This document discusses portal hypertension, its causes, signs, symptoms, diagnosis, and management. It provides an overview of normal portal circulation and defines portal hypertension as a portal pressure greater than 12 mmHg. It describes various etiologies of portal hypertension including presinusoidal, sinusoidal, postsinusoidal, and posthepatic causes. Complications of portal hypertension like variceal bleeding, ascites, and hepatic encephalopathy are discussed. The management of portal hypertension and its complications is also summarized.

1. Prof.Dr. V.Padma,MD,FRCP(Glasgow),FIMSA
Professor of Medicine,SBMCH

2.  The hepatic portal
circulation carries blood
from GI tract (i.e. from the
distil esophagus to
anorectal junction) to the
liver.
 Porto–systemic
anastomosis occurs in
junctional areas of venous
drainage.
 Portal venous blood drain
into venous sinusoids of
liver and hence in to the
hepatic veins.

3. NORMAL PORTAL HYPERTENSION
 Pressure = Flow X
Resistance
 Portal pressure : 5-10
mm Hg
 Normal elevation:
 Eating
 Exercise
 Valsalva
 10 mmHg (prolonged) →
Shunting
 Lt Gastric → esophageal
 Short Gastric → Gastric
Submucosal
 Lt portal → epigastric
 Retroperetoneal and
anorectal collateral
 12 mm Hg → Bleeding

4.  Portal hypertension develop
when there is elevation of
portal pressure is greater
than 12 mmHg, while normal
portal pressure is 5 –
10mmHg.
 As portal hypertension
produce no symptoms it is
usually diagnosed following
presentation with
decompensated chronic liver
disease encephalopathy,
ascites or variceal bleeding.

5. PRESINUSOIDAL SINUSOIDAL
 Extra-hepatic:
 Portal vein thrombosis,
 Splenic vein Thrombosis,
congenital atresia, extrinsic
compression, schistosomiasis,
superior mesenteric vein
thrombosis
 Intra-hepatic:
 Congenital hepatic fibrosis
 Primary biliary cirrhosis
 Sarcoidosis
 Schistosomiasis
 Metastatic carcinoma
 Steatohepatitis
 Wilson disease
 Cirrhosis
 Primary biliary disease
 Cryptogenic cirrhosis

6. POSTSINUSOIDAL POSTHEPATIC
Intra-hepatic:
 Heamochromatosis
 Alcoholic cirrhosis
 Post-hepatitic cirrhosis
 Hepatic vein thrombosis
 Veno occlusive disease
Extra-hepatic:
 Budd Chiari syndrome
 Rt heart Failure

7.  Cirrhosis results in scarring (perisinusoidal
deposition of collagen)
 Scarring narrows and compresses hepatic
sinusoids (fibrosis)
 Progressive increase in resistance to portal
venous blood flow results in PH
 Portal vein thrombosis, or hepatic venous
obstruction also cause PH by increasing the
resistance to portal blood flow
 As pressure increases, blood flow decreases
and the pressure in the portal system is
transmitted to its branches
 Results in dilation of venous tributaries
 Increased blood flow through collaterals and
subsequently increased venous return cause an
increase in cardiac output and total blood
volume and a decrease in systemic vascular
resistance
 With progression of disease, blood pressure
usually falls

8.  Oesophageal and gastric varices(lt
gastric vein+short gastric vein(P)-
intercostal,diaphragmatic,esophageal
,azygos vein(VC) )
 Haemorrhoids (superior hemohhoidal
vein(P)-middle and inferior
hemorrhoidal veins(VC) )
 Caput medusae (remnant of umbilical
circulation-large paraumbilical vein-
epigastric venous system around the
umbilicus )
 Retroperitoneal veins-
gastrointestinal veins through the
bare areas of the liver
 Omental and lumbar veins

9.  Hepatomegaly initially later shrunken liver
 Jaundice
 Ascites
 Circulatory changes
 Spider telangiectasia, palmar erythema, cyanosis
 Endocrine changes
 Loss of libido, hair loss
 Men: gynaecomastia, testicular atrophy,
impotence
 Women: breast atrophy, irregular menses,
amenorrhoea
 Haemorrhagic tendency
 Bruises, purpura, epistaxis, menorrhagia
 Portal hypertension
 Splenomegaly, collateral vessels, variceal
bleeding, fetor hepaticus
 Hepatic (portosystemic) encephalopathy
 Pigmentation, digital clubbing

11.  Specific treatment in some pre cirrhotic lesions:
Wilson disease—D penicillamine,
Hemochromatosis---phlebotomy,
Antiviral drugs for chronic viral hepatitis
 In established cirrhosis-treatment of complications
 Screening for hepatocellular carcinoma
 Liver transplantation
 Maintenance of nutrition

12.  Caused by hepatic venous obstruction at the level of the inferior
vena cava, the hepatic veins, or the central veins within the liver itself
 Result of congenital webs (in Africa and Asia), acute or chronic
thrombosis (in the West), and malignancy
 Acute symptoms include hepatomegaly, RUQ abdominal pain,
nausea, vomiting, ascites
 Chronic form present with the sequelae of cirrhosis and portal
hypertension, including variceal bleeding, ascites, spontaneous
bacterial peritonitis, fatigue, and encephalopathy
 Diagnosis is most often made by US evaluation of the liver and its
vasculature. Cross-sectional imaging using contrast-enhanced CT or
MRI . Gold standard for the diagnosis has been angiography
 Management has traditionally been surgical intervention (surgical
decompression with a side-to-side portosystemic shunt)
 Minimally invasive treatment using TIPS may be first-line therapy now
 Response rates to medical therapy are poor

13.  Most common cause in children (fewer than 10% of adult pts.)
 Normal liver function and not as susceptible to the development of
complications, such as encephalopathy
 Diagnosis by sonography, CT and MRI
 Often, the initial manifestation of portal vein thrombosis is variceal
bleeding in a noncirrhotic patient with normal liver function
 Causes:
 Umbilical vein infection (the most common cause in children)
 Coagulopathies (protein C and antithrombin III deficiency),
 Hepatic malignancy, myeloproliferative disorders
 Inflammatory bowel disease
 pancreatitis
 trauma
 Most cases in adults are idiopathic
 Therapeutic options are esophageal variceal ligation and sclerotherapy
 Distal splenorenal shunt
 Rex shunt in patients whose intrahepatic portal vein is patent (most
 commonly children)

14.  Most often caused by disorders of the
pancreas (acute and chronic pancreatitis,
trauma, pancreatic malignancy, and pseudocysts)
 Related to the location of the splenic vein
 Gastric varices are present in 80% of patients
 Occurs in the setting of normal liver function
 Readily cured with splenectomy (variceal
hemorrhage), although observation for
asymptomatic patients is acceptable.

15.  Decrease or reverse portal blood flow to the liver
promote the development of the portosystemic
anastomosis between the portal system and
systemic circulation-dilated veins around umbilicus.
 Liver cell dysfunction/liver failure occurs in
hepatic and post – hepatic causes
 Ascites
 Splenomegaly (hypersplenism may be result)
 The CHILD – PUGH classification is used to asses the
severity.
 Jaundice
 Anemia
 Signs of encephalopathy-asterexsis

16. Conditions 1 2 3
Bilirubin (md/dl) <2 2-3 >3
Albumin (g/L) >3.5 2.8 – 3.4 <2.8
Prothrombin
index(%)
>70 40-70 <40
Ascites None Slight-
Moderate
Moderate –
severe
Encephalopathy None Slight-
Moderate
Moderate –
severe
 5-7 =A
 7-10 =B
 >10 =C
 Child A – mild
B → non-
transplant
surgery
 Child C –
advanced B →
transplant

17.  GI bleeding due to gastric and esophageal varices
 Ascites
 Hepatic encephalopathy
 portal hypertensive gastropathy and colopathy.
 congestive splenomegaly,hypersplenism
 Hepatorenal syndrome
 Hepato pulmonary syndrome

18.  1- FBC, Urea & electrolytes ,LFT and clotting fn.tests
 2- Screening tests for the causes of the cirrhosis
 3- CT & ultrasound scan to assess liver morphology,
diagnose portal hypertension and assess cause.
 4- Transabdominal Doppler ultrasound to assess blood
flow in the portal vein and hepatic artery.
 5-Gastroscopy in acute variceal bleeding
 6-portal venogram
 7-measurement of portal venous pressure through
transjugular cannulation of hepatic veins(high in
sinusoidal and postsinusoidal PH)

19.  General resuscitation
 Anti – coagulation for Budd – Chiari syndrome
 Treatment of hepatic cause
 Treatment Of Chronic Complication such as Esophageal gastric
varices:
1- Beta – blocker (propranolol or nadolol), reduce portal venous
pressure due to vasodilatory effects on both splanchnic arterial bed and
portal venous system and reduced cardiac output.
2- Repeated injection sclerotherapy or variceal ligation
3- Elective porto – systemic shunt (spleno – renal anastomosis)
4- Liver transplant may be considered for treatment if associated with
severe liver diseases.
 Rectal Varices: Injection sclerotherapy
 Symptomatic splenomegaly: laparoscopic or open splenectomy.
 Ascites: Oral spironolactone, in cases of ascites, paracentesis may be
required with IV albumin replacement.

20.  Hemorrhage from the varices is acute complication of the portal
hypertension.
 Mortality rate of first variceal bleed established portal hypertension is
30%.
 Bleeding arises from oesophageal varices mostly or from gastric varices
Causes & Features:
 Typical variceal bleeding is rapid in onset, copious dark blood with little
mixing with food.
 Feature of established portal hypertension e.g. capute medusae
 Feature of developing hepatic encephalopathy
 Factors like NSAIDS intake,high portal pressure,large varices,endoscopic
variceal stigma(red spots,red stripes),tense ascites precipitate bleeding
 Symptoms and signs of shock(tachycardia,systolic Bp <90mmHg,urine
 output <30ml/hr)

21.  Established large caliber IV access, give crystalloid fluid up to 1000
mL, if tachycardic or hypotensive.
 Only use O - ve blood if the patient is in extreme shock, otherwise
wait for cross – match blood.
 Catheterize and place on fluid balance chart if hypotensive.
 Send blood for FBC, HB conc. WCC, U&E, Na, K, LFT, albumin and
clotting.
 Monitor pulse rate, BP and urinary output.
 Insertion Of Sengstaken Blackemore gastro-esophageal tube may
be a life saving. To be deflated after 24 hrs.If bleeding stops remove
in another 24 hrs.

22.  Decreases the rate of bleeding
 Enhances the endoscopic ability to visualize the site of bleeding
 1.Vasopressin - potent splanchnic vasoconstrictor; decreases portal
venous blood flow and pressure.0.1-0.5 units/min for 4-12 hrs then
reduce dose upto 48 hrs. Terlipressin is better in hepatorenal syndrome
 Somatostatin: direct splanchnic vasoconstrictor.250 microgm bolus
followed by 250 microgm/hr infusion
 Octreotide:Synthetic somatostatin analogue.50 microgm bolus IV foll by
50 microgm/hr
 Short acting nitrates(NTG)-lower portal pressure by direct vasodilation of
porto systemic collaterals

23.  Endoscopic Sclerotherapy with sodum morrhuate,absolute
alcohol,ethanolamine oleate: complications occur in 10-30%
and include retrosternal chest pain, perforation,ARDS,sepsis
 Endoscopic band variceal ligation: becoming the initial
intervention of choice; success rates range from 80-100%
Surgery
 Porto systemic shunt-selective,nonselective
 Totally diverting (end-side portacaval)
 Partially diverting (side-side portacaval)
 Selective (distal splenorenal shunt)
 Splenectomy –splenic vein thrombosis
 Liver transplantation

24.  Primary prophylaxis: prevent 1st episode of bleeding
 Secondary prophylaxis: prevent recurrent episodes of
bleeding
 Include control of underlying cause of cirrhosis and
pharmacological, surgical interventions to lower portal
pressure
 Beta blockade: Beta blockade (Nadolol, Propranolol)
 Sclerotherapy
 Endoscopy band variceal ligation
 TIPSS
 Portosystemic Shunt Surgery
 Prevent encephalopathy by giving lactulose

25.  Neuropsychiatric complication of cirrhosis
 Results from spontaneous or surgical / radiological
portal-systemic shunt + chronic liver failure
 Failure to metabolize neurotoxic substances
 Alterations of astrocyte morphology and function
(Alzheimer type II astrocytosis)
 Types:
 1.acute or subacute-reversible
 2.chronic-progressive leading to coma and death

27.  Increased nitrogen load(GI
Bleed,uremia,constipation,in
creased protein intake)
 Electrolyte
imbalance(hypokalemia,hyp
ovolemia,hypoxia,alkalosis)
 Drugs(narcotics,diuretics,se
datives)
 Large binge of alcohol
 Large volume paracentesis
 TIPSS
 Infection,surgery ,acute liver
disease

28.  Identify and treat
precipitating factor
 Infection
 GI hemorrhage
 Prerenal azotemia
 Sedatives
 Constipation
 Lactulose (adjust to 2-3
bowel movements/day)
 Protein restriction, short-
term (if at all)

30. Hepatorenal Syndrome Hepatopulmonary syndrome
6 criterias
 1.Cirrhosis with ascites
 2.Creatinine>1.5 mg%
 3.Absence of other cause of renal
failure
 4.No treatment with nephrotoxic
drugs
 5.Absence of shock
 6.Kidney is anatomically, histologically
functionally normal
 Type 1:acute,rapid,poor prognosis
 Type 2:chronic,stable,better prognosis
 Decreased blood volume and
increased sympathetic tone
 Treat precipitating factors,
saline/albumin,midodrine,octreotide
 Liver transplant
4 criterias
1.Advanced chronic liver disease
2.Arterial hypoxemia
3.Intra pulmonary vasodilation
(defective clearance of vasodilatory
substance by liver)
4.No primary cardiopulmonary disorder
Clinical
Platypnoea(dyspnoea in upright)
Orthodeoxia(desaturation in upright)
Inv
Contrast enhanced ECHO
Technetium-99m macroaggregated
albumin lung perfusion scan
Treat
Oxygen,drugs like almitrine,methylene
blue, garlic-increase pulmonary vasc
resistance+pulm art pressure
TIPS,liver transplant

31. CAUSES
 Prehepatic, hepatic ,posthepatic
COMPLICATIONS
 GI bleeding due to gastric and esophageal varices
 Ascites
 Hepatic encephalopathy
 portal hypertensive gastropathy and colopathy.
 congestive splenomegaly
 Hepato renal,hepato pulmonary syndrome
TREATMENT
 Treat the cause, reduce portal pressure, liver
transplant