Portal Hypertension

July 5, 2024

Normal Portal pressure = 5-10 mm hg
Portal HTN = HVPG>5 mm hg ( normally 1-5 mm hg)
HVPG = [Wedge pressure ( pressure at sinusoids) - Free venous pressure]
Clinically Significant PHTN when HVPG is 10 mm hg
Varices start to bleed = 12mm hg
Causes:
- Post hepatic :
  - MC = Budd Chiari
  - Constrictive pericarditis
- Intra hepatic
  - Pre sinusoidal = HVPG will be Normal here
    - NCPF
    - Schistosomiasis
    - Hepatic Fibrosis
  - Sinusoidal
    - only cause is Cirrhosis d/t multiple etiologies
  - Post Sinusoidal:
    - MC = Veno Occlusive disease
- Pre hepatic:
  - EHPVO =HVPG here also will be normal ‘
  - Portal or splenic vein thrombosis
  - AV fistula
HVPG is normal in EHPVO, NCPF ( site of obstruction is pre sinusoidal)

HVPG HELPS IN DIAGNOSIS OF PHTN BY:

	Hepatic Vein Pressure	HVPG	Portal Pressure
Post sinusoidal	Increased	Increased	Increased
Sinusoidal	Increased	Increased	Increased
Pre Sinusoidal	Normal	Normal	Increased
Pre hepatic	Normal	Normal	Increased
- MC cause of :
- PHTN = Cirrhosis
- PHTN in Children = EHPVO
- Intrahepatic Presinusoidal PHTN = Schistosomiasis

VARICEAL BLEED

Management of Acute bleed :
- ABCDE
- Resuscitate = transfusions to maintain Hb just above 7-8 gm/dl
- IV antibiotics (50% chance of infection)
- IV Terlipressin
- IV Somatostatin / Octreotide ( 5days)
- EVL >> EST = within 12 hrs [ EVL but not EST can also be done as primary prophylaxis]
- Non responder
  - Cirrhosis = TIPS > shunt
  - Non cirrhotics [EHPVO / NCPF] = Shunt surgery
- No role of Beta blockers in acute bleed and if the pt has successful EVL = Secondary prophylaxis can then be added [ also used in Primary prophylaxis]
- if there is a very high CTP score then we can also directly go for Upfront TIPPS [Bavano guidelines - for PHTN]
- Algorithm of Prevention of recurrent variceal bleeding:
  
  TIPS FOR CIRRHOTICS SHUNTS FOR EHPVO AND NCPF BRTO FOR BLEEDING GASTRIC VARICES
Prophylaxis of Variceal Bleed:
- EVL / Beta blocker
- No role of TIPS , surgical shunting , EST in prophylaxis of variceal bleed.
Secondary Prophylaxis
- EVL / Beta Blocker
- TIPS can never be used as a prophylactic therapy even in decompensated liver disease with high risk varices = here also we use EVL only
- Risk of Rebleeding is 70% within first 6 weeks
- Beta blockers reduce Risk of rebleeding to 40%
- Beta Blockers combined with nitrates is more effective than beta blocker alone to reduce risk of rebleeding
TIPS:
- TIPS = Side to side Portocaval Shunt = Nonselective
- preferred treatment when endoscopy fails
- Not used as Primary prophylaxis d/t risk of encephalopathy
- Can be used in
  - medical refractory ascites,
  - hydrothorax,
  - gastric varices,
  - Budd chiari sx = RxOC now a days.
- MC early complication : Intraperitoneal H’ge ( Bailey)
- MC late complication: Shunt stenosis ( 50% at 1 year)
- 50% chance of Shunt stenosis > shunt thrombosis
- Rebleed rate is less than endoscopic treatment
- Short term portal decompression
- Bridge to Transplant
- Indications for TIPS:
  - Control of bleeding varices
  - Ectopic Varices
  - Refractory ascites
  - Refractory hepatic hydrothorax
  - Portal vein occlusion = cannulate PV and give anticoagulants
- Disadvantages:
  - Increase Encephalopathy
- In case of ascites with PHTN = usually managed medically = but medical refractory then best shunt is TIPS =but if a surgical shunt has to be done then it should be S-S PC Shunt ( IF we do E-S shunt then the liver will not be decompressed)

SELECTIVE VS NON SELECTIVE SHUNTS VS PARTIALLY SELECTIVE SHUNTS:

Partially selective :
- if graft diameter = 8mm = Sarfeh Shunt = PCS
- Linton’s = PSRS = Splenorenal shunt with Splenectomy
- Rex shunt = Mesenterico-left Portal Bypass = SMV to LPV by passing Main PV
Selective:
- DSRS = Warren’s
  - Preserve spleen
  - Connect splenic vein to Left Renal Vein
  - also cut Left Coronary Vein to decompress GE varices
  - NOT done in ascites
  - Pancreatic siphon = over time converts to Non selective
    - Loss of portal flow by 50% at 1 year
    - specially in pt’s of Alcoholic cirrhosis
  - Equivalent to Non selective shunt for recurrent bleed
  - Lower encephalopathy rates
  - Compared to TIPS = similar rebleed rate/ encephalopathy rate
- Inokuchi shunt = Left coronary vein to IVC

Non Selective shunts:

Portocaval
- S-S PC shunt
- Eck’s Fistula = E-S PC shunt
Mesocaval = SMV - IVC
Mesorenal
DEVASCULARIZATION PROCEDURES
- if there is a Small Splenic vein / damaged splenic vein during sx / non shuntable splenic vein is present then Devascularization procedures are of help
- we devascularise the lower esophagus and stomach and also remove spleen
  1. Hassab:
    1. ligate collaterals from lower (abdominal) esophagus and stomach (cardia)
    2. ligate Left Gastric vein
    3. ligate Left Coronary vein
    4. Splenectomy
    Disadvantage : Pt may have reccurence d/t remaining PHTN
  2. Siguiara: Original - 2 stages = thorax and abdomen ( Sigiuara and Futagawa) Modified = 1 stage = all through abdomen incision ( by Ginsberg and Umeyama)
    1. Ligate the perforating veins of esophagus upto inferior pulmonary vein ( 7-8 cms)
    2. Devascularization of Upper 2/3rd of greater and lesser curve
    3. DID NOT ligate Left Coronary Vein
    4. Esophageal Transection 2 cms above GEJ done and Reanastomosis done with circular stapler
    5. Highly Selective Vagotomy +/- drainage procedures
    6. Splenectomy done

EHPVO vs NCPF

EHPVO	NCPF	Cirrhosis
1st or 2nd decade Child
mean age =19 yrs	Middle aged pt 3rd or 4th decade
Mean age = 28-32 yrs
present with signs of Phtn
Variceal bleed	present with signs of Phtn	present with signs of Phtn
USG =
- Portal Vein thrombosis with involvement of Splenic & mesenteric veins
- Cavernomatous transformation of Portal vein
- Splenomegaly & Hypersplenism = mild -moderate	USG =
- **Dilated Portal Vein
- Withered tree** appearance (cannot see 2nd & 3rd order branches)
-Splenomegaly is disproportionate and massive
No Cirrhosis signs = jaundice, ascites, encephalopathy	No Cirrhosis signs = jaundice, ascites, encephalopathy	Cirrhosis signs = jaundice, ascites, encephalopathy +nt
effects main portal vein
liver is completely normal	Biopsy =
- **Portal Sclerosis of 2nd and 3rd order branches
- Obliterative portovenopathy**
-fibrosis at presinusoidal level in liver
ESSENTIAL FOR DIAGNOSIS
**Growth retardation
Portal Biliopathy in 90-100%**	Autoimmune features +nt

Treatment of EHPVO & NCPF

These conditions are PHTN with Non Cirrhotic livers = therefore Surgical Shunts are preferred over other modalities
Shunts or Devascularization procedures
Shunts:
- Non selective = best shunts d/t global decompression of PV
  - here splenomegaly is also a problem to be addressed; hence PSRS ( linton’s) - BEST
  - Mesocaval Shunt
- Selective :
  - DSRS = warrens can also be done in cases of small spleen
- Rex shunt
  - physiological shunt
  - Mesenterico - left portal bypass
  - Mostly used in cases of EHPVO
  - we should use a graft here and so risk of thrombosis
  - Splenomegaly has to be addressed separately here
Devascularization procedures:
- Hassab
- Siguiara
- Modified Siguiara

PORTAL BILIOPATHY

Definition: abnormality in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. For the Diagnosis to be established, all of the following criteria have to be fulfilled :

presence of portal cavernoma

typical cholangiographic changes in ERCP or MRCP ( table 1)

absence of other causes of these biliary changes like Bile Duct Injury , PSC, Sclerosing Cholangitis, Cholangiocarcinoma etc.

Table 1: Cholangiographic abnormalities of Portal Cavernoma Cholangiopathy

Extrinsic impressions/ indentations

Shallow impressions/ indentations

Irregular ductal contour

Stricture

Filling defects

Bile duct angulation

Upstream dilatation

Ectasia

Exp : primary diagnosis is EHPVO which lead to changes in Biliary system causing strictures which leads to PORTAL BILIOPATHY. this case is symptomatic. but there is no cholangitis

Most cases 70% are Asymptomatic = which needs no treatment
If symptomatic = look for cholangitis
- Cholangitis present = do ERCP + Stenting
- Cholangitis absent = Do any Non Selective Shunt ;
  - PSRS shunt = best = with follow up after 3 months = 30-40% will have resolution of symptoms with shunt alone
  - mesocaval
- We CANNOT do a DSRS shunt in portal biliopathy because it doesnt cause global decompression
- We dont require anything other than shunt procedures like for eg; they always give HJ in the options
- There are 2 kinds of strictures in Portal biliopathy
  - Fibrotic strictures = Sx needed
    - we need to do Surgery but not a upfront hepaticojejunostomy
    - first do shunt surgery and repeat imaging after 3 months and if still there is a fibrotic stricture then do a HJ
  - Non fibrotic strictures = no need for additional procedure

Stages of Portal Cavernoma Cholangiopathy:

Stage	Portal Cavernoma	Cholangiopathy	LIver Biochemistry	Symptoms	Complications	Treatment
Pre clinical	+	-	Normal	Absent	-	-
Asymptomatic	+	early changes	N/ Abnormal	Absent	-	-
Symptomatic	+	Advanced changes	Abnormal	Present	-	Required
Complicated
= Biliary stricture	+	Advanced changes	Abnormal	Present	+	Required

Untitled

Budd Chiari Syndrome

In india , Most Commonly d/t sequale of Infections; eg: Amebic Liver abscess / some other abdominal infections which causes thrombosis of veins
In western = MC is Myeloprofilerative disease ; Polycythemia vera is Overall most common cause of thrombosis and in west also; OC pills
Acute BCS = MC in west = more fulminant
Chronic BCS = MC in east = more of subacute
Membranous IVC obstruction = MC in east
Clinical Triad of Budd chiari :
- Pain
- Hepatomegaly
- Ascites = MC feature
  - Generally jaundice is not a part of triad; but if it is present then there is liver decompensation