Portal Hypertension

Meet the patient

A 54-year-old man with known alcohol-related cirrhosis is brought to ED at 2am having vomited bright red blood onto the bedroom floor — three bowlfuls, his wife says. He is pale, sweaty, heart rate 112, blood pressure 88 over 52, and a spider naevus sits on his chest.[1][2]

Two questions now decide the next two hours: is this a variceal bleed? and can I keep him alive until the endoscopist arrives? Everything below exists to answer those two questions at consultant depth. Hold them, and the four numbers, the five anastomoses, and the acute-bleed bundle all fall into place.[1]

The HVPG ladder — "five defines, ten threatens, twelve bleeds, sixteen kills"

Portal hypertension is a pressure gradient, and four numbers on that gradient decide everything that follows. The gradient is the hepatic venous pressure gradient (HVPG): the wedged (balloon-occluded) hepatic venous pressure, which reads sinusoidal pressure, minus the free hepatic venous pressure, which reads intra-abdominal IVC pressure. Normal is 3 to 5 mmHg.[1]

The mantra to carry into the viva: over 5 defines portal hypertension, over 10 is clinically significant (varices and ascites appear), over 12 is the bleeding threshold, over 16 kills. A patient who drops to under 12 mmHg, or by over 20 percent, on a non-selective beta-blocker is a responder — and responders rebleed rarely.[1]

The portal vein forms behind the neck of the pancreas from the superior mesenteric vein (draining the midgut) and the splenic vein (draining the spleen and foregut via the left gastric and short gastric veins). It is valveless and sits between two capillary beds — splanchnic and hepatic sinusoid — so any obstruction, before or within or after the liver, transmits pressure backwards and prises open the embryonic portosystemic anastomoses. Portal flow carries roughly 75 percent of hepatic inflow; the hepatic artery supplies the remaining 25 percent.[1]

Where the pressure escapes — the five portosystemic anastomoses (OPERA)

A raised portal pressure decompresses through five named sites, and the first one kills patients. These are the embryonic portosystemic channels that re-open when the portal vein cannot drain — and the oesophago-gastric junction is the lethal one.[1]

The five sites, each a portocaval meeting point:[1]

1. Oesophago-gastric junction — left gastric (coronary) vein (portal) meets the oesophageal veins draining to the azygos and hemi-azygos (systemic). These dilate into oesophageal and gastric varices — the commonest source of life-threatening haemorrhage.
2. Rectum and anal canal — superior rectal vein (portal, via inferior mesenteric) meets middle and inferior rectal veins (systemic, internal iliac), producing rectal varices.
3. Periumbilical region — paraumbilical veins in the falciform ligament (portal) meet superficial epigastric and thoraco-epigastric veins (systemic), producing caput medusae and the audible Cruveilhier-Baumgarten murmur.
4. Retroperitoneum — veins of the second part of the duodenum, pancreas, and bare areas dilate; typically silent but bleed at surgery.
5. Bare areas of the liver — diaphragmatic and retroperitoneal venous communications.[1]

[1]

The classic trap: rectal varices are not haemorrhoids. Varices lie above the dentate line, are portosystemic collaterals, and bleed back into the portal system. Treating them as piles misses the portal hypertension driving them.[1]

Three sites of obstruction — pre, intra, post

The level of the block predicts whether liver synthetic function is preserved — and that single fact changes the whole management. Pre-hepatic and pre-sinusoidal obstruction leaves hepatocytes intact; sinusoidal cirrhosis and post-hepatic outflow obstruction do not.[1]

[1]

The discriminator line: a cirrhotic patient with a variceal bleed has abnormal liver function; a schistosomal or portal-vein-thrombosis patient with the same bleed often has perfectly normal albumin and INR. That single split tells you whether transplant is ever on the table — it is not, when the liver is structurally preserved.[1]

Who gets it — cirrhosis in the West, schistosomiasis in the tropics

In most of the world portal hypertension is a complication of cirrhosis, and cirrhosis is a top-ten killer of the middle-aged. The global aetiological burden is shifting: alcohol dominates in Europe and the Americas; chronic hepatitis B drives disease in sub-Saharan Africa, East and South-East Asia; hepatitis C is declining where direct-acting antivirals reach patients but persists where they do not; and metabolic dysfunction-associated steatohepatitis (MASH, formerly NASH) tied to obesity and type 2 diabetes is the fastest-rising cause in the West.[1]

Once cirrhosis is established, varices develop at roughly 6 to 8 percent per year, and about one-third of patients with varices will bleed. Without secondary prophylaxis, 60 percent of survivors rebleed within a year.[1]

[1]

The two-hit mechanism — resistance up, inflow up

Portal pressure follows Ohm's law: pressure equals flow multiplied by resistance. Cirrhosis raises both. That dual hit is why a single drug rarely controls it and why beta-blockers — which cut inflow — are the backbone of prophylaxis.[1]

Hit one — intrahepatic resistance rises. The structural component is obvious: fibrous septa, regenerative nodules, and sinusoidal capillarisation physically distort the vascular bed. But there is also a dynamic, reversible component — injured hepatocytes and sinusoidal endothelial cells activate hepatic stellate cells, which trans-differentiate into contractile myofibroblasts wrapped around the sinusoids. These constrict in response to endothelin-1, angiotensin II, and thromboxane, and relax to nitric oxide. In cirrhosis the intrahepatic NO balance falls, so the sinusoid constricts. This dynamic element is therapeutically important — it is why drugs that lower intrahepatic resistance (carvedilol, statins in trial) have a target.[1]

Hit two — splanchnic inflow rises. As portal pressure climbs, the splanchnic circulation paradoxically vasodilates, driven by excess nitric oxide, glucagon, and prostacyclin from a hyperdynamic splanchnic endothelium. More blood arrives at a higher-resistance liver, amplifying the hypertension and producing the hyperdynamic circulation of cirrhosis — bounding pulse, warm peripheries, low systemic vascular resistance, high cardiac output — that ultimately decompensates into cardiomyopathy and hepatorenal failure. This is the rationale for non-selective beta-blockers: beta-2 blockade leaves unopposed alpha-mediated splanchnic vasoconstriction, cutting inflow.[1]

Variceal rupture follows Laplace's law. Wall tension is proportional to transmural pressure multiplied by radius, divided by wall thickness. A varix therefore ruptures when portal pressure is high, the varix is large, and its wall is thin — the basis of endoscopic grading by size and the red wale signs and cherry-red spots that mark wall thinning. It is also why band ligation works: it obliterates the thin-walled varix and replaces it with a fibrotic nodule.[1]

Four complications declare the disease

Portal hypertension declares itself through four complications, overlaid on the stigmata of chronic liver disease when the cause is cirrhosis. Learn the four as a cluster: varices, ascites, splenomegaly, encephalopathy.[1]

[1] [1]

When the cause is cirrhosis, the examination also reveals the stigmata of hepatocellular failure: palmar erythema, spider naevi (in the superior vena cava distribution), gynaecomastia and testicular atrophy from impaired oestrogen metabolism, parotid enlargement, Dupuytren contracture, jaundice, fetor hepaticus, and asterixis. Caput medusae — a crown of dilated periumbilical veins radiating from a recanalised umbilical vein — with a venous hum and thrill over the umbilicus is the Cruveilhier-Baumgarten sign, virtually pathognomonic of portal hypertension.[1]

Examiners test the corners. Consider portal hypertension in a child with painless haematemesis (extrahepatic portal vein obstruction), in a young woman with sudden hepatomegaly and ascites (Budd-Chiari, often post-partum or in a myeloproliferative disorder), in a patient with a known pancreatic mass and isolated gastric varices (splenic vein thrombosis), and in chronic constrictive pericarditis presenting with ascites out of proportion to leg oedema. A patient who bleeds from varices with entirely normal liver synthetic function should prompt a search for a pre-sinusoidal (schistosomiasis) or pre-hepatic (portal vein thrombosis) cause.[1]

The differential — what else bleeds, what else swells

The acute presentation is upper GI haemorrhage; the chronic is ascites with splenomegaly. Both generate wide differentials, and varices are one cause of each. The table below is the cornerstone of the SAQ.[1]

| Condition | Key distinguishing feature |[1] |---|---| | Oesophageal or gastric varices | Known chronic liver disease; massive painless haematemesis; signs of portal HTN; SAAG over 11 g per litre if ascites present | | Peptic ulcer (gastric or duodenal) | Epigastric pain, melaena more than haematemesis, normal liver function, NSAID or H. pylori history | | Mallory-Weiss tear | Post-emesis haematemesis after initial non-bloody vomit; normal liver usually | | Gastric or oesophageal malignancy | Weight loss, dysphagia, anaemia; mass on endoscopy | | Aorto-enteric fistula | Prior aortic graft, sentinel bleed then catastrophic haematemesis; urgent CT angiography | | Dieulafoy lesion or angiodysplasia | Painless bleed, normal liver, diagnosis at endoscopy | | Budd-Chiari syndrome | Acute painful hepatomegaly, ascites, tender liver; hepatic vein thrombosis on imaging | | Right heart failure or constrictive pericarditis | Raised JVP, hepatomegaly, ascites; echocardiography diagnostic | | Intra-abdominal malignancy with peritoneal deposits | SAAG under 11 g per litre (exudate), cytology positive, fixed pelvic mass |

[1]

The discriminator line: SAAG over 11 g per litre means the ascites is portal hypertensive; under 11 g per litre means it is peritoneal — malignancy or TB — and chasing portal hypertension is the wrong road.[1]

The bedside round — look for the four, smell the liver

Bedside examination in suspected portal hypertension rarely provides one diagnostic sign; its job is to map the four complications and the stigmata of the underlying cause. Begin with a focused general examination: pallor, jaundice, scratch marks, spider naevi, palmar erythema, gynaecomastia, testicular atrophy, parotid enlargement, Dupuytren contracture, clubbing. Smell for fetor hepaticus — sweet, musty, mercaptan breath. In any bleeder, assess hydration and the signs of shock: tachycardia, hypotension, cold peripheries, oliguria, confusion.[1]

Abdominal examination looks for hepatosplenomegaly, ascites (shifting dullness, fluid thrill), caput medusae, and superficial abdominal wall collaterals. A venous hum over the umbilicus (Cruveilhier-Baumgarten) is pathognomonic. Test for asterixis (flapping tremor with wrists dorsiflexed) and constructional apraxia (Reitan trail, clock drawing) for encephalopathy. A digital rectal examination may reveal melaena, and anoscopy distinguishes true rectal varices (above the dentate line, blanching bluish) from haemorrhoids.[1]

Bedside pearl: what does a platelet count of 70 with splenomegaly tell you? (answer)

In a cirrhotic patient, thrombocytopaenia under 150 with splenomegaly is a robust non-invasive marker of clinically significant portal hypertension (CSPH) — Baveno VII endorses platelet count plus liver stiffness as a rule-out test for high-risk varices, sparing endoscopy when both are reassuring.[2]

Investigations — confirm, locate, grade

Investigation serves three purposes: confirm portal hypertension, define its cause and site, and grade severity for prognosis and transplant listing. Run them in that order and you will not miss a step.[1]

Bloods. A full blood count shows thrombocytopaenia (hypersplenism), anaemia (blood loss, alcohol, folate deficiency, haemodilution), and leucopaenia. Liver function tests show a cholestatic or hepatocellular pattern depending on aetiology. Coagulation (INR) and albumin gauge synthetic function and feed the Child-Pugh and MELD scores. Renal function and electrolytes screen for hepatorenal syndrome and hyponatraemia. Send viral serology (HBsAg, anti-HCV), autoimmune and metabolic markers (ferritin, caeruloplasmin, alpha-1-antitrypsin, immunoglobulins, anti-mitochondrial antibody), and alpha-fetoprotein for hepatocellular carcinoma surveillance.[1]

Endoscopy (OGD). The cornerstone for diagnosis and therapy. Varices are graded by size (small, medium, large) and inspected for red signs (red wale marks, cherry-red spots, haematocystic spots) that predict bleeding. Gastric varices are classified by Sarin (GOV1, GOV2, IGV1, IGV2). At the same session, band ligation or glue injection delivers therapy.[1]

Ultrasound with Doppler is first-line imaging. It shows liver nodularity and echotexture, portal vein patency and flow direction (hepatofugal — away from the liver — in advanced disease), splenomegaly, ascites, and screens for hepatocellular carcinoma. Portal vein thrombosis appears as an echogenic clot or absence of flow with cavernous transformation when chronic. Splenic vein patency should be checked specifically when isolated gastric varices are found.[1]

Cross-sectional imaging — CT and MR portography, contrast-enhanced ultrasound. These define vascular anatomy, confirm thrombosis, plan TIPSS or surgery, screen for hepatocellular carcinoma, and quantify collaterals. MR elastography and transient elastography (FibroScan) estimate liver stiffness, which — combined with platelet count — stratifies the probability of CSPH and high-risk varices non-invasively, per Baveno VII.[2]

HVPG is the gold standard for diagnosing and grading portal hypertension. A balloon-tipped catheter is passed under fluoroscopy via the internal jugular vein to a hepatic vein; the wedged pressure reflects sinusoidal pressure and the free pressure the IVC. The difference is the HVPG. It is reproducible and prognostic and is the metric by which beta-blocker response is judged — but it is invasive and requires expertise, so it is reserved for diagnostic uncertainty, research, and assessing treatment response in specialised centres.[1]

Severity scores — Child-Pugh and MELD

[2]

Child-Pugh combines bilirubin, albumin, INR, encephalopathy, and ascites to give a 5 to 15 score (A, B, or C). MELD (Model for End-stage Liver Disease) uses bilirubin, INR, and creatinine (plus sodium in MELD-Na); it predicts three-month mortality and drives transplant allocation — a MELD over 15 generally warrants transplant assessment.[2]

The acute bleed bundle — ABC-TAB

An acute variceal bleed is a medical emergency with a fixed bundle, started before endoscopy and continued through it. The four pillars — airway, restrictive transfusion, vasoactive drug, and antibiotic — are delivered simultaneously, not sequentially, alongside plans for urgent endoscopy and a defined rescue pathway.[1][2]

[1] [1]

Two principles are examiner-favourite traps. First, transfusion is restrictive — a target haemoglobin of 70 to 80 g per litre, because over-transfusion raises central venous and portal pressure, worsens bleeding, and impairs coagulation. Second, antibiotics are mandatory in every variceal bleed, independent of aspiration risk or ascites: infection accompanies up to half of bleeds, drives rebleeding, and short-term ceftriaxone reduces mortality by roughly a third.[2]

The vasoactive drug. Terlipressin, a synthetic vasopressin analogue, is the preferred agent in most of the world: 2 mg IV every four hours for the first 48 hours, then 1 mg every four hours, discontinued by day five. It causes splanchnic vasoconstriction and reduces portal inflow. A systematic review and meta-analysis of vasoactive agents found no difference between terlipressin or vasopressin versus octreotide or somatostatin in mortality, bleeding control, rebleeding, transfusion, or length of stay — though the terlipressin group had more adverse events (ischaemia, hyponatraemia). Octreotide (50 microgram bolus then 50 microgram per hour infusion) or somatostatin are acceptable alternatives where terlipressin is contraindicated (ischaemic heart disease, peripheral vascular disease).[4]

Refractory bleeding — the rescue ladder

If bleeding continues despite vasoactive drug and endoscopic therapy, escalate in this fixed order. Do not improvise — each rung has a defined role and a defined time limit.[1]

1. Balloon tamponade (Sengstaken-Blakemore or Minnesota tube, or the self-expanding Cook tube). The gastric balloon is inflated to tamponade the varix at the gastro-oesophageal junction. Use only as a bridge for a maximum of 24 hours while arranging definitive treatment; risks include aspiration, oesophageal ulceration and perforation, and pressure necrosis. Endotracheal protection should be in place.
2. Self-expanding covered oesophageal metal stent — an alternative bridge that is easier to place and carries a lower perforation risk.
3. Transjugular intrahepatic portosystemic shunt (TIPSS) — the definitive rescue therapy, decompressing the portal system by stenting a tract between the portal and hepatic veins.[1]

The classic trap: the Sengstaken-Blakemore tube is a bridge, not a treatment. Leave it in beyond 24 hours and you trade a bleeding varix for an oesophageal perforation. The tube buys you time to reach TIPSS — nothing more.[1]

Pre-emptive TIPSS — the 72-hour fork for high-risk bleeders

For the highest-risk patients, TIPSS is not rescue — it is the plan from hour one. In a cirrhotic bleeder who is Child-Pugh B with active bleeding at endoscopy, or Child-Pugh C scoring 10 to 13, a TIPSS placed within 72 hours of admission reduces treatment failure, rebleeding, and mortality.[2]

This recommendation rests on the landmark Early TIPS trial (Garcia-Pagan, NEJM 2010): 63 high-risk patients randomised within 24 hours to covered-stent TIPSS within 72 hours versus pharmacotherapy plus EVL. Early TIPSS gave a 97 percent versus 50 percent one-year probability of remaining free of the composite endpoint (failure to control bleeding or rebleeding) and improved one-year survival (86 percent versus 61 percent), without excess serious adverse events.[3]

Consultant confession: the hardest part of this decision is not the evidence — it is recognising, at 3am, that the patient in front of you qualifies. Calculate Child-Pugh on admission, before the bleed distorts the labs, and flag the high-risk patient for pre-emptive TIPSS the moment endoscopy confirms active bleeding.[3]

Prophylaxis — stop the first bleed, stop the next one

Primary prophylaxis — prevent the first bleed

All patients with cirrhosis should be screened for varices. The aim of primary prophylaxis is to prevent the first variceal haemorrhage in those found to have medium or large varices, or small varices with red signs or Child-Pugh B or C disease. Baveno VII endorses a non-invasive rule-out — liver stiffness under 25 kPa and platelet count over 150 — to spare endoscopy in low-risk patients.[2]

Non-selective beta-blocker (NSBB). Propranolol 20 to 40 mg twice daily, titrated to reduce resting heart rate by about 25 percent to 55 to 60 bpm; nadolol 40 mg once daily (longer half-life, less CNS penetration); or carvedilol 6.25 mg once daily (additive alpha-1 blockade, greater portal-pressure reduction — now the preferred agent where tolerated). Mechanism: beta-2 blockade removes splanchnic vasodilatory tone, leaving unopposed alpha-mediated vasoconstriction, which cuts portal inflow and pressure; carvedilol also lowers intrahepatic resistance. Side effects: fatigue, hypotension, bronchospasm, erectile dysfunction; contraindicated in asthma, second-degree heart block, bradycardia.[1]

Endoscopic variceal ligation (EVL) is first-line for large varices, or where NSBB are contraindicated or not tolerated. Bands are applied at two- to eight-week intervals until variceal eradication (typically two to four sessions).[1]

Secondary prophylaxis — prevent rebleed

Every patient who survives a variceal bleed needs secondary prophylaxis — without it, 60 percent rebleed within a year. The evidence-based standard is combination therapy: NSBB plus serial EVL, which is superior to either modality alone.[2]

TIPSS, shunts, and transplant — the decompressive ladder

TIPSS places a covered stent between an intrahepatic portal vein branch and a hepatic vein under fluoroscopic guidance via the internal jugular vein, decompressing the portal system. It controls refractory bleeding, refractory ascites, hepatic hydrothorax, and Budd-Chiari syndrome, and is the bridge to transplant.[1]

It controls bleeding in over 90 percent but causes new or worsening encephalopathy in 30 to 50 percent — by shunting nitrogenous toxins straight to the systemic circulation. Stenosis or thrombosis requires surveillance Doppler. Contraindications include severe heart failure, severe liver failure, polycystic liver disease, uncontrolled encephalopathy, and active sepsis.[1]

Surgical shunts are now rarely used — supplanted by TIPSS, which is less invasive and carries lower peri-procedural mortality. They are reserved for failed TIPSS, TIPSS not technically feasible, or specialised centres with surgical expertise:[1]

• Distal splenorenal (Warren) shunt — selectively decompresses gastro-oesophageal varices into the left renal vein while preserving mesenteric-portal perfusion; lowest encephalopathy rate of the surgical shunts.
• Mesocaval shunt — jugular or prosthetic graft between SMV and IVC.
• Portocaval shunt — side-to-side or end-to-side; the most effective decompression but the highest encephalopathy rate.[1]

Devascularisation procedures (oesophageal transection, Sugiura-Futagawa) are historical salvage options now virtually obsolete in centres with TIPSS.[1]

Liver transplant is the only definitive cure for portal hypertension arising from cirrhosis. Reserved for decompensated cirrhosis (Child-Pugh C, MELD over 15, or a decompensating event such as refractory ascites, recurrent encephalopathy, or hepatorenal syndrome). Allocation is by MELD or MELD-Na score. Portal hypertension from pre-hepatic or pre-sinusoidal causes (portal vein thrombosis, schistosomiasis) does not require transplant — the liver is structurally preserved.[1]

Ascites and encephalopathy — the stepwise ladders

Ascites is managed in a stepwise ladder keyed to severity. Do not jump to paracentesis first — the ladder exists because each rung has a cost and a failure mode.[2]

1. Sodium restriction to under 88 mmol (2 g) per day, and fluid restriction only if hyponatraemic (sodium under 120 to 125).
2. Diuretics — spironolactone plus furosemide in a 100:40 ratio (e.g. spironolactone 100 mg and furosemide 40 mg each morning), titrated to a maximum of 400 mg and 160 mg respectively, targeting 0.5 kg per day weight loss (up to 1 kg per day if peripheral oedema). The ratio maintains normokalaemia.
3. Large-volume paracentesis (LVP) for tense or refractory ascites — drain over 5 litres in one sitting, with 6 to 8 g of intravenous albumin per litre removed if over 5 litres, to prevent post-paracentesis circulatory dysfunction and hepatorenal syndrome.
4. TIPSS for refractory ascites (diuretic-resistant or diuretic-intractable) in selected patients.
5. Liver transplant for refractory ascites as a decompensating event.[1]

Hepatic encephalopathy combines precipitant removal with ammonia-lowering therapy. Most episodes resolve when you find and fix the trigger — so hunt for it first.[2]

1. Identify and treat the precipitant — infection (including SBP), gastrointestinal bleeding, constipation, dehydration, electrolyte disturbance (hypokalaemia), sedatives or opioids, TIPSS, and worsening liver failure. This alone resolves many episodes.
2. Lactulose 15 to 30 mL orally two to three times daily, titrated to two to three soft stools per day. The disaccharide is fermented by colonic bacteria to organic acids that lower colonic pH, convert absorbable ammonia to non-absorbable ammonium, and have a cathartic effect.
3. Rifaximin 550 mg twice daily — a minimally absorbed antibiotic that reduces ammonia-producing gut flora; add to lactulose for recurrent or persistent encephalopathy and for secondary prevention.
4. Supportive care — airway protection in grade III to IV encephalopathy, nutrition with adequate protein (do not restrict protein), correction of electrolytes.[1]

The classic trap: do not restrict protein in hepatic encephalopathy. Protein restriction is obsolete and worsens sarcopenia — and sarcopenia is itself a mortality predictor in cirrhosis. Treat the precipitant, lower the ammonia, feed the patient.[1]

The complications that kill — SBP, HRS, encephalopathy

Variceal bleeding carries 15 to 25 percent six-week mortality per episode, even with modern therapy. Sixty percent rebleed within a year without secondary prophylaxis. Bleeding also precipitates encephalopathy, infection, and hepatorenal syndrome — so the post-bleed patient is never "stable", only "between complications".[1]

Spontaneous bacterial peritonitis (SBP) is infection of ascitic fluid without an obvious source. Presents with fever, abdominal pain, worsening encephalopathy, or simply deterioration. Diagnostic tap: an ascitic polymorphonuclear count over 250 cells per cubic millimetre defines SBP. Treat with cefotaxime 2 g IV every eight hours (or ceftriaxone 2 g IV daily) for five to seven days, with albumin 1.5 g per kg on day one and 1 g per kg on day three to prevent hepatorenal syndrome. Secondary prophylaxis with oral norfloxacin 400 mg daily or ciprofloxacin 500 mg daily; primary prophylaxis is given to high-risk groups (low ascitic protein, prior SBP, acute GI bleed).[1]

Hepatorenal syndrome (HRS-AKI) is functional renal failure from intense splanchnic vasodilation reducing effective circulating volume and renal perfusion; the kidneys are histologically normal. Diagnosed by the ICA criteria (rise in creatinine, no response to albumin challenge, exclusion of other causes). HRS-AKI (formerly type 1) is rapid and fatal untreated; treated with terlipressin plus albumin (or noradrenaline in ICU). HRS with refractory ascites (formerly type 2) is indolent. Definitive treatment is liver transplant.[1]

Other complications to name. Hepatopulmonary syndrome (intrapulmonary shunting, hypoxaemia, orthodeoxia) and portopulmonary hypertension (pulmonary arterial hypertension from portosystemic shunting) are pulmonary vascular complications. Hepatic hydrothorax (usually right-sided) and umbilical hernia with risk of rupture are mechanical complications of ascites. Gastric antral vascular ectasia and portal hypertensive gastropathy cause chronic occult bleeding.[1]

The six classic pitfalls

• Over-transfusing a variceal bleed — raises portal pressure and worsens bleeding.
• Forgetting antibiotics — a mortality-reducing, evidence-mandated component of the bundle.
• Mistaking rectal varices for haemorrhoids — varices lie above the dentate line, are portosystemic collaterals, and bleed into the portal system.
• Misattributing ascites with a low SAAG to portal hypertension — a SAAG under 11 g per litre is peritoneal (malignancy, TB), not portal.
• Delaying TIPSS in a high-risk Child-Pugh B or C bleeder who qualifies for pre-emptive TIPSS within 72 hours.
• Restricting protein in encephalopathy — protein restriction is obsolete and worsens sarcopenia; treat the precipitant instead.[1]

Special situations — the corners examiners test

Portal vein thrombosis (pre-hepatic). Acute PVT presents with abdominal pain or is found incidentally; anticoagulation (low-molecular-weight heparin transitioning to warfarin or a direct oral anticoagulant for at least six months) aims for recanalisation and prevents extension. Chronic thrombosis produces cavernous transformation (a tangle of periportal collaterals) and portal hypertension with varices but normal liver synthetic function; management focuses on variceal prophylaxis and eradication. Anticoagulation is safe and beneficial even in cirrhotic PVT when varices are adequately treated.[1]

Budd-Chiari syndrome (post-hepatic). Hepatic vein or IVC thrombosis, classically in a young woman with a myeloproliferative disorder (JAK2 mutation), on oral contraceptives, or post-partum. The classic triad is abdominal pain, hepatomegaly, and ascites. Diagnosis is by Doppler and CT or MR venography showing hepatic vein occlusion. Treatment is anticoagulation, angioplasty or stenting of short stenoses, TIPSS as the default decompressive procedure, and liver transplant for fulminant or end-stage disease.[1]

Splenic vein thrombosis (sinistral, left-sided portal hypertension). Usually a complication of pancreatitis or pancreatic cancer. Isolated gastric (fundal) varices develop with normal liver function. Splenectomy is curative.[1]

Gastric varices bleed less often but more severely than oesophageal varices and are harder to band. GOV2 and IGV1 (fundal varices) are treated with cyanoacrylate (glue) injection as first-line endoscopic therapy; TIPSS is the preferred rescue and is used earlier than for oesophageal varices. Balloon-occluded retrograde transvenous obliteration (BRTO) is an alternative where there is a spontaneous gastrorenal shunt.[1]

Children (extrahepatic portal vein obstruction) — the commonest cause of portal hypertension in children, usually from neonatal portal vein thrombosis (cannulation, sepsis). Liver function is normal; presentation is with variceal bleeding, often well tolerated. Managed conservatively (EVL, beta-blockers). The Meso-Rex bypass (mesenteric-to-left-portal-vein autologous jugular vein graft) restores physiological portal inflow and is the definitive treatment for refractory bleeding in expert centres.[1]

Pregnancy. Portal hypertension in pregnancy carries a higher risk of variceal bleeding because of increased blood volume and intra-abdominal pressure. Varices should be screened and eradicated before conception where possible. Terlipressin is avoided (uterine vasospasm); octreotide or endoscopic therapy are preferred in pregnancy. TIPSS and surgery are deferred where possible. Vaginal delivery is acceptable with good pain control; caesarean is for obstetric indications.[1]

The anticoagulated patient. Anticoagulation is not contraindicated in cirrhosis once varices are treated; portal vein thrombosis on anticoagulation should be managed jointly with hepatology. Reverse warfarin with vitamin K and prothrombin complex concentrate for an acute bleed; direct oral anticoagulants have specific reversal strategies.[1]

The elderly and comorbid. Frail, elderly patients tolerate bleeds poorly; beta-blocker dosing should start low and titrate slowly; pre-emptive TIPSS decisions weigh encephalopathy risk against bleeding risk.[1]

Prognosis and disposition

Variceal bleeding carries a six-week mortality of 15 to 25 percent per episode in modern series, lower with adherence to the full bundle (restrictive transfusion, vasoactive drug, antibiotic, EVL, and pre-emptive TIPSS where indicated). Survivors need structured secondary prophylaxis and surveillance endoscopy.[1]

Cirrhosis by Child-Pugh class (approximate one- and two-year survival): A 100 percent then 85 percent; B 80 percent then 60 percent; C 45 percent then 35 percent. MELD predicts three-month mortality more precisely and drives transplant allocation.[2]

TIPSS controls bleeding in over 90 percent and refractory ascites in the majority; one-year survival is 60 to 80 percent, limited by the underlying liver disease. Thirty to 50 percent develop encephalopathy.[1]

Pre-hepatic and pre-sinusoidal causes (portal vein thrombosis, schistosomiasis, congenital hepatic fibrosis) carry a far better prognosis because hepatocellular function is preserved; patients tolerate bleeds well and live normal spans with variceal control.[1]

Disposition. Every acute variceal bleed is admitted to a unit with 24-hour endoscopy and interventional radiology. High-risk patients (Child-Pugh B active bleed, Child-Pugh C 10 to 13) are flagged early for pre-emptive TIPSS. Survivors are enrolled in a surveillance and secondary prophylaxis programme, with transplant referral when MELD exceeds 15 or a decompensating event occurs.[1]

Evidence, guidelines, and regional deltas

Baveno VII consensus (2022) is the current international standard for portal hypertension — "Personalised Care for Portal Hypertension" — superseding Baveno VI. Key recommendations: CSPH is defined non-invasively by liver stiffness over 25 kPa (rule-in) or under 15 kPa with platelets over 150 (rule-out); NSBBs (preferably carvedilol) are first-line for CSPH; EVL for large varices or NSBB intolerance; the acute bleed bundle includes vasoactive drug, antibiotic, and EVL with pre-emptive TIPSS for high-risk patients; secondary prophylaxis combines NSBB and EVL; and aetiological cure (antivirals, alcohol abstinence, weight loss) can reverse portal hypertension in early disease.[2]

The Early TIPS trial (Garcia-Pagan, NEJM 2010). The pivotal randomised trial establishing pre-emptive TIPSS: 63 high-risk cirrhotic patients (Child-Pugh C under 14, or Child-Pugh B with active bleeding) randomised within 24 hours to covered-stent TIPSS within 72 hours versus pharmacotherapy plus EVL. Early TIPSS gave 97 percent versus 50 percent one-year freedom from failure-to-control-bleeding-or-rebleeding and improved one-year survival (86 percent versus 61 percent), without excess serious adverse events.[3]

Vasoactive agent meta-analysis (Huaringa-Marcelo 2021). Twenty-one RCTs comparing terlipressin or vasopressin with octreotide or somatostatin found no difference in mortality, bleeding control, rebleeding, transfusion, or length of stay; the terlipressin group had more adverse events. The choice of vasoactive agent is therefore often driven by availability, cost, and contraindications rather than efficacy.[4]

The mantra

Over 5 defines, over 10 threatens, over 12 bleeds, over 16 kills. The four numbers, the five anastomoses (OPERA), the four complications (varices, ascites, splenomegaly, encephalopathy), and the acute-bleed bundle (ABC-TAB) — restrictive transfusion, terlipressin, mandatory antibiotics, EVL within 12 hours — are the spine of every portal-hypertension answer. Hold them, and the rest is unpacking.[1][2]

Ward-round test

Stem 1 — the man from the top of the topic (answer)

The 54-year-old cirrhotic who vomited three bowlfuls of blood at 2am. Heart rate 112, blood pressure 88 over 52. What do you do in the next 30 minutes? Model: This is an acute variceal bleed until proven otherwise. Run the ABC-TAB bundle simultaneously: two large-bore cannulae and crossmatch 4 to 6 units with a restrictive transfusion target of Hb 70 to 80 g per litre (over-transfusion raises portal pressure); terlipressin 2 mg IV every 4 hours for splanchnic vasoconstriction; ceftriaxone 1 g IV daily — mandatory, reduces mortality by about a third; and call the endoscopist for EVL within 12 hours. Calculate his Child-Pugh now — if he is Child-Pugh B with active bleed at OGD or Child-Pugh C 10 to 13, flag him for pre-emptive TIPSS within 72 hours.[1][2]

Stem 2 — the well-looking patient who bled from varices with normal liver function (answer)

A 28-year-old man from a schistosomiasis-endemic region presents with painless haematemesis. Endoscopy shows oesophageal varices. His albumin, INR, and bilirubin are entirely normal. What is the mechanism, and what does this tell you about transplant? Model: This is pre-sinusoidal portal hypertension from schistosomiasis — Schistosoma mansoni or japonicum eggs embolise to the pre-sinusoidal portal venules, where granulomatous reaction obstructs flow before the sinusoid. Hepatocytes are intact, so synthetic function is preserved. Treat the parasite with praziquantel 40 mg per kg and manage varices as for cirrhotic portal hypertension. Transplant is never indicated — the liver is structurally normal.[1]

Stem 3 — the ascites with SAAG under 11 (answer)

A cirrhotic patient has tense ascites. The diagnostic tap returns fluid with a SAAG of 8 g per litre. The registrar attributes it to portal hypertension. What is the error? Model: A SAAG under 11 g per litre is peritoneal, not portal. Portal hypertensive ascites has a SAAG over 11 g per litre because sinusoidal hypertension pushes a protein-rich transudate. A low-SAAG ascites means malignancy or tuberculosis peritonitis until proven otherwise — send cytology and culture, and stop chasing portal pressure as the cause of the fluid. The classic trap is misattributing low-SAAG ascites to portal hypertension and missing the peritoneal disease.[1]

Stem 4 — the post-TIPSS confusion (answer)

Three days after a TIPSS for refractory ascites, a 60-year-old cirrhotic becomes drowsy and develops a flapping tremor. What happened, and what do you do? Model: This is post-TIPSS hepatic encephalopathy — 30 to 50 percent incidence, because the shunt delivers nitrogenous toxins straight to the systemic circulation. Hunt for a precipitant (infection, constipation, sedatives), start lactulose 15 to 30 mL two to three times daily titrated to two to three soft stools, and add rifaximin 550 mg twice daily. Most episodes resolve with medical therapy; recurrent or refractory encephalopathy post-TIPSS may require shunt reduction. Do not restrict protein — feed the patient.[1][2]

References

1. [1]Brzdęk M, Dobrowolska K, Janczura J, et al. Advances in portal hypertension management: Evolution of the Baveno guidelines World J Gastroenterol, 2025.PMID 41024757
2. [2]de Franchis R, Bosch J, Garcia-Tsao G, Reiberger T, Ripoll C; Baveno VII Faculty. Baveno VII - Renewing consensus in portal hypertension J Hepatol, 2022.PMID 35120736
3. [3]García-Pagán JC, Caca K, Bureau C, et al. Early use of TIPS in patients with cirrhosis and variceal bleeding N Engl J Med, 2010.PMID 20573925
4. [4]Huaringa-Marcelo J, Huaman MR, Brañez-Condorena A, et al. Vasoactive Agents for the Management of Acute Variceal Bleeding: A Systematic Review and Meta-analysis J Gastrointestin Liver Dis, 2021.PMID 33723542