PORTAL HYPERTENSION Anatomy Definition Aetiology Pathophysiology Clinical presentation Treatment - What to treat ? - How to treat ? - When to treat ? considering the indication for surgical treatment, the questions are: WHAT - HOW - WHEN What must we or what can we treat Which techniques and When should we propose surgery?

1- Portal Venous System - ANATOMY V cave V mes sup V splen

2- Portal Hypertension - DEFINITION GRADIENT = “Raised Pressure” in Portal Venous System ! Reference = “pressure gradient” PV pressure – IVC pressure NL = + 2 to + 8 mmHg AbNL = + 8 to … 40 mmHg V cave V mes sup V splen

F R Flow x Resistance = Pressure 3- ETIOLOGY

3- ETIOLOGY a Banti syndrome Arterio-portal fistula Portal Hypertension related to Increased portal flow Banti syndrome splenomegaly, normal liver, portal hypertension Arterio-portal fistula arterio-portal vascular malformation splanchnic arterio-venous fistula To make a long story short THERE ARE TWO MAIN SITUATIONS EITHER There is a block BUT the liver is normal OR The liver is abnormal and THIS ABNORMAL LIVER is the cause of portal hypertension The next slide give some details

Portal hypertension caused by a Venous block 3- ETIOLOGY b Portal hypertension caused by a Venous block Sinusoids Post-sinusoidal Sinusoidal Pre-sinusoidal (Pre-Hepatic)

Portal hypertension caused by a Venous block 3- ETIOLOGY b Portal hypertension caused by a Venous block NORMAL LIVER HEPATIC VEIN OBSTRUCTION VENO-OCCLUSIVE DISEASE CIRRHOSIS CHOLESTASIS METABOLIC DISEASE HEPATITIS NORMAL LIVER “FIBROSE” HEPATIQUE PORTAL VEIN OBSTRUCTION Post-sinusoidal Sinusoidal Pre-sinusoidal (Pre-Hepatic)

4- PATHO-PHYSIOLOGY of CIRRHOSIS Liver cirrhosis  Resistance + Hyperdynamic circulation + Circulating vasodilators = Flow Flow x Resistance = Pressure

1- Effects of Raised Pressure 2- +/- Liver Dysfunction 5- CLINICAL SYMPTOMS 1- Effects of Raised Pressure 2- +/- Liver Dysfunction (variable according etiology and evolution) What are the consequences or complications associated with portal hypertension. It is important now to remember that alteration of liver function and portal hypertension are often associated. Thus a patient can present with symptoms which reflects EITHER one problem predominantly -- OR both problems , and this in a combinaison with variable proportions. The liver dysfunction can be related to the primary disease -for example the cirrhosis - or it can also be a secondary problem. The quality of the liver function is a VERY IMPORTANT parameter for the medical decision of choosing one or another treatment. But this is another story. Lets concentrate only on the direct effects of the portal hypertension

Effects of Raised Pressure I . 5- CLINICAL SYMPTOMS Effects of Raised Pressure I . Splenomegaly - Hypersplenism Venous collaterals development Oesophageal varices Hypertensive Gastropathy Hypertensive Enteropathy Hemorroids GI bleeding Chronic anaemia

Liver Dysfunction . 5- CLINICAL SYMPTOMS Jaundice Associated symptoms Stellar angiomas, Cruveilhier-Baumgarten Arthropathy… Coagulopathy Hypo-albuminemia Hyper-gammaglobulinemia Hyper-ammonemia …

6- DIAGNOSTIC IMAGING Doppler ultrasonography Oeso-gastroscopy Sus-hepatic catheterism (pressure gradient) Selective celio-mesenteric arteriography (portal angiography) RMN +/- angiography

Echo doppler ultrasound 6- DIAGNOSTIC IMAGING Echo doppler ultrasound Liver structure and anatomy - ? anomalies ? Splenomegaly, ? Ascites ? Varices (omentum / aorta ratio < 1.7 ) ? Portal flow - hepatopedal (10 - 30 cm/sec) - stagnant flow / thrombosis - hepatofugal … Hepatic artery : resistivity index (RI) Bile ducts

- Sclerotherapy / banding - Shunt surgery / TIPPS - Other surgery 7- MANAGEMENT - Wait & see - B Blockers - Sclerotherapy / banding - Shunt surgery / TIPPS - Other surgery - Liver Transplantation

7- MANAGEMENT First question = WHAT do you want TO TREAT ? Sequence of events Clinical problems Management alternatives Liver cirrhosis PRIMARY DISEASE Liver transplant Flow Resistance RAISED PRESSURE B-Blockers, Shunt Varices Splenomegaly RELATED PROBLEMS Alternative surgery COMPLICATIONS Bleeding Ascites Banding, Diuretics

To Take into account - ? Liver disease - ? Liver function 7- MANAGEMENT To Take into account - ? Liver disease - ? Liver function - ? Complications - ? Prognosis

7- MANAGEMENT Wait & see Compensated liver disease No complications yet Minor/moderate risk of bleeding Wait & see

7- MANAGEMENT Medical treatment B-Blockers Diuretics ? TIPPS Compensated liver disease Previous bleeding, banding Ascites Medical treatment B-Blockers Diuretics ? TIPPS ? Shunt surgery

7- MANAGEMENT Transplant End-stage liver disease Recurrent varices & bleeding Refractory ascites Transplant

Sites: oesophageal varices duodenal ulcer ectopic varices - MANAGEMENT of Gastro-Intestinal Bleeding Sites: oesophageal varices duodenal ulcer ectopic varices hypertensive gastropathy

Emergency Therapy of Variceal Hemorrhage Gastro-Intestinal Bleeding Emergency Therapy of Variceal Hemorrhage 1. Fluid resuscitation 2. Platelet transfusion (if Platelet count< 50.000/mm3) 3. Vit. K / Fresh frozen plasma 4. Oesogastroscopy (diagnostic + variceal obliteration) 5. Pharmacologic intervention - Somatostatin - Vasopressin 6. Rescue therapy - Balloon compression - TIPSS - Shunt surgery

Gastro-Intestinal Bleeding Sclerotherapy/Banding Limits: - ectopic varices - recurrence Complications of sclerotherapy ulcer stricture mediastinitis Oes. dysmotility GE reflux

Place of Surgery ? 7- MANAGEMENT What is the place of surgery. Remember that by “ conventional surgery” I mean the Surgical TECHNIQUES FOR TREATING PORTAL HYPERTENSION All but liver transplantation Of course liver transplantation is also surgery but it must be considered separately BECAUSE IT IS MUCH MORE THAN ONLY TREATING PORTAL HYPERTENSION

Portal Hypertension Management A- Non-Transplant Surgery Comparison of techniques Disconnection Splenectomy X Shunt Effect on Portal pressure -- . + Oesoph. Varices + . + Hypersplenism -- + +

- Direct bypass Porto-caval SHUNT SURGERY - types - Direct bypass Porto-caval Spleno-renal distal Spleno-renal proximal - H type bypass Porto-caval Meso-caval Spleno-renal atypical derivations

Shunt surgery RISKS ? SHUNT THROMBOSIS LIVER DECOMPENSATION ENCEPHALOPATHY What are the most important reported risks related to shunt surgery There is a risk of shunt thrombosis but this risk is low. If the shunt thromboses, then portal hypertension recurs. As we have seen, the creation of a shunt reroutes the blood away from the liver and deprives partly the liver of the portal flow and this can lead to liver failure mostly in patient with advanced cirrhosis. This is avoided by adequate selection of the candidates Re-routing the blood flow directly to the heart also carries some risk of encephalopathy and other problems at long term.

Well compensated cirrhosis SHUNT SURGERY - Limited Indications Well compensated cirrhosis Bleeding ectopic varices Unsuccessful sclerotherapy/banding Untractable ascites Non-cirrhotic portal hypertension Inadequate local medical support Refractory/recurrent bleeding

If a Shunt is indicated consider TIPPS …

TIPSS - Indications Unsuccessful sclerotherapy/banding Refractory ascites Bleeding ectopic varices Preparation to transplant

Liver Transplant B- Transplant Surgery Sequence of events Clinical problems Liver Transplant Liver cirrhosis PRIMARY DISEASE Flow Resistance RAISED PRESSURE Cures the primary disease, thus all related complications as well Varices Splenomegaly RELATED PROBLEMS COMPLICATIONS Bleeding Ascites

C- Other Primary Disease Management Normal liver + Pre-hepatic portal hypertension Arterio-venous fistula  embolise or surgery Portal vein thrombosis  meso-rex bypass or shunt

Indication = Portal vein thrombosis meso-rex bypass Indication = Portal vein thrombosis Cavernoma

Portal vein thrombosis / cavernoma Normal hepatic parenchyma Portal cavernoma Portal hypertension Splenomégaly Hypersplenism Oesophageal varices Spontaneous shunts Liver atrophy ! Malabsorption Cholestasis Coagulopathy Subclinical encephalopathy Hepato-pulmonary problems IVC SMV Splenic vein

Portal vein thrombosis / cavernoma Normal hepatic parenchyma Portal cavernoma Portal hypertension Splenomégaly Hypersplenism Oesophageal varices Spontaneous shunts Liver atrophy ! Malabsorption Cholestasis Coagulopathy Subclinical encephalopathy Hepato-pulmonary problems

Portal vein thrombosis / cavernoma meso-rex bypass Bypass