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.