Endovascular treatment of pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia
PRINCIPLES: To assess the efficiency and complication rates of vaso-occlusion of pulmonary arteriovenous malformations (PAVMs) in Rendu-Osler-Weber disease (hereditary haemorrhagic telangectasia; HHT).
METHODS: Seventy-two patients were investigated in our institution for HHT between March 2000 and November 2011. Sixteen presented PAVMs (22.2%), and 11 (68.8%) were treated with vaso-occlusion for a total of 18 procedures. Procedures included coils, plugs and combined approaches. Immediate success and recurrence rate, complication were recorded, as well as persistent and new PAVMs during clinical and computed tomography (CT) follow-up.
RESULTS: Eighteen procedures were performed and a total of 37 PAVMs were treated, 19 with coils, 16 with plugs and 2 with combined treatment. Mean CT follow-up time was 41 months (1‒164). No major complication was observed. One distal translocation was treated during the same intervention. Two PAVMs persisted after treatment (5.7%), both treated by means of plug embolisation. One new PAVM was observed during follow-up CT. PAVMs with an afferent artery of less than 3mm or asymptomatic PAVMs were not treated.
CONCLUSION: Recent studies have demonstrated that vaso-occlusion has become the gold standard treatment for PAVM. This study is in accordance with previous results and shows a minimal complication rate and little recurrence, whether by coils, plugs, or combined treatments.
- Gossage JR, Kanj G. Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med. 1998;158:643–61.
- Bayrak-Toydemir P, Mao R, Lewin S, McDonald J. Hereditary hemorrhagic telangiectasia: an overview of diagnosis and management in the molecular era for clinicians. Genet Med. 2004;6:175–91.
- Trerotola SO, Pyeritz RE. PAVM embolization: an update. AJR Am J Roentgenol. 2010;195:837–45.
- Lacombe P, Lacout A, Marcy PY, 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;94:835–48.
- Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ. et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. 2000;91:66–7.
- Faughnan ME, Palda VA, Garcia-Tsao G, Geisthoff UW, McDonald J, Proctor DD, et al. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet. 2011;48:73–87.
- Bideau A, Plauchu H, Brunet G, Robert J. Epidemiological investigation of Rendu-Osler disease in France: its geographical distribution and prevalence. Popul. 1989;44:3–22.
- Cottin V, Dupuis-Girod S, Lesca G, Cordier JF. Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). Respiration. 2007;74:361–78.
- Drouet A, Le Moigne F, Donat A, Guilloton L, Felten D. Brain abscess as the first clinical manifestation of isolated pulmonary arteriovenous malformation without Rendu-Osler disease. Rev Neurol. (Paris) 2011;167:29–34.
- Dupuis-Girod S, Giraud S, Decullier E, et al. Hemorrhagic hereditary telangiectasia (Rendu-Osler disease) and infectious diseases: an underestimated association. Clin Infect Dis. 2007;44:841–5.
- Luthra S, Antippa P, Tatoulis J. Pulmonary arteriovenous aneurysm as manifestation of Osler-Weber-Rendu syndrome. Heart Lung Circ. 2008;17:336–9.
- Gupta P, Mordin C, Curtis J, Hughes JM, Shovlin CL, Jackson JE. Pulmonary arteriovenous malformations: effect of embolization on right-to-left shunt, hypoxemia, and exercise tolerance in 66 patients. AJR Am J Roentgeno. 2002;179:347–55.
- Andersen PE, Kjeldsen AD, Oxhoj H, Vase P, White RI Jr. Embolotherapy for pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Acta Radiol. 1998;39:723–6.
- Abdel Aal AK, Hamed MF, Biosca RF, Saddekni S, Raghuram K. Occlusion time for Amplatzer vascular plug in the management of pulmonary arteriovenous malformations AJR Am J Roentgeno. 2009;192:793–9.
- Pollak JS, Saluja S, Thabet A, Henderson KJ, Denbow N, White RI, Jr. Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J Vasc Interv Radiol 2006;17:35–44; quiz 5.
- Trerotola SO, Pyeritz RE. Does use of coils in addition to amplatzer vascular plugs prevent recanalization? AJR American journal of roentgenology. 2010;195:766–71.
- Qanadli SD, Mesurolle B, Mignon F, Barre O, Bruckert F, Dubourg O, et al. [Bronchial and pulmonary vaso-occlusions]. Rev Mal Respir. 1999;16:719–29. French.
- White RI Jr., Lynch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L, et al. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988;169:663–9.
- White RI Jr. Pulmonary arteriovenous malformations: how do I embolize? Tech Vasc Interv Radiol. 2007;10:283–90.
- Saluja S, Sitko I, Lee DW, Pollak J, White RI, Jr. Embolotherapy of pulmonary arteriovenous malformations with detachable balloons: long-term durability and efficacy. J Vasc Interv Radiol. 1999;10:883–9.
- Lubarsky M, Ray C, Funaki B. Embolization agents-which one should be used when? Part 2: small-vessel embolization. Semin Intervent Radiol. 2010;27:99–104.
- Hu S, Davis C. Endovascular embolization with a vascular plug corrects a PAVM. JAAPA 2009;22:27–8, 31, 5.
- Kucukay F, Ozdemir M, Senol E, Okten S, Ereren M, Karan A. Large Pulmonary Arteriovenous Malformations: Long-Term Results of Embolization with AMPLATZER Vascular Plugs. J Vasc Interv Radiol. 2014.