Clinical Physiology of Circulation

ISSN 1814-6910 (Print)
ISSN 2410-9134 (Online)

 

Chief Editor

Leo A. Bockeria, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, President of Bakoulev National Medical Research Center for Cardiovascular Surgery


Issue's catalogue ➤ КФК. 2021; 18 (4): 257-336 ➤ Артериовенозные фистулы легких – современное состояние проблемы

Arteriovenous pulmonary fistulas – state of the art

Authors: Petrosyan K.V., Khakimov Kh.A.

Company:
Bakoulev National Medical Research Center for Cardiovascular Surgery, Moscow, Russian Federation

E-mail: Сведения доступны для зарегистрированных пользователей.

DOI: https://doi.org/10.24022/1814-6910-2021-18-4-273-280

UDC: 616.24:612.133/.134-089.86

Link: Clinical Physiology of Blood Circulaiton. 2021; 4 (18): 273-280

Quote as: Petrosyan K.V., Khakimov Kh.A. Arteriovenous pulmonary fistulas – state of the art. Clinical Physiology of Circulation. 2021; 18 (4): 273–80 (in Russ.). DOI: 10.24022/1814-6910-2021-18-4-273-280

Keywords:  vascular malformations arteriovenous fistulas endovascular embolization endovascular closure

Received / Accepted:  26.08.2021 / 04.10.2021

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Abstract

An arteriovenous fistula is an abnormal vascular connection between an artery and a vein without any intermediate capillary network. Such uncommon vascular fistulas can form both in the pulmonary and in the systemic circulation. In turn, arteriovenous pulmonary fistulas (AVPF), or arteriovenous pulmonary malformation are a very rare anomaly in the development of pulmonary vessels, represented by direct communication between the branches of the pulmonary arteries and pulmonary veins at different levels of division of the vascular tree of the lungs. According to several instrumental diagnostic methods, a definite diagnosis of AVPF is established, including contrast multispiral computed tomography, pulmonary angiography, and transthoracic echocardiography. Statistically, this malformation is found in the lower lobes of both lungs or the right middle lobe, more often in the right lung. This literary review presents a detailed review of literary sources devoted to such a rare but very formidable problem.

References

  1. Пурсанов М.Г., Соболев А.В., Белкина М.В., Шмальц А.А., Матаева Т.В., Горбачевский С.В. Этапный подход эндоваскулярного лечения сложной формы множественных артериовенозных фистул правого легкого с использованием окклюдера Amplatzer Vascular plug II. Креативная кардиология. 2017; 11 (1): 71–8. DOI: 10.15275/kreatkard.2017.01.07
  2. Gossage J.R., Kanj G. Pulmonary arteriovenous malformations. A state of the art rewiew. Am. J. Respir. Crit. Care Med. 1998; 158 (2); 643–61. DOI: 10.1164/ajrccm.158.2.9711041
  3. Gartin-Ceba R., Swanson K.L., Krowka M.J. Pulmonary artiriovenous malformations. Chest. 2013; 144 (3): 1033–44. DOI: 10.1378/chest.12-0924
  4. Enjolras O., Wassef M., Chapot R. Introduction: ISSVA Classification. London: Cambridge University Press; 2007. DOI: 10.1017/cbo9780511722073.001
  5. Lacout A., Marcy P.Y., Thariat J., El Hajjam M., Lacombe P. VEGF target in HHT lung patients: the role of bevacizumab as a possible alternative to embolization. Med. Hypotheses. 2012; 78 (5): 689–90. DOI: 10.1016/j.mehy.2012.02.011
  6. Giordano P., Lenato G.M., Pierucci P., Suppressa P., Altomare M., Del Vecchio G. et al. Effects of VEGF on phenotypic severity in children with hereditary hemorrhagic telangiectasia. J. Pediatr. Hematol. Oncol. 2009; 31 (8): 577–82. DOI: 10.1097/mph.0b013e3181a1c104
  7. Sabba C., Pasculli G., Lenato G.M., Suppressa P., Lastella P., Memeo M. et al. Hereditary hemorrhagic telangiectasia: clinical features in ENG and ALK1 mutation carriers. J. Thromb. Haemost. 2007; 5 (6): 1149–57. DOI: 10.1111/j.1538-7836.2007.02531.x
  8. McDonald J., Pyeritz R.E. Hereditary hemorrhagic telangiectasia. In: Pagon R.A., Bird T.D., Dolan C.R., Stephens K., Adam M.P. (Eds) Gene Reviews™ [Internet]. Seattle (WA): University of Washington, Seattle; 1993. Available at: http://www.ncbi.nlm.nih.gov/books/NBK1351/(accessed 07.08.2021)
  9. Dupuis-Girod S., Ginon I., Saurin J.C., Marion D., Guillot E., Decullier E. et al. Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA. 2012; 307 (9): 948–55. DOI: 10.1001/jama.2012.250
  10. Cottin V., Plauchu H., Bayle J.-Y., Barthelet M., Revel D., Cordier J.-F. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am. J. Respir. Crit. Care Med. 2004; 169: 994–1000. DOI: 10.1164/rccm.200310-1441oc
  11. Circo S., Gossage J.R. Pulmonary vascular complications of hereditary haemorrhagic telangiectasia. Curr. Opin. Pulm. Med. 2014; 20: 421–8. DOI: 10.1097/mcp.0000000000000076
  12. Kjeldsen A.D., Oxho/j H., Andersen P.E., Elle B., Jacobsen J.P., Vase P. Pulmonary arteriovenous malformations: screening procedures and pulmonary angiography in patients with hereditary hemorrhagic telangiectasia. Chest. 1999; 116 (2): 432–9. DOI: 10.1378/chest.116.2.432
  13. Cummings K.W., Bhalla S. Pulmonary vascular diseases. Clin. Chest. Med. 2015; 36: 235–48. DOI: 10.1016/j.ccm.2015.02.007
  14. Lee E.Y., Boiselle P.M., Cleveland R.H. Multidetector CT evaluation of congenital lung anomalies. Radiology. 2008; 247: 632–48. DOI: 10.1148/radiol.2473062124
  15. Krishnan A.S., Babar J.L., Gopalan D. Imaging of congenital and acquired disorders of the pulmonary artery. Curr. Probl. Diagn. Radiol. 2012; 41: 165–78. DOI: 10.1067/j.cpradiol.2011.11.001
  16. Charlton R.W., Du Plessis L.A. Multiple aneurysms of pulmonary artery. Br. Med. J. 1897; 1: 1223. DOI: 10.1136/thx.16.4.364
  17. Lacombe P., Lacout A., Marcy P.-Y., Binsse S., Sellier J., Bensalah M. et al. Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: an overview. Diagn. Interv. Imaging. 2013; 94 (9): 835–48. DOI: 10.1016/j.diii.2013.03.014
  18. White R.I., Lynch-Nyhan A., Terry P., Buescher P.C., Farmlett E.J., Charnas L. et al. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988; 169: 663–9. DOI: 10.1148/radiology.169.3.3186989
  19. Faughnan M.E., Lui Y.W., Wirth J.A., Pugash R.A., Redelmeier D.A., Hyland R.H. et al. Diffuse pulmonary arteriovenous malformations: characteristics and prognosis. Chest. 2000; 117: 31–8. DOI: 10.1378/chest.117.1.31
  20. White R.I., Mitchell S.E., Barth K.H., Kaufman S.L., Kadir S., Chang R. et al. Angioarchitecture of pulmonary arteriovenous malformations: an important consideration before embolotherapy. Am. J. Roentgenol. 1983; 140: 681–6. DOI: 10.2214/ajr.140.4.681
  21. Remy J., Remy-Jardin M., Wattinne L., Deffontaines C. Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology. 1992; 182: 809–16. DOI: 10.1148/radiology. 182.3.1535899
  22. Anderson R.H., Baker E.J., Penny D.J., Redington A.N., Rigby M.L., Wernovsky G. Paediatric cardiology. 3rd Ed. Churchill Livingstone; 2010: 1048–52. DOI: 10.1016/b978-0-7020-3064-2.00073-4
  23. Montani D., Price L.C., Girerd B., Chinet T., Lacombe P., Simonneau G. et al. Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH. Eur. Respir. Rev. 2009; 18: 42–6. DOI: 10.1183/09059180.00011113
  24. Knoss N., Hoffmann B., Krauss B., Heller M., Biederer J. Dual energy computed tomography of lung nodules: differentiation of iodine and calcium in artificial pulmonary nodules in vitro. Eur. J. Radiol. 2011; 80: e516–9. DOI: 10.1016/j.ejrad.2010.11.001
  25. Faughnan M.E., Palda V.A., Garcia-Tsao G., Geisthoff U.W., McDonald J., Proctor D.D. et al. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J. Med. Genet. 2011; 48: 73–87. DOI: 10.1136/jmg.2009.069013
  26. Gill S.S., Roddie M.E., Shovlin C.L., Jackson J.E. Pulmonary arteriovenous malformations and their mimics. Clin. Radiol. 2015; 70: 96–110. DOI: 10.1016/j.crad.2014.09.003
  27. Nanthakumar K., Graham A.T., Robinson T.I., Grande P., Pugash R.A., Clarke J.A. et al. Contrast echocardiography for detection of pulmonary arteriovenous malformations. Am. Heart J. 2001; 141: 243–6. DOI: 10.1067/mhj.2001.112682
  28. Velthuis S., Vorselaars V.M.M., van Gent M.W.F., Westermann C.J.J., Snijder R.J., Mager J.J. et al. Role of transthoracic contrast echocardiography in the clinical diagnosis of hereditary hemorrhagic telangiectasia. Chest. 2013; 144: 1876–82. DOI: 10.1378/chest.13-0716
  29. Remy-Jardin M., Dumont Ph., Brillet P.-Y., Dupuis Ph., Duhamel A., Remy J. Pulmonary arteriovenous malformations treated with embolotherapy: helical CT evaluation of long-term effectiveness after 2-21-year follow-up. Radiology. 2006; 239 (2): 576–85. DOI: 10.1148/radiol.2391050333
  30. Pollak J.S., Saluja S., Thabet A., Henderson K.J., Denbow N., White R.I., Jr. Clinical andanatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J. Vasc. Interv. Radiol. 2006; 17 (1): 35–44. DOI: 10.1097/01.rvi.0000191410.13974.b6
  31. Baba Ya., Hayashi S., Nakajo M. Pulmonary and other non-neurological vascular malformations: diagnosis and endovascular treatment. Tjoumakaris, IntechOpen; 2013. DOI: 10.5772/56730
  32. Sagara K., Miyazono N., Inoue H., Ueno K., Nishida H., Nakajo M. Recanalization aftercoil embolotherapy of pulmonary arteriovenous malformations: study of long-termoutcome and mechanism for recanalization. Am. J. Roentgenol. 1998; 170 (3): 727–30. DOI: 10.2214/ajr.170.3.9490963
****
  1. Pursanov M.G., Sobolev A.V., Belkina M.V., Shmal’ts A.A., Mataeva T.V., Gorbachevskiy S.V. Step approach in treatment of complex multiple arteriovenous fistulas of the right lung using Amplatzer Vascular plug II occluder. Creative Cardiology. 2017; 11 (1): 71–8 (in Russ.). DOI: 10.15275/kreatkard.2017.01.07
  2. Gossage J.R., Kanj G. Pulmonary arteriovenous malformations. A state of the art rewiew. Am. J. Respir. Crit. Care Med. 1998; 158 (2); 643–61. DOI: 10.1164/ajrccm.158.2.9711041
  3. Gartin-Ceba R., Swanson K.L., Krowka M.J. Pulmonary artiriovenous malformations. Chest. 2013; 144 (3): 1033–44. DOI: 10.1378/chest.12-0924
  4. Enjolras O., Wassef M., Chapot R. Introduction: ISSVA Classification. London: Cambridge University Press; 2007. DOI: 10.1017/cbo9780511722073.001
  5. Lacout A., Marcy P.Y., Thariat J., El Hajjam M., Lacombe P. VEGF target in HHT lung patients: the role of bevacizumab as a possible alternative to embolization. Med. Hypotheses. 2012; 78 (5): 689–90. DOI: 10.1016/j.mehy.2012.02.011
  6. Giordano P., Lenato G.M., Pierucci P., Suppressa P., Altomare M., Del Vecchio G. et al. Effects of VEGF on phenotypic severity in children with hereditary hemorrhagic telangiectasia. J. Pediatr. Hematol. Oncol. 2009; 31 (8): 577–82. DOI: 10.1097/mph.0b013e3181a1c104
  7. Sabba C., Pasculli G., Lenato G.M., Suppressa P., Lastella P., Memeo M. et al. Hereditary hemorrhagic telangiectasia: clinical features in ENG and ALK1 mutation carriers. J. Thromb. Haemost. 2007; 5 (6): 1149–57. DOI: 10.1111/j.1538-7836.2007.02531.x
  8. McDonald J., Pyeritz R.E. Hereditary hemorrhagic telangiectasia. In: Pagon R.A., Bird T.D., Dolan C.R., Stephens K., Adam M.P. (Eds) Gene Reviews™ [Internet]. Seattle (WA): University of Washington, Seattle; 1993. Available at: http://www.ncbi.nlm.nih.gov/books/NBK1351/(accessed 07.08.2021)
  9. Dupuis-Girod S., Ginon I., Saurin J.C., Marion D., Guillot E., Decullier E. et al. Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA. 2012; 307 (9): 948–55. DOI: 10.1001/jama.2012.250
  10. Cottin V., Plauchu H., Bayle J.-Y., Barthelet M., Revel D., Cordier J.-F. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am. J. Respir. Crit. Care Med. 2004; 169: 994–1000. DOI: 10.1164/rccm.200310-1441oc
  11. Circo S., Gossage J.R. Pulmonary vascular complications of hereditary haemorrhagic telangiectasia. Curr. Opin. Pulm. Med. 2014; 20: 421–8. DOI: 10.1097/mcp.0000000000000076
  12. Kjeldsen A.D., Oxho/j H., Andersen P.E., Elle B., Jacobsen J.P., Vase P. Pulmonary arteriovenous malformations: screening procedures and pulmonary angiography in patients with hereditary hemorrhagic telangiectasia. Chest. 1999; 116 (2): 432–9. DOI: 10.1378/chest.116.2.432
  13. Cummings K.W., Bhalla S. Pulmonary vascular diseases. Clin. Chest. Med. 2015; 36: 235–48. DOI: 10.1016/j.ccm.2015.02.007
  14. Lee E.Y., Boiselle P.M., Cleveland R.H. Multidetector CT evaluation of congenital lung anomalies. Radiology. 2008; 247: 632–48. DOI: 10.1148/radiol.2473062124
  15. Krishnan A.S., Babar J.L., Gopalan D. Imaging of congenital and acquired disorders of the pulmonary artery. Curr. Probl. Diagn. Radiol. 2012; 41: 165–78. DOI: 10.1067/j.cpradiol.2011.11.001
  16. Charlton R.W., Du Plessis L.A. Multiple aneurysms of pulmonary artery. Br. Med. J. 1897; 1: 1223. DOI: 10.1136/thx.16.4.364
  17. Lacombe P., Lacout A., Marcy P.-Y., Binsse S., Sellier J., Bensalah M. et al. Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: an overview. Diagn. Interv. Imaging. 2013; 94 (9): 835–48. DOI: 10.1016/j.diii.2013.03.014
  18. White R.I., Lynch-Nyhan A., Terry P., Buescher P.C., Farmlett E.J., Charnas L. et al. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988; 169: 663–9. DOI: 10.1148/radiology.169.3.3186989
  19. Faughnan M.E., Lui Y.W., Wirth J.A., Pugash R.A., Redelmeier D.A., Hyland R.H. et al. Diffuse pulmonary arteriovenous malformations: characteristics and prognosis. Chest. 2000; 117: 31–8. DOI: 10.1378/chest.117.1.31
  20. White R.I., Mitchell S.E., Barth K.H., Kaufman S.L., Kadir S., Chang R. et al. Angioarchitecture of pulmonary arteriovenous malformations: an important consideration before embolotherapy. Am. J. Roentgenol. 1983; 140: 681–6. DOI: 10.2214/ajr.140.4.681
  21. Remy J., Remy-Jardin M., Wattinne L., Deffontaines C. Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology. 1992; 182: 809–16. DOI: 10.1148/radiology. 182.3.1535899
  22. Anderson R.H., Baker E.J., Penny D.J., Redington A.N., Rigby M.L., Wernovsky G. Paediatric cardiology. 3rd Ed. Churchill Livingstone; 2010: 1048–52. DOI: 10.1016/b978-0-7020-3064-2.00073-4
  23. Montani D., Price L.C., Girerd B., Chinet T., Lacombe P., Simonneau G. et al. Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH. Eur. Respir. Rev. 2009; 18: 42–6. DOI: 10.1183/09059180.00011113
  24. Knoss N., Hoffmann B., Krauss B., Heller M., Biederer J. Dual energy computed tomography of lung nodules: differentiation of iodine and calcium in artificial pulmonary nodules in vitro. Eur. J. Radiol. 2011; 80: e516–9. DOI: 10.1016/j.ejrad.2010.11.001
  25. Faughnan M.E., Palda V.A., Garcia-Tsao G., Geisthoff U.W., McDonald J., Proctor D.D. et al. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J. Med. Genet. 2011; 48: 73–87. DOI: 10.1136/jmg.2009.069013
  26. Gill S.S., Roddie M.E., Shovlin C.L., Jackson J.E. Pulmonary arteriovenous malformations and their mimics. Clin. Radiol. 2015; 70: 96–110. DOI: 10.1016/j.crad.2014.09.003
  27. Nanthakumar K., Graham A.T., Robinson T.I., Grande P., Pugash R.A., Clarke J.A. et al. Contrast echocardiography for detection of pulmonary arteriovenous malformations. Am. Heart J. 2001; 141: 243–6. DOI: 10.1067/mhj.2001.112682
  28. Velthuis S., Vorselaars V.M.M., van Gent M.W.F., Westermann C.J.J., Snijder R.J., Mager J.J. et al. Role of transthoracic contrast echocardiography in the clinical diagnosis of hereditary hemorrhagic telangiectasia. Chest. 2013; 144: 1876–82. DOI: 10.1378/chest.13-0716
  29. Remy-Jardin M., Dumont Ph., Brillet P.-Y., Dupuis Ph., Duhamel A., Remy J. Pulmonary arteriovenous malformations treated with embolotherapy: helical CT evaluation of long-term effectiveness after 2-21-year follow-up. Radiology. 2006; 239 (2): 576–85. DOI: 10.1148/radiol.2391050333
  30. Pollak J.S., Saluja S., Thabet A., Henderson K.J., Denbow N., White R.I., Jr. Clinical andanatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J. Vasc. Interv. Radiol. 2006; 17 (1): 35–44. DOI: 10.1097/01.rvi.0000191410.13974.b6
  31. Baba Ya., Hayashi S., Nakajo M. Pulmonary and other non-neurological vascular malformations: diagnosis and endovascular treatment. Tjoumakaris, IntechOpen; 2013. DOI: 10.5772/56730
  32. Sagara K., Miyazono N., Inoue H., Ueno K., Nishida H., Nakajo M. Recanalization aftercoil embolotherapy of pulmonary arteriovenous malformations: study of long-termoutcome and mechanism for recanalization. Am. J. Roentgenol. 1998; 170 (3): 727–30. DOI: 10.2214/ajr.170.3.9490963

About Authors

Karen V. Petrosyan, Dr. Med. Sci., Endovascular Surgeon; ORCID

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