多脾并肺动静脉畸形
Polysplenia with pulmonary arteriovenous malformations.
作者信息
Papagiannis J, Kanter R J, Effman E L, Pratt P C, Marcille R, Browning I B, Armstrong B E
机构信息
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710.
出版信息
Pediatr Cardiol. 1993 Mar;14(2):127-9. doi: 10.1007/BF00796995.
A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.
一名患有多脾综合征、右位心、左心房异构、大血管关系正常且无心脏内分流的患者出现了进行性发绀和杵状指。通过血管造影诊断为肺动静脉畸形（PAVM），并经肺活检证实。肠系膜上动脉造影显示肝内门静脉分支发育不全和门体分流。讨论了PAVM的可能病因。
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