Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome
Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome
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单心室多脾综合征患者肺动静脉畸形
DOI：
10.1136/bcr-2019-229491
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发表时间：
2019
期刊：
影响因子：
0.9
通讯作者：
Hiroshi Ono
中科院分区：
文献类型：
--
作者：
Shinichiro Sakaki;Taiyu Hayashi;Hiroshi Ono
一个5个月大的单心室，下腔静脉中断和多脾综合征的女婴，通过双侧Blalock-Taussig分流术缓解，尽管肺血流明显增加，但仍出现严重的紫绀。血管造影示弥漫性肺小动脉毛细血管扩张及早期肺静脉充盈，提示存在肺动静脉畸形。6个月时的腹部血管造影显示一个大的肝外门系统分流，经皮用血管塞关闭。门静脉分流关闭2周后行川岛手术。虽然紫绀暂时改善，但患者在9个月大时遭受恶化的紫绀并接受了Fontan完成治疗。此后，她的血氧饱和度在3周内逐渐提高到95%。先天性肝外门静脉分流术和川岛手术均可导致肺动静脉畸形的发生。
A 5-month-old girl with single ventricle, interrupted inferior vena cava and polysplenia syndrome palliated with bilateral Blalock-Taussig shunts developed severe cyanosis despite apparently increased pulmonary blood flow. Angiography revealed diffuse pulmonary arteriolar capillary dilatation and early pulmonary venous filling, suggesting the presence of pulmonary arteriovenous malformations. Abdominal angiography at 6 months demonstrated a large extrahepatic portosystemic shunt, which was percutaneously closed with a vascular plug. Kawashima operation was performed 2 weeks after portosystemic shunt closure. Although cyanosis improved temporarily, the patient suffered from deteriorating cyanosis at 9 months of age and underwent Fontan completion. Thereafter, her oxygen saturation gradually improved to 95% over the course of 3 weeks. Both the congenital extrahepatic portosystemic shunt and Kawashima operation contributed to the development of pulmonary arteriovenous malformations.
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影响因子：
0.3
作者：
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通讯作者：
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DOI：
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