Pulmonary arteriovenous shunting in children with liver disease

Abstract Pulmonary arteriovenous shunting (PAVS) with hypoxemia is a severe complication of cirrhosis that may regress after liver transplantation. We report PAVS in 25 children with cirrhosis and in 1 with portal vein obstruction; proof of shunting was obtained by technetium Tc 99m microaggregated albumin pulmonary scanning or a high alveoloarterial O 2 gradient or both. Cyanosis or dyspnea or both occurred at ages ranging from 6 months to 14 years, earlier in children with biliary atresia and polysplenia syndrome ( p o 2 ) was 57 mm Hg (range, 42 to 81 mm Hg) during breathing of 21% O 2 and 367 mm Hg (range, 179 to 535 mm Hg) in 100% O 2 . Cardiac index was always raised, significantly more in children with biliary atresia and polysplenia syndrome ( p o 2 value during breathing of 100% O 2 was >300 mm Hg in the survivors and p o 2 while the patient is breathing 100% O 2 . The results indicate that systematic screening for PAVS should be part of the examination of these children. ( J PEDIATR 1995;126:571-9)