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. 2002 Apr;78(918):191–197. doi: 10.1136/pmj.78.918.191

Pulmonary arteriovenous malformation

I Khurshid 1, G Downie 1
PMCID: PMC1742331  PMID: 11930021

Abstract

Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies. Although most patients are asymptomatic, PAVMs can cause dyspnoea from right-to-left shunt. Because of paradoxical emboli, various central nervous system complications have been described including stroke and brain abscess. There is a strong association between PAVM and hereditary haemorrhagic telangiectasia. Chest radiography and contrast enhanced computed tomography are essential initial diagnostic tools but pulmonary angiography is the gold standard. Contrast echocardiography is useful for diagnosis and monitoring after treatment. Most patients should be treated. Therapeutic options include angiographic embolisation with metal coil or balloon occlusion and surgical excision.

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Selected References

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  1. ANABTAWI I. N., ELLISON R. G., ELLISON L. T. PULMONARY ARTERIOVENOUS ANEURYSMS AND FISTULAS. ANATOMICAL VARIATIONS, EMBRYOLOGY, AND CLASSIFICATION. Ann Thorac Surg. 1965 May;1:277–285. doi: 10.1016/s0003-4975(10)66755-0. [DOI] [PubMed] [Google Scholar]
  2. BOSHER L. H., Jr, BLAKE D. A., BYRD B. R. An analysis of the pathologic anatomy of pulmonary arteriovenous aneurysms with particular reference to the applicability of local excision. Surgery. 1959 Jan;45(1):91–104. [PubMed] [Google Scholar]
  3. Barzilai B., Waggoner A. D., Spessert C., Picus D., Goodenberger D. Two-dimensional contrast echocardiography in the detection and follow-up of congenital pulmonary arteriovenous malformations. Am J Cardiol. 1991 Dec 1;68(15):1507–1510. doi: 10.1016/0002-9149(91)90287-u. [DOI] [PubMed] [Google Scholar]
  4. Brown J. J., Gilbert T., Gamsu G., Golden J. A., Higgins C. B. MR imaging of low signal intensity pulmonary lesions using flow-sensitive techniques. J Comput Assist Tomogr. 1988 Jul-Aug;12(4):560–564. doi: 10.1097/00004728-198807000-00003. [DOI] [PubMed] [Google Scholar]
  5. Brown S. E., Wright P. W., Renner J. W., Riker J. B. Staged bilateral thoracotomies for multiple pulmonary arteriovenous malformations complicating hereditary hemorrhagic telangiectasia. J Thorac Cardiovasc Surg. 1982 Feb;83(2):285–289. [PubMed] [Google Scholar]
  6. COOLEY D. A., McNAMARA D. G. Pulmonary telangiectasia: report of case proved by pulmonary biopsy. J Thorac Surg. 1954 Jun;27(6):614–622. [PubMed] [Google Scholar]
  7. Dines D. E., Arms R. A., Bernatz P. E., Gomes M. R. Pulmonary arteriovenous fistulas. Mayo Clin Proc. 1974 Jul;49(7):460–465. [PubMed] [Google Scholar]
  8. Dines D. E., Seward J. B., Bernatz P. E. Pulmonary arteriovenous fistulas. Mayo Clin Proc. 1983 Mar;58(3):176–181. [PubMed] [Google Scholar]
  9. Dutton J. A., Jackson J. E., Hughes J. M., Whyte M. K., Peters A. M., Ussov W., Allison D. J. Pulmonary arteriovenous malformations: results of treatment with coil embolization in 53 patients. AJR Am J Roentgenol. 1995 Nov;165(5):1119–1125. doi: 10.2214/ajr.165.5.7572487. [DOI] [PubMed] [Google Scholar]
  10. Dutton J. A., Jackson J. E., Hughes J. M., Whyte M. K., Peters A. M., Ussov W., Allison D. J. Pulmonary arteriovenous malformations: results of treatment with coil embolization in 53 patients. AJR Am J Roentgenol. 1995 Nov;165(5):1119–1125. doi: 10.2214/ajr.165.5.7572487. [DOI] [PubMed] [Google Scholar]
  11. Esplin M. S., Varner M. W. Progression of pulmonary arteriovenous malformation during pregnancy: case report and review of the literature. Obstet Gynecol Surv. 1997 Apr;52(4):248–253. doi: 10.1097/00006254-199704000-00022. [DOI] [PubMed] [Google Scholar]
  12. Ference B. A., Shannon T. M., White R. I., Jr, Zawin M., Burdge C. M. Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia. Chest. 1994 Nov;106(5):1387–1390. doi: 10.1378/chest.106.5.1387. [DOI] [PubMed] [Google Scholar]
  13. HODGSON C. H., KAYE R. L. Pulmonary arteriovenous fistula and hereditary hemorrhagic telangiectasia: a review and report of 35 cases of fistula. Dis Chest. 1963 May;43:449–455. doi: 10.1378/chest.43.5.449. [DOI] [PubMed] [Google Scholar]
  14. Haitjema T. J., Overtoom T. T., Westermann C. J., Lammers J. W. Embolisation of pulmonary arteriovenous malformations: results and follow up in 32 patients. Thorax. 1995 Jul;50(7):719–723. doi: 10.1136/thx.50.7.719. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Halbsguth A., Schulze W., Ungeheuer E., Höer P. W. Pitfall in the CT diagnosis of pulmonary arteriovenous malformation. J Comput Assist Tomogr. 1983 Aug;7(4):710–712. doi: 10.1097/00004728-198308000-00025. [DOI] [PubMed] [Google Scholar]
  16. Hoffman R., Rabens R. Evolving pulmonary nodules: multiple pulmonary arteriovenous fistulas. Am J Roentgenol Radium Ther Nucl Med. 1974 Apr;120(4):861–864. doi: 10.2214/ajr.120.4.861. [DOI] [PubMed] [Google Scholar]
  17. Kamei K., Kusumoto K., Suzuki T. Pulmonary amyloidosis with pulmonary arteriovenous fistula. Chest. 1989 Dec;96(6):1435–1436. doi: 10.1378/chest.96.6.1435. [DOI] [PubMed] [Google Scholar]
  18. LEROUX B. T. PULMONARY "HAMARTOMATA. Thorax. 1964 May;19:236–243. doi: 10.1136/thx.19.3.236. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. LINDSKOG G. E., LIEBOW A., KAUSEL H., JANZEN A. Pulmonary arteriovenous aneurysm. Ann Surg. 1950 Oct;132(4):591–610. doi: 10.1097/00000658-195010000-00002. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Le Roux B. T., Gibb B. H., Wainwright J. Pulmonary arteriovenous fistula with bilharzial pulmonary hypertension. Br Heart J. 1970 Jul;32(4):571–574. doi: 10.1136/hrt.32.4.571. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Lee D. W., White R. I., Jr, Egglin T. K., Pollak J. S., Fayad P. B., Wirth J. A., Rosenblatt M. M., Dickey K. W., Burdge C. M. Embolotherapy of large pulmonary arteriovenous malformations: long-term results. Ann Thorac Surg. 1997 Oct;64(4):930–940. doi: 10.1016/s0003-4975(97)00815-1. [DOI] [PubMed] [Google Scholar]
  22. Lee D. W., White R. I., Jr, Egglin T. K., Pollak J. S., Fayad P. B., Wirth J. A., Rosenblatt M. M., Dickey K. W., Burdge C. M. Embolotherapy of large pulmonary arteriovenous malformations: long-term results. Ann Thorac Surg. 1997 Oct;64(4):930–940. doi: 10.1016/s0003-4975(97)00815-1. [DOI] [PubMed] [Google Scholar]
  23. MURI J. W. Arteriovenous aneurysm of the lung. Am J Surg. 1955 Jan;89(1):265–271. doi: 10.1016/0002-9610(55)90529-8. [DOI] [PubMed] [Google Scholar]
  24. Mansour K. A., Hatcher C. R., Jr, Logan W. D., Jr, Abbott O. A. Pulmonary arteriovenous fistula. Am Surg. 1971 Apr;37(4):203–208. [PubMed] [Google Scholar]
  25. Marchuk D. A. The molecular genetics of hereditary hemorrhagic telangiectasia. Chest. 1997 Jun;111(6 Suppl):79S–82S. doi: 10.1378/chest.111.6_supplement.79s. [DOI] [PubMed] [Google Scholar]
  26. Minami M., Fujii Y., Mizuta T., Kishima H., Matsuda H. Video-assisted thoracoscopic local excision of pulmonary arteriovenous fistula. J Thorac Cardiovasc Surg. 1996 Nov;112(5):1395–1397. doi: 10.1016/S0022-5223(96)70161-7. [DOI] [PubMed] [Google Scholar]
  27. Moore B. P. Pulmonary arterio-venous fistula. Thorax. 1969 May;24(3):381–381. doi: 10.1136/thx.24.3.381-c. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Moore J. W., Kirby W. C., Madden W. A., Gaither N. S. Development of pulmonary arteriovenous malformations after modified Fontan operations. J Thorac Cardiovasc Surg. 1989 Dec;98(6):1045–1050. [PubMed] [Google Scholar]
  29. PIERCE J. A., REAGAN W. P., KIMBALL R. W. Unusual cases of pulmonary arteriovenous fistulas, with a note on thyroid carcinoma as a cause. N Engl J Med. 1959 Apr 30;260(18):901–907. doi: 10.1056/NEJM195904302601802. [DOI] [PubMed] [Google Scholar]
  30. Porteous M. E., Curtis A., Williams O., Marchuk D., Bhattacharya S. S., Burn J. Genetic heterogeneity in hereditary haemorrhagic telangiectasia. J Med Genet. 1994 Dec;31(12):925–926. doi: 10.1136/jmg.31.12.925. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Prager R. L., Laws K. H., Bender H. W., Jr Arteriovenous fistula of the lung. Ann Thorac Surg. 1983 Aug;36(2):231–239. doi: 10.1016/s0003-4975(10)60465-1. [DOI] [PubMed] [Google Scholar]
  32. Puskas J. D., Allen M. S., Moncure A. C., Wain J. C., Jr, Hilgenberg A. D., Wright C., Grillo H. C., Mathisen D. J. Pulmonary arteriovenous malformations: therapeutic options. Ann Thorac Surg. 1993 Aug;56(2):253–258. doi: 10.1016/0003-4975(93)91156-h. [DOI] [PubMed] [Google Scholar]
  33. Remy-Jardin M., Wattinne L., Remy J. Transcatheter occlusion of pulmonary arterial circulation and collateral supply: failures, incidents, and complications. Radiology. 1991 Sep;180(3):699–705. doi: 10.1148/radiology.180.3.1871280. [DOI] [PubMed] [Google Scholar]
  34. Remy J., Remy-Jardin M., Giraud F., Wattinne L. Angioarchitecture of pulmonary arteriovenous malformations: clinical utility of three-dimensional helical CT. Radiology. 1994 Jun;191(3):657–664. doi: 10.1148/radiology.191.3.8184042. [DOI] [PubMed] [Google Scholar]
  35. Remy J., Remy-Jardin M., Wattinne L., Deffontaines C. Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology. 1992 Mar;182(3):809–816. doi: 10.1148/radiology.182.3.1535899. [DOI] [PubMed] [Google Scholar]
  36. Rotondo A., Scialpi M., Scapati C. Pulmonary arteriovenous malformation: evaluation by MR angiography. AJR Am J Roentgenol. 1997 Mar;168(3):847–849. doi: 10.2214/ajr.168.3.9057556. [DOI] [PubMed] [Google Scholar]
  37. SHUMACKER H. B., Jr, WALDHAUSEN J. A. PULMONARY ARTERIOVENOUS FISTULAS IN CHILDREN. Ann Surg. 1963 Oct;158:713–720. doi: 10.1097/00000658-196310000-00017. [DOI] [PMC free article] [PubMed] [Google Scholar]
  38. STRINGER C. J., STANLEY A. L., BATES R. C., SUMMERS J. E. Pulmonary arteriovenous fistula. Am J Surg. 1955 May;89(5):1054–1080. doi: 10.1016/0002-9610(55)90580-8. [DOI] [PubMed] [Google Scholar]
  39. Saluja S., Sitko I., Lee D. W., Pollak J., White R. I., Jr Embolotherapy of pulmonary arteriovenous malformations with detachable balloons: long-term durability and efficacy. J Vasc Interv Radiol. 1999 Jul-Aug;10(7):883–889. doi: 10.1016/s1051-0443(99)70132-6. [DOI] [PubMed] [Google Scholar]
  40. Saluja S., Sitko I., Lee D. W., Pollak J., White R. I., Jr Embolotherapy of pulmonary arteriovenous malformations with detachable balloons: long-term durability and efficacy. J Vasc Interv Radiol. 1999 Jul-Aug;10(7):883–889. doi: 10.1016/s1051-0443(99)70132-6. [DOI] [PubMed] [Google Scholar]
  41. Silverman J. M., Julien P. J., Herfkens R. J., Pelc N. J. Magnetic resonance imaging evaluation of pulmonary vascular malformations. Chest. 1994 Nov;106(5):1333–1338. doi: 10.1378/chest.106.5.1333. [DOI] [PubMed] [Google Scholar]
  42. Sluiter-Eringa H., Orie N. G., Sluiter H. J. Pulmonary arteriovenous fistula. Diagnosis and prognosis in noncomplainant patients. Am Rev Respir Dis. 1969 Aug;100(2):177–188. doi: 10.1164/arrd.1969.100.2.177. [DOI] [PubMed] [Google Scholar]
  43. Symbas P. N., Goldman M., Erbesfeld M. H., Vlasis S. E. Pulmonary arteriovenous fistula, pulmonary artery aneurysm, and other vascular changes of the lung from penetrating trauma. Ann Surg. 1980 Mar;191(3):336–340. doi: 10.1097/00000658-198003000-00013. [DOI] [PMC free article] [PubMed] [Google Scholar]
  44. Taylor B. G., Cockerill E. M., Manfredi F., Klatte E. C. Therapeutic embolization of the pulmonary artery in pulmonary arteriovenous fistula. Am J Med. 1978 Feb;64(2):360–365. doi: 10.1016/0002-9343(78)90066-9. [DOI] [PubMed] [Google Scholar]
  45. White R. I., Jr, Lynch-Nyhan A., Terry P., Buescher P. C., Farmlett E. J., Charnas L., Shuman K., Kim W., Kinnison M., Mitchell S. E. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988 Dec;169(3):663–669. doi: 10.1148/radiology.169.3.3186989. [DOI] [PubMed] [Google Scholar]
  46. Whyte M. K., Peters A. M., Hughes J. M., Henderson B. L., Bellingan G. J., Jackson J. E., Chilvers E. R. Quantification of right to left shunt at rest and during exercise in patients with pulmonary arteriovenous malformations. Thorax. 1992 Oct;47(10):790–796. doi: 10.1136/thx.47.10.790. [DOI] [PMC free article] [PubMed] [Google Scholar]

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