Management of Pulmonary Arteriovenous Malformations

ABSTRACT

Pulmonary arteriovenous malformations are rare lesions with significant clinical complications. These lesions are commonly seen in patients with hereditary hemorrhagic telangiectasia (formerly Osler-Weber-Rendu syndrome). Interventional radiologists are a key part of the treatment team in this complex disease, and a thorough understanding of the disease process is critical to providing good patient care. In this article, the authors review the disease course and its association with hereditary hemorrhagic telangiectasia, discusses the clinical evaluation and treatment of these complex patients, and outlines complications and follow-up.

KEYWORDS

Pulmonary arteriovenous malformation - embolotherapy - arteriovenous malformation - telangiectasia

REFERENCES

• 1 Kwon B S, Bae E J, Kim G B, Noh C I, Choi J Y, Yun Y S. Development of bilateral diffuse pulmonary arteriovenous fistula after Fontan procedure: is there nonhepatic factor?.  Ann Thorac Surg. 2009;  88 (2) 677-680
• 2 Gossage J R, Kanj G. Pulmonary arteriovenous malformations. A state of the art review.  Am J Respir Crit Care Med. 1998;  158 (2) 643-661
• 3 Swanson K L, Prakash UBS, Stanson A W. Pulmonary arteriovenous fistulas: Mayo Clinic experience, 1982-1997.  Mayo Clin Proc. 1999;  74 (7) 671-680
• 4 White Jr R I, Lynch-Nyhan A, Terry P et al.. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy.  Radiology. 1988;  169 (3) 663-669
• 5 White Jr R I, Pollak J S, Wirth J A. Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy.  J Vasc Interv Radiol. 1996;  7 (6) 787-804
• 6 Faughnan M E, Palda V A, Garcia-Tsao G et al.. International guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia.  J Med Genet. 2011;  48 (2) 73-87
• 7 Carette M-F, Nedelcu C, Tassart M, Grange J-D, Wislez M, Khalil A. Imaging of hereditary hemorrhagic telangiectasia.  Cardiovasc Intervent Radiol. 2009;  32 (4) 745-757
• 8 Shovlin C L, Guttmacher A E, Buscarini E et al.. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome).  Am J Med Genet. 2000;  91 (1) 66-67
• 9 Ference B A, Shannon T M, White Jr R I, Zawin M, Burdge C M. Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia.  Chest. 1994;  106 (5) 1387-1390
• 10 Dutton JAE, Jackson J E, Hughes JMB et al.. Pulmonary arteriovenous malformations: results of treatment with coil embolization in 53 patients.  AJR Am J Roentgenol. 1995;  165 (5) 1119-1125
• 11 Haitjema T, Disch F, Overtoom T TC, Westermann C JJ, Lammers J-WJ. Screening family members of patients with hereditary hemorrhagic telangiectasia.  Am J Med. 1995;  99 (5) 519-524
• 12 Nawaz A, Litt H I, Stavropoulos S W et al.. Digital subtraction pulmonary arteriography versus multidetector CT in the detection of pulmonary arteriovenous malformations.  J Vasc Interv Radiol. 2008;  19 (11) 1582-1588
• 13 Khalil A, Farres M T, Mangiapan G, Tassart M, Bigot J-M, Carette M-F. Pulmonary arteriovenous malformations.  Chest. 2000;  117 (5) 1399-1403
• 14 Zuckerman D A, Sterling K M, Oser R F. Safety of pulmonary angiography in the 1990s.  J Vasc Interv Radiol. 1996;  7 (2) 199-205
• 15 Dinkel H P, Triller J. Pulmonary arteriovenous malformations: embolotherapy with superselective coaxial catheter placement and filling of venous sac with Guglielmi detachable coils.  Radiology. 2002;  223 (3) 709-714
• 16 Cil B, Canyigit M, Ozkan O S, Pamuk G A, Dogan R. Bilateral multiple pulmonary arteriovenous malformations: endovascular treatment with the Amplatzer Vascular Plug.  J Vasc Interv Radiol. 2006;  17 (1) 141-145
• 17 Gupta P, Mordin C, Curtis J, Hughes J MB, Shovlin C L, Jackson J E. Pulmonary arteriovenous malformations: effect of embolization on right-to-left shunt, hypoxemia, and exercise tolerance in 66 patients.  AJR Am J Roentgenol. 2002;  179 (2) 347-355