Prevalence of hepatopulmonary syndrome in cirrhosis and extrahepatic portal venous obstruction.

OBJECTIVE: Hepatopulmonary syndrome (HPS) is characterized by arterial hypoxemia in patients with chronic liver disease caused by abnormal intrapulmonary vasodilations. Data on its frequency vary from 5% to 29%. Most of these studies are from the West and in patients with cirrhosis. We, therefore, studied the prevalence of HPS in patients with liver cirrhosis and extrahepatic portal venous obstruction (EHPVO).
METHODS: We studied 54 consecutive patients with liver cirrhosis (42 men and 12 women; mean age = 44.2 +/- 13 yr; Child grade A: 13, B: 22, and C: 19) and 50 patients with EHPVO (31 men and 19 women; mean age = 23.3 +/- 7.8 yr) Diagnosis of cirrhosis was made by history, liver function abnormalities, endoscopy, and sonography, whereas EHPVO was diagnosed by demonstration of a block in the splenoportovenous axis on sonography. Each of the patients underwent chest x-ray, arterial blood gas analysis, contrast-enhanced echocardiography (CEE), and pulmonary function tests. HPS was diagnosed in a patient with positive CEE, in the presence of hypoxia (PaO2 < 70 mm Hg) and/or elevated alveolar arterial oxygen gradient of > 20 mm Hg in the absence of any underlying cardiopulmonary disease.
RESULTS: Ten of 54 patients (18.5%) with cirrhosis were positive on CEE compared with two of 50 patients (4%) with EHPVO. Six of the 10 patients positive with cirrhosis for CEE had associated hypoxia, whereas only one EHPVO patient with positive CEE had an elevated pulmonary alveolar arterial oxygen gradient of > 20 mm Hg. Thus, the incidence of HPS was 11.1% in patients with cirrhosis, compared with 2% in patients with EHPVO. One patient with HPS and cirrhosis had clinical cyanosis.
CONCLUSION: HPS occurs more commonly in patients with cirrhosis but can also be seen in patients with EHPVO.