Circulatory disorders (END)

Question 1

What is the effect of hepatic artery inflow interruption?

Answer 1

Rarely leads to ischemic necrosis due to dual blood supply from the hepatic artery and portal vein.
Exception: Hepatic artery thrombosis in a transplanted liver can cause organ loss.

Question 2

What happens if there is thrombosis or compression of an intrahepatic branch of the hepatic artery?

Answer 2

Leads to a localized parenchymal infarct.

Question 3

What are the causes of extra-hepatic portal vein obstruction?

Answer 3

Peritoneal Sepsis:
Conditions like acute diverticulitis or appendicitis causing pylephlebitis in the splanchnic circulation.
Pancreatitis:
Leading to splenic vein thrombosis & subsequent portal vein thrombosis.
Thrombogenic diseases & post-surgical thrombosis.
Vascular invasion by primary or secondary cancer in the liver.
Cirrhosis:
Reduces portal vein blood flow, predisposing to thrombosis.

Question 4

What are the clinical features of portal vein (or major branch) occlusion?

Answer 4

Abdominal pain.
Ascites.
Esophageal varices.
Acute visceral blood flow impairment, leading to congestion and bowel infarction.

Question 5

What are the effects of acute intra-hepatic thrombosis of a portal vein branch?

Answer 5

Causes an infarct of Zahn, characterized by:
Sharply demarcated area of red-blue discoloration.
No necrosis but hepatocellular atrophy & sinusoidal congestion.

Question 6

What is hepato-portal sclerosis?

Answer 6

A chronic, idiopathic (possibly autoimmune) condition involving progressive portal tract sclerosis, impairing portal vein inflow.

Question 7

Causes of Passive congestion & centrilobular necrosis

Answer 7

Systemic circulatory disorders:
Right-sided cardiac Decompensation → Passive Congestion of the Liver
Long-standing cases → Centri-Lobular necrosis and perivenular fibrosis in the necrotic areas

Question 8

Lab findings of Passive congestion & centrilobular necrosis

Answer 8

Mild elevation of serum transaminase levels – Hyper-bilirubinaemia & elevated alkaline phosphatase (some cases)

Question 9

What are the macroscopic features of the liver in right-sided cardiac failure?

Answer 9

Slightly enlarged liver.
Tense and cyanotic appearance.

Question 10

What are the microscopic findings in right-sided cardiac failure?

Answer 10

Congestion of centri-lobular sinusoids.
Atrophic centri-lobular hepatocytes with markedly attenuated liver cell cords.

Question 11

What is “Cardiac Sclerosis” or “Cardiac Cirrhosis”?

Answer 11

Chronic severe congestive heart failure causes centri-lobular fibrosis.
Rarely forms bridging fibrous septa and cirrhosis.

Question 12

What liver changes occur in left-sided cardiac failure?

Answer 12

Hepatic hypo-perfusion and hypoxia lead to:
Ischemic necrosis of centri-lobular hepatocytes and bile ducts.

Question 13

What results from combined left-sided hypo-perfusion and right-sided retrograde congestion?

Answer 13

Centri-lobular hemorrhagic necrosis.

Question 14

What is the macroscopic appearance of the liver in centri-lobular hemorrhagic necrosis?

Answer 14

Variegated, mottled appearance.
“Nutmeg liver” pattern: Centri-lobular areas of hemorrhage and necrosis alternate with pale mid-zonal areas.

Question 15

What is the synonym for Hepatic Vein Thrombosis?

Answer 15

Budd-Chiari Syndrome.

Question 16

What are common causes or associations with Budd-Chiari Syndrome?

Answer 16

Polycythaemia Vera.
Pregnancy.
Use of oral contraceptives.
Paroxysmal Nocturnal Haemoglobinuria.
Hepatocellular Carcinoma.
Idiopathic in 10% of cases.

Question 17

What are the clinical features of Budd-Chiari Syndrome?

Answer 17

Hepatomegaly.
Ascites.
Abdominal pain.

Question 18

What are the macroscopic features of the liver in Budd-Chiari Syndrome?

Answer 18

Swollen and red-purple liver.
Tense organ capsule.

Question 19

What are the microscopic findings in Budd-Chiari Syndrome?

Answer 19

Severe centri-lobular congestion and necrosis.
Centri-lobular fibrosis in slow-developing thrombosis.
Completely or incompletely occlusive fresh thrombi in the major vein lumen.
Organized, adherent thrombi in chronic cases.

Question 20

How is Budd-Chiari Syndrome treated?

Answer 20

Surgical creation of a porto-systemic venous shunt.
Angiography for direct dilation of inferior vena cava obstruction.

Question 21

What is the prognosis for Budd-Chiari Syndrome?

Answer 21

Untreated acute cases: High mortality.
Chronic cases: Far less severe, with >2/3 survival at 5 years.

Question 22

What was the former name for Sinusoidal Obstruction Syndrome?

Answer 22

Veno-Occlusive Disease.

Question 23

What are the common causes of Sinusoidal Obstruction Syndrome?

Answer 23

Chemotherapeutic agents (e.g., Cyclophosphamide, Actinomycin D, Mithramycin).
Total body radiation (used in pre- or post-transplantation regimens).

Question 24

What is the pathogenesis of Sinusoidal Obstruction Syndrome?

Answer 24

Toxic injury to sinusoidal endothelium leads to:
Sloughing of damaged endothelial cells → Thrombus formation → Obstruction of sinusoidal flow.
Erythrocyte leakage into the space of Disse.
Proliferation of stellate cells and fibrosis of terminal hepatic vein branches.

Question 25

When does Sinusoidal Obstruction Syndrome typically present?

Answer 25

Within the first 20 to 30 days after bone marrow transplantation (occurs in ~20% of recipients).

Question 26

How does the clinical presentation of Sinusoidal Obstruction Syndrome compare to other conditions?

Answer 26

Resembles Budd-Chiari Syndrome and can range from mild to severe forms

Question 27

What is the prognosis for severe cases of Sinusoidal Obstruction Syndrome?

Answer 27

Severe cases without resolution after three months of treatment can be fatal.

Question 28

Hepatic insufficiency progressing within 2–3 weeks (from onset of symptoms to hepatic encephalopathy) in persons without chronic liver disease.

Answer 28

Fulminant Hepatic Failure

Question 29

What are the main causes of Fulminant Hepatic Failure?

Answer 29

Viral Hepatitis (12%): HBV (8%) and HAV (4%).
Unknown etiology in 15% of cases.

Question 30

What are the macroscopic features of Fulminant Hepatic Failure?

Answer 30

Liver shrinkage due to massive loss of mass (500–700 g).
Limp, red organ with a wrinkled, large capsule.
Necrotic areas have a muddy red, mushy appearance with hemorrhage.

Question 31

What are the microscopic findings in Fulminant Hepatic Failure?

Answer 31

Complete destruction of hepatocytes in neighboring lobules.
Collapsed reticulin framework with preserved portal tracts.
Minimal inflammatory reaction (except in cases of survival, where inflammatory cells accumulate).

Question 32

How does the disease progress if survival exceeds one week?

Answer 32

Prolonged disease can lead to fibrous scarring and eventual cirrhosis.

Question 33

What is the primary treatment option for Fulminant Hepatic Failure?

Answer 33

Liver transplantation

Question 34

What is the mortality rate for Fulminant Hepatic Failure?

Answer 34

Without liver transplantation: ~80%.
With liver transplantation: ~35%.