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Portal Hypertension: Causes, Features, Complications, and Management 🩺

Introduction

Portal hypertension is defined as an elevation of the hepatic venous pressure gradient (HVPG) to >5 mmHgΒΉ. This pathological condition represents a major complication of chronic liver disease and is responsible for many of the clinical manifestations seen in patients with cirrhosis. Understanding portal hypertension is crucial for clinicians, as it drives the transition from compensated to decompensated cirrhosis, with significant implications for patient prognosis and management.

Pathophysiology πŸ”¬

Portal hypertension results from two simultaneously occurring hemodynamic processes:

  1. Increased intrahepatic resistance to blood flow through the liver due to:
  2. Cirrhosis and regenerative nodules
  3. Microthrombi formation
  4. Hepatic stellate cell activation leading to smooth-muscle contraction

  5. Decreased endothelial nitric oxide synthetase (eNOS) production

  6. Increased splanchnic blood flow secondary to:

  7. Vasodilation within the splanchnic vascular bed
  8. Increased systemic nitric oxide levels
  9. Elevated vascular endothelial growth factor
  10. Increased tumor necrosis factor levels

Classification and Causes πŸ“‹

Portal hypertension is classified based on the anatomical location of the obstruction:

Prehepatic Causes (affecting portal venous system before entering the liver)

  • Portal vein thrombosis
  • Splenic vein thrombosis
  • Massive splenomegaly (Banti's syndrome)

Intrahepatic Causes (>95% of cases)

Presinusoidal:

  • Schistosomiasis
  • Congenital hepatic fibrosis

Sinusoidal:

  • Cirrhosis (various etiologies) - most common cause
  • Alcoholic hepatitis

Postsinusoidal:

  • Veno-occlusive disease (sinusoidal obstruction syndrome)

Posthepatic Causes

  • Budd-Chiari syndrome
  • Inferior vena caval webs
  • Cardiac causes:
  • Restrictive cardiomyopathy
  • Constrictive pericarditis
  • Severe congestive heart failure

Clinical Features πŸ₯

Portal hypertension manifests through three primary complications:

1. Gastroesophageal Varices 🩸

  • Present in approximately one-third of cirrhotic patients at diagnosis
  • 5-15% of cirrhotics develop varices annually
  • Risk factors for bleeding include:
  • Severity of cirrhosis (Child-Pugh class, MELD score)
  • Height of HVPG (>12 mmHg indicates bleeding risk)
  • Varix size and location
  • Endoscopic stigmata (red wale signs, cherry red spots)
  • Presence of tense ascites

2. Ascites πŸ’§

  • Results from portal hypertension and renal sodium/water retention
  • Patients typically notice increased abdominal girth
  • Often accompanied by peripheral edema
  • Can lead to:
  • Spontaneous bacterial peritonitis (SBP)
  • Hepatorenal syndrome
  • Respiratory compromise

3. Splenomegaly and Hypersplenism

  • Usually the first indication of portal hypertension
  • Results in:
  • Thrombocytopenia
  • Leukopenia
  • Left-sided abdominal pain in some patients

Diagnosis πŸ”

Diagnosis involves multiple modalities:

  1. Clinical Assessment
  2. Development of thrombocytopenia
  3. Enlarged spleen on physical examination

  4. Signs of decompensation (ascites, encephalopathy, varices)

  5. Endoscopy

  6. Recommended at diagnosis of compensated cirrhosis
  7. Follow-up every 2-3 years depending on disease activity

  8. Essential for identifying varices at risk for bleeding

  9. Imaging

  10. CT/MRI: Demonstrates nodular liver and collateral circulation

  11. Doppler ultrasound: Assesses portal vein patency

  12. HVPG Measurement

  13. Gold standard but rarely performed
  14. HVPG β‰₯10 mmHg indicates clinically significant portal hypertension
  15. HVPG >12 mmHg indicates risk for variceal hemorrhage

Complications and Their Management πŸ’Š

Variceal Hemorrhage

Management is divided into:

Primary Prophylaxis:

  • Non-selective beta blockers (NSBB): Target heart rate 55-60 bpm
  • Endoscopic variceal ligation (EVL) for high-risk varices
  • Contraindicated: NSBBs in refractory ascites or hypotension

Acute Bleeding Management:

  • Resuscitation and airway protection
  • Vasoactive drugs (octreotide, terlipressin)
  • Antibiotic prophylaxis (reduces mortality)
  • Endoscopic therapy within 12 hours
  • TIPS for refractory bleeding

Secondary Prophylaxis:

  • Combination of EVL + NSBB preferred
  • TIPS for recurrent bleeding despite medical therapy

Portal Hypertensive Gastropathy

  • Can cause acute or chronic GI bleeding
  • Treatment: NSBBs and iron supplementation
  • Refractory cases may require TIPS

Ascites Management

Medical Management:

  1. Sodium restriction (<2g/day)
  2. Diuretics:
  3. Spironolactone 100-400 mg/day
  4. Furosemide 40-160 mg/day

  5. Goal: 0.5-1 kg weight loss/day

  6. Large Volume Paracentesis (LVP):

  7. For tense or refractory ascites

  8. Albumin replacement (6-8g per liter removed)

  9. TIPS:

  10. For refractory ascites
  11. Risk of hepatic encephalopathy (20%)

Spontaneous Bacterial Peritonitis

  • Diagnosis: Ascitic fluid PMN >250/ΞΌL
  • Treatment: Third-generation cephalosporins + IV albumin
  • Prophylaxis indicated in high-risk patients

Hepatorenal Syndrome

  • Type 1: Rapidly progressive (doubling of creatinine in <2 weeks)
  • Type 2: More gradual progression
  • Treatment: Vasoconstrictors (terlipressin) + albumin
  • Definitive treatment: Liver transplantation

Hepatopulmonary Syndrome

  • Arterial hypoxemia due to intrapulmonary vascular dilations
  • Diagnosis: Hypoxemia + bubble echocardiography
  • Treatment: Oxygen supplementation and liver transplantation

Portopulmonary Hypertension

  • Occurs in ~5% of advanced cirrhosis
  • Diagnosis: Elevated mean pulmonary artery pressure on right heart catheterization
  • Treatment: Pulmonary vasodilators followed by liver transplantation

Special Considerations 🎯

TIPS (Transjugular Intrahepatic Portosystemic Shunt)

Indications: - Refractory variceal bleeding - Refractory ascites - Bridge to transplantation

Contraindications: - Severe hepatic encephalopathy - Severe heart failure - Severe pulmonary hypertension

Liver Transplantation

  • Definitive treatment for portal hypertension
  • Should be considered in all patients with decompensated cirrhosis
  • MELD score used for organ allocation

Prognosis πŸ“Š

  • Compensated cirrhosis (HVPG 5-10 mmHg): Can remain asymptomatic for β‰₯10 years
  • Clinically significant portal hypertension (HVPG β‰₯10 mmHg): High risk of decompensation
  • Decompensated cirrhosis: Median survival <2 years without transplantation
  • Variceal hemorrhage: 20-30% mortality per bleeding episode

Conclusion

Portal hypertension represents a critical milestone in the natural history of chronic liver disease. Its development marks the transition from compensated to decompensated cirrhosis, with profound implications for patient management and prognosis. Early recognition and appropriate management of portal hypertension and its complications can significantly improve outcomes. The management requires a multidisciplinary approach, with liver transplantation remaining the only definitive cure. As our understanding of the pathophysiology continues to evolve, new therapeutic targets and management strategies continue to emerge, offering hope for improved outcomes in this challenging condition.

References πŸ“š

ΒΉGarcia-Tsao G et al: Portal hypertensive bleeding in cirrhosis: Risk stratification, diagnosis and management: 2016 practice guidance by the American Association for the Study of Liver Diseases. Hepatology 65:310, 2017.

Section 3 Liver and Biliart Tract Disease/iMD - Harrison_s Principles of Internal Medicine, 21e - Chapter 344_ Cirrhosis and Its Complications.md