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Pulmonary arteriovenous malformations

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Abstract

Pulmonary arteriovenous malformation, a condition most commonly associated with hereditary hemorrhagic telangiectasia, is an abnormal communication between the pulmonary artery and pulmonary vein without an intervening capillary communication. Although asymptomatic in ~ 50% individuals, it can present with the dreaded complications of stroke or intracranial abscess in high-risk individuals including pregnant women, if untreated. The mainstay of treatment is now endovascular embolization of the feeding artery which can alleviate the symptoms and prevent these complications. In this review, we describe the pathophysiology, methods of screening, diagnostic workup and treatment of these vascular lesions with a particular focus on the currently used embolization techniques and their outcomes.

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References

  1. Churton T (1896) Multiple aneurysms of pulmonary artery. Br Med J 1897(v.1):1223

    Google Scholar 

  2. Gossage JR, Kanj G (1998) Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med 158(2):643–661. https://doi.org/10.1164/ajrccm.158.2.9711041

    Article  CAS  PubMed  Google Scholar 

  3. Pollak JS, Saluja S, Thabet A, Henderson KJ, Denbow N, White RI Jr (2006) Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J Vasc Interv Radiol 17(1):35–44. https://doi.org/10.1097/01.Rvi.0000191410.13974.B6

    Article  PubMed  Google Scholar 

  4. Moussouttas M, Fayad P, Rosenblatt M, Hashimoto M, Pollak J, Henderson K, Ma TYZ, White RI (2000) Pulmonary arteriovenous malformations. Neurology 55(7):959

    Article  CAS  Google Scholar 

  5. Goodenberger DM, Chakinala M (2015) Pulmonary arteriovenous malformations. In: Grippi MA, Elias JA, Fishman JA et al (eds) Fishman’s pulmonary diseases and disorders, 5th ed. McGraw-Hill Education, New York

    Google Scholar 

  6. Plauchu H, de Chadarevian JP, Bideau A, Robert JM (1989) Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population. Am J Med Genet 32(3):291–297. https://doi.org/10.1002/ajmg.1320320302

    Article  CAS  PubMed  Google Scholar 

  7. Shovlin CL, Letarte M (1999) Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations: issues in clinical management and review of pathogenic mechanisms. Thorax 54(8):714–729

    Article  CAS  Google Scholar 

  8. Haitjema T, Westermann CJ, Overtoom TT, Timmer R, Disch F, Mauser H, Lammers JW (1996) Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): new insights in pathogenesis, complications, and treatment. Arch Intern Med 156(7):714–719

    Article  CAS  Google Scholar 

  9. Shovlin CL (2014) Pulmonary arteriovenous malformations. Am J Respir Crit Care Med 190(11):1217–1228. https://doi.org/10.1164/rccm.201407-1254CI

    Article  PubMed  PubMed Central  Google Scholar 

  10. Shovlin CL, Sodhi V, McCarthy A, Lasjaunias P, Jackson JE, Sheppard MN (2008) Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): suggested approach for obstetric services. BJOG 115(9):1108–1115. https://doi.org/10.1111/j.1471-0528.2008.01786.x

    Article  CAS  PubMed  Google Scholar 

  11. Cottin V, Plauchu H, Bayle JY, Barthelet M, Revel D, Cordier JF (2004) Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am J Respir Crit Care Med 169(9):994–1000. https://doi.org/10.1164/rccm.200310-1441OC

    Article  PubMed  Google Scholar 

  12. Shovlin CL (2010) Hereditary haemorrhagic telangiectasia: pathophysiology, diagnosis and treatment. Blood Rev 24(6):203–219. https://doi.org/10.1016/j.blre.2010.07.001

    Article  CAS  PubMed  Google Scholar 

  13. Bayrak-Toydemir P, McDonald J, Markewitz B, Lewin S, Miller F, Chou LS, Gedge F, Tang W, Coon H, Mao R (2006) Genotype-phenotype correlation in hereditary hemorrhagic telangiectasia: mutations and manifestations. Am J Med Genet A 140(5):463–470. https://doi.org/10.1002/ajmg.a.31101

    Article  PubMed  Google Scholar 

  14. Nakayama M, Nawa T, Chonan T, Endo K, Morikawa S, Bando M, Wada Y, Shioya T, Sugiyama Y, Fukai S (2012) Prevalence of pulmonary arteriovenous malformations as estimated by low-dose thoracic CT screening. Intern Med 51(13):1677–1681

    Article  Google Scholar 

  15. Cottin V, Dupuis-Girod S, Lesca G, Cordier JF (2007) Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). Respiration 74(4):361–378. https://doi.org/10.1159/000103205

    Article  CAS  PubMed  Google Scholar 

  16. Swinburne AJ, Fedullo AJ, Gangemi R, Mijangos JA (1986) Hereditary telangiectasia and multiple pulmonary arteriovenous fistulas. Clinical deterioration during pregnancy. Chest 89(3):459–460

    Article  CAS  Google Scholar 

  17. McAdams HP, Erasmus J, Crockett R, Mitchell J, Godwin JD, McDermott VG (1996) The hepatopulmonary syndrome: radiologic findings in 10 patients. AJR Am J Roentgenol 166(6):1379–1385. https://doi.org/10.2214/ajr.166.6.8633451

    Article  CAS  PubMed  Google Scholar 

  18. Lundell C, Finck E (1983) Arteriovenous fistulas originating from Rasmussen aneurysms. AJR Am J Roentgenol 140(4):687–688. https://doi.org/10.2214/ajr.140.4.687

    Article  CAS  PubMed  Google Scholar 

  19. McAllister KA, Grogg KM, Johnson DW, Gallione CJ, Baldwin MA, Jackson CE, Helmbold EA, Markel DS, McKinnon WC, Murrell J et al (1994) Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet 8(4):345–351. https://doi.org/10.1038/ng1294-345

    Article  CAS  Google Scholar 

  20. White RI Jr, Lynch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L, Shuman K, Kim W, Kinnison M, Mitchell SE (1988) Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology 169(3):663–669. https://doi.org/10.1148/radiology.169.3.3186989

    Article  PubMed  Google Scholar 

  21. White RI Jr, Mitchell SE, Barth KH, Kaufman SL, Kadir S, Chang R, Terry PB (1983) Angioarchitecture of pulmonary arteriovenous malformations: an important consideration before embolotherapy. AJR Am J Roentgenol 140(4):681–686. https://doi.org/10.2214/ajr.140.4.681

    Article  PubMed  Google Scholar 

  22. Pierucci P, Murphy J, Henderson KJ, Chyun DA, White RI Jr (2008) New definition and natural history of patients with diffuse pulmonary arteriovenous malformations: twenty-seven-year experience. Chest 133(3):653–661. https://doi.org/10.1378/chest.07-1949

    Article  PubMed  Google Scholar 

  23. Faughnan ME, Lui YW, Wirth JA, Pugash RA, Redelmeier DA, Hyland RH, White RI Jr (2000) Diffuse pulmonary arteriovenous malformations: characteristics and prognosis. Chest 117(1):31–38

    Article  CAS  Google Scholar 

  24. Burke CM, Safai C, Nelson DP, Raffin TA (1986) Pulmonary arteriovenous malformations: a critical update. Am Rev Respir Dis 134(2):334–339. https://doi.org/10.1164/arrd.1986.134.2.334

    Article  CAS  PubMed  Google Scholar 

  25. Dines DE, Arms RA, Bernatz PE, Gomes MR (1974) Pulmonary arteriovenous fistulas. Mayo Clin Proc 49(7):460–465

    CAS  PubMed  Google Scholar 

  26. Guttmacher AE, Marchuk DA, White RI Jr (1995) Hereditary hemorrhagic telangiectasia. N Engl J Med 333(14):918–924. https://doi.org/10.1056/nejm199510053331407

    Article  CAS  PubMed  Google Scholar 

  27. Faughnan ME, Palda VA, Garcia-Tsao G, Geisthoff UW, McDonald J, Proctor DD, Spears J, Brown DH, Buscarini E, Chesnutt MS, Cottin V, Ganguly A, Gossage JR, Guttmacher AE, Hyland RH, Kennedy SJ, Korzenik J, Mager JJ, Ozanne AP, Piccirillo JF, Picus D, Plauchu H, Porteous ME, Pyeritz RE, Ross DA, Sabba C, Swanson K, Terry P, Wallace MC, Westermann CJ, White RI, Young LH, Zarrabeitia R (2011) International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet 48(2):73–87. https://doi.org/10.1136/jmg.2009.069013

    Article  CAS  PubMed  Google Scholar 

  28. Langiulli M, Aronow WS, Das M, Salomon P, McClung JA, Kim-Schluger L, Wolf DC, Belkin RN (2006) Prevalence and prognosis of intrapulmonary shunts in patients with hepatic cirrhosis. Cardiol Rev 14(2):53–54. https://doi.org/10.1097/01.crd.0000173188.52195.7a

    Article  PubMed  Google Scholar 

  29. Aller R, Moya JL, Moreira V, Boixeda D, Cano A, Picher J, Garcia-Rull S, de Luis DA (1999) Diagnosis of hepatopulmonary syndrome with contrast transesophageal echocardiography: advantages over contrast transthoracic echocardiography. Dig Dis Sci 44(6):1243–1248

    Article  CAS  Google Scholar 

  30. Nanthakumar K, Graham AT, Robinson TI, Grande P, Pugash RA, Clarke JA, Hutchison SJ, Mandzia JL, Hyland RH, Faughnan ME (2001) Contrast echocardiography for detection of pulmonary arteriovenous malformations. Am Heart J 141(2):243–246. https://doi.org/10.1067/mhj.2001.112682

    Article  CAS  PubMed  Google Scholar 

  31. Trerotola SO, Pyeritz RE (2010) PAVM embolization: an update. Am J Roentgenol 195(4):837–845. https://doi.org/10.2214/AJR.10.5230

    Article  Google Scholar 

  32. Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ, Kjeldsen AD, Plauchu H (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 91(1):66–67

    Article  CAS  Google Scholar 

  33. Remy J, Remy-Jardin M, Wattinne L, Deffontaines C (1992) Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology 182(3):809–816. https://doi.org/10.1148/radiology.182.3.1535899

    Article  CAS  PubMed  Google Scholar 

  34. Primack SL, Muller NL, Mayo JR, Remy-Jardin M, Remy J (1994) Pulmonary parenchymal abnormalities of vascular origin: high-resolution CT findings. Radiographics 14(4):739–746. https://doi.org/10.1148/radiographics.14.4.7938765

    Article  CAS  PubMed  Google Scholar 

  35. Gazzaniga P, Buscarini E, Leandro G, Reduzzi L, Grosso M, Pongiglione G, Pedrinazzi C, Lanzarini L, Portugalli V, Blotta P, Forner P, Boccardi E, Pagella F, Manfredi G, Olivieri C, Zambelli A, Danesino C, Inama G (2009) Contrast echocardiography for pulmonary arteriovenous malformations screening: does any bubble matter? Eur J Echocardiogr 10(4):513–518. https://doi.org/10.1093/ejechocard/jen317

    Article  CAS  PubMed  Google Scholar 

  36. Hofmann LV, Kuszyk BS, Mitchell SE, Horton KM, Fishman EK (2000) Angioarchitecture of pulmonary arteriovenous malformations: characterization using volume-rendered 3-D CT angiography. Cardiovasc Intervent Radiol 23(2):165–170

    Article  CAS  Google Scholar 

  37. Remy J, Remy-Jardin M, Giraud F, Wattinne L (1994) Angioarchitecture of pulmonary arteriovenous malformations: clinical utility of three-dimensional helical CT. Radiology 191(3):657–664. https://doi.org/10.1148/radiology.191.3.8184042

    Article  CAS  PubMed  Google Scholar 

  38. Boussel L, Cernicanu A, Geerts L, Gamondes D, Khouatra C, Cottin V, Revel D, Douek P (2010) 4D time-resolved magnetic resonance angiography for noninvasive assessment of pulmonary arteriovenous malformations patency. J Magn Reson Imaging 32(5):1110–1116. https://doi.org/10.1002/jmri.22384

    Article  PubMed  Google Scholar 

  39. Ohno Y, Hatabu H, Takenaka D, Adachi S, Hirota S, Sugimura K (2002) Contrast-enhanced MR perfusion imaging and MR angiography: utility for management of pulmonary arteriovenous malformations for embolotherapy. Eur J Radiol 41(2):136–146

    Article  Google Scholar 

  40. Schneider G, Uder M, Koehler M, Kirchin MA, Massmann A, Buecker A, Geisthoff U (2008) MR angiography for detection of pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. AJR Am J Roentgenol 190(4):892–901. https://doi.org/10.2214/ajr.07.2966

    Article  PubMed  Google Scholar 

  41. Tanabe Y, Landeras L, Ghandour A, Partovi S, Rajiah P (2018) State-of-the-art pulmonary arterial imaging—part 1. VASA 47(5):345–359. https://doi.org/10.1024/0301-1526/a000708

    Article  PubMed  Google Scholar 

  42. Taylor BG, Cockerill EM, Manfredi F, Klatte EC (1978) Therapeutic embolization of the pulmonary artery in pulmonary arteriovenous fistula. Am J Med 64(2):360–365

    Article  CAS  Google Scholar 

  43. Terry PB, Barth KH, Kaufman SL, White RI Jr (1980) Balloon embolization for treatment of pulmonary arteriovenous fistulas. N Engl J Med 302(21):1189–1190. https://doi.org/10.1056/nejm198005223022107

    Article  CAS  PubMed  Google Scholar 

  44. Trerotola SO, Pyeritz RE, Bernhardt BA (2009) Outpatient single-session pulmonary arteriovenous malformation embolization. J Vasc Interv Radiol 20(10):1287–1291. https://doi.org/10.1016/j.jvir.2009.06.026

    Article  PubMed  Google Scholar 

  45. Faughnan ME, Thabet A, Mei-Zahav M, Colombo M, Maclusky I, Hyland RH, Pugash RA, Chait P, Henderson KJ, White RI Jr (2004) Pulmonary arteriovenous malformations in children: outcomes of transcatheter embolotherapy. J Pediatr 145(6):826–831. https://doi.org/10.1016/j.jpeds.2004.08.046

    Article  PubMed  Google Scholar 

  46. Mowers KL, Sekarski L, White AJ, Grady RM (2018) Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study. Pulmonary Circulation 8(3):2045894018786696. https://doi.org/10.1177/2045894018786696

    Article  PubMed  PubMed Central  Google Scholar 

  47. Gefen AM, White AJ (2017) Asymptomatic pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia. Pediatr Pulmonol 52(9):1194–1197. https://doi.org/10.1002/ppul.23686

    Article  PubMed  Google Scholar 

  48. Lee DW, White RI Jr, Egglin TK, Pollak JS, Fayad PB, Wirth JA, Rosenblatt MM, Dickey KW, Burdge CM (1997) Embolotherapy of large pulmonary arteriovenous malformations: long-term results. Ann Thorac Surg 64(4):930–939 (discussion 939-940)

    Article  CAS  Google Scholar 

  49. Chamarthy MR, Park H, Sutphin P, Kumar G, Lamus D, Saboo S, Anderson M, Kalva SP (2018) Pulmonary arteriovenous malformations: endovascular therapy. Cardiovasc Diagn Therapy 8(3):338–349

    Google Scholar 

  50. Trerotola SO, Pyeritz RE (2010) Does use of coils in addition to amplatzer vascular plugs prevent recanalization? AJR Am J Roentgenol 195(3):766–771. https://doi.org/10.2214/ajr.09.3953

    Article  PubMed  Google Scholar 

  51. Shovlin CL, Jackson JE, Bamford KB, Jenkins IH, Benjamin AR, Ramadan H, Kulinskaya E (2008) Primary determinants of ischaemic stroke/brain abscess risks are independent of severity of pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia. Thorax 63(3):259–266. https://doi.org/10.1136/thx.2007.087452

    Article  CAS  PubMed  Google Scholar 

  52. Gupta P, Mordin C, Curtis J, Hughes JM, Shovlin CL, Jackson JE (2002) Pulmonary arteriovenous malformations: effect of embolization on right-to-left shunt, hypoxemia, and exercise tolerance in 66 patients. AJR Am J Roentgenol 179(2):347–355. https://doi.org/10.2214/ajr.179.2.1790347

    Article  CAS  PubMed  Google Scholar 

  53. Swanson KL, Prakash UB, Stanson AW (1999) Pulmonary arteriovenous fistulas: Mayo Clinic experience, 1982–1997. Mayo Clin Proc 74(7):671–680. https://doi.org/10.4065/74.7.671

    Article  CAS  PubMed  Google Scholar 

  54. Remy-Jardin M, Dumont P, Brillet PY, Dupuis P, Duhamel A, Remy J (2006) Pulmonary arteriovenous malformations treated with embolotherapy: helical CT evaluation of long-term effectiveness after 2-21-year follow-up. Radiology 239(2):576–585. https://doi.org/10.1148/radiol.2391050333

    Article  PubMed  Google Scholar 

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Correspondence to Sreeshma Tellapuri.

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Tellapuri, S., Park, H.S. & Kalva, S.P. Pulmonary arteriovenous malformations. Int J Cardiovasc Imaging 35, 1421–1428 (2019). https://doi.org/10.1007/s10554-018-1479-x

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