portal htn by magdi sasi 2015

PORTAL HYPERTENSION AND OESOPHAGEAL VARICES DR MAGDI AWAD SASI 2015

PORTAL HTN

PHYSIOLOGY:

Portal vein flow 900 --- 1200 ml/min.

Hepatic arterial flow 500 --- 700 ml /min.

Normal pressure 8---12 (( cmH2o))------ 5 –10 mmHg.

Pressure = flow(( rarely due to increase flow AV fistula and splenomegally x resistance(( )).

PORTAL HTN--- an increase in resting portal venous pressure > 12 mmHg.

PORTAL HTN results from:

A. Increased resistance to portal flow

B. Increased portal flow

measurement of hepatic venous pressure are useful for determining the site of resistance . The

hepatic venous pressure gradient is the difference between the wedged hepatic venous pressure and

free hepatic venous pressure.

DEFINITION:

Portal HTN is an increase in the blood pressure within the portal venous system . Normally

the veins come from the stomach ,intestine ,spleen and pancrease ,merge into the portal

veins. If the vessels in the liver are blocked ,it is hard for the blood to flow causing pressure

in the portal system.

When the pressure becomes too high ,the blood backs up and finds other ways to flow

back to the heart ,where it is pumped to the lungs ,where it gets rid of waste products and

picks up oxygen .

The blood travel to the veins in the oesophagus ,in the skin of the abdomen , and the veins

of the rectum to get around the blockages in the liver.

It is Product of portal flow volume and resistance to out flow from the portal vein.

The hydrostatic pressure is more than 5 mmHg , results initially from obstruction to portal

venous out flow.

WHAT IS THE CAUSE?

The most common symptom is cirrhosis.

PRE SINUOIDAL------ portal vein thrombosis

Portal fibrosis

Infiltrative lesions

SINUSOIDAL -------------cirrhosis

POST SINUSOIDAL ------ budd chiari syndrome

Venoocclusive lesions

In cirrhosis,

1.There is increased resistance to out flow through distorted hepatic

sinusoids

2.Enhanced portal flow due to splanchnic arteriolar dilatation


CAUSES OF PORTAL HTN: A.EXTRAHEPATIC OBSTRUCTION OF PORTAL VEIN AND ITS BRANCHES:---- 1. Postal vein thrombosis 2. Splenic vein thrombosis B. HEPATIC VENOUS OUTFLOW OBSTRUCTION:----- 1. Suprahepatic ----

i. Budd chiarri syndrome ii. Constrictive

iii. Right heart failure 2.Smaller hepatic vein and venules--- A. Veno-occlusive disease

i. Pyrrlizidine alkaloids ii. Radiation

iii. Anti leukemic drugs B. Sclerosing hyaline necrosis C. Hepatic causes: There may be presinusoidal and sinusoidal resistance to flow

1) Presinusoidal : 1. Schistosomiasis 2. Myeloproliferative 3. Primary biliary cirrhosis 4. Sarcoid 5. Arsenic ,Vinyl chloride 6. Firopolycystic disease 7. Ideopathic P.HTN

2) Sinusoidal: 1. Cirrhosis 2. Chronic active hepatitis 3. Partial nodular transfusion

D.Increased hepatic blood flow: Tropical splenmegally Hematological disease with massive splenomegally Hepatorenal A/V fistula

PORTAL VEIN THROMBOSIS:

1. Abdominal sepsis—omphalitis ,pancreatitis, umbilical sepsis ,portal sepsis

2. Collagen vascular disease, inflammatory bowel disease

3. Cirrhosis ,retroperitoneal fibrosis

4. Hepatocellular carcinoma

5. Myeloproliferative syndromes ,PNH

6. Compression or invasion of portal vein by tumor

7. Sclerotherapy

8. Hypercoagulable--Factor V leide deficiency ,protein C deficiency

9. Pregnancy

10. Trauma

Liver cirrhosis is the cause of 80% of cases.


With presinusoidal P.HTN , due to blocked portal vein or narrowing of the smaller intrahepatic

portal vein branches, the WHVP is normal.

P.HTN in cirrhosis may be:

a. Sinusoidal (( high WHVP + HPVG ))

b. Pruning of portal vein branches

c. Narrowing of sinusoids due to :

Hepatocyte enlargement

Collagen deposition in the space of disse

Distortion of intrahepatic vasculature by fibrosis / nodule formation

WHAT ARE THE SYMPTOMS OF PORTAL HTN?

The onset may not always be associated with specific symptoms .the main symptoms and

complications of PORTAL HTN include:

1. GIT bleeding----black tarry stools or blood in the stools ,hematemsis

2. Ascites

3. Encephalopathy ,confusion ,forgetfulness

4. Bleeding tendency----peteichea , ecchymosis

CLINICAL SIGNS:

Signs may be related to the underlying cause or complication of P.HTN. The spleen is usually palpable ,may be very large in young patients with :

1. Extrahepatic portal block

2. Small ,shrunken livers

Prominent abdominal wall veins are often found with P.HTN

Veins radiating away from the umbilicus are a valuable sign as they indicate that

the block is distal to the main portal vein branches. They disappears if P.V.

thrombosed.

With extrahepatic portal vein block ,there are no abdominal wall collaterals .

Collaterals may occur at the site of surgical scar and indicate only a high portal

pressure.

Prominent abdominal wall veins result on the side of an ileofemoral block ;or with

IVC obstruction " when they are bilateral and visible over the back".

Budd chiarri syndrome----Hepatomegally ,Ascitis ,Signs of IVC obstruction

The sudden development of umbilical collaterals in patients with cirrhosis suggests

an acute hepatic vein block ; inferior vena caval collaterals suggest a blocked cava.

A venous hum ;loudest during inspiration ; Heard over large upper abdominal

collaterals (( Cruveilheir --Baumarten syndrome )). There may be accompanying

thrill

HOW IS PORTAL HTN TREATED?

Most causes can't be treated. Treatment focuses on preventing or managing

complications especially bleeding from the varices.


RICESVA

od to systemic olHTN portal vein and return bseen to decompress the -----VARICES

circulation.

Varices in the oesophagus tend to develop in cirrhosis when HPVG rises> 12 mmHg.

They are seen when the pressure gradient B/W portal veins and hepatic veins > 12mmHg.


The portal –hepatic venous pressure gradient is obtained by hepatic venous

catheterization and with measurement of the difference between the wedged

hepatic venous pressure (( which approximates the sinusoidal and portal pressures

in cirrhosis and the free hepatic venous pressure)).

Oesophageal varices----important collateral in P. HTN

90% Oesophagus ,10% gastric area

Oesophageal varices are dilatation of normal submucosal oesophageal veins which

extend 10—15cm below gastroes-ophageal junction.

Bleeding can be slow oozing or sudden sever.

Bleeding occurs nearly always from the lower 5cm of the oesophagus.

CLINICAL PRESENTATION:

Hematemsis

Melena

Hematochezia

Upper GIT bleeding can be:

1. Bleeding varices.

2. Portal hypertensive gastropathy.

The severity depends on the amount and rapidity of blood loss.

CONSEQUENCES OF PORTAL HTN:

Increased P.V.P----distends the veins proximal to the block and spleen enlarges

Capillary pressure raises in the organs drained by the obstructed V.


Fluid exudation and lymph flow both increase.

Small anastomoses connecting the portal and systemic circulation may enlarge (( often

markedly )) because of the increased portal pressure and allow portal blood to pass

directly into the systemic circulation.

Patients with cirrhosis tend to have a high cardiac output ,and increased splanchanic blood

flow may maintain an elevated portal HTN despite the development of large anastomoses.

There are three main types of anastomosis:

1. Gastro –oesophageal junction:

Veins at the oesophagogastric junction shunt blood from the left gastric and short

gastric veins into the inferior oesophageal plexus and onto the superior vena cava

via the azygous system.

Varices are dilated(( submucosal veins projecting into the lumen of oesophagus

and stomach)).

2. Venous channels from retroperitoneal visera may communicate directly with

systemic veins on the posterior abdominal wall.

3. The obliterated umbilical vein and paraumbilical veins may open up, allowing

blood to pass from the left branch of the portal vein to the umbilicus and thence

into abdominal –wall veins.

Anterior abdominal wall collaterals also occurs where adhesions exists between

abdominal viscera and parietal peritoneum or at ileostomy or colostomy site.

Localized varices in colon may be related to previous operation.

When hepatic veins are occlude "budd chiarri syndrome" ;collaterals open up

within the liver ; blood tends to be diverted through the caudate lobe whose short

hepatic veins drain directly into the inferior vena cava.

A varix explodes as a result of:

1. Increased pressure

2. Increased size

3. Thinning of its wall

Sites of bleeding:

1. Oesophageal varices

2. Gastric varices

3. Portal HTN gastropathy

4. Sclero-therapy ulcer

5. Peptic ulcer

6. Oesophageal erosions

7. Gastric erosions

May be more sever because of high IVP.

Seen in Alcoholics and NSAIDS ingestion.

8. Rectal varices


IS THERE A RISK FACTORS FOR BLEEDING VARICES?

1.LOCATION OF VARICES:

Distal oesophagus ,stomach ,rectum

Gastroesophageal junction have the thinnest coat of supporting tissue.

It is most likely to rupture and bleed.

The varices in the gastric fundus bleed frequently.

Gastric varices are classified according to their location.

A. Varices in different continuity with esophagus along the lesser and greater curves of the

stomach ((gastroesophageal varices --GOV)) type 1 & 2

B. Isolated gastric varices in the fundus occur less frequently than GOV (( 10% Vx 90%))

Bleeding from isolated gastric varices in the fundus occurred much more frequently than others.

RISK FACTORS FOR RECURRENT VARICEAL BLEEDING

2.SIZE OF VARICES:

Small increase in the vessel radius result in large increase in wall tension.

F1 small straight varices

F2 enlarged tortuous varices that occupy < 1/3 of lumen

F3 large coil –shaped varices that occupy > 1/3 of lumen

Causes of hematemsis: 1. Peptic ulcer disease 2. Erosive gastritis due to hypergastrinemia , alcohol intake

Hypergastrinemia occurs due to defective metabolism by cirrhotic liver.

REBLEEDING > 6 WEEKS(LATE) REBLEEDING < 6 WEEKS(EARLY) ITEM

no yes Age >60 year

no yes Severity if initial bleed Anemia / blood pr low

no yes Renal failure

yes yes Severity of hepatic failure

yes yes ascites

yes not hepatoma

yes no alcoholism

no yes Active bleeding on endoscopy

yes yes Increased varices size

Not known yes Red signs

Not known yes Platelet clot on varix

no unclear Portal pressure


3.APPEARANCE OF VARICES:

Red wale marks

Cherry red spots

Hematocystic spots

Diffuse erythema

4.CLINICAL FEATURES:

Degree of liver dysfunction

H/O previous variceal bleed

5.VARICEAL PRESSURE:

By endoscopic gauge

0 13 mmHg≥

9% 13 ----14

17% >14---- <15

50% >15----<16

72% >16

MANAGEMENT:

Investigation-----

CBP , coagulation profile , RFT ,electrolytes ,BUN ,LFT ,blood group ,Cx matching

When portal HTN is suspected ,endoscopy should be done to look for

oesophageal and gastric varices.

Increased risk of bleeding: 1) Large varices---5 mm in diameter 2) Blue varices 3) Have red wheals

P.HTN gastropathy causes chronic anemia or frank bleeding .

Red spots similar to petechiae , particularly over antrum and fundus.

Diagnostic endoscopy is mandatory as identification of the source of

bleeding and directly influence therapy.

If it is delayed for 24 hours, it may be impossible to identify the source

of bleeding.

Early endoscopy always identify source of bleeding after few hours.

Delirium tremens ----------------------------------controlled by Chloromethiazole


Bleeding varices------ definitive if there is :

A. A venous { non pulsatile spurt } ; A venous ooze a varix

B. A white fibrin –platelet plug on the varix

C. Adherent blood clot ---rare

In cirrhotic patients ,there is gastric varices 10% and non variceal source 30%.

The major detrminent of subsequent mortality depends on:

A. The severity of bleeding

B. Pugh childs grading

A 10% B 25% C 50%

In patient with splenomegally or prominent abdominal wall veins ,but no varices ,P.HTN

can be confirmed by direct measurement of portal pressure by

1. Splenic puncture

2. Transhepatic cannulation of portal vein branch

Liver biopsy helpful but can be normal in:

A. Ideopathic portal HTN

B. Extrahepatic portal venous obstruction

Portography -----to detect the site of block to portal flow

Abdominal uss and CTscan

Doppler USS give information about the direction of flow.


Catheterization of hepatic vein with venography.

You should asses RFT , evidence of infection –CXR ,U C/S , BLOOD CULTURE

INVESTIGATIONS: CBC LFT USS ABDOMEN –asctes, splenomegally ,liver cirrhosis Detection of oesophgeal varices: Fibroptic endoscopy Barium swallow Duplex scan Liver biopsy/ hepatitis marker Portal venography (( prior to surgery ))

CLIP SCORING SYSTEM: Child –Pugh stage Score

A 0

B 1

C 2

Tumor morphology :

Uninodular with extention ≤ 50% 0

Multinodular with extention ≤ 50% 1

Massive or extention > 50% 2

Alpha feto protein:

<400 0

≥400 1

Portal vein thrombosis;

NO 0

YES 1

TREATMENT :

1. Ensure clear airway

2. I.V. line ---central or peripheral (( large bore venous canula )).

3. Replacement of loss –Blood cross matching

After restoring the systolic pressure to 80—90 mmHg ;Packed cells should be given to

maintain HB 10 g/dl and the urine output 40ml/hr or more.

Monitor CVP ---I.V.ringer lactate

Fresh frozen plasma given to maintain and correct the effect of large transfusion of stored

blood .

Fresh frozen plasma If P.T. prolonged or platelet count < 50000/ml


Platelet transfusion if massive transfusion or thrombocytopenia

VIT K 10 mg should be given.

4. Lactulose and mg sulphate to decrease encephalopathy

5. Na containing fluid should be avoided

6. Insert Foleys catheter

7. Arrest the haemorrhage:

A. Black moor stengstaken tube---gastric and oesophageal ballon

Balloon tamponade

If the patient uncooperative or comatosed ,intubation is the solution.

Bleeding is controlled in 90% of cases.

If the bleeding is continuing ,the cause is:

a. Incorrect placement

b. Presence of coagulopathy

c. Wrong diagnosis about source of bleeding

It should not be maintained for more than 12 hours

Temporary measures is done until definitive therapy planned.

1. Linton tube(( gastric balloon ---alternative ))

Aspiration of gastric and oesophageal content

Inflation of gastric then oesophageal balloon

Not more than 24 hrs

2. VASOACTIVE DRUGS—splanchnic arteriolar vasoconstrictors

A. VASPRESSIN :

20U in 200ml fluid over 20 min---0.2 -0.4U/min for 24-48hr

C/I--- coronary artery vasospasm ,so give glyceryl nitrate" infusion or patch "

Systolic pressure should be maintained at 100mmHg or more.

Morphin is contraindicated.

Once is hemostasis stabilized ,urgent endoscopy is done with sclerotherapy

(( ethanolamine oleate)) injected into varices.

B. Glypressin 2mg/6hr/24---48hr

C. Somatostatin 250 microgm/hr for 2 ---5 days

D. Octeotide 5o microgm /hr for 2 –5 days

They don’t alter hospital mortality.

3. Operation :

Emergency operation is avoided because of higher mortality but if sclerotherapy is

failed ,there is no alternative.

Types:

Devascularization or transaction

Portosystemic shunting

4. General measures to:

A. Encephalopathy ----neomycin ,lactulose ,mg sulphate

B. Correction of clotting defect ---VIT K injection& prevention of rebleeding

C. Endoscopic therapy:

5% Ethanolamine ,1% polidocanal

Sclerotherapy is the mainstay of acute management of bleeding varices.

Oesophageal ---- inject into the varix or along side it.

Gastric -tissue adhesive(( Bucrylate)) , polymerizes in contact with bloodRepeated

sclerotherapy with regular check.

Single session of sclerotherapy 65% affective

Two session of sclerotherapy 85% affective


No more than two sclerotherapy sessions should be used in 5 days.

This is to minimize oesophageal complications.

1. Mediastinitis 2. Deep oesophageal ulceration 3. Oesophageal stenosis

D. Devascularization and transection operation

A surgical procure that removes the bleeding varices. .This procedure done when a

TIPS or surgical shunt is no possible or is unsuccessful in controlling the bleeding.

Upper 1/3 of stomach and lower part of oesophagus are devascularized.

Gastroeosophageal junction is transected and reanastomosis by ((stapling gun)).

E. Portosystemic shunt

TIPS ---transjugular intrahepatic portosystemic stent shunt

To achive shunting with out surgery.

It is very affective.

It has higher thrombosis rate within 12 hours.

If transplantation is contemplated ,abdominal surgery should be avoided.

Portacaval ---- end to end ,side to side Mesocaval Proximal splenorenal Distal splenorenal

PORTOCAVAL---portal vein is patent with good liver function.

It is very effective in lowering portal pressure.

Disadvantage ---high incidence of encephalopathy

MESOCAVAL---- used when portal vein is thrombosed.

PROXIMAL SOLENORENAL---less encephalopathy .It is less effective in preventing

further bleeding.

DISTAL SPLENORENAL ----SELECTIVE SHUNT

Right and left gastric vessels are ligated ,the distal splenic vein is jointed to renal

vein and short gastric veins are preserved.

Low incidence of encephalopathy , liver function remains normal

TRANSJUGUKAR INTRAHEPATIC PORTOSYSTEMIC SHUNT

F. Splenectomy :

It is only effective in sectorial HTN (( LT side portal HTN)) that occurs after

pancreatitis leads to splenic vein thrombosis---fundal varices

THE MAJOR COMPLICATIONS OF VARICEAL BLEEDING: Which may lead to multiorgan failure and cause death

1. PNEUMONIA: Especially during endoscopy or placement of balloon tamponade tube Tracheal intubation should be used if patient comatosed. Pulse oximetry is mandatory ,even in absence of pneumonia As hypoxemia is common ,sepsis ,shock ,massive transfusion

2. Hepatic encephalopathy: Lactulose Twicw daily cleaning enema

3. Infection due to: Enteric organism causing septicemia Spontaneous bacterial peritonitis Cephalosporin I.V. is the antibiotic of choice. Nephrotoxic antibiotics should be avoided .


As soon as infection is suspected ,it should be treated. 4. Water / electrolyte imbalance:

With ascitis and development of renal failure Saline solutions should be avoided. Aim ---maintain an adequate circulating volume & avoid volume overload. Ascites should be treated. Electrolyte abnormalities should be corrected.

5. Under nutrition: Nutrition should be strated as soon as possible within 24 hrs wether enteral or parenteral according to the renal function and bleeding.

REBLEEDING:

Rebleeding is the most frequent in 1st 6 weeks,accounting for 20—30% of deaths during

follow up. Patients who survived the first bleed from oesophageal varices are at significant

risk of recurrent haemorrhage: 70% of patients will experience recurrent haemorrhage and

about a third of further bleeding episodes are fatal.

Up to fifty percent of recurrent haemorrhage occurs within the first 6 weeks after the

index bleed.

The risk of re-bleeding is highest during the first five days, decreases slowly over the first 6

weeks, and becomes virtually equal to that before the index bleed after the sixth week.

Risk factors predictive of re-bleeding include the degree of hepatic decompensation, age greater than 60, severity of initial bleed, renal insufficiency, level of portal pressure, size of varices, active bleeding at the time of initial endoscopy and the presence of hepatoma.

All therapy decrease rebleeding and choice depends on:

1. Individual circumstances

2. Severity of liver disease

3. Feasibility of different methods of treatment

Portal systemic shunts decrease 10% + not proloning survival

Repeated sclerotherapy decrease 50% + reduce mortality

Drug therapy ----more convinent and cheaper

B—BLOCKER

Decrease cardiac output ----decrease variceal flow

Allowing unopposed α vasoconstriction of splanchanic arterioles.

Acts directly on collaterals feeding the varices.

Decrease portal pressure with increase intra hepatic resistance.

Appears that lowering the hepatic vein pressure gradient to < 80%.

Pretreatment values at 3 months reduce risk of rebleeding.

Decrease rebleeding 50% and lowers the mortality.

Portal hypotensive effect may be enhanced by isosorbide mononitrate.

affective to reduce bleeding and anemia in gastropathy.

In trials ,comparing scleropathy with B blockers ,((higher risk patients were

included)) ,there were no differences in survival /rebleeding rates.

No advantage occurred by combinig B blocker withsclerotherapy.


CLINICAL POINTS: HVPG—hepatic venous pressure gradient should be reduced to 12mmHg or below to eliminate the risk of bleeding. 1ry prevention with B blocker show reduction in bleeding from varices and portal hypertensive gastropathy and decrease in mortality. B blocker should be given to patients with varices at risk. 1ry prevention with scleropathy /surgical shunts ,it have shown no overall benefit and increased mortality.

Prognosis in patients with esophageal varices Approximately 30% of patients with esophagieal varices will bleed within the first year after diagnosis. The mortality resulting from bleeding episodes depends on the severity of the underlying liver disease The mortality resulting from any bleeding episode may range from < 10% in well- compensated cirrhotic patients with Child–Pugh grade A to > 70% in those in the advanced Child–Pugh C cirrhotic stage. The risk of re-bleeding is high, reaching 80% within 1 year Patients with a hepatic venous pressure gradient > 20 mmHg within 24 h of variceal hemorrhage, in comparison with those with lower pressure, are at higher risk for recurrent bleeding within the first week of admission, or of failure to control bleeding (83% vs. 29%) and have a higher 1-year mortality rate (64% vs. 20%) Approximately 60% of untreated patients develop “late rebleeding '” within 1–2 years of the index hemorrhage

TIPS:

Transjugular intrahepatic portosystemic shunt:

This procure involving placing a stent in the middle of the liver. The stent connects the hepatic

vein with the portal vein.


DSRS---distal splenorenal shunt:

WHAT TESTS ARE REQUIRED BEFORE THE TIPS AND DSRS PROCEDURES?

Before receiving either of these procures ,the following tests may be performaed to

determine the extent and severity of the condition.

1. Evaluation of the medical history

2. Physical examination

3. Blood tests

4. Angiogram

5. Ultrasound

6. Endoscopy

7. Chest x ray

8. ECG

9. Keep plasma ready for O.T.

WHAT HAPPENS IN TIPS?

A radiologist makes a tunnel through the liver with a needle ,connecting the portal vein to

one of the hepatic veins . A metal stent often covered with a thin plastic material ,is placed

in this tunnel to keep the tunnel open.

The procedure reroutes blood flow in the liver and reduces pressure in all abnormal veins

,not only in the stomach and oesophagus ,burt also in the bowel and the liver

This not surgery , The radiologist performs the procedure within the vessels under X –ray

guidance. This process lasts one to three hours ,but you should expect to stay in the

hospital over night after the procedure.

HOW SUCCESSFUL IS THE TIPS PROCEDURE?

It controls bleeding immediately in 90% of patients.

20% of cases develop rebleeding due to narrowing of the shunt

COMPLICATIONS OF TIPS:

1. Shunt narrowing /occlusion within the 1st year

Follow up UU examinations are performed frequently

The signs of occlusion include increased ascites and rebleeding.

Treatment ---by radiologist who re-expand the shunt with a ballon or

repeats the procedure to place a new stent

2. Encephalopathy ----can occur with sever liver disease

It can become worse when blood flow to the liver is reduced by TIPS which

may result in toxic substances reaching the brain without being metabolise

1st by the liver.

Treated by diet ,medications ,or occluding the shunt


WHAT HAPPENS IN THE DSRS PROCEDURE?

DSRS--- a surgical procedure during which the splenic vein is detached from

the portal vein and connected to the renal vein. .This selectively reduces

the pressure in the varices and control the bleeding. It is done only in

patients with good liver function.

HOW SUCCESSFUL IS THE DSRS SURGERY ?

It controls the bleeing in 90% of patients with the highest risk of rebleeding

occurring in the first month. It provides good long term control of bleeding.

COMPLICATIONS OF DSRS?

Ascites can occur.

FOLLOW UP:

10 days after hospital discharge , patient should follow hepatologist to

evaluate the progress .Lab work will be done at this time.

6 weeks after TIPS , 3 months ,USS should be to check the shunt.

Angiogram is done if USS indicates a problem.

6 weeks after DSRS ,3 months, USS should be done to check the shunt.

12 months after either procedures , USS to be done to check shunt.

If the shunt is working well every 6 months after the 1st year of follow up

appointements ,have an USS ,lab work and visit the doctor

portal htn by magdi sasi 2015

Health & Medicine