalopathy
Hepatocellular carcinoma
if solitary tumor < 5 cm, or
up to three tumors < 3 cm