Imoto Yutaka, Sese Akira, Joh Kunitaka
Cardiovascular Surgery, Kyushu Koseinenkin Hospital, Yahatanishi-ku, Kitakyushu, Japan.
Pediatr Cardiol. 2006 Jul-Aug;27(4):490-2. doi: 10.1007/s00246-006-1242-2. Epub 2006 Jul 6.
A 45-month-old boy who had complex cardiac anomalies with interrupted inferior vena cava and polysplenia underwent extracardiac Fontan operation. He redeveloped deep cyanosis postoperatively. Cardiac catheterization showed a preferential flow of the hepatic venous blood to the right lung and arteriovenous malformations (PAVMs) in the left lung. He underwent revision of the conduit to attain balanced hepatic venous drainage with subsequent regression of the PAVMs and disappearance of cyanosis. Our experience shows that deficiency of hepatic venous flow played a crucial role in the development of PAVMs and that redistribution of the flow retained potential to ameliorate the pathological condition.
一名45个月大的男孩患有复杂心脏畸形，包括下腔静脉中断和多脾症，接受了心外Fontan手术。术后他再次出现严重发绀。心导管检查显示肝静脉血优先流向右肺，左肺存在动静脉畸形（PAVM）。他接受了管道修复手术，以实现肝静脉引流平衡，随后PAVM消退，发绀消失。我们的经验表明，肝静脉血流不足在PAVM的发生发展中起关键作用，血流重新分布具有改善病理状况的潜力。
