The hepatopulmonary syndrome.
The hepatopulmonary syndrome is a triad of liver disease, increased alveolar-arterial oxygen gradient and intrapulmonary vascular dilatations. Manifestations include orthodeoxia, platypnoea and hyperdynamic circulation. Intrapulmonary vascular abnormalities, perhaps mediated by nitric oxide, cause hypoxaemia by shunting, a perfusion-diffusion defect, and ventilation-perfusion mismatching. Contrast-enhanced echocardiography is the method of choice for demonstrating pulmonary vascular abnormalities, although perfusion lung scanning is a more specific and sensitive test. Angiography is best reserved for patients with poor response to 100% oxygen and defines whether vascular dilatations are of the diffuse 'spongy' type or, less commonly, discrete arteriovenous communications amenable to embolization. About 80% of patients with the hepatopulmonary syndrome eventually have improved oxygenation after liver transplantation, thereby making worsening hypoxaemia the primary indication for transplantation in many instances. Nevertheless, severe hypoxaemia carries a peri-operative mortality of 30% and reliable predictors of successful outcome after transplantation remain to be determined.
Aboussouan
L S
LS
Division of Pulmonary and Critical Care Medicine, Wayne State University School of Medicine, Harper Hospital, 3-Hudson, 3990 John R, Detroit, MI 48201, USA.
Stoller
J K
JK
D003131:Combined Modality Therapy; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D006801:Humans; D008297:Male; D011379:Prognosis; D012720:Severity of Illness Index; D015996:Survival Rate