1. 门脉高压症 the First Affiliated Hospital, College of Medicine,
Portal Hypertension
门脉高压症 the First Affiliated Hospital, College of Medicine,
Zhejiang University
浙江大学医学院附属第一医院
Prof. Zhang Min, MD
张 珉

2. What do we know about Portal Hypertension?

3. Then, What's Portal Hypertension?
Introduction of Portal Hypertension
As early as the 17th century, it was realized that structural changes in the portal circulation could cause gastrointestinal bleeding.
In 1902, Gilbert and Carnot introduced the term "portal hypertension" to describe this condition.
Then, What's Portal Hypertension?

4. Definition Portal hypertension is defined as a pressure > 12 mmHg.
This increased pressure results from a functional obstruction to blood flow from any point in the portal system's origin (in the splanchnic bed) through the hepatic veins (exit into the systemic circulation) or from an increase in blood flow in the system.

5. Pathophysiology The normal pressure of portal vein:
5-10mmHg (13~24 cmH2O)

6. Anatomy of Portal System
The portal vein supplies 70% of the blood flow to the liver, but only 40% of the liver oxygen supply. The remainder of the blood comes from the hepatic artery, and blood from both of these vessels mixes in the sinusoids.

7. Ducts and vessles of the liver
Tow concepts:
Glisson’s sheath
Lobule of the liver

8. Sinus
Central v.
Portal triad
Liver lobule

10. Four ramus communicans between portal and systemic circulations
esophageal and gastric veins
anterior abdominal wall veins
retroperitoneal venous plexus
inferior rectal-anal veins

11. Etiology & Types
Portal hypertension should be divided into three types as its blood flow obstructed positions.

12. Types pre-hepatic intra-hepatic post-hepatic pre-sinusoidal Sinusoidal
post-sinusoidal
post-hepatic

13. Pre-hepatic type the portal vein trunk itself obstruction.
Congenital deformity: obliteration, stenosis
先天性畸形：闭塞、狭窄
Thrombosis: portal vein thrombosis, splenic vein thrombosis, ect
血栓形成：门静脉、脾静脉
Infection: Tropical splenomegaly, ect
感染：热带巨脾综合症
Trauma: arterio-venous fistula, ect
创伤：动静脉瘘

14. Intra-hepatic type
About 95% patients belong to this type of portal hypertension
A classification by pathology:
pre-sinusoidal obstructions
sinusoidal obstructions
post-sinusoidal obstructions

15. Pre-sinusoidal obstruction
Schistosomial cirrhosis.
血吸虫病
Schistosomial eggs deposit in small branches of portal vein, resulting in obstruction of blood flow and increase of portal vein pressure.
Primary biliary cirrhosis
原发性胆汁性肝硬化
Chronic active hepatitis
慢性活动性肝炎

16. Schistosomiasis cirrhosis
Schistosomiasis eggs
Schistosomiasis cirrhosis

17. Sinusoidal obstruction
Cirrhosis
Post hepatitic cirrhosis,
肝炎后肝硬化
Alcohol cirrhosis,
酒精性肝硬化
Cryptogenic cirrhosis,
隐匿性肝硬化
Metabolic liver disease (e.g. Wilson's Disease)
代谢性疾病（Wilson’s 病）
Non-cirrhotic
Cytotoxic drugs,
细胞毒性药物
Vitamin A intoxication.
维生素A中毒

18. liver cirrhosis

19. Pseudolobules formation in liver cirrhosis
The regeneration nodules distort the vascular tree, impeding the flow of blood.

20. Post-sinusoidal obstruction
Budd-Chiari syndrome
Hepatic veins or inferior vena cava in hepatic level obstruction included congenital deformity or thrombosis, called Budd-Chiari syndrome.
Veno-occlusive disease

21. Post-hepatic type
Posthepatic obstruction occurs at any level between liver and right heart, including
Hepatic vein thrombosis,
肝静脉血栓形成
IVC thrombosis,
下腔静脉血栓形成
IVC congenital malformation,
下腔静脉先天性畸形
Constrictive pericarditis.
缩窄性心包炎

22. Pathophysiology congestive splenomegaly 充血性脾肿大
ramus communicans dilatation
交通支扩张
ascites 腹水

23. Symptoms and Signs （1） congestive splenomegaly and hypersplenism
Splenomegaly is defined as the spleen size >12cm in length.
Hypersplenism is a type of disorder which causes the spleen to rapidly and prematurely destroy blood cells

24. Splenomegaly

25. Splenomegaly

26. Symptoms and Signs （2） ramus communicans dilatation:
esophageal and gastric veins: varices rupture and gastrointestinal hemorrhage
inferior rectal-anal veins: hemorrhoid & bleeding
anterior abdominal wall veins: paraumbilical varices (caput medusae)
retroperitoneal veins plexus: dilatation & congestion

27. Caput Medusae

28. Esophageal varices

29. Symptoms and Signs （3） Ascites
disordered albumin synthesis and decreased plasma colloid osmotic pressure caused by hepatocellular function damage
increased capillary filter pressure due to increased portal hypertension
lymph liquid leakage into abdominal cavity from surface of the liver because of lymph back-flow obstruction
salt and water retention by aldosterone and antidiuretic hormones deactivation disturbance

30. Ascites

31. Symptoms and Signs（4） Non-specific systemic symptoms: Fatigue 乏力
Lethargy 嗜睡
loss of appetite 纳差

32. Diagnosis and Differential Diagnosis

33. Diagnosis Medical history: hepatitis, schistosomiasis,
alcohol, or drugs?
Clinical presentation: splenomegaly and hypersplenism, hematemesis and melena, ascites
Lab exams and images

34. Laboratory Exams & Images
Blood RT : WBC↓, Plt↓
Liver Function: albumin↓, A/G ratio reversing, prothrombin time↑
Markers of hepatitis B or C
Ultrasound
CT/MRI
Esophageal endoscopy
Esophageal barium swallow
Angiography

35. Images
Ultrasound and Doppler: cirrhosis, splenomegaly, ascites, thrombosis and occlusion of the portal, superior mesenteric and splenic vein, enlargement of portal vein>13mm and of splenic vein>10mm

36. Images
CT scan

37. Images
Esophageal endoscopy:
white, pink, red, cherry red varices

38. Images
Esophageal endoscopy

39. Images esophageal barium swallow
multiple irregular filling defects as “string of beads” or “earthworm”

40. Images
Angiography
Normal
Portal hypertension

41. Differential Diagnosis
 peptic ulcer  gastritis  gastric cancer  biliary tract

42. Treatment Non-surgical treatment during Massive Variceal Bleeding
Management of Ascites
Surgical treatment

43. Evaluation of liver function reserve
What for？
How？

44. Child-Pugh classification of liver function
Child-Pugh classification is a scoring system developed for evaluating surgical risk in patients with cirrhosis.
Child’ grade
1 point
2 points
3 points
Serum bilirubin (umol/L)
<34.2
>51.3
Albumin (g/l)
>35
30-35
<30
Prothrombin (s' prolonged)
1-4
4-6
>6
Ascites
absent
slight
moderate
Encephalopathy
none
none or minimal
coma

45. Child-Pugh classification of liver function
Class A ( low operative risk ): 5 or 6 points
Class B ( moderate risk ): 7 to 9 points
Class C (high risk ): 10 to 15 points

46. Non-surgical treatment for Massive Variceal Bleeding
Anti-shock
Pharmacotherapy and control of bleeding • vasopressin • sandostatin
Beta blockers (propranolol) • general hemostatic drugs
Local treatment • endoscopic variceal sclerosis or banding • hemostatic drugs injection per oral or stomach tube mg% noradrenaline ice saline? • balloon tamponade
TIPS(Transjugular interhepatic portasystemic shunts)

47. endoscopic variceal sclerosis or ligation

48. balloon tamponade
(life-saving procedure)

49. balloon tamponade Sengstaken-Blakemore tube (1950)
esophageal balloon ( ml)
gastric balloon ( ml)
one lumen to gastric balloon
one lumen for gastric aspiration
one lumen to esophageal balloon

50. Attention!
 check the balloons for air leakage before use of the tube  h of placement and 10-20min /12h removal of air  filling the air firstly to the gastric balloon and removing the air firstly from esophageal balloon  observation of the patient’s breath and enhancing the respiratory tract nursing

51. TIPSS Transjugular intrahepatic portasystemic stent-shunt
TIPSS is a small, tubular metal device commonly called a stent that is placed in veins in the middle of the liver to permit blood flow to bypass the liver.
In a TIPSS procedure, interventional radiologists use image guidance to make a tunnel through the liver to connect the portal vein to one of the hepatic veins. A stent is then placed in this tunnel to keep the pathway open.

52. TIPSS
Transjugular intrahepatic portasystemic stent-shunt

53. TIPSS
stent

54. TIPSS Transjugular intrahepatic portasystemic stent-shunt
A TIPSS is used to treat the complications of portal hypertension, including:
variceal bleeding, bleeding from any of the veins that normally drain the stomach, esophagus, or intestines into the liver.
portal gastropathy, an engorgement of the veins in the wall of the stomach, which can cause severe bleeding.
severe ascites (the accumulation of fluid in the abdomen) and/or hydrothorax (in the chest).
Budd-Chiari syndrome, a blockage in one or more veins that carry blood from the liver back to the heart

55. Management of Ascites
 salt restriction  diuretic therapy  paracentesis  peritoneal venous shunt

56. Surgical treatment
◈ splenectomy ◈ portosystemic shunt or bypass ◈ extensive devascularization around the cardia
◈ liver transplantation

57. ◈ reduction of portal blood flow
Splenectomy
◈ reduction of portal blood flow
Because of “Higher volume, Higher pressure”

58. Portosystemic shunt or bypass
Anastomose the portal vein or its main branches (splenic vein and superior mesenteric vein) to vena cava or its main branches (renal vein) by use of operative procedures, and put the hypertensive portal blood flow into the low-pressured inferior vena cava.
To reduce the portal vein pressure and thus decrease the blood flow through collateral venous beds

59. portacaval side-to-side shunt portacaval end-to-side shunt

60. mesocaval shunt
splenorenal shunt

61. Limited Side-to-Side Portacaval Shunt

62. Selective distal splenorenal shunt (Warren’s operation)
During the surgery, the splenic vein is detached from the portal vein and reattached to the left renal vein.

63. Devascularization operations
◈ Disconnection of the venous circulation of the distal esophagus and cardiac from the hypertensive portal circulation by division of all the feeding vessels.
ligation of lower esophageal and gastric variceal veins
disconnection of cardiac portal systemic venous shunt
resection of lower esophagus and gastric fundus esophagogastrostomy

64. Devascularization operations

65. Comparing the shunt with devascularization Shunt devascularization decrease pressure clear, obvious none or increase encephalopathy maybe none hepatic perfusion decrease increase operative procedure difficult simple anastomotic obstruction maybe none

66. Liver transplantation
Liver transplantation is potentially applicable to any acute or chronic condition with irreversible liver dysfunction.
Most liver transplants are performed for chronic liver diseases that lead to irreversible scarring of the liver, or cirrhosis of the liver.

67. Liver transplantation

68. Thanks for your attention!