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Pulmonary arteriovenous malformations: what the interventional radiologist needs to know

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Abstract

Pulmonary arteriovenous malformations (PAVMs) or fistulas are rare direct pathological connections between pulmonary arterial and venous circulation. Most of PAVMs are congenital and closely associated with hereditary hemorrhagic telangiectasia, but acquired PAVMs have also been described in the literature. Diagnosis of PAVMs is a priority for clinicians, in order to prevent potentially fatal events such as cerebrovascular stroke, systemic septic embolization, hemoptysis and hemothorax. In this scenario, the radiologist plays a key role in both diagnostic and therapeutic workups of PAVMs: Chest X-ray, computed tomography and magnetic resonance are effective tools for PAVMs identification and confirmation of the suspected diagnosis. Furthermore, imaging modalities provide most of the elements for PAVMs classification according to their angioarchitecture (simple and complex) and help the clinicians in establishing which lesion requires prompt treatment and which one will benefit of imaging follow-up alone. Endovascular management of PAVMs has grown up as the first-line treatment in respect of surgery during last decades, showing lower risk of intra- and post-procedural complications and offering a wide number of treatment options and materials, ensuring effective management in virtually any clinical situation; interventional treatment aims to exclude PAVMs from pulmonary circulation, and specific technique and embolic agents should be selected according to pre-treatment imaging, in order to obtain the best procedural outcome. This paper proposes a review of the clinical and radiological features that a radiologist needs to know for PAVMs diagnosis and proper management, also showing an overview of the most common endovascular treatment strategies and embolization materials.

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References

  1. Churton T (1897) Leeds and west-riding medico-chirurgical society: multiple aneurysms of pulmonary artery. Brit Med J 1:1223

    Google Scholar 

  2. Gossage JR, Kani G (1998) Pulmonary arteriovenous malformations. Am J Respir Crit Care Med 158:643–661

    Article  CAS  Google Scholar 

  3. Khurshid I, Downie GH (2002) Pulmonary arteriovenous malformation. Postgrad Med J 78:191–197

    Article  CAS  Google Scholar 

  4. Holzer RJ et al (2016) Pulmonary arteriovenous malformations and risk of stroke. Cardiol Clin 34:241–246

    Article  Google Scholar 

  5. Naito J et al (2017) Pulmonary arteriovenous malformations complicated by splenic infarction and abscess. Respirol Case Rep 5:e00254

    PubMed  PubMed Central  Google Scholar 

  6. Brothers M, Peeler B, Paolillo J (2017) Renal thromboembolism from a large pulmonary artery to a pulmonary vein fistula in an asymptomatic adolescent. Cardiol Young 27:199–201

    Article  Google Scholar 

  7. Elmali M, Akan H, Findik S et al (2008) Hereditary hemorrhagic telangiectasia associated with pulmonary arteriovenous malformations presenting as hemothorax. J Thorac Imaging 23:295–297

    Article  Google Scholar 

  8. Iqbal M, Leonard JR, Harry NS et al (2000) Pulmonary arteriovenous malformations: a clinical review. Postgrad Med J 76:390–394

    Article  CAS  Google Scholar 

  9. de Gussem EM, Lausman AY, Beder AJ et al (2014) Outcomes of pregnancy in women with hereditary hemorrhagic telangiectasia. Obstet Gynecol 123:514–520

    Article  Google Scholar 

  10. Carette MF, Nedelcu C, Tassart M et al (2009) Imaging of hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol 32:745–757

    Article  Google Scholar 

  11. van Gent MW, Post MC, Snijder RJ, Westermann CJ et al (2010) Real prevalence of pulmonary right-to-left shunt according to genotype in patients with hereditary hemorrhagic telangiectasia: a transthoracic contrast echocardiography study. Chest 138:833–839

    Article  Google Scholar 

  12. Ruiz-Llorente L, Gallardo-Vara E, Rossi E et al (2017) Endoglin and alk1 as therapeutic targets for hereditary hemorrhagic telangiectasia. Expert Opin Ther Targets 21:933–947

    Article  CAS  Google Scholar 

  13. Gallione C, Aylsworth AS, Beis J et al (2010) Overlap** spectra of SMAD4 mutations in juvenile polyposis (JP) and JP-HHT syndrome. Am J Med Genet A 152A:333–339

    Article  CAS  Google Scholar 

  14. Gallione CJ, Repetto GM, Legius E et al (2004) A combined syndrome of juvenile polyposis and hereditary haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet 363:852–859

    Article  CAS  Google Scholar 

  15. Ryan DJ, O’Connor TM, Murphy MM, Brady AP (2017) Follow-up interval for small untreated pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia. Clin Radiol 72:236–241

    Article  CAS  Google Scholar 

  16. Handa T, Okano Y, Nakanishi N et al (2014) BMPR2 gene mutation in pulmonary arteriovenous malformation and pulmonary hypertension: a case report. Eur Respir J 52:195–198

    Google Scholar 

  17. Matsutani N, Dejima H, Takahashi Y et al (2016) Birt-Hogg–Dube syndrome accompanied by pulmonary arteriovenous malformation. J Thorac Dis 8:E1187–E1189

    Article  Google Scholar 

  18. Inami T, Yokoyama S, Seino Y, Mizuno K (2013) Unique case of acquired pulmonary arteriovenous malformation developed by calcific constrictive pericarditis. BMJ Case Rep 15:2013

    Google Scholar 

  19. Ghersin E, Hildoer DJ, Fishman JE (2010) Pulmonary arteriovenous fistula within a pulmonary cyst-evaluation with CT pulmonary angiography. Br J Radiol 83:e114–e117

    Article  CAS  Google Scholar 

  20. Sharma A, Gulati GS, Parakh N, Aggarwal A (2016) Pulmonary arteriovenous malformation in chronic thromboembolic pulmonary hypertension. Indian J Radiol Imaging 26:195–197

    Article  Google Scholar 

  21. Esplin MS, Varner MW (1997) Progression of pulmonary arteriovenous malformation during pregnancy: case report and review of the literature. Obstet Gynecol Surv 52:248–253

    Article  CAS  Google Scholar 

  22. Shovlin CL, Sodhi V, McCarthy A et al (2008) Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler–Weber–Rendu syndrome): suggested approach for obstetric services. BJOG 115:1108–1115

    Article  CAS  Google Scholar 

  23. White RI Jr, Pollak JS, Wirth JA (1996) Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. J Vasc Interv Radiol 7:787–804

    Article  Google Scholar 

  24. Gill SS, Roddie ME, Shovlin CL, Jackson JE (2015) Pulmonary arteriovenous malformations and their mimics. Clin Radiol 70:96–110

    Article  CAS  Google Scholar 

  25. Faughnan ME, Palda VA, Garcia-Tsao G et al (2011) International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet 48:73–87

    Article  CAS  Google Scholar 

  26. Remy-Jardin M, Dumont P, Brillet PY et al (2006) Pulmonary arteriovenous malformations treated with embolotherapy: helical CT evaluation of long-term effectiveness after 2–21-year follow-up. Radiology 239:576–585

    Article  Google Scholar 

  27. Khoja AM, Jalan RK, Jain DL, Kajale OV (2016) Osler–Weber–Rendu disease: a rare cause of recurrent hemoptysis. Lung India 33:313–316

    Article  Google Scholar 

  28. Shovlin CL, Guttmacher AE, Buscarini E et al (2009) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu–Osler–Weber syndrome). Am J Med Genet 91:66–67

    Article  Google Scholar 

  29. Shovlin CL (2014) Pulmonary arteriovenous malformations. Am J Respir Crit Care Med 190:1217–1228

    Article  Google Scholar 

  30. Swanson KL, Prakash UB, Stanson AW (1999) Pulmonary arteriovenous fistulas: Mayo Clinic experience, 1982-1997. Mayo Clin Proc 74:671–680

    Article  CAS  Google Scholar 

  31. Cottin V, Plauchu H, Bayle JY et al (2004) Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am J Respir Crit Care Med 169:994–1000

    Article  Google Scholar 

  32. Cartin-Ceba R, Swanson KL, Krowka MJ (2013) Pulmonary arteriovenous malformations. Chest 144:1033–1044

    Article  Google Scholar 

  33. European guidelines on quality criteria for diagnostic radiographic images. http://www.sprmn.pt/pdf/EuropeanGuidelineseur16260.pdf. Accessed Aug 24 2018

  34. Shovlin CL, Condliffe R, Donaldson JW et al (2017) British thoracic society clinical statement on pulmonary arteriovenous malformations. Thorax 72:1154–1163

    Article  Google Scholar 

  35. Cummings KW, Bhalla S (2010) Multidetector computed tomographic pulmonary angiography: beyond acute pulmonary embolism. Radiol Clin North Am 48:51–65

    Article  Google Scholar 

  36. Vase P, Holm M, Arendrup H (1985) Pulmonary arteriovenous fistulas in hereditary hemorrhagic telangiectasia. Acta Med Scand 218:105–109

    Article  CAS  Google Scholar 

  37. Lacombe P, Lacout A, Marcy PY et al (2013) Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: an overview. Diagn Interv Imaging 94:835–848

    Article  CAS  Google Scholar 

  38. Nawaz A, Litt HI, Stavropoulos SW et al (2008) Digital subtraction pulmonary arteriography versus multidetector CT in the detection of pulmonary arteriovenous malformations. J Vasc Interv Radiol 19:1582–1588

    Article  Google Scholar 

  39. Cummings KW, Bhalla S (2015) Pulmonary vascular diseases. Clin Chest Med 36:235–248

    Article  Google Scholar 

  40. Hanneman K, Faughnan ME, Prabhudesai V (2014) Cumulative radiation dose in patients with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations. Can Assoc Radiol J 65:135–140

    Article  Google Scholar 

  41. Parra JA, Cuesta JM, Zarrabeitia R et al (2016) Screening pulmonary arteriovenous malformations in a large cohort of Spanish patients with hemorrhagic hereditary telangiectasia. Int J Cardiol 218:240–245

    Article  Google Scholar 

  42. Inarejos Clemente EJ, Ratjen F, Manson DE (2016) Utility of MDCT MIP postprocessing reconstruction images in children with hereditary hemorrhagic telangiectasia. J Comput Assist Tomogr 40:375–379

    Article  Google Scholar 

  43. Lell MM, May M, Deak P et al (2011) High-pitch spiral computed tomography: effect on image quality and radiation dose in pediatric chest computed tomography. Invest Radiol 46:116–123

    Article  Google Scholar 

  44. Balci NC, Yalçin Y, Tunaci A, Balci Y (2003) Assessment of the anomalous pulmonary circulation by dynamic contrast-enhanced MR angiography in under four seconds. Magn Reson Imaging 21:1–7

    Article  Google Scholar 

  45. Hamamoto K, Matsuura K, Chiba E et al (2016) Feasibility of non-contrast-enhanced MR angiography using the time-SLIP technique for the assessment of pulmonary arteriovenous malformation. Magn Reson Med Sci 15:253–265

    Article  Google Scholar 

  46. Schneider G, Uder M, Koehler M et al (2008) MR angiography for detection of pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. AJR Am J Roentgenol 190:892–901

    Article  Google Scholar 

  47. Shimohira M, Kawai T, Hashizume T et al (2015) Reperfusion rates of pulmonary arteriovenous malformations after coil embolization: evaluation with time-resolved MR angiography or pulmonary angiography. J Vasc Interv Radiol 26:856–864

    Article  Google Scholar 

  48. Kawai T, Shimohira M, Kan H et al (2014) Feasibility of time-resolved MR angiography for detecting recanalization of pulmonary arteriovenous malformations treated with embolization with platinum coils. J Vasc Interv Radiol 25:1339–1347

    Article  Google Scholar 

  49. Maki DD, Siegelman ES, Roberts DA et al (2001) Pulmonary arteriovenous malformations: three-dimensional gadolinium-enhanced MR angiography-initial experience. Radiology 219:243–246

    Article  CAS  Google Scholar 

  50. Nagano M, Ichinose J, Sasabuchi Y, Nakajima J, Yasunaga H (2017) Surgery versus percutaneous transcatheter embolization for pulmonary arteriovenous malformation: analysis of a national inpatient database in Japan. J Thorac Cardiovasc Surg 154:1137–1143

    Article  Google Scholar 

  51. Dutton JA, Jackson JE, Hughes JM et al (1995) Pulmonary arteriovenous malformations: results of treatment with coil embolization in 53 patients. AJR Am J Roentgenol 165:1119–1125

    Article  CAS  Google Scholar 

  52. Sandeep V, Kathleen RT, Jarvis C (2008) An overview of embolic agents. Semin Intervent Radiol 25:204–215

    Article  Google Scholar 

  53. White IR (2007) Pulmonary arteriovenous malformations: how do I embolize? Tech Vasc Interv Radiol 10:283–290

    Article  Google Scholar 

  54. Tal MG, Saluja S, Henderson KJ et al (2002) Vein of Galen technique for occluding the aneurysmal sac of pulmonary arteriovenous malformations. J Vas Interv Radiol 13:1261–1264

    Article  Google Scholar 

  55. Masayuki K, Hiroshi K, Satoshi G, Yusuke T, Haruo W, Noriyuki M (2012) Giant high-flow type pulmonary arteriovenous malformation: coil embolization with flow control by balloon occlusion and an anchored detachable coil. Korean J Radiol 13:111–114

    Article  Google Scholar 

  56. Vikash P, Raymond PC, Marie EF (2004) Embolotherapy of pulmonary arteriovenous malformations: efficacy of platinum versus stainless steel coils. J Vasc Interv Radiol 15:153–160

    Article  Google Scholar 

  57. Lopera JE (2015) The Amplatzer vascular plug: review of evolution and current applications. Semin Intervent Radiol 32:356–369

    Article  Google Scholar 

  58. Rabellino M, Serra M, Peralta O et al (2014) Early experience with the AMPLATZER vascular plug IV for the occlusion of pulmonary arteriovenous malformations. J Vasc Interv Radiol 25:1333–1337

    Article  Google Scholar 

  59. Barrett L, Brown NI, Joseph VP (2018) First use of micro vascular plugs in Australia: endovascular treatment of pulmonary arteriovenous malformation. J Med Imaging Radiat Oncol 62:72–76

    Article  Google Scholar 

  60. Conrad MB, Ishaque BM, Surman AM et al (2015) Intraprocedural safety and technical success of the MVP micro vascular plug for embolization of pulmonary arteriovenous malformations. J Vasc Interv Radiol 26:1735–1739

    Article  Google Scholar 

  61. Corvino F, Silvestre M, Cervo A, Giurazza F, Corvino A, Maglione F (2016) Endovascular occlusion of pulmonary arteriovenous malformations with the ArtVentive endoluminal occlusion system™. Diagn Interv Radiol 22:463–465

    Article  Google Scholar 

  62. Takahashi K, Tanimura K, Honda M et al (1999) Venous sac embolization of pulmonary arteriovenous malformation: preliminary experience using interlocking detachable coils. Cardiovasc Intervent Radiol 22:210–213

    Article  CAS  Google Scholar 

  63. Dinkel HP, Triller J (2002) Pulmonary arteriovenous malformations: embolotherapy with superselective coaxial catheter placement and filling of venous sac with Guglielmi detachable coils. Radiology 223:709–714

    Article  Google Scholar 

  64. Kajiwara K, Urashima M, Yamagami T et al (2014) Venous sac embolization of pulmonary arteriovenous malformation: safety and effectiveness at mid-term follow-up. Acta Radiol 55:1093–1098

    Article  Google Scholar 

  65. Hayashi S, Baba Y, Senokuchi T, Nakajo M (2012) Efficacy of venous sac embolization for pulmonary arteriovenous malformations: comparison with feeding artery embolization. J Vasc Interv Radiol 23:1566–1577

    Article  Google Scholar 

  66. Bélanger C, Chartrand-Lefebvre C, Soulez G et al (2016) Pulmonary arteriovenous malformation (PAVM) reperfusion after percutaneous embolization: sensitivity and specificity of non-enhanced CT. Eur J Radiol 85:150–157

    Article  Google Scholar 

  67. Stein EJ, Chittams JL, Miller M, Trerotola SO (2017) Persistence in coil-embolized pulmonary arteriovenous malformations with feeding artery diameters of 3 mm or less: a retrospective single-center observational study. J Vasc Interv Radiol 28:442–449

    Article  Google Scholar 

  68. Gamondès D, Si-Mohamed S, Cottin V et al (2016) Vein diameter on unenhanced multidetector CT predicts reperfusion of pulmonary arteriovenous malformation after embolotherapy. Eur Radiol 26:2723–2729

    Article  Google Scholar 

  69. Kawai N, Sato M, Minamiguchi H et al (2014) Percutaneous sclerotherapy of a fingertip high-flow arteriovenous malformation with ethanol plus lipiodol. J Vasc Interv Radiol 25:1477–1478

    Article  Google Scholar 

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Correspondence to Andrea Contegiacomo.

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Contegiacomo, A., del Ciello, A., Rella, R. et al. Pulmonary arteriovenous malformations: what the interventional radiologist needs to know. Radiol med 124, 973–988 (2019). https://doi.org/10.1007/s11547-019-01051-7

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