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

What do we know about 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.

Introduction of Portal Hypertension

Then, What's Portal Hypertension?

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.

Pathophysiology

• The normal pressure of portal vein:

5-10mmHg (13~24 cmH2O)

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.

Ducts and vessles of the liver

Tow concepts:

Glisson’s sheath

Lobule of the liver

Liver lobule

Portal triad

Central v.

Sinus

Four ramus communicans betweenportal and systemic circulations

esophageal and gastric veins

inferior rectal-anal veins

anterior abdominal wall veinsretroperitoneal venous plexus

• Portal hypertension should be divided into three types as its blood flow obstructed positions.

Etiology & Types

Types

pre-hepatic

intra-hepaticpre-sinusoidal

Sinusoidal

post-sinusoidal

post-hepatic

• 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

Pre-hepatic type

• About 95% patients belong to this type of portal hypertension

• A classification by pathology:pre-sinusoidal obstructions

sinusoidal obstructions

post-sinusoidal obstructions

Intra-hepatic type

• 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

Pre-sinusoidal obstruction

Schistosomiasis cirrhosis

Schistosomiasis eggs

• 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

Sinusoidal obstruction

liver cirrhosis

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

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

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

Pathophysiology

congestive splenomegaly

充血性脾肿大 ramus communicans dilatation

交通支扩张 ascites

腹水

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

Splenomegaly

Splenomegaly

Symptoms and Signs （ 2）

• ramus communicans dilatation:– esophageal and gastric veins: varices rupture and g

astrointestinal hemorrhage – inferior rectal-anal veins: hemorrhoid & bleeding– anterior abdominal wall veins: paraumbilical

varices (caput medusae)– retroperitoneal veins plexus: dilatation & congestio

n

Caput Medusae

Esophageal varices

Symptoms and Signs （ 3）

• Ascites– disordered albumin synthesis and decreased plas

ma 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

Ascites

Symptoms and Signs （ 4）• Non-specific systemic symptoms:

– Fatigue

乏力– Lethargy

嗜睡– loss of appetite

纳差

Diagnosis and Differential Diagnosis

Diagnosis

• Medical history: hepatitis, schistosomiasis,

alcohol, or drugs?

• Clinical presentation: splenomegaly and hypersplenism, hematemesis and melena, ascites

• Lab exams and images

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

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

Images

• CT scan

Images

• Esophageal endoscopy:

white, pink, red, cherry red varices

Images

• Esophageal endoscopy

Images

• esophageal barium swallow

multiple irregular filling defects as “string of beads” or “earthworm”

Images

Normal Portal hypertension

• Angiography

Differential Diagnosis

• peptic ulcer gastritis gastric cancer biliary tract

Treatment

• Non-surgical treatment during Massive Vari

ceal Bleeding

• Management of Ascites

• Surgical treatment

Evaluation of liver function reserve

What for ？

How ？

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 34.2-51.3 >51.3

Albumin (g/l) >35 30-35 <30

Prothrombin (s' prolonged)

1-4 4-6 >6

Ascites absent slight moderate

Encephalopathy nonenone or minimal

coma

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

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 8mg% noradrenaline ice saline? • balloon tamponade

• TIPS(Transjugular interhepatic portasystemic shunts)

endoscopic variceal sclerosis or ligation

balloon tamponade

(life-saving procedure)

balloon tamponade

Sengstaken-Blakemore tube(1950)

esophageal balloon (100-150ml) gastric balloon (150-200ml) one lumen to gastric balloon one lumen for gastric aspiration one lumen to esophageal balloon

Attention!

check the balloons for air leakage before use of the

tube

24-72 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

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.

TIPSS

Transjugular intrahepatic portasystemic stent-shunt

TIPSS

stent

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

Management of Ascites

salt restriction diuretic therapy paracentesis peritoneal venous shunt

Surgical treatment

◈ splenectomy portosystemic shunt or bypass◈ extensive devascularization around ◈

the cardia ◈ liver transplantation

Splenectomy

◈ reduction of portal blood flowBecause of “Higher volume, Higher pressure”

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

portacaval end-to-side shunt portacaval side-to-side shunt

mesocaval shunt splenorenal shunt

Limited Side-to-Side Portacaval Shunt

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.

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 veinsdisconnection of cardiac portal systemic venous shuntresection of lower esophagus and gastric fundus esophagogastrostomy

Devascularization operations

Comparing the shunt with devascularization

Shunt devascularization

decrease pressure clear, obvious none or increaseencephalopathy maybe nonehepatic perfusion decrease increaseoperative procedure difficult simpleanastomotic obstruction maybe none

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.

Liver transplantation