What is Hepatopulmonary Syndrome?

Hepatopulmonary syndrome is a medical condition characterized by decreased oxygen levels in the blood and difficulty breathing, both caused by changes to the vascular system in the lungs. These changes are the result of advanced liver disease. This condition can be managed, but the only effective treatment is liver transplant. Treatment for patients with hepatopulmonary syndrome can require the services of a hepatologist, a physician who specializes in liver care, along with pulmonary specialists and surgeons. The prognosis is variable, depending on the patient's general level of health.

In patients with advanced liver disease, research suggests that the liver begins producing increased amounts of vasodilators, chemical compounds designed to widen the blood vessels. In the lungs, widening to the blood vessels inhibits gas exchange. This limits the amount of oxygen in the blood and can lead to shortness of breath as the patient struggles for more oxygen.

If a patient with known liver disease presents with low blood oxygen levels and shortness of breath, a physician may suspect hepatopulmonary syndrome. An echocardiogram of the heart may be recommended to check on heart function and look for signs of dilated blood vessels and a nuclear medicine scan using a radioactive contrast agent to highlight bloodflow in the lungs can also be used as a diagnostic test. The outcome of both tests can confirm hepatopulmonary syndrome in the patient.

Immediate treatment for this condition is supplemental oxygen, to help the patient get more oxygen into the blood. This condition can also be exacerbated by certain body positions and the patient may be advised to change position to address the symptoms. Sitting or standing upright can make hepatopulmonary syndrome worse. Ultimately, the goal is to replace the patient's liver. Many patients with this condition are already candidates for transplant and the development of hepatopulmonary syndrome can be a sign to push the patient up the transplant list.

The presence of other complications in the patient may necessitate hospitalization so the patient can be provided with a high level of care. For other patients, it may be possible to go home while waiting for news on a transplant. The patient can use supplemental oxygen comfortably at home and may feel less stressed and nervous in a home environment. While preparing for transplant, patients will also learn about the anti-rejection drugs needed to keep the new liver healthy. Information about surgical aftercare can be provided as well to help patients prepare effectively.