What is a hepatic hydrothorax (fluid accumulation in the pleural space due to liver disease)?

From the Guidelines

Hepatic hydrothorax is a serious complication of liver cirrhosis characterized by the accumulation of transudative fluid in the pleural space, affecting approximately 5-12% of patients with advanced liver disease, and is associated with a poor prognosis and high mortality risk. This condition occurs when ascites fluid from the abdomen moves through small defects in the diaphragm into the pleural cavity due to the pressure gradient between the abdominal and thoracic cavities 1. The prevalence of hepatic hydrothorax in cirrhosis is estimated to be around 4-12% 1. Patients typically experience shortness of breath, chest discomfort, and decreased exercise tolerance, with the severity of symptoms depending on the volume of fluid and how quickly it accumulates.

Key Characteristics of Hepatic Hydrothorax

• It is a transudative pleural effusion that occurs in portal hypertension 1
• The pleural fluid originates in the peritoneal cavity and is drawn through defects in the diaphragm by the negative intrathoracic pressure at inspiration 1
• A serum to pleural fluid albumin gradient of >1.1 g/dL is suggestive of hepatic hydrothorax 1
• Pleural fluid in hepatic hydrothorax may have higher protein content than concurrent ascites 1

Management and Treatment

• Initial management is similar to that of ascites, with sodium restriction and diuretics 1
• Therapeutic thoracentesis may be required to provide symptomatic relief from dyspnea, but the effect is transient and repeated procedures increase the risks of complications 1
• In refractory cases, transjugular intrahepatic portosystemic shunt (TIPS) may be considered, though liver transplantation remains the definitive treatment for eligible patients with end-stage liver disease 1
• The management of hepatic hydrothorax should prioritize controlling ascites with diuretics and/or large volume paracentesis (LVP), and considering TIPS or liver transplantation in refractory cases, with the goal of improving morbidity, mortality, and quality of life.

From the Research

Definition and Diagnosis of Hepatic Hydrothorax

• Hepatic hydrothorax (HH) is defined as a pleural effusion greater than 500 ml in association with cirrhosis and portal hypertension 2.
• It is an uncommon complication of cirrhosis, most frequently seen in association with decompensated liver disease 2.
• The diagnosis of HH should be suspected in any patient with established cirrhosis and portal hypertension presenting with unilateral pleural effusion, especially on the right side 2.
• Diagnostic thoracentesis should be performed in all patients with suspected HH to confirm the diagnosis and rule out infection and alternative diagnoses 2.

Clinical Presentation and Pathophysiology

• The clinical presentation of HH is quite variable, with some patients remaining relatively asymptomatic in the presence of small or incidental effusions, while others present with frank respiratory failure requiring pleural intervention 3.
• The development of HH remains incompletely understood and involves a complex pathophysiological process, with the most acceptable explanation being the passage of ascitic fluid through small diaphragmatic defects 2.
• The pathophysiology involves the passage of ascitic fluid from the peritoneal cavity to the pleural space through diaphragmatic defects 4.

Treatment and Management

• The mainstay of management is focused on optimizing fluid balance through dietary salt restriction and diuretic therapy 3.
• Liver transplantation remains the definitive treatment option for HH 2, 3, 4.
• Other therapeutic modalities that can be considered include transjugular intrahepatic portosystemic shunt placement, videoassisted thoracoscopic surgery repair, pleurodesis, and vasoconstrictors (e.g., octreotide and terlipressin) 4.
• The primary therapeutic goals are to reduce ascitic fluid production and improve symptoms to bridge the time for liver transplantation 4.

Complications and Outcomes

• Spontaneous bacterial empyema and spontaneous bacterial pleuritis can complicate HH and increase morbidity and mortality 2, 3.
• The overall outcome of HH is extremely poor, except in those undergoing liver transplantation or transjugular intrahepatic portosystemic shunt (TIPS) 5.
• The median survival of patients with HH is 8-12 months 3.