Portal hypertension and variceal bleed

1. Portal Hypertension and
Variceal bleeding
Dr Rohit Gupta
JR(Acad)
General surgery
AIIMS Patna

2. Hemodynamics
• Portal flow - 75% to 80% of the inflow to liver,
remainder from hepatic artery, outflow through
hepatic veins to IVC
• Normal flow is hepatopetal.
• Normal pressure
• Portal venous pressure = 5 – 10 mm of Hg
indirectly measured by HPVG
• Normal HPVG = 3- 5mm of Hg

3. •
 Wedge hepatic pressure obtained by wedging the catheter into smallest branch of
hepatic vein = sinusoidal pressure
 Free hepatic pressure is systemic pressure acts a zero referrence point

4. Clinically significant portal hypertension: HVPG >10-12 mm Hg

6. Etiology

7. Prehepatic
1.Portal /splenic vein thrombosis - most common prehepatic cause .
- associated with umbilical vein catheterization, sepsis and dehydration in infancy.
- In adult patient associated with hypercoagulable syndromes
- Other etiologies - pancreatitis and pancreatic tumors
2 .Extrinsic compression - On the portal vein from (lymph nodes , tumors ) can occasionally
lead to portal Hypertension
3.. Arteriovenous fistula - hepatic artery to portal venous fistulas, usually secondary to
liver biopsy

8. 4. Sinistral portal hypertension - leftsided (sinistral) portal hypertension
- Isolated splenic vein thrombosis, portal vein normal and no intrahepatic block.
- Most common causes - pancreatitis and carcinoma of the body and tail of pancreas
- Large collaterals from splenic hilus to fundus of stomach

9. Extrahepatic Portal Vein Obstruction (EHPVO)
• Childhood disorder -chronic blockage of PV blood supply leading to PHT and its sequelae
with well-preserved liver function.
• As per APASL - defined as ‘‘ vascular disorder of liver, characterized by obstruction of the
extra-hepatic PV with or without involvement of intra-hepatic PV radicles or splenic or
superior mesenteric veins’’
• EHPVO is a distinct disease with well tolerated episodes of variceal bleed, splenomegaly
anemia, with accompanied growth retardation.
• Formation of portal cavernoma and development of PHT differentiates EHPVO from portal
vein thrombosis.

10. Pathogenesis
Initial acute PVT event leading to thrombus
Hepatopetal collaterals around PV in 6–20 days and cavernoma in 3 weeks
Insufficient to decompress high pressure in the splanchnic bed
Hepatofugal vessels develop at the sites of portosystemic communications
Transform into varices, lower GI bleed, splenomegaly
Cavernomatous transformation and biliopathy.

11. Diagnosis
• Doppler ultrasound (USG) - Portal cavernoma , thrombosis
• Endoscopy- Identification of gastric and esophageal varices.
• Liver Function - elevations of alkaline phosphatase and gamma-
glutamyl transpeptidase are seen with development of portal biliopathy
• CT angiography - patency of the venous and arterial systems, cavernous
transformation can be identified, planning shunt procedures

12. Hepatic - Pathophysiology
Obstruction to portal venous flow eg cirrhosis
Activated hepatic stellate cells and myofibroblasts – Fibrosis
Production of vasoconstrictors (endothelin, norepinephrine, angiotensin)
Release of splanchnic vasodilators -(NO ,VEGF) , increased splanchnic inflow.
Systemic hyperdynamic circulation
Development of Portosystemic collaterals

13. Collaterals in portal hypertension
1. Esophageal and gastric varices
2. Paraumbilical and abdominal wall.
3. Retroperitoneal collaterals
4. Mesenteric and omental varices
5. splenic collaterals
6. Rectal varices

14. Complications
• Gastric and esophageal varices
• Splenomegaly and hypersplenism
• Ascites
• Hepatic hydrothorax
• Hepatic encephalopathy
• Hepatorenal syndrome
• Hepatopulmonary Syndrome
• Portal hypertensive gastropathy

15. Clinical manifestation
Variceal bleeding-
 Incidence 8% to 11% in the cirrhotic patients
 Upper gastrointestinal (GI) bleeding one of the most common and life-threatening
complication
 Usually present as hematemesis and/or melena, but can also present with shock
Ascites-
 Late sign of portal hypertension.
 Abdominal distention, weight gain, and shortness of breath - increased fluid and
intra-abdominal pressure.
 Refractory ascites - sign of decompensation of the underlying liver disease.

16. Hepatic encephalopathy
 Cognitive changes, loss of coordination, and asterixis leading to coma.
 Precipitated by bleeding, infection, renal failure, and other manifestations of liver
failure.
Hepatopulmonary syndrome
 Triad of liver disease, arterial hypoxemia, and intrapulmonary vascular dilation .
 Oxygen desaturation, shortness of breath, and dyspnea on exertion caused by
intrapulmonary shunting.
Hydrothorax (pleural effusion) - movement of fluid from the abdominal cavity into
the pleural space, usually on the right side.
Spontaneous bacterial peritonitis - fever, pain, and tenderness.

17. Non-cirrhotic portal fibrosis (NCPF)
• Known as Idiopathic PHT (IPH), hepatoportal sclerosis and obliterative venopathy-disorder of
unknown etiology
• Features of PHT, moderate to massive splenomegaly, with or without hypersplenism, preserved
liver functions, and patent hepatic and portal veins.
• More common in young males (3 to 4 decade) belonging to low socioeconomic groups.
• Infections and prothrombotic states, toxins especially arsenic and human leukocyte antigen
(HLA)-DR3.
• Absence of Cavernomatous changes and stigmata of cirrhosis
• Some Variant of NCPF develop hepatofugal flow – Hepatic enchephalopathy ( 2%)

18. APASL criteria
• Presence of moderate to massive splenomegaly
• Evidence of portal hypertension, varices, and/or collaterals
• Patent spleno-portal axis and hepatic veins on ultrasound Doppler
• Test results indicating normal or near-normal liver functions
• Normal or near-normal HVPG
• Liver histology-no evidence of cirrhosis or parenchymal injury

19. Diagnosis and evaluation
Goals of diagnostic studies
 determine the presence of hepatic disease
 level of obstruction to flow
 presence and extent of intra-abdominal portosystemic collaterals
 direction of blood flow in the portal vein (PV) (hepatopetal/hepatofugal)
 Presence of thrombosis

20. Investigations
• Duplex Ultrasound- first-line examination in diagnosis and follow-up
 Grayscale- evaluate overall morphology and locate focal lesion in liver
 Color Doppler - Portosystemic collaterals are readily identified
• Findings -
Splenomegaly (diameter >12 cm and/or area >45 cm2 )
Dilatation of the PV (diameter >13 mm)
Reduced PV velocity
• MDCT and CT angiography-
 Identify morphologic changes, regenerative and dysplastic nodules, HCC.
 Portosystemic collaterals (varices) - well-defined tubular or serpentine structures.

21. Elastography
• Transient elastography performed by Fibroscan – assess liver and spleen stiffness.
(normal value - 2 and 6 kPa).
• Cirrhosis is invariably associated with liver stiffness .
• Other causes like portal vein thrombosis associated with spleen stiffness..
• Equiment uses ultrasound mounted on a vibrator-velocity of propagation of wave
directly related to tissue stiffness.
• Correlation of elastography with HPVG good till 10 mm of hg.
• Liver stiffness correlates with degree of esophageal varices

22. Endoscopy
• Esophagogastroduodenoscopy (EGD) - gold standard
– esophageal and gastric varices and hemorrhage
• Identification of risk factor, provides immediate
therapy of at-risk variceal columns( sclerotherapy,
band ligation)
• Increased variceal diameter and thin variceal wall
thickness indicated by a red color sign - predictive of
variceal bleeding
• Portal gastropathy can be identified on upper GI
endoscopy

23. Endoscopic classification
Paquet’s classification
(Esophageal varices)
•Grade I: Microcapillaries located in distal
oesophagus or oesophago-gastric junction.
•Grade II: One or two small varices located in
the distal oesophagus.
•Grade III: Medium-sized varices of any number.
•Grade IV: Large-sized varices in any part of
oesophagus.
Sarins Classification
( Gastric Varices)
•Gastro-oesophageal varices Type 1: Continuation of
oesophageal varices into lesser curvature (GOV1).
•Gastro-oesophageal varices Type 2: Oesophageal
and fundal varices are present in continuity with the
greater curvature (GOV2).
•Isolated gastric varices Type 1: Fundal varices are
present in the cardia in the absence of oesophageal
varices (IGV1).
•Isolated gastric varices Type 2: Fundal varices
present in the stomach outside of cardio-fundal
region or first part of duodenum (IGV2).

24. Other investigations
• Liver Function Test- status: bilirubin, albumin, prothrombin time, enzymes.
• Hematologic parameters - hemoglobin, platelet, white blood cell count.
• Hepatitis panel, antinuclear antibody, antimitochondrial antibody
• Metabolic disease markers - iron, copper, alpha1-antitrypsin,α-fetoprotein.
• Calculation of child pugh score – prognosis and treatment.

25. Post Hepatic – Budd Chiari syndrome
Defined as hepatic venous outflow obstruction.
- Primary – venous process ( thrombosis, phebilitis of hepatic veins)
- Secondary – compression or invasion ( malignancy)
Etiology
• Hereditary or acquired hypercoagulable state.
• Primary myeloproliferative disease
• Factor V Leiden with protein C and S defeciency
• Compression ( tumor, polycystic kidney disease)
• Abdominal trauma

26. Categorization
Acute ( fulminant)-
 acute liver failure with jaundice and hepatic encephalopathy or intractable ascitis
and hepatic necrosis .
 Abdominal pain( tender hepatomegaly) , distension ( ascites), variceal bleeding.
Sub acute -
 Minimal ascitis with hepatic vein collaterals
 Generally asymptomatic , right upper quadrant pain ( caudate lobe hypertrophy)
Chronic ( fibrosis)
 Complication of cirrhosis
 Portal hypertension and ascitis

27. Diagnosis
• Doppler usg – enlargment of caudate lobe, inability of visualize juction of hepatic
veins with IVC, spider we apperance nera hepatic vein ostia , flat hepatic wave form
• CT scan – delayed or absent filling of majot hepatic veins , relative clearance of
contrast from caudate lobe, intrahepatic collaterals
• Venography – if non invasive test are negative with strong clinical suspicion, gold
standard – hepativ vein venography.
• Liver fuction – elevated liver enzymes (ischemic hepatocellular damage)

28. Acute variceal bleeding
• Resuscitation – ABC protocol, iv Fluids and blood products.
• Pharmacologic intervention-somatostatin analogue and vasoconstrictive therapy
 Octreotide – Initial IV bolus of 50 ug then continuous infusion 50 ug/h X 2-5 days
 Terlipressin – 2 mg IV every 4 hr until bleeding control ( 48 hrs)
Maintenance – 1 mg IV every 4 hours X 2 - 5 days.
 Proton pump inhibitors – continuous IV infusion
 Antibiotics - Norfloxacin 400 mg X BD x 7 days or ceftriaxone (1g/day)

29. Endoscopic ligation/ sclerotherapy
 Should be performed within 12 hours,
variceal ligation performed to control
bleeding
Ø EVL controls bleeding in approximately 80%
to 100% of patients better results
than sclerotherapy
Ø Multiple follow-up sessions every 1 to 2
weeks until obliteration

30. Balloon tamponade
• Sengstaken-Blakemore or Minnesota tube -
unresponsive to pharmacologic therapy and
EVL.
• Two balloons—a gastric balloon and an
esophageal balloon—to tamponade the
submucosal veins.
• Initial control of bleeding accomplished in 80%
of patients, but over 50% rebleed after the
balloons are deflated.
• Used as a temporizing measure- preparing the
patient for an emergent TIPS or surgical shunt

32. Portal hypertension Emmanuel A Tsochatzis, Jaime Bosch, Andrew K Burroughs. Liver
cirrhosis. Lancet 2014; 383: 1749 36

33. Decompression of varices
Transjugular intrahepatic portosystemic shunt (TIPS)
Indications
• Refractory acute variceal bleeding (gastric or esophageal)
• Secondary prevention of variceal bleeding (gastric or esophageal)
• Portal hypertensive gastropathy
• Refractory ascites
• Budd-Chiari syndrome
• Hepatic venoocclusive disease
• Hepatopulmonary syndrome

34. Absolute contraindications
• Primary prevention of variceal bleeding
• Congestive heart failure
• Severe pulmonary hypertension
• Multiple hepatic cysts
• Active infections or sepsis
• Unrelieved biliary obstruction
Relative contraindications
• Single hepatic cyst or central hepatoma
• Hepatic vein thrombosis
• Portal vein thrombosis
• Severe coagulopathy or thrombocytopenia
• Moderate pulmonary hypertension

35. Outcome and Complications
• Clinical resolution of bleeding is achieved in 90% of cases
• Technical success of TIPS to decrease portal pressure less than 12 mm of Hg achieved in
95% of cases
Complications include-
• Rebleeding ( 9% to 40.6%)
• Intraabdominal hemorrhage
• Right heart failure
• Hepatic encephalopathy
• TIPS dysfunction(defined as ≥50% stenosis ,increase in HVPG to >12 mm Hg, or recurrence )
• Stent Thrombosis or migration.

36. Surgical management
Indications
• Symptomatic PHTN in the noncirrhotic patient with preserved liver function (eg EHPVO,
schistosomiasis)
• Budd−Chiari syndrome
• Total mesenteric venous occlusion
• Failed medical management
Divided into -
• Decompressive procedure (Selective and Non selective)
• Devascularization procedures( Modified Sugiura procedure)

37. Selective Shunts
Sarfeh mesocaval shunt
• Side-to-side shunt from SMV to IVC -
maintain forward portal flow.
• Decreases portal pressure via small, 8 mm
Dacron or PTFE grafts
• Do not expand like side-to-side portacaval
shunts to become complete shunt as it
has a prosthesis.
• No portal dissection requires and less
complicated future transplant
• lower rate of postoperative
encephalopathy, improved ascites and
variceal decompression.

38. DSRS(Distal splenorenal shunt)
• Developed by Dean Warren- selective
gastroesophageal variceal decompression and
preservation of portal flow.
• Includes
 Mobilizing the SV along the inferior border of the
pancreas
 Disconnecting all branches to the pancreas,
 Anastomosis btw the SV and the left renal vein
 Dividing other collaterals like coronary (left
gastric) vein.

39. Advantages-
 Control of variceal hemorrhage( 94%)
 avoidance of portal dissection
 antegrade portal flow (90%)
 lower incidence (15%) of hepatic
encephalopathy.
Disadvantages
 Increased risk of ascites

40. Nonselective Shunts
 End-to-side portacaval
 Side-to-side portacaval (>10 mm)
 Mesocaval shunts
 Central splenorenal shunts
Disadvantages
 Hepatic encephalopathy
 Risk of ascites
 Accelerated need for transplantation

41. Devascularization
Sugiura technique
 Ligation of esophageal , short gastric, lesser and
greater curve veins.
 Esophageal transection and anastomosis
 splenectomy, vagotomy, and pyloroplasty
Modified Sugiura technique
 Single-incision operations
 Devascularization without transection.
 Preservation of the vagus.

42. Definitive management