CLINICAL OUTCOME OF TRANSFEMORAL EMBOLIZATION IN PATIENTS WITH ARTERIOVENOUS-MALFORMATIONS OF THE LIVER IN HEREDITARY HEMORRHAGIC TELANGIECTASIA (WEBER-RENDU-OSLER-DISEASE)

Background - Arteriovenous malformations of the liver in Osler's disea se may present as high output cardiac failure. A few case reports sugg ested that treatment with arterial embolisation may have beneficial ef fects in such patients. Aims - To investigate the efficacy and safety of this treatment modality in a prospective pilot study. Patients and methods - Four women and one man (aged 39 - 59 years) with the dominan t hepatic manifestation of Osler's disease presented with symptoms of cardiac failure and elevated cardiac output. Arteriovenous malformatio ns were treated in three to five sessions with arterial embolisation u sing coils. The outcome was analysed by measurement of cardiac output and scoring of clinical symptoms. Results - Embolisation was technical ly feasible in all patients and adequate occlusion of vascular malform ations was achieved in four patients. After completion of therapy symp toms improved in four patients, while one patient suffered from abdomi nal pain due to cholangitis. One patient died seven months after the e mbolisation treatment from variceal bleeding, Mean cardiac output sign ificantly decreased from 14.2 (range 12 - 17.3) 1/min to 8 (range 5.9 - 10.6) 1/min (p = 0.043). After a median follow up of 23 months (rang e 7 - 50 months), three of five patients had a long lasting improvemen t of clinical symptoms and cardiac function. Conclusions - This first treatment series of patients with dominant hepatic involvement in Osle r's disease indicates that arterial embolisation may prevent cardiac f ailure by significantly lowering cardiac output.