Perioperative Management of the Fontan Operation in an Adolescent With a Single Lung

Abstract

The Fontan operation is performed to correct complex cardiac malformations characterized by single ventricle physiology. Following the procedure, the systemic venous blood flows directly into the lungs without passing through the single ventricle. Consequently, the Fontan circulation requires passive venous flow to the lungs, making it imperative for the pulmonary vascular resistance to be kept low in order to maintain adequate pulmonary blood flow and cardiac output. Given that the pulmonary circulation is important for a successful Fontan physiology, it would be intuitive that a single lung Fontan operation would do poorly because of the major loss in pulmonary vascular bed and the subsequent increase in pulmonary vascular resistance. The authors present a 14-year-old adolescent who was born with tricuspid atresia, pulmonary atresia, and right ventricular hypoplasia who underwent a successful Fontan operation into a single right lung, the left pulmonary artery being hypoplastic and disconnected from the...