The Fontan Procedure in Patients with Single-Lung Physiology: Insights from a Case Report and Scoping Review

The Fontan operation represents the third stage of single-ventricle palliation and requires optimal cardiovascular and pulmonary conditions to achieve success. We report a 6-year-old boy with tricuspid atresia, pulmonary atresia, and functional single-lung circulation secondary to severe left pulmonary artery (LPA) hypoplasia and disconnection after previous palliation. Following comprehensive clinical, hemodynamic, and anatomic evaluation, including unsuccessful attempts at LPA recruitment, the patient underwent an extracardiac fenestrated single-lung Fontan procedure. At 2 years of follow-up, he remained in good functional status without Fontan-related complications or the need for reintervention. Additionally, we present a scoping review of published case reports and case series of children with single-lung physiology undergoing the Fontan procedure, focusing on early and follow-up survival. This case, together with the available literature, suggests that single-lung Fontan completion may be feasible in carefully selected patients following meticulous multidisciplinary evaluation, although long-term outcomes remain uncertain.