[Anesthetic Management of a Patient with Pulmonary Atresia and Intact Ventricular Septum Accompanying Sinusoidal Communication].

We experienced a case of a patient with pulmonary atresia and intact ventricular septum (PAIVS) accompanying sinusoidal communication. PAIVS can be associated with coronary artery anomalies, including sinu- soidal communications from the right ventricle to coronary. In addition, the coronary circulation depends on the blood supply from the right ventricle. The patient was a 1-year-old boy with PAIVS (9.0 kg in weight and 74 cm in height). He had undergone balloon atrial septostomy, central shunt and Glenn procedure. Preopera- tive percutaneous oxygen saturation was 85% which decreased to 80% with agitation. He was scheduled for cheiloplasty. The goals of anesthetic management for the patient were to maintain a decrease in pulmonary vascular resistance and an increase in pulmonary blood flow to sinusoidal communications. Stable Pa0(2) was maintained by adjusting FI0(2) : 0.5, and Paco(2) : 30-35 mmHg. Hemodynamics was maintained by fluid trans- fusion, maintaining urine volume and alpha(1)-adrenoceptor agonist We used Aesculon(R) for monitoring cardiac output which is a noninvasive cardiac function monitor based on impedance technology.