PULMONARY ARTERIOVENOUS SHUNTING IN CHILDREN WITH LIVER-DISEASE

Pulmonary arteriovenous shunting (PAVS) with hypoxemia is a severe com plication of cirrhosis that may regress after liver transplantation. W e report PAVS in 25 children with cirrhosis and in 1 with portal vein obstruction; proof of shunting was obtained by technetium Tc 99m micro aggregated albumin pulmonary scanning or a high alveoloarterial O-2 gr adient or both. Cyanosis or dyspnea or both occurred at ages ranging f rom 6 months to 14 years, earlier in children with biliary atresia and polysplenia syndrome (p <0.01). Mean arterial oxygen tension (Pao(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 alw ays raised, significantly more in children with biliary atresia and po lysplenia syndrome (p <0.01). Seven untreated children died 3 months t o 8 years after the diagnosis of PAVS. Eleven underwent liver transpla ntation: seven are alive (follow-up, 1 to 4 years) and have no signs o f PAVS. The Pao(2) value during breathing of 100% O-2 was >300 mm Hg i n the survivors and <200 mm Hg in the four nonsurvivors (p <0.01). The se results indicate (1) that PAVS can occur at any age in children wit h portal hypertension, and that the risk is highest and earliest in ch ildren with biliary atresia and polysplenia syndrome, (2) that early l iver transplantation allows regression of PAVS, and (3) that the progn osis may in part be related to the level of Pao(2) while the patient i s breathing 100% O-2. The results indicate that systematic screening f or PAVS should be part of the examination of these children.