The single ventricle presenting late: surgical options

The term single ventricle (SV) comprises a wide variety of congenital anomalies with a structurally or functionally univentricular heart where a biventricular repair is not possible or undesirable. The surgical management of these patients is often staged and the ultimate palliation is the Fontan operation (FO). Despite advances in surgical techniques, significant challenges persist in the management of SV patients, particularly in low- and middle-income countries (LMICs), where delayed presentation and limited healthcare access are common. This review explores current management strategies for patients beyond childhood, including those who have never undergone palliation and post-Fontan patients facing long-term complications. Key topics include the surgical approaches to SV, such as pulmonary artery banding (PAB), bidirectional Glenn (BDG), and FO, with particular focus on considerations for late-presenting adults in LMICs. The disparities in healthcare resources between high-income countries and LMICs are highlighted, demonstrating that resource constraints significantly impact timely interventions and on-going follow-up care.