Refractory hypoxemia during liver cirrhosis. Hepatopulmonary syndrome or "primary" pulmonary hypertension?
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.
Raffy
O
O
Service de Pneumologie et Réanimation Respiratoire, Hôpital Beaujon, Clichy, France.
Sleiman
C
C
Vachiery
F
F
Mal
H
H
Roue
C
C
Hadengue
A
A
Jebrak
G
G
Fournier
M
M
Pariente
R
R
D000319:Adrenergic beta-Antagonists / Q000009:adverse effects / Q000627:therapeutic use; D003287:Contrast Media; D003937:Diagnosis, Differential; D004452:Echocardiography; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000000981:diagnostic imaging; D006801:Humans; D006975:Hypertension, Portal; D006976:Hypertension, Pulmonary / Q000209:etiology; D000860:Hypoxia / Q000209:etiology*; D008103:Liver Cirrhosis / Q000150:complications*; D008875:Middle Aged; D013577:Syndrome
D000319:Adrenergic beta-Antagonists; D003287:Contrast Media