Portal Hypertension: Clinical Presentation and Management
Definition and Pathophysiology
Portal hypertension is defined as an increase in portal pressure above 5 mmHg, with clinically significant portal hypertension (CSPH) occurring at ≥10 mmHg, measured by hepatic venous pressure gradient (HVPG). 1 The condition results primarily from increased resistance to portal blood flow due to morphological changes in chronic liver disease, aggravated by active contraction of elements in the porto-hepatic bed and increased portal blood flow from splanchnic vasodilation. 2
Clinical Manifestations
Primary Symptoms and Signs
Portal hypertension presents with distinct clinical features that vary by severity:
- Variceal bleeding is the most dreaded complication, occurring when the portal-systemic gradient exceeds 12 mmHg, affecting 30% of cirrhotic patients with a 30-day mortality of 20%. 3
- Splenomegaly with hypersplenism leads to reduction in blood cell counts (thrombocytopenia, leukopenia, anemia). 4, 5
- Ascites develops as a major decompensation event, particularly in advanced disease. 6, 5
- Hepatic encephalopathy occurs as a consequence of portosystemic shunting. 6, 5
- Portal hypertensive gastropathy represents mucosal changes in the stomach due to elevated portal pressure. 7
- Growth retardation may occur in pediatric patients with chronic portal hypertension. 5
- Jaundice can develop secondary to portal hypertensive biliopathy. 5
Physical Examination Findings
Key clinical indicators include:
- Platelet count <100,000/mm³ with significant splenomegaly suggests clinically significant portal hypertension without need for invasive measurement. 1
- Presence of varices on endoscopy or requirement for diuretics to control ascites indicates severe portal hypertension. 1
- Collateral portosystemic vessels detectable on abdominal imaging. 4
Treatment Approach by Disease Stage
Stage 1: Compensated Cirrhosis with Mild Portal Hypertension (HVPG 5-10 mmHg)
In this early stage, non-selective beta-blockers (NSBBs) are mostly ineffective because the hyperdynamic circulatory state is not fully developed; therefore, elimination of the etiologic agent is the mainstay of therapy. 1, 6 Patients at this stage have very low risk of clinical decompensation over 5 years. 1
Stage 2: Compensated Cirrhosis with CSPH but No Varices (HVPG ≥10 mmHg)
- NSBBs are NOT recommended for preventing varix formation based on a large multicenter trial showing no benefit of timolol versus placebo. 1
- Carvedilol has emerged as superior to traditional NSBBs (nadolol, propranolol) in reducing portal pressure and preventing hepatic decompensation among patients with CSPH. 8
- Liver stiffness measurement combined with platelet count can non-invasively identify CSPH, avoiding the need for invasive HVPG measurement. 8
Stage 3: Varices Present (Primary Prophylaxis)
NSBBs are the preferred first-line therapy over endoscopic band ligation (EBL) for primary prophylaxis because they reduce portal pressure and prevent other complications of portal hypertension beyond just variceal bleeding. 6 The target is HVPG reduction to ≤12 mmHg or ≥20% reduction from baseline. 6
Stage 4: Acute Variceal Bleeding
Immediate management requires combination therapy with vasoactive agents (octreotide or terlipressin) plus endoscopic therapy, which improves 5-day hemostasis rates to 77% versus 58% with endoscopy alone. 6, 4
Critical management steps:
- Initiate vasoactive agents immediately before endoscopy. 4, 2
- Terlipressin is more effective than octreotide with longer half-life and fewer adverse effects. 6
- Short-course prophylactic antibiotics are strongly recommended as they reduce mortality, bacterial infections, and rebleeding. 6
- Endoscopic band ligation is first-line endoscopic treatment with high success rates (up to 85%). 4, 3
- No more than two endoscopic sessions should be attempted; if bleeding persists, proceed to TIPS or surgery. 2
Stage 5: Secondary Prophylaxis (Prevention of Rebleeding)
Combined therapy with NSBBs plus endoscopic band ligation is recommended as it significantly decreases rebleeding compared to monotherapy. 6
Stage 6: Refractory Complications
TIPS is strongly recommended for variceal bleeding refractory to endoscopic and drug therapy. 6
TIPS Indications and Timing
- Early or pre-emptive TIPS within 72 hours should be considered in high-risk patients (Child-Pugh C or MELD ≥19). 6
- TIPS is recommended for refractory or recurrent ascites. 6
- TIPS may be considered for hepatic hydrothorax, though evidence is limited. 6
TIPS Contraindications
Absolute contraindications include: 6
- Bilirubin >50 μmol/L
- Platelets <75×10⁹/L
- Pre-existing encephalopathy
- Active infection
- Severe cardiac failure
- Severe pulmonary hypertension
Special Consideration: Porto-Pulmonary Hypertension
Porto-pulmonary hypertension occurs in 2-8% of patients with portal hypertension and requires careful evaluation before TIPS, as the procedure may worsen pulmonary hypertension. 1, 6
Management includes:
- Supplemental oxygen to maintain saturations >90%. 1, 6
- Cautious diuretic use for volume control. 1, 6
- Avoid anticoagulation in patients with impaired hepatic function, low platelets, or variceal bleeding risk. 1, 6
- Beta-blockers for portal hypertension may be poorly tolerated in porto-pulmonary hypertension. 1
Post-TIPS Complications
Hepatic encephalopathy affects approximately one-third of patients after TIPS. 6 Most cases respond to medical therapy (lactulose, rifaximin), but severe cases may require TIPS diameter reduction or occlusion. 6
Common Pitfalls
- Do not use NSBBs in mild portal hypertension (HVPG <10 mmHg) as they are ineffective at this stage. 1, 6
- Do not delay TIPS in high-risk acute bleeders (Child-Pugh C, MELD ≥19); early TIPS within 72 hours improves outcomes. 6
- Do not perform more than two endoscopic sessions for acute bleeding; escalate to TIPS promptly. 2
- Screen for porto-pulmonary hypertension before TIPS as it is a relative contraindication. 1, 6
- Recognize that platelet count <100,000/mm³ with splenomegaly reliably indicates CSPH without invasive testing. 1