In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.
Keywords: Down syndrome; Fontan circulation; biventricular repair; double-inlet left ventricle; heterotaxy syndromes; hypoplastic left heart syndrome; mitral atresia with normal aortic root; single ventricle; tricuspid atresia; unbalanced atrioventricular septal defect.