[Clinical analysis of 23 cases of hepatopulmonary syndrome].
OBJECTIVE
To describe the clinical features and to highlight the main criteria for diagnosis of hepatopulmonary syndrome (HPS).


METHODS
Twenty-three patients with HPS were retrospectively investigated.


RESULTS
Twenty-two cases had cirrhosis while one had acute hepatitis. The male to female ratio was 1.3:1, with an mean age of (42 +/- 21) years. 82.6% (19/23) of the patients were complicated with portal hypertension. The liver function of 78.2% of the cases was Child-Pugh grade B and C. The main clinical manifestations were dyspnea (91.3%), cyanosis (91.3%), liver palms (69.5%), clubbing fingers (65.1%), telangiectasis on face (56.5%), and spider angiomata (56.5%). The mean PaO(2) was (50.8 +/- 14.1) mm Hg (1 mm Hg = 0.133 kPa). Incidence of orthodeoxia was 85.7% (12/14). Mean diffusing capacity of lung function test was 43.1%. Chest X-ray was abnormal in 9 of the 39.1 (9/23) patients. Faint "mottled" shadows, nodular or reticulonodular opacities on bilateral lower zones were shown. All the 23 cases were diagnosed by radionuclide lung perfusion scanning technetium-labled macroaggregated albumin particles ((99m)Tc-MAA) and the mean shunt ratio was 36.3%.


CONCLUSIONS
HPS is often associated with advanced Child-Pugh grade or portal hypertension. When "unexplained" hypoxaemia, portal hypertension, spider angiomata (or telangiectasis on face), or clubbing finger are present in these patients, HPS should be highly suspected. The presence of orthodeoxia is suggestive of HPS. (99m)Tc-MAA lung perfusion scanning is able to determine the presence of intrapulmonary shunting and the diagnosis of HPS.
Zhang
Wei-hua
WH
Department of Internal Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Lu
Wei-xuan
WX
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult