Management of pulmonary blood flow for a patient with congenital pulmonary vein stenosis undergoing pulmonary venous obstruction release and pulmonary artery banding

Congenital pulmonary vein stenosis (CPVS) is a rare fetal congenital heart disease with a prevalence of 1.7 per 100,000 children younger than two years of age. Because of the difficulty of maintaining the pulmonary blood flow, CPVS is associated with a 50% survival rate within five years of diagnosis. We describe a successful management of pulmonary blood flow for a 4-month-old-girl with CPVS, combined with atrial septal defect and ventricular septal defect undergoing pulmonary vein obstruction release (PVOR). In this case, CPVS was the only cause for pulmonary hypertension because there was no significant pressure gradient between each pulmonary capillary wedge pressure and the paired pulmonary vein pressure, indicating the normal pulmonary vascular structure prior to pulmonary vein stenosis. As pulmonary blood flow was estimated to be high after PVOR, pulmonary artery banding was also performed. Management of pulmonary blood flow is the most important issue for anesthesia of this surgery, especially in postcardiopulmonary bypass period, when the pulmonary vasoconstriction is induced by endothelial dysfuncion.