Fontan Operation: A Comprehensive Review

Abstract

Since the first description of the Fontan operation in the early 1970s, a number of modifications have been introduced and currently staged, total cavopulmonary connection with fenestration has become the most commonly used multistage surgery in diverting the vena caval blood flow into the lungs. The existing ventricle, whether it is left or right, is utilized to supply systemic circuit. During Stage I, palliative surgery is performed, usually at presentation in the neonatal period/early infancy, on the basis of pathophysiology of the cardiac defect. During Stage II, a bidirectional Glenn procedure is undertaken in which the superior vena caval flow is diverted into the lungs at an approximate age of 6 months. During Stage IIIA, the blood flow from the inferior vena cava (IVC) is rerouted into the pulmonary arteries, typically by an extra-cardiac conduit along with a fenestration, generally around 2 years of age. During Stage IIIB, the fenestration is closed by transcatheter methodology 6–12 months after Stage IIIA. The evolution of Fontan concepts, the indications for Fontan surgery, and the results of old and current types of Fontan operation form the focus of this review.