Refractory hypoxemia during liver cirrhosis. Hepatopulmonary syndrome or "primary" pulmonary hypertension?

• Published in: American journal of respiratory and critical care medicine Vol. 153; no. 3; p. 1169
• Main Authors: Raffy, O, Sleiman, C, Vachiery, F, Mal, H, Roue, C, Hadengue, A, Jebrak, G, Fournier, M, Pariente, R
• Format: Journal Article
• Language: English
• Published: United States 01.03.1996
• Subjects: Adrenergic beta-Antagonists - adverse effects; Adrenergic beta-Antagonists - therapeutic use; Contrast Media; Diagnosis, Differential; Echocardiography; Female; Heart Septal Defects, Atrial - complications; Heart Septal Defects, Atrial - diagnostic imaging; Humans; Hypertension, Portal; Hypertension, Pulmonary - etiology; Hypoxia - etiology; Liver Cirrhosis - complications; Middle Aged; Syndrome
• ISSN: 1073-449X
• DOI: 10.1164/ajrccm.153.3.8630562

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.