HEREDITARY HEMORRHAGIC TELANGIECTASIA - EFFECTIVE PROTOCOL FOR EMBOLIZATION OF HEPATIC VASCULAR MALFORMATIONS - EXPERIENCE IN 5 PATIENTS

PURPOSE: To develop a hepatic artery embolization protocol and investi gate its efficacy in a prospective study treating patients with heredi tary hemorrhagic telangiectasia and predominant hepatic involvement. M ATERIALS AND METHODS: One man and four women with hereditary hemorrhag ic telangiectasia presented with symptoms of high-output heart failure , abdominal angina, or severe portal hypertension. The hepatic arterie s were embolized in stages in three to five sessions at 1- to 15-week intervals. After peripheral embolization with polyvinyl alcohol partic les, proximal arteries were embolized with coils. Computed tomography and assessment of cardiac output were performed before and after thera py and at the end of follow-up (median, 25 months; range, 12-55 months ). RESULTS: After embolization, analgesics and antiemetics were necess ary for a median of 5 and 2 days, respectively. Other than ischemic ch olangitis (one patient), no complications were observed. The mean card iac output decreased significantly (P < .05) from 14.2 L/min to 8.0 L/ min. Symptoms of high-output heart failure, abdominal angina, and port al hypertension resolved in all patients. Seven months after embolizat ion, one patient died of postoperative sepsis after an unsuccessful su rgical attempt to create a portacaval shunt. Delayed recurrence of sym ptoms was not noted in the other patients. CONCLUSION: In symptomatic patients with hereditary hemorrhagic telangiectasia and predominant he patic involvement embolization of the hepatic arteries in stages is we ll tolerated by the patients and results in good clinical improvement at midterm follow-up.