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

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Abstract

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.

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