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 hemorrhagic telangiectasia (HHT), the Rendu-Osler-Weber syndrome. Material and Methods: Twelve patients in the county of Fyn, Denmark, were treated with transcatheter embolization of 20 PAVMs using 12 detachable silicone balloons and 26 steel coils. Results: All PAVMs were completely occluded and we observed a significant rise in PaO2 after treatment and a significant decrease in right-to-left shunt estimated by contrast echocardiography. All patients experienced an improved functional level. One patient experienced severe pleurisy and another a rise in temperature following treatment, but otherwise no symptomatic complications were observed. Conclusion: Embolotherapy is a definitive treatment for PAVMs: it is very effective with a high success rate and few complications. Patients with HHT are at risk of PAVM and should be screened and treated for PAVMs when these reach a size that is associated with complications. In the detection of PAVMs, contrast echocardiography is a very sensitive method, and follow-up of these patients can be done with contrast echocardiography.