EMBOLOTHERAPY FOR PULMONARY ARTERIOVENOUS-MALFORMATIONS IN PATIENTS WITH HEREDITARY HEMORRHAGIC TELANGIECTASIA (RENDU-OSLER-WEBER-SYNDROME)

Purpose: To evaluate the clinical results of embolization of pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemor rhagic telangiectasia (HHT), the Rendu-Osler-Weber syndrome. Material and Methods: Twelve patients in the county of Fyn, Denmark, were treat ed with transcatheter embolization of 20 PAVMs using 12 detachable sil icone balloons and 26 steel coils. Results: All PAVMs were completely occluded and we observed a significant rise in PaO2 after treatment an d a significant decrease in right-to-left shunt estimated by contrast echocardiography. All patients experienced an improved functional leve l. One patient experienced severe pleurisy and another a rise in tempe rature following treatment, but otherwise no symptomatic complications were observed. Conclusion: Embolotherapy is a definitive treatment fo r PAVMs: it is very effective with a high success rate and few complic ations. Patients with HHT are at risk of PAVM and should be screened a nd treated for PAVMs when these reach a size that is associated with c omplications. In the detection of PAVMs, contrast echocardiography is a very sensitive method, and follow-up of these patients can be done w ith contrast echocardiography.