Portal Hypertension Prof.Dr. V.Padma,MD,FRCP(Glasgow),FIMSA

Professor of Medicine,SBMCH

Introduction 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.

Physiology

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

Portal Hypertension 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.

Portal Hypertension

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

Portal Hypertension

POSTSINUSOIDAL POSTHEPATIC

Intra-hepatic: Heamochromatosis Alcoholic cirrhosis Post-hepatitic cirrhosis Hepatic vein

thrombosis Veno occlusive

disease

Extra-hepatic: Budd Chiari syndrome Rt heart Failure

Pathophysiology 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

Portal Vein Collaterals 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

Cirrhosis Liver 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

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

Treatment

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

Budd Chiari Syndrome

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)

Portal Vein Thrombosis

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.

Splenic Vein Thrombosis

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

Clinical Features

CHILD – PUGH Classification Of Portal Hypertension

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

GI bleeding due to gastric and esophageal varices

Ascites Hepatic encephalopathy portal hypertensive gastropathy and

colopathy. congestive splenomegaly,hypersplenism Hepatorenal syndrome Hepato pulmonary syndrome

COMPLICATIONS

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)

Diagnosis & Investigation

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.

Treatment

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)

Acute Variceal Hemorrhage

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.

Emergency management (Resuscitation)

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

Drug Therapy

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

Endoscopic Therapy,Surgery

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

Prevention of Varices

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

Hepatic Encephalopathy

PRECIPITANTS Increased nitrogen load(GI

Bleed,uremia,constipation,increased protein intake)

Electrolyte imbalance(hypokalemia,hypovolemia,hypoxia,alkalosis)

Drugs(narcotics,diuretics,sedatives)

Large binge of alcohol Large volume paracentesis TIPSS Infection,surgery ,acute

liver disease

Treatment 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)

Hepatorenal Syndrome Hepatopulmonary syndrome6 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 criterias1.Advanced chronic liver disease2.Arterial hypoxemia3.Intra pulmonary vasodilation (defective clearance of vasodilatory substance by liver)4.No primary cardiopulmonary disorder

ClinicalPlatypnoea(dyspnoea in upright)Orthodeoxia(desaturation in upright)

InvContrast enhanced ECHOTechnetium-99m macroaggregated albumin lung perfusion scan

TreatOxygen,drugs like almitrine,methylene blue, garlic-increase pulmonary vasc resistance+pulm art pressureTIPS,liver transplant

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

SUMMARY

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