What is the difference between hepatopulmonary syndrome and Portopulmonary hypertension?

Abnormal intrapulmonary vascular dilatation, the hallmark of hepatopulmonary syndrome, can cause profound hypoxaemia that can be very difficult to treat. By contrast, portopulmonary hypertension results from excessive pulmonary vasoconstriction and vascular remodelling that eventually leads to right-heart failure.

What causes hepatopulmonary syndrome?

Hepatopulmonary syndrome is caused by blood vessels in the lungs expanding (dilating) and increasing in number, making it hard for red blood cells to properly absorb oxygen. This leaves the lungs unable to deliver adequate amounts of oxygen to the body, which leads to low oxygen levels (hypoxemia).

Does hepatopulmonary syndrome cause pulmonary hypertension?

Interestingly, approximately 10% of POPH patients have portal hypertension without cirrhosis. This picture is found most frequently in patients infected with Schistosoma mansoni[5]. Furthermore, the development of pulmonary hypertension is not related to the severity of liver disease.

How long can you live with hepatopulmonary syndrome?

The diagnosis of the hepatopulmonary syndrome significantly worsens the prognosis. One observational study demonstrated that patients with the hepatopulmonary syndrome who were not candidates for liver transplantation had a median survival of 24 months and a 5-year survival rate of 23%.

When does hepatopulmonary syndrome occur in cirrhosis?

The hepatopulmonary syndrome (HPS) is a pulmonary complication of cirrhosis and/or portal hypertension whereby patients develop hypoxemia as a result of alterations in pulmonary microvascular tone and architecture. HPS occurs in up to 30% of patients with cirrhosis. Although the degree of hypoxemia …

How is portal hypertension related to liver disease?

Liver disease and portal hypertension can be associated with pulmonary vascular complications, including portopulmonary hypertension (POPH), characterised by an elevated mean pulmonary artery pressure secondary to an increased pulmonary vascular resistance, and hepatopulmonary syndrome (HPS), characterised by hypoxaemia due to pulmonary

How is hepatopulmonary syndrome different from portopulmonary hypertension?

However, hepatopulmonary syndrome and portopulmonary hypertension are not the same disease. Although both are abnormalities of the pulmonary vasculature resulting from liver disease, HPS is characterized by vasodilatation and hypoxemia whereas portopulmonary hypertension is characterized by obstruction or narrowing (vasoconstriction) of blood

How to tell if you have hepatopulmonary syndrome?

To begin with, evidence for (1) liver disease is sought through abdominal imaging for liver abnormalities including cirrhosis, as well as ancillary signs of portal hypertension, such as enlarged veins (varices) and/or enlarged spleen (splenomegaly).