[A case report of infective endocarditis complicated with Klippel-Trenaunay-Weber syndrome]

We reported an experience of successful aortic valve replacement due to active infective endocarditis complicated with Klippel-Trenaunay-Weber (KTW) syndrome which was characterized by limb hypertrophy, hemangioma, arteriovenous fistula and varicose veins. A 27-year-old man was admitted to our hospital because of severe dyspnea and high grade fever. Echocardiogram revealed severe aortic regurgitation and destruction of aortic valve due to active infective endocarditis. We performed aortic valve replacement and patch closure of annular abscess. As to vascular malformation of lower limb including arteriovenous fistula and varicose veins, surgical treatment was not undergone to avoid postoperative limb dysfunction. Although the origin of infective endocarditis was uncertain, the patient had peripheral vascular malformations. It was postulated that valvular endocardial injury might be occurred by cardiac volume overload due to arteriovenous fistula. The patients with KTW syndrome should be followed under careful observation since infective endocarditis may be one of the complications of the syndrome.