Am. J. Respir. Crit. Care Med., Vol 153, No. 3, 03 1996, 1169-1171.
Refractory hypoxemia during liver cirrhosis. Hepatopulmonary syndrome or "primary" pulmonary hypertension?
O Raffy, C Sleiman, F Vachiery, H Mal, C Roue, A Hadengue, G Jebrak, M Fournier and R Pariente
Service de Pneumologie et Reanimation Respiratoire, Hopital Beaujon, Clichy, France.
We report an uncommon mechanism of severe hypoxemia in two cirrhotic
patients under long-term beta-blocker therapy. Our patients presented with
profound hypoxemia refractory to oxygen therapy, normal lung radiography
and pulmonary function tests, and evidence of right-to-left anatomic shunt.
Although these features are highly suggestive of hepatopulmonary syndrome,
pulmonary hypertension was present, and a right-to-left shunt through a
patent foramen ovale was demonstrated by contrast-enhanced
echocardiography. No cause of pulmonary hypertension other than portal
hypertension was identified. Pulmonary hypertension and intracardiac
right-to-left shunt eventually regressed after discontinuation of
beta-blocker therapy. We conclude that "primary" pulmonary hypertension
associated with portal hypertension may because of severe hypoxemia during
liver cirrhosis. Differential diagnosis of hepatopulmonary syndrome relies
upon contrast-enhanced echocardiography and may be of critical importance
because of possible therapeutic implications.
