title: Polysplenia with pulmonary arteriovenous malformations.
abstract: 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.
authors: Department of Pediatrics, Duke University Medical Center, Durham, NC 27710.
authors: J
authors: J
authors: Papagiannis
authors: R J
authors: RJ
authors: Kanter
authors: E L
authors: EL
authors: Effman
authors: P C
authors: PC
authors: Pratt
authors: R
authors: R
authors: Marcille
authors: I B
authors: IB
authors: Browning
authors: B E
authors: BE
authors: Armstrong
mesh_terms: D001165:Arteriovenous Malformations / Q000175:diagnosis* / Q000473:pathology; D001706:Biopsy; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008168:Lung / Q000473:pathology; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000473:pathology; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome