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http://dx.doi.org/10.4070/kcj.2010.40.12.684

A Case of a Pulmonary Arteriovenous Malformation With Ebstein's Anomaly  

Park, Kwon-Oh (Department of Internal Medicine, Kangdong Sacred Heart Hospital)
Kim, Chang-Hwan (Department of Internal Medicine and Sejong Medical Research Institute, Sejong General Hospital)
Lim, Dal-Soo (Department of Internal Medicine and Sejong Medical Research Institute, Sejong General Hospital)
Ro, Young-Moo (Department of Internal Medicine and Sejong Medical Research Institute, Sejong General Hospital)
Park, Jong-Won (Department of Internal Medicine, Kangdong Sacred Heart Hospital)
Chun, Seung-Yun (Department of Internal Medicine, Kangdong Sacred Heart Hospital)
Lim, Seung-Jin (Department of Internal Medicine, Kangdong Sacred Heart Hospital)
Cho, Hyun-Jung (Department of Internal Medicine, Kangdong Sacred Heart Hospital)
Lee, Sang-Ho (Department of Internal Medicine, Kangdong Sacred Heart Hospital)
Kim, Sung-Eun (Department of Internal Medicine, Kangdong Sacred Heart Hospital)
Publication Information
Korean Circulation Journal / v.40, no.12, 2010 , pp. 684-686 More about this Journal
Abstract
A pulmonary arteriovenous malformation (PAVM) is a rare pulmonary vascular anomaly presenting as dyspnea or recurrent epistaxis. Ebstein's anomaly (EA), a congenital cardiac malformation, is also a rare condition. There have been no reports concerning the co-existence of PAVM with hereditary hemorrhagic telangiectasia (HHT) and EA. A 40-year-old woman was admitted with a 2-month history of increasing dyspnea and several years of recurrent epistaxis. On transthoracic echocardiography, she was diagnosed with EA and agreed to undergo surgical treatment. A chest CT angiography showed a 12-mm serpig-inous vascular structure suspicious for a PAVM and a liver CT suggested HTT. Although it is unclear whether or not a concur-rent PAVM and EA have an embryologic or genetic relationship, we report a case of a PAVM with EA. Further genetic and em-bryonic studies are needed to identify a possible relationship of the two medical conditions.
Keywords
Arteriovenous malformation ration; Lung; Telangiectasia, hereditary hemorrhagic; Ebstein anomaly;
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1 Andelfinger G, Wright KN, Lee HS, Siemens LM, Benson DW. Canine tricuspid valve malformation, a model of human Ebstein anomaly, maps to dog chromosome 9. J Med Genet 2003;40:320-4.   DOI
2 Marchuk DA. The molecular genetics of hereditary hemorrhagic telangiectasia. Chest 1997;111(6 Suppl):79S-82S.   DOI
3 Kim MA, Cho SW, Lee WS, et al. Ebastein's anomaly in adults. Korean Circ J 1988;18:673-80.   DOI
4 Shovlin CL, Guttmacher AE, Buscarini E, et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 2000;91:66-7.   DOI   ScienceOn
5 Pick A, Deschamps C, Stanson AW. Pulmonary arteriovenous fistula: presentation, diagnosis, and treatment. World J Surg 1999;23: 1118-22.   DOI   ScienceOn
6 Khurshid I, Downie GH. Pulmonary arteriovenous malformation. Postgrad Med J 2002;78:191-7.   DOI   ScienceOn
7 Jang HJ, Kim MS, Kim SY, et al. A case of embolization seen in pulmonary arteriovenous malformation in a patient with Osler-Rendu-Weber syndrome. Korean Circ J 2006;36:820-2.   DOI
8 Gossage JR, Kanj G. Pulmonary arteriovenous malformations: a state of the art review. Am J Respir Crit Care Med 1998;158:643-61.   DOI
9 Swanson KL, Prakash UB, Stanson AW. Pulmonary arteriovenous fistulas: Mayo clinic experience, 1982-1997. Mayo Clin Proc 1999;74: 671-80.   DOI   ScienceOn
10 Memeo M, Scardapane A, De Blasi R, Sabba C, Carella A, Angelelli G. Diagnostic imaging in the study of visceral involvement of hereditary haemorrhagic telangiectasia. Radiol Med 2008;113:547-66.   DOI   ScienceOn
11 Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly. Circulation 2007;115:277-85.
12 Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein's anomaly: review of a multifaceted congenital cardiac condition. Swiss Med Wkly 2005;135:269-81.