Hepatopulmonary syndrome

Hepatopulmonary syndrome is a condition which occurs in advanced stages of liver failure. This condition indicates a very bad prognosis and only treatment is liver transplant. Let us discuss Hepatopulmonary failure.

Hepatopulmonary failure is a triad of liver disease with deranged pulmonary gas exchange mechanism with abnormality in blood gas levels in arteries.

What are features of hepatopulmonary syndrome?

Hepatopulmonary syndrome occurs in severe liver disease. In this case the patient shows the following symptoms.

  • Difficulty in breathing especially when the patient is sitting or standing. This condition is called platypnea.
  • Difficulty in breathing is seen as increased efforts to breath and there is intercostal retraction of chest. There are increased movements of chest and abdomen suggesting increased efforts to breath.
  • The condition worsens when the patient is sitting or standing and there is somewhat relief when the patient is lying down, posing or sleeping.
  • The blood oxygen level deteriorates when the patient is sitting or standing.

Why does hepatorenal syndrome occur?

Yet the mechanism in which hepatorenal syndrome occurs is not clearly understood. Still the studies are going on to define the exact mechanism causing this syndrome.

One of the findings in this syndrome is dilated blood vessels in the lungs. Because of this dilated blood vessels in the lung the blood gas exchange becomes difficult when the patient sits or stands.

What are other associated symptoms?

Hepatorenal syndrome occurs in advanced cases of liver failure so other signs and symptoms of liver failure occur with this syndrome. Thy are as follows:

  • Altered sleep wake cycle with periods of lethargy and euphoria. In more advanced stages patients may show agitated behaviour and irritability or drowsiness and coma.
  • There are tremors and difficulty in drawing figures and writing. In more advanced stages there is hyperreflexia and then flaccidity with loss of reflexes.
  • EEG of the patient may show changes with generalized slowing or triphasic waves and bilateral delta waves or complete electrological silence may be seen.
  • Other signs like asterixis may be found in some patients.

What is treatment of hepatopulmonary syndrome?

Hepatopulmonary syndrome is a manifestation of the advanced liver disease. So the only treatment which is definitive is treating the underlying disease.

Liver transplant

Currently liver transplant is the only modality of treatment that can reverse the hepatopulmonary syndrome. 

Patients with this condition should be shifted and admitted to ICU where a liver transplant facility is available. 

Artificial liver support system

Till the transplant is available, an artificial liver support system may help patients. This facility is yet not available in many centers.

Supportive treatment

As we know liver transplant is the only definitive treatment, the supportive treatment is aimed at sustaining life till liver transplant is available. If needed ventilatory support should be provided.

For this particular problem hepatorenal syndrome patients should be in lying down position and be provided supportive oxygen therapy as needed. 

Other supportive treatments in liver failure include maintaining hydration and blood sugar level of the patient. Maintaining electrolyte balance should be the focus in managing the patient.

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