Hepatopulmonary syndrome is marked by chronic liver disease combined with intrapulmonary vasodilation and impairment of arterial oxygenation. Hepatopulmonary syndrome is a disorder affecting individuals with chronic liver problems. Recurrent or chronic liver infection can cause intrapulmonary dilations of veins and increase in hepatic production can also cause this problem. Improper vascular dilations can lead to hypoxemia resulting in increase in cardiac problems. Hepatopulmonary problem can lead to various heart diseases like dyspnea and hypoxemia. Often people with chronic liver disease may get this syndrome and spider angiomas as well.
Symptoms :
People with long term liver diseases and infection are likely to develop hepatopulmonary syndrome. They are prone to show symptoms like rapid heartbeat, breathlessness and hypoxemia and dyspnea in addition to the symptoms caused by chronic liver disease. The patient might develop liver cirrhosis combined with progressive dyspnea. It is reported that 15-20% of the patients with liver cirrhosis can develop hepatopulmonary syndrome.
Causes :
Increased production of nitric oxide is likely to be the cause for pulmonary vascular dilatation leading to HPS. Patients with hepatopulmonary syndrome would exhale volumes of nitric oxide until the liver problems could be managed. Due to pulmonary vascular dilatation the veins would get supply of mixed blood leading to reduced oxygenation. Ventilation perfusion occurs due to increased flow of blood and conserved alveolar ventilation being accompanied with inhibited hypoxic vasoconstriction.
Diagnosis :
Depending on the symptoms and progression of the liver problem, the doctor would order for various testing procedures like pulse oximetry and echocardiography to check the condition of heart. Imaging tests like CT scan or MRI scan of the heart is taken to check the condition of arteriolar dilation.
Treatment :
Firstly the condition of the patient is stabilized by giving supplemental oxygen. To prevent vasodilation drugs like somatostatin can be given. There is no evidence that giving any other medications like Beta blockers, systemic corticosteroids, cyclo oxygenase inhibitors or No inhibitors can improve the symptoms.
If the patient suffers from severe hypoxemia liver transplant is the only best choice available. Without liver transplantation the survival rate of patients with HPS is poor and often the person collapse due to hepatic (liver) problems only. In most of the cases, the symptoms of hepatopulmonary syndrome would reduce if the liver problems are set right.
Prognosis :
The rate of survival is very low if the liver cirrhosis has progressed. Without treatment, the patient may collapse within few months. The patient who tolerates liver transplant can escape from the consequences of hepatopulmonary syndrome.