Portal Hypertension

Definition

pressure gradiant between PV and IVC of more than 10-12 mm Hg
- if >10mm Hg - varices
- if >12 mm Hg variceal bleed and ascites
- if 6-12 mm Hg - subclinical PHTN
Intrasplenic pressure >17 mm Hg
Hepatic vein pressure gradient > 4 mm Hg

gastrointestinal bleeding
- well compensated in EHPVO unlike variceal bleed due to cirrhosis

portal vein is formed by SMV and spleenic vein
spleenic vein is formed by IMV
varices - abnormal connection between systemic and portal circulation
develop when pressure is high in portal system to decompress it
sites (most to least common)
- lower esophagus
- stomach
- rectum
- peribiliary
- retroperitoneal
- bare area of liver
prone to bleeding due to their postition and exposure to food and acid
- in stomach this can cause vascular ectasia - congestive gastropathy
sites around the splenorenal and retroperitoneal cause more compression symptoms

development of portal cavernoma in the absence of associated liver disease
obstruction of extrahepatic portal vein with or without the involvement of the intrahepatic portal vein
MC in india of PHTN

pulmonary arterial pressure greater than 25 mm Hg at rest or left ventricular end diastolic pressure less than 15 mm Hg
presents with
- clubbing
- cyanosis
- dyspnea
- spider nevi
histopathology
- laminar intimal fibrosis of pulmonary artery

h/o umblical sepsis, UVC, recurrent diarrhoes, trauma, abdominal surgery
h/o chronic liver disease lincluding maternal history of Hep B, hep c,
h/o drug abuse and tattooing
h/o consangunity, family history, history of liver disease, wilson disease
h/o recurrent jaundice, high coloured urine, pale stool, ictching and steatorrhea

small liver, splenomegaly without ascites and anterior abdominal veins - EHPVO
firm liver, splenomegaly, ant. abdominal vein, with ascites - Cirrhosis
dysmorphic facies - Alagille syndrome
Pruritogenic skin marks, thick hyperpigmented skin over extremities - cholestasis
children, firm liver well appreciated below xiphisternum with moderate splenomegaly - congenital hepatic fibrosis
similar presentation in young adult - NCPF
tender hepatomegaly, ascites, dialated abdominal wall veins and presence of veins over the back - budd chiary syndrome
elevated jugular vein pulsation, tender hepatomegaly - cardiac cirrhosis

CBC
- low Hb due to blood loss or impared absorbtion due to PHTN
- pancytopenia or bicytopenia due to hypersplenism
LFT
- normal in Non cirrhotic
- reversal of albumin globulin ration indicated chronic liver disease
USG
- size of liver
- surface of liver
- collaterals
  - doppler can demonstrate the flow of blood
  - reversal of portovenous flow (heptaofugal) has been assocaited with varices
- spleen
- spleenomegaly, omental thickening and splenorenal collaterals - chronic portal hypertension
- altered or increased liver echoes, spleenomegaly, dialted portal vein with or without ascites - Cirrhosis
- presence of hepatic venous outlow tract obstruction iwth or without IVC obstruction - BCS
- liver cyst, renal cyst and colletrals - congenital heptic fibrosis
- biliary dialtion with similar features - cholangitic CHF
- altered liver echoes and dialted hepatic veins
Upper GI endoscopy

Grading of varices

CECT
- not required routinely
- selective arteriography of specifuc vessels
- required in liver transplant
- advantage of displaying varices and parenchymal hepatic abnormalities
MRCP
- done in chidren with portal bilopathy, cholangitic type of portal bilopathy, cholangitic type of portal vein hypertension
- to also look for vascular anomaly
prothrombotic workup
- in familial cases
serum ceruloplasmin
KF ring
24 hour urinary copper
Viral markers
serum bile acids
GGT
Cholestrol
liver biopsy
- autoimmune, wilsons

emergency treatment
- fluid resusitation
- PRBC transfusion
- avoid over transfusion - 7 to 9 mg/dl is a reasonable goal for hemoglobin
- PPI
- Vitamin K to stop bleeding
pharmacological
- vasopressin - 0.33 units/kg over 20 mins then followed by same dose hourly maintainance
- octreotide - 1 mcg/kg as slow bolus or 1-5 mcg/kg/h
- terlipressin
endoscopic
- ligation (preferred)
  - application of tight band around the varices to cut off the blood flow
  - less session needed
  - less complications
- sclerotherapy (can be done in smaller children when banding is not possible)
  - inject sclerosing agent into varices
  - require multiple session
  - control 80-90% bleed
balloon tamponade
primary prophylaxis
- before first bleeding
- proponolol - 20 mg BD - watch for bradycardia
- endoscopic variceal oblitration - every 2-4 weeks
secondary prophylaxis
- after first bleeding
- proponalol and nadalol
- 1-3 mg/kg/day
- MOA - block beta 2 receptor and splanchnic vasoconstricton
Surgery
- recurrent variceal bleed, failed endotheraphy
- decompressive shunt surgery
- splectomy - hyperslenism is not a indication
  - indication - profound thrombocytopenia
  - repeated infection
  - physical discomfort caused by massive splenomegaly
- orthotopic liver transplant

percutaneously created connection between liver and system
less invasive
indication
- acute variceal bleed not controlled medically
- recurrent variceal bleed
- refractory ascites
- portal decompression in budd chiari
contraindication
- right sided heart disease
- polycystic liver disease
- severe liver failure (relative)
complications
- obstruction of shunt
- vascular injury
- biliary injury
- dislocation of shunt
- gi bleed
- massive spleenomegaly
- ascites
- hepatopulmonary syndrome
- portopulmonary syndrome
- growth failure
- portal biliopathy

compression of bile duct due to varices around the common bile duct resulting in ischemia
lead to strictures, stone in bile duct, obstructive jaundice, cholangitis
treatment
- shunting
- endoscopic sphincterotomy