Portal Hypertension — Clinical Case

DCE Long Case

Patient brief (provided to trainee)

Patient: Mr David Nguyen, 58 years old, retired carpenter. [1]

Presenting complaint: Acute haematemesis (approximately 500 mL of fresh blood) and melaena this morning, followed by presyncope. Brought by ambulance. [1]

Past history:

• Alcohol-related cirrhosis diagnosed 3 years ago — current alcohol intake 60 grams/day (reduced from 100 g/day), has not engaged with alcohol services
• Large oesophageal varices (last OGD 6 months ago) — on carvedilol 12.5 mg OD for primary prophylaxis
• Mild ascites — on spironolactone 100 mg OD
• Hypertension, diet-controlled type 2 diabetes
• No prior hepatic encephalopathy, no known HCC [1]

Current medications:

• Carvedilol 12.5 mg OD
• Spironolactone 100 mg OD
• Thiamine 100 mg OD [1]

Examination findings (trainee elicits):

• Conscious, alert but pale, cool peripheries, capillary refill 4 seconds
• BP 86/52, HR 118 (regular), RR 22, SpO2 96 percent on room air, temp 36.8
• Spider naevi on anterior chest wall, palmar erythema, parotid enlargement, bilateral Dupuytren contracture, no gynaecomastia
• Abdomen: distended with shifting dullness (moderate ascites), spleen palpable 3 cm below left costal margin, liver edge not palpable. No caput medusae visible. Bowel sounds present.
• Rectal examination: melaena
• No asterixis, no obvious encephalopathy
• Bilateral pitting oedema to mid-shin [1]

Investigations:

• Hb 58 g/L (baseline 108 from 2 months ago), platelets 72, INR 1.7
• Na 131, K 4.2, creatinine 95 (baseline 80), urea 12.5
• Bilirubin 65, ALT 45, ALP 140, albumin 26
• Glucose 7.2
• Lactate 2.8
• Blood group O positive, cross-match requested, 4 units [1]

Candidate's structured presentation (model)

Opening statement: [1]

"Mr Nguyen is a 58-year-old retired carpenter who presents with an acute upper gastrointestinal haemorrhage — haematemesis and melaena with presyncope — in the context of known alcohol-related cirrhosis and large oesophageal varices. He is currently on carvedilol for primary prophylaxis of variceal bleeding. [1]

His main problems are:

1. Acute variceal haemorrhage with hypovolaemic shock — medical emergency
2. Decompensated alcohol-related cirrhosis — Child-Pugh B (score 9), MELD-Na approximately 19
3. High-risk bleeder (known large varices, Child-Pugh B) — consider pre-emptive TIPS
4. SBP risk requiring prophylactic antibiotics
5. Hepatorenal syndrome risk — monitor creatinine closely
6. Alcohol dependence — withdrawal prophylaxis and thiamine required
7. Malnutrition and sarcopenia
8. Liver transplant candidacy assessment needed [1]

My immediate priorities are: resuscitate with restrictive transfusion to haemoglobin 70 to 80, commence vasoactive therapy (terlipressin or octreotide) and prophylactic ceftriaxone immediately, proceed to urgent endoscopy within 12 hours for band ligation, assess for pre-emptive TIPS eligibility as he is a high-risk bleeder, and simultaneously plan for alcohol withdrawal prophylaxis, HRS surveillance, and transplant referral." [1]

Integrated management plan

1. Resuscitation and acute bleeding management: [1]

• Airway protection: consider intubation if ongoing haematemesis or reduced conscious state
• Restrictive transfusion: target haemoglobin 70 to 80 g/L (he is at 58 — transfuse 2 units of packed red cells). Avoid over-transfusion — raises portal pressure and worsens rebleeding
• Vasoactive therapy: terlipressin 2 mg IV every 4 hours (or octreotide 50 mcg bolus then 50 mcg/hour infusion). Start immediately, before endoscopy. Continue for 2 to 5 days
• Prophylactic antibiotics: ceftriaxone 1 g IV daily for 7 days — reduces mortality, not just infection. Essential in all cirrhotic patients with GI bleeding
• Urgent endoscopy within 12 hours: band ligation for oesophageal varices. If bleeding uncontrolled, balloon tamponade (Sengstaken-Blakemore) as bridge to TIPS
• Assess for pre-emptive TIPS: he is Child-Pugh B with known active variceal bleeding. Per Garcia-Pagan (PMID 20573925), patients with Child-Pugh C (10 to 13) or Child-Pugh B with active bleeding benefit from early TIPS within 72 hours — reduces treatment failure from 50 percent to 3 percent, improves one-year survival from 61 percent to 86 percent. I would discuss urgently with interventional radiology and hepatology [1]

2. Hepatorenal syndrome surveillance: [1]

• His creatinine is 95 (baseline 80) — not yet AKI but at high risk. Monitor creatinine every 12 to 24 hours
• If creatinine rises above 106 (meeting ICA-AKI criteria): stop diuretics, give albumin 1 g/kg/day for 2 days, then assess for HRS-AKI. Treat with terlipressin plus albumin if criteria met (CONFIRM trial, PMID 33657294)
• Check for SBP with diagnostic paracentesis — send PMN count, culture, albumin (SAAG) [1]

3. Alcohol withdrawal prophylaxis: [1]

• CIWA-Ar monitoring
• Diazepam or lorazepam protocol for withdrawal (hepatically metabolised agents at reduced doses; lorazepam preferred in significant liver dysfunction due to glucuronidation pathway)
• Continue thiamine 100 mg IV daily for 3 to 5 days to prevent Wernicke encephalopathy — give before any glucose
• Folate supplementation [1]

4. Longer-term management and transplant assessment: [1]

• Once bleeding controlled: secondary prophylaxis with NSBB plus serial EVL
• Liver transplant referral — calculate MELD-Na (currently approximately 19), assess abstinence (typically 6 months required), psychosocial evaluation
• Alcohol liaison team involvement — the single most important prognostic intervention for alcohol-related cirrhosis
• HCC surveillance: liver ultrasound plus AFP every 6 months
• Vaccinations: hepatitis A and B (if non-immune), influenza, pneumococcal, COVID-19
• Nutritional support: high-protein diet (1.2 to 1.5 g/kg/day), avoid fasting; dietician referral [1]

Examiner discussion questions

Q: "He is on carvedilol for primary prophylaxis and still bled. Does this mean the beta-blocker failed?" [1]

"Not necessarily — NSBB reduce but do not eliminate bleeding risk. Carvedilol reduces the risk of first variceal bleed by approximately 50 percent, but patients can still bleed, particularly if they have large varices with red signs or if the HVPG is not reduced below 12 mmHg (the bleeding threshold). The fact that he bled despite NSBB means he now requires secondary prophylaxis, which is combination therapy with NSBB plus serial EVL — this is more effective than either alone. I would also verify adherence and whether his carvedilol dose was adequate — 12.5 mg daily is a reasonable dose, but some patients require dose titration based on heart rate response. Once the acute episode resolves, I would recommence NSBB (continuing carvedilol or switching to propranolol or nadolol) combined with serial EVL every 2 to 4 weeks until variceal eradication." [1]

Q: "How would you decide between terlipressin and octreotide as the vasoactive agent?" [1]

"Terlipressin is the preferred agent where available. It is a synthetic vasopressin analogue with greater evidence for efficacy in both acute variceal bleeding and hepatorenal syndrome. Meta-analyses show terlipressin reduces all-cause mortality compared to placebo in acute variceal bleeding, while octreotide has more mixed evidence. However, terlipressin is not available in all centres or countries — it was only FDA-approved in the US in 2022 (as Terlivaz), and octreotide remains more widely used there. In ANZ and the UK, terlipressin is available and preferred. Both work by causing splanchnic vasoconstriction, reducing portal inflow and pressure. Key contraindications to terlipressin include ischaemic heart disease, peripheral vascular disease, and volume overload with hypoxia (due to the respiratory failure risk highlighted in the CONFIRM trial). Octreotide is generally better tolerated with fewer ischaemic side effects." [1]

Q: "He develops SBP (PMN 380). How does this change his management?" [1]

"SBP is both a complication and a precipitant of further decompensation, including hepatorenal syndrome. I would: continue ceftriaxone 1 g IV daily for 5 to 7 days; give intravenous albumin 1.5 g/kg on day 1 and 1 g/kg on day 3 (Sort trial, PMID 10432325 — reduces renal impairment from 33 to 10 percent and in-hospital mortality from 29 to 10 percent); repeat paracentesis at 48 hours to confirm PMN is falling by at least 25 percent; monitor renal function closely as SBP is a common trigger for HRS-AKI. After recovery, commence secondary SBP prophylaxis with norfloxacin 400 mg orally daily indefinitely — the one-year recurrence rate without prophylaxis is 70 percent." [1]

Q: "What is his prognosis and how would you discuss transplantation?" [1]

"His prognosis depends critically on whether he can maintain alcohol abstinence. After a variceal bleed, the one-year mortality is 30 to 40 percent for patients who continue drinking, but drops substantially for those who abstain and achieve recompensation. His MELD-Na of approximately 19 predicts a 90-day mortality of approximately 6 to 10 percent with optimal management, but this rises if he develops further complications (HRS, SBP, recurrent bleeding). I would initiate liver transplant assessment immediately — the indications are his decompensation events (bleeding, ascites). For alcohol-related cirrhosis, most Australian transplant centres require 6 months of documented abstinence, though this is increasingly individualised based on psychosocial assessment rather than a rigid timeframe. I would involve the alcohol liaison team, hepatology, transplant surgery, social work, and psychiatry. I would discuss prognosis honestly — emphasising that abstinence is the single most important prognostic factor, that transplant is the definitive treatment for decompensated cirrhosis, and that advance care planning is appropriate if he is not a transplant candidate." [1]

DCE Short Case — Abdominal Examination in Portal Hypertension

Instruction

"Examine this patient's abdominal system. You have 7 minutes for examination and 8 minutes for discussion." [1]

Systematic examination routine

Preparation:

• Position the patient flat with one pillow, abdomen exposed from xiphisternum to pubic symphysis
• Warm hands and stethoscope
• Stand on the patient's right side [1]

1. General inspection (30 seconds):

• Body habitus — cachectic in advanced cirrhosis
• Skin — jaundice, spider naevi (above nipple line, central distribution), palmar erythema, bruising, scratch marks (pruritus)
• Hands — asterixis (ask patient to hold hands dorsiflexed for 30 seconds), leukonychia, clubbing, Dupuytren contracture
• Face — parotid enlargement, sialorrhoea, fetor hepaticus (sweet musty breath)
• Chest — gynaecomastia, loss of axillary hair
• Neck — assess for elevated JVP (if posthepatic cause suspected) [1]

2. Abdominal inspection:

• Contour — distended (ascites), scaphoid (cachexia)
• Skin — caput medusae (dilated periumbilical veins), striae, surgical scars, dilated superficial veins
• Visible peristalsis, visible pulsations [1]

3. Palpation — superficial then deep:

• Start away from any tender area
• Palpate all nine regions systematically
• Liver: start in right iliac fossa, move upward toward right costal margin with inspiration. Assess size, consistency (hard, nodular in cirrhosis), surface, edge (tender?), presence of pulsatility (tricuspid regurgitation)
• Spleen: start in right iliac fossa, move diagonally toward left costal margin. A palpable spleen is enlarged. Confirm with percussion (Traube's area dullness) and position patient in right lateral decubitus if uncertain
• Kidneys: ballot each kidney
• Assess for any masses, tenderness, guarding [1]

4. Percussion:

• Liver span — measure in the midclavicular line (normally 10 to 12 cm in men; reduced in advanced cirrhosis)
• Shifting dullness: percuss from the umbilicus outward to the flank. If dull in the flank, keep your finger there and ask the patient to roll onto their side (away from you), wait 30 seconds, re-percuss. If previously dull area becomes resonant, shifting dullness is present — confirms ascites
• Fluid thrill: if ascites is large, tap one flank and feel for a transmitted impulse with the edge of your hand on the patient's abdomen [1]

5. Auscultation:

• Bowel sounds
• Cruveilhier-Baumgarten murmur: venous hum heard between xiphisternum and umbilicus, radiating toward the umbilicus — pathognomonic of portosystemic collateral through recanalised umbilical vein
• Hepatic bruit: may indicate hepatocellular carcinoma or alcoholic hepatitis
• Periumbilical venous hum: portal hypertension [1]

6. Complete the examination:

• Examine for peripheral oedema
• DRE: melaena, rectal varices
• Examine for signs of hepatic encephalopathy (asterixis, constructional apraxia — ask patient to draw a five-pointed star)
• Check urine output (if catheterised)
• Offer to examine the cardiovascular and respiratory systems, examine for testicular atrophy [1]

Presentation template (model)

"I examined Mr Nguyen's abdominal system. He is a cachectic man with multiple spider naevi on the anterior chest wall above the nipple line, palmar erythema, bilateral parotid enlargement, and Dupuytren contracture. There is no asterixis. [1]

On inspection, the abdomen is distended with an everted umbilicus. There are visible dilated periumbilical veins radiating from the umbilicus in a caput medusae distribution. There are no surgical scars. [1]

On palpation, the abdomen is soft and non-tender. The liver edge is not palpable. The spleen is enlarged, palpable 3 cm below the left costal margin. There are no other masses or organomegaly. [1]

On percussion, there is shifting dullness in the right and left flanks, confirming ascites. The liver span is reduced to approximately 8 cm in the midclavicular line, consistent with a shrunken cirrhotic liver. [1]

On auscultation, bowel sounds are present. There is a venous hum in the epigastrium consistent with a Cruveilhier-Baumgarten murmur. [1]

There is bilateral pitting oedema to mid-shin. [1]

In summary, these findings are consistent with decompensated chronic liver disease, likely alcohol-related, with portal hypertension evidenced by splenomegaly, caput medusae, the Cruveilhier-Baumgarten murmur, and ascites. [1]

I would like to complete my examination by checking for asterixis and constructional apraxia, performing a digital rectal examination, and examining the cardiovascular and respiratory systems." [1]

Discussion questions

Examiner: "What is the significance of a palpable spleen in this patient?" [1]

"A palpable spleen in the context of cirrhosis is one of the most reliable clinical indicators of portal hypertension. The spleen enlarges because portal venous congestion increases splenic venous pressure, causing congestive splenomegaly. This correlates with clinically significant portal hypertension — HVPG at or above 10 mmHg. Hypersplenism may cause thrombocytopenia and leucopenia from splenic sequestration, which is why a low platelet count is used as a non-invasive marker of portal hypertension severity in Baveno VII criteria." [1]

Examiner: "What investigations would you order?" [1]

"Bloods: full blood count (thrombocytopenia from hypersplenism), coagulation (elevated INR from impaired synthesis), U&E and creatinine (baseline renal function for HRS monitoring), liver function tests (hypoalbuminaemia, elevated bilirubin, coagulopathy — markers of synthetic failure), iron studies (haemochromatosis screening), hepatitis B and C serology, autoimmune screen (ANA, SMA, anti-LKM), caeruloplasmin (Wilson disease in younger patients), alpha-1-antitrypsin, IgG4, and alpha-fetoprotein for HCC screening. Imaging: abdominal ultrasound with Doppler to assess liver texture, portal vein patency and flow direction, splenomegaly, ascites, and any focal liver lesions. Transient elastography (FibroScan) to quantify liver stiffness — LSM at or above 25 kPa rules in clinically significant portal hypertension per Baveno VII. Upper endoscopy to screen for and classify varices. Diagnostic paracentesis of ascitic fluid — cell count for PMN, culture, albumin for SAAG, total protein, and cytology if malignancy suspected. If the aetiology is unclear, consider liver biopsy." [1]

Examiner: "What are the four decompensation events of cirrhosis?" [1]

"The four decompensation events are: development of ascites, variceal bleeding, hepatic encephalopathy, and jaundice. Each marks the transition from compensated to decompensated cirrhosis and is associated with significantly worse prognosis. Once a patient decompensates, the median survival drops from greater than 12 years to approximately 1.5 to 2 years without transplant. Multiple decompensation events carry an even worse prognosis. Baveno VII also defines 'further decompensation' — a second or subsequent event — as a distinct, high-mortality prognostic stage. The goal of management is to prevent decompensation (via etiological treatment and NSBB in those with CSPH) and, once decompensated, to manage complications and refer for transplant." [1]