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Hepatopulmonary syndrome
Last reviewed: 23.04.2024
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[What causes hepatopulmonary syndrome?
Hepatopulmonary syndrome is a consequence of the formation of microscopic intrapulmonary arteriovenous dilatation in patients with chronic liver disease. The mechanism is unknown, but it is believed that this is due to an increase in production in the liver or a decrease in the hepatic clearance of vasodilators, possibly also nitrogen oxide. Vascular dilatations cause excess perfusion relative to ventilation, this leads to hypoxemia. Since lesions are often more numerous in the bases of the lungs, hepatopulmonary syndrome causes platypnea (dyspnea in a supine or sitting position) and orthoedexia (hypoxemia) in an elevated or upright position that disappear in the prone position. Most patients also have stigma of chronic liver disease, such as spasmodic angiomas. Approximately 20% of patients, however, have isolated pulmonary symptoms.
Symptoms of Hepatopulmonary Syndrome
Hepatopulmonary syndrome is suspected in any patient with liver disease, which indicates dyspnoea (especially platypnea). Patients with clinically significant symptoms should undergo pulsoximetry. If the syndrome is expressed, a study of the gas composition of the blood should be performed in air and 100% O2 to determine the discharge fraction.
Diagnosis of hepatopulmonary syndrome
A useful diagnostic test is contrast echocardiography. Intravenous microbubbles of foamed saline solution are injected, which are normally quickly recovered by pulmonary capillaries, pass through the lungs and appear in the left atrium within seven contractions. Similarly, the intravenously injected albumin labeled with technetium-99 can pass through the lungs and appear in the kidney and brain. Pulmonary angiography can show a diffusely good or spotted vascular configuration. Vasography is generally not required in the absence of suspicion of thromboembolism.
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Treatment of hepatopulmonary syndrome
The main treatment for hepatopulmonary syndrome is an additional O 2 for symptomatic patients. Another therapy, such as somatostatin, to inhibit vasodilatation, yields modest results only in some patients. Embolization is virtually impossible because of the number and size of lesions. In the future, inhaled inhibitors of nitric oxide synthesis may become the drug of choice. Hepatopulmonary syndrome can regress after liver transplantation or if the underlying liver disease decreases .
What is the prognosis of hepatopulmonary syndrome?
Without treatment, hepatopulmonary syndrome is marked by an unfavorable prognosis (survival less than 2 years).