Complications

• Definition: the development of ascites, hepatic encephalopathy, or GI bleeding in patients with cirrhosis

• Onset: can be triggered by an acute event (e.g., bleeding, sepsis) or develop gradually (e.g., ascites, encephalopathy) [38][39]

• Common precipitating factors[39]

  • Infection (e.g., spontaneous bacterial peritonitis) or sepsis

  • Alcohol consumption

  • Medications (e.g., NSAIDs)

  • GI bleeding

  • Dehydration

  • Constipation

  • Acute PVT

  • HCC

• Diagnostics: to establish the cause of decompensation

  • CBC, BMP, liver chemistries

  • Magnesium and phosphate levels

  • Coagulation panel

  • Consider type and screen.

  • Paracentesis in patients with ascites

  • CRP, blood cultures, urinalysis and culture, chest x-ray

  • Abdominal ultrasound to assess for PVT

• Acute management:

  • Gastroenterology consult

  • Treat the underlying cause and complications (if applicable).

• Prognosis: In patients with decompensated cirrhosis, survival rates are usually poor unless liver transplantation is performed.

Definition

Hepatopulmonary syndrome is a condition characterized by hypoxemia, intrapulmonary vasodilatation, and portal hypertension in the presence of cirrhosis.

Pathophysiology

• Not completely understood

• Portal hypertension and liver damage → translocation of bacterial endotoxins → changes in the production of cytokines and pulmonary vasodilators → ↑ nitric oxide in the lung vessels → pulmonary vasodilation → hepatopulmonary syndrome

Clinical features

• Dyspnea

• Platypnea

• Orthodeoxia

Diagnostics

• Diagnostic criteria: All criteria must be fulfilled.

  • Presence of liver disease

  • Intrapulmonary vascular dilatation detected on contrast echocardiography

  • Abnormal arterial oxygenation confirmed by elevated A-a gradient

• Pulse oximetry (screening test): Hypoxemia

• Imaging

  • Contrast echocardiogram (gold standard)

  • Lung perfusion scintigraphy (Tc-99m macroaggregated albumin): helps diagnose and quantify intrapulmonary vasodilation

Treatment

• Supportive management

  • Supplemental oxygen to maintain O2 saturation > 88%

  • Pulse oximetry monitoring

• Definitive treatment: liver transplantation

Definition [41]

Portopulmonary hypertension is a diagnosis of exclusion made in patients with pulmonary arterial hypertension and portal hypertension after other causes have been ruled out.

Pathophysiology

• Not completely understood

• High cardiac output in advanced liver disease → wall shear stress in pulmonary vasculature → ↑ vasoactive and angiogenic substances (e.g., endothelin-1) → hypertrophy of smooth muscle cells and fibroblasts, fibrosis of intimal sheath, and microaneurysms of pulmonary arterioles

Clinical features [32][41]

• May be asymptomatic

• Features of pulmonary hypertension

• Features of right heart failure

Risk factors [41]

• Female sex

• Autoimmune liver disease

Diagnostics [41]

• Transthoracic Doppler echocardiography (initial): Typical findings include right ventricular hypertrophy or dysfunction, and increased right ventricular systolic pressure.

• Right heart catheterization (gold standard) [41]

  • Typical findings include:

    • ↑ Mean pulmonary artery pressure (mPAP)

    • ↑ Pulmonary vascular resistance (PVR)

    • Normal pulmonary artery wedge pressure (PAWP)

  • Severity of POPH

    • Mild: mPAP < 35 mm Hg

    • Moderate: mPAP 35–44 mm Hg

    • Severe: mPAP ≥ 45 mm Hg

• Diagnostic criteria: All criteria must be fulfilled.

  • Clinical features of portal hypertension or elevated portal venous pressure

  • mPAP > 25 mm Hg

  • PVR > 3 Wood units

  • PAWP < 15 mm Hg

Congestive heart failure and severe POPH (mPAP ≥ 45 mm Hg) are absolute contraindications to elective TIPS. [41]

Treatment [32][41]

• Pharmacotherapy

  • Drugs used in primary pulmonary arterial hypertension (e.g., epoprostenol, bosentan, or sildenafil) may be effective in POPH.

  • Avoid beta blockers for the treatment of varices in POPH.

• Definitive treatment: liver transplantation

Hepatic hydrothorax [42]

• Definition: pleural effusions (typically one-sided; 70% right, 18% left) with transudate characteristics in the absence of any other cardiac, pulmonary, or pleural disease [42]

• Pathophysiology: increased permeability of the diaphragm (small defects, increased abdominal pressure)

• Clinical presentation

  • May be asymptomatic

  • Signs and symptoms of pleural effusion: cough, dyspnea, hypoxia

• Diagnosis: Thoracocentesis shows transudate.

• Treatment

  • Medical management: sodium restriction and diuretics

  • Thoracocentesis for symptomatic relief

  • Surgical management for recurrent accumulation

    • Percutaneous drainage

    • Pleurodesis

    • Video-assisted thoracoscopic surgery for the closure of diaphragm defects

    • TIPS

  • Definitive treatment: liver transplantation

• Maternal complications

  • Worsening of liver cirrhosis; pregnancy in individuals with advanced disease is associated with increased risk of:

    • New-onset ascites or worsening of ascites

    • Liver failure

    • Hepatorenal syndrome

    • Variceal bleeding (during pregnancy and/or labor)

  • Increased risk of pregnancy-related complications, including:

    • Gestational diabetes

    • Placental abruption

    • Preeclampsia

    • Postpartum hemorrhage

    • Spontaneous abortion, need of cesarean delivery, preterm delivery

• Fetal complications: associated with increased rates of newborn asphyxia and small size for gestational age