Aortic valve replacement for the patient with Osler-Rendu-Weber disease

Osler Rendu-Weber (O-R W) disease (hereditary haemorrhagic telangiectasia) is characterized by an anormality of the peripheral blood vessels which often causes recurrent epistaxis. We presented a case of aortic valve replacement for the patient with O-R-W disease. A 75-year-old woman diagnosed as O-R-W disease with chest pain and dyspnea was admitted. Preoperative transthoratic echocardiography showed severe aortic valve stenosis (AS) with a pressure gradient (from left ventricle to ascending aorta) of 72.5 mmHg. Urgent aortic valve replacement (AVR) was performed after preoperative hormonal (estrogen-progesterone) therapy, that might be effective for bleeding from vascular ectasia. No hemorrhagic tendency was recognized and the postoperative course was uneventful.