Key result
Single-lung fenestrated Fontan maintains good 2-year functional status without complications in a pediatric case.
Why the study?
The Fontan procedure requires optimal cardiovascular and pulmonary conditions, but its feasibility and outcomes in patients with single-lung physiology remain uncertain.
Is the single-lung Fontan procedure feasible and safe in children with single-lung physiology?
Case Report (n=1)
Is the single-lung Fontan procedure feasible and safe in children with single-lung physiology?
Single-lung Fontan completion may be feasible in carefully selected pediatric patients with single-lung physiology following meticulous multidisciplinary evaluation.
Answers are grounded in this paper and cite their sources.
Supports feasibility in select single-lung cases; leaves open long-term durability and broader applicability.
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.
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Guerrero et al. (2026) conducted a case report in Tricuspid atresia, pulmonary atresia, and functional single-lung circulation (n=1). Extracardiac fenestrated single-lung Fontan procedure was evaluated on Early and follow-up survival, functional status, and complications. An extracardiac fenestrated single-lung Fontan procedure was successfully performed in a 6-year-old boy, who remained in good functional status without complications at 2 years of follow-up.
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