Contrast-Enhanced Magnetic Resonance Angiography in Management of Pulmonary Arterio-Venous Malformations in Patients with HHT (Osler Disease)

Guenther Schneider¹, Arno Buecker¹, Alexander Massmann¹, Peter Fries¹

Purpose of our study was to evaluate CE-MRA for management of pulmonary arterio-venous malformations (PAVM) in patients with hereditary hemorrhagic telangiectasia (HHT). 286 patients (mean-age 45,9 y, male/female = 118/168) with confirmed HHT or first degree relatives underwent screening pulmonary CE-MRA (MultiHance 0.1 mmol/kg bodyweight). Patients with relevant PAVM were referred for DSA. CE-MRA detected 323 PAVM in 97 patients. 74 patients with 263 PAVMs detected on CE-MRA underwent DSA. Significantly fewer PAVMs (205/263 [78%]) were demonstrated on global DSA of which 191 were embolized. Follow-up CE-MRA showed 57 newly developed PAVM in 14 patients (interval 1-6years) and 32 reperfused PAVM in 24 patients (interval 3months-7years). All reperfused PAVMs were confirmed by DSA and reembolized successfully. CE-MRA can be considered method of choice for management of PAVMs in HHT.