Hepatopulmonary syndrome: a rare complication of chronic liver disease in children.
An 11-year-old boy with congenital hepatic fibrosis presented with cyanosis at the National University Hospital. Echocardiogram revealed a structurally normal heart with good ventricular function. A pulmonary cause of his cyanosis was suggested on macroaggregated albumin scan and selective pulmonary artery angiogram. Arterial hypoxaemia secondary to intrapulmonary arteriovenous shunting in chronic liver cirrhosis can lead to permanent cyanosis. The potential for a complete reversal of this condition after liver transplantation indicates that arterial hypoxaemia, rather than being a contraindication, should be a reason for early liver transplantation.
Yap
F K
FK
Department of Paediatrics, National University Hospital, Singapore.
Aw
M M
MM
Quek
S C
SC
Quak
S H
SH
Quak
S C
SC
D000792:Angiography; D001164:Arteriovenous Fistula / Q000150:complications; D002648:Child; D003490:Cyanosis / Q000209:etiology; D004452:Echocardiography; D020065:Hepatopulmonary Syndrome / Q000209:etiology*; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D008103:Liver Cirrhosis / Q000151:congenital*; D008168:Lung / Q000098:blood supply; D008297:Male; D019275:Radiopharmaceuticals; D013668:Technetium Tc 99m Aggregated Albumin; D016276:Ventricular Function
D019275:Radiopharmaceuticals; D013668:Technetium Tc 99m Aggregated Albumin