Diagnosis

Portal hypertension

Etiology

Cause [y0v][5kp]

1. Prehepatic:

   Portal atresia, portal vein thrombosis, tumor compression, AV-fistula (increased flow)

2. Intrahepatic: Changes in hepatic microcirculation --> structural changes --> fibrosis, regenerative nodules, vascular occlusion, angiogenesis, vasoconstriction

   • Presinusoidal: Schistosomiasis, fibrosis (hemochromatosis, Wilson, congenital), early primary biliary cholangitis, sarcoidosis, chronic active hepatitis, and toxins (vinyl chloride, arsenic, and copper)

   • Sinusoidal: Cirrhosis, alcohol-related hepatitis, vitamin A intoxication, cytotoxic drugs, and advanced primary biliary cholangitis

   • Postsinusoidal: sinusoidal obstruction syndrome due to veno-occlusive disease

3. Posthepatic: Budd-Chiari syndrome, tumor compression of IVC, heart failure (constrictive pericarditis, tricuspid insufficiency, restrictive cardiomyopathy)

4. Idiopathic portal hypertension

Pathophysiology [5kp][y0v]

1. The portal vein pressure is normally between 1 and 4 mm Hg (higher than the hepatic vein pressure)

2. Increased resistance to portal blood flow --> portal hypertension

3. Portosystemic collaterals: caput medusa, hemorrhoids, esophageal varices

4. Sustained portal hypertension result in

   • Decreased systemic blood pressure

   • Reduced effective arterial blood volume.

   • Activation of the renin-angiotensin-aldosterone system --> Na and H2O retention

   • Bacterial translocation from the gut lumen to the systemic circulation

5. Hepatic venous pressure gradient ≥12 mm Hg --> decompensation:

   • Ascites

   • Variceal bleeding

   • Hepatic encephalopathy

Complications [5kp]

• Thrombocytopenia due to hypersplenism

• Variceal bleeding (gastroesophageal, anorectal, retroperitoneal)

• Anemia

• Ascites

• Spontaneous bacterial peritonitis

• Hepatic hydrothorax

• Hepatorenal syndrome

• Hepatic encephalopathy

• Hepatopulmonary syndrome

• Portopulmonary hypertension

• Cirrhotic cardiomyopathy

Epidemiology

Incidence per 100.000 [vcd][sgc][ids][f5r]

Approach

• Blood tests: WBC, liver function tests (ASAT, ALAT, ALT, ALP, PT-INR, APTT, albumin, bilirubin, creatinine, electrolytes, antibodies (ANA, AMA, ASMA), iron, ceruloplasmin, Alpha1-antitrypsin deficiency

• Arterial blood gas

• Ultrasound abdomen

• Liver biopsy

• CT abdomen

• Visceral angiography

• Hepatic venous pressure gradient (HVPG)

• Esophagogastroduodenoscopy

Treatment

1. Identify and address the underlying etiology [5kp]

   • Thrombosis --> anticoagulation

   • Hepatitis C infection --> antiviral therapy

2. Medical treatment: [ccf][y0v]

   • Splanchnic vasoconstrictors:

     • Vasopressin (IV)

     • Somatostatin (IV): Octreotide, Vasopressin, Terlipressin

     • Nonselective beta-adrenergic blockers: propranolol or nadolol (oral)

       β1-blockade (reduction of cardiac output)

       β2-blockade (splanchnic vasoconstriction)

   • Analogues of nitric oxide (NO): nitrates and simvastatin

   • Block adrenergic activity: prazosin and clonidine

   • Block angiotensin: captopril, losartan, and irbesartan (induce intrahepatic vasodilatation)

3. Local therapy: risk of recurrence --> endoscopic surveillance

   • Esophageal variceal ligation (endoscopic elastic bands)

   • Variceal sclerotherapy (endoscopic)

   • Balloon tamponade

   • Expandable esophageal stents

   • Percutaneous transhepatic embolization

4. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

5. Surgery: severe cirrhosis/esophageal varices and/or liver failure

   • Portosystemic shunts

   • Liver transplantation

Differential diagnoses

Acute liver failure, Ascites, Budd-Chiari syndrome, Cirrhosis, Congenital atresia, Congenital fibrosis, Hemochromatosis, Leukemia, Lymphoreticular cancer, Pericarditis, Polycystic kidney disease, Portal vein thrombosis, Sarcoidosis, Schistosomiasis, Splenomegaly, Tricuspid regurgitation, Tuberculosis, Vitamin deficiency, Wilson's disease