Barbé T, Losay J, Grimon G, Devictor D, Sardet A, Gauthier F, Houssin D, Bernard O
Département de Pédiatrie, Hôpital de Bicêtre, France.
J Pediatr. 1995 Apr;126(4):571-9. doi: 10.1016/s0022-3476(95)70351-9.
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 O2 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 < 0.01). Mean arterial oxygen tension (PaO2) was 57 mm Hg (range, 42 to 81 mm Hg) during breathing of 21% O2 and 367 mm Hg (range, 179 to 535 mm Hg) in 100% O2. Cardiac index was always raised, significantly more in children with biliary atresia and polysplenia syndrome (p < 0.01). Seven untreated children died 3 months to 8 years after the diagnosis of PAVS. Eleven underwent liver transplantation: seven are alive (follow-up, 1 to 4 years) and have no signs of PAVS. The PaO2 value during breathing of 100% O2 was > 300 mm Hg in the survivors and < 200 mm Hg in the four nonsurvivors (p < 0.01). These results indicate (1) that PAVS can occur at any age in children with portal hypertension, and that the risk is highest and earliest in children with biliary atresia and polysplenia syndrome, (2) that early liver transplantation allows regression of PAVS, and (3) that the prognosis may in part be related to the level of PaO2 while the patient is breathing 100% O2. The results indicate that systematic screening for PAVS should be part of the examination of these children.
伴有低氧血症的肺动静脉分流（PAVS）是肝硬化的一种严重并发症，肝移植后可能会消退。我们报告了25例肝硬化患儿和1例门静脉梗阻患儿出现PAVS的情况；通过锝Tc 99m微聚白蛋白肺扫描或高肺泡动脉氧梯度或两者来证实分流。发绀或呼吸困难或两者均有，发生年龄在6个月至14岁之间，在患有胆道闭锁和多脾综合征的儿童中出现得更早（p<0.01）。在吸入21%氧气时，平均动脉血氧分压（PaO2）为57毫米汞柱（范围为42至81毫米汞柱），在吸入100%氧气时为367毫米汞柱（范围为179至535毫米汞柱）。心脏指数总是升高，在患有胆道闭锁和多脾综合征的儿童中升高更为显著（p<0.01）。7例未经治疗的患儿在诊断为PAVS后3个月至8年死亡。11例接受了肝移植：7例存活（随访1至4年），且无PAVS迹象。存活者在吸入100%氧气时的PaO2值>300毫米汞柱，4例非存活者<200毫米汞柱（p<0.01）。这些结果表明：（1）门静脉高压患儿任何年龄都可能发生PAVS，且在患有胆道闭锁和多脾综合征的儿童中风险最高且出现最早；（2）早期肝移植可使PAVS消退；（3）预后可能部分与患者吸入100%氧气时的PaO2水平有关。结果表明，对这些儿童进行系统的PAVS筛查应成为检查的一部分。
