General anesthesia in a patient with Parkes Weber syndrome with high-output cardiac failure due to multiple arteriovenous fistulas complicated by severe aortic regurgitation

Jun Ninagawa, Yoshitsugu Yamada

Parkes Weber syndrome is a rare disease characterized by overgrowth of an extremity linked to the presence of an arteriovenous malformation with multiple arteriovenous fistulas (AVFs). We report a patient with Parkes Weber syndrome with high-output cardiac failure due to multiple AVFs complicated by severe aortic regurgitation (AR) who required surgical treatment for AVFs. Division of the left deep femoral artery and banding of the left superficial femoral artery were performed. Such procedures can cause aggravation of AR and left ventricular failure due to the sudden increase in cardiac afterload. Pulmonary artery pressure, mixed venous oxygen saturation and cardiac index monitored by a thermodilution catheter, and a transesophageal echocardiography were useful in evaluating the effect of the surgical procedure and resultant acute increase in cardiac afterload on cardiac output and left ventricular function.