Korean Circulation Journal

The Korean Society of Cardiology (대한심장학회)
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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)
  • Received : 2010.04.14
  • Accepted : 2010.05.24
  • Published : 2010.12.30
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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

References

  1. Pick A, Deschamps C, Stanson AW. Pulmonary arteriovenous fistula: presentation, diagnosis, and treatment. World J Surg 1999;23: 1118-22. https://doi.org/10.1007/s002689900634
  2. Khurshid I, Downie GH. Pulmonary arteriovenous malformation. Postgrad Med J 2002;78:191-7. https://doi.org/10.1136/pmj.78.918.191
  3. 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. https://doi.org/10.4070/kcj.2006.36.12.820
  4. Gossage JR, Kanj G. Pulmonary arteriovenous malformations: a state of the art review. Am J Respir Crit Care Med 1998;158:643-61. https://doi.org/10.1164/ajrccm.158.2.9711041
  5. Swanson KL, Prakash UB, Stanson AW. Pulmonary arteriovenous fistulas: Mayo clinic experience, 1982-1997. Mayo Clin Proc 1999;74: 671-80. https://doi.org/10.4065/74.7.671
  6. 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. https://doi.org/10.1007/s11547-008-0276-3
  7. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly. Circulation 2007;115:277-85.
  8. 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.
  9. 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. https://doi.org/10.1136/jmg.40.5.320
  10. Marchuk DA. The molecular genetics of hereditary hemorrhagic telangiectasia. Chest 1997;111(6 Suppl):79S-82S. https://doi.org/10.1378/chest.111.6_Supplement.79S
  11. Kim MA, Cho SW, Lee WS, et al. Ebastein's anomaly in adults. Korean Circ J 1988;18:673-80. https://doi.org/10.4070/kcj.1988.18.4.673
  12. 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. https://doi.org/10.1002/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P