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The population of adults with congenital heart disease (CHD) continues to grow in size but also evolves in anatomic and complexity case composition. Most patients with significant CHD are nowadays diagnosed prenatally, what often enables safe delivery and even occasionally intrautero therapy. There has been ongoing innovation and improvement of surgical and percutaneous interventions matched with better long-term follow-up and, with it, better understanding and treatment of late sequelae. The resulting survival benefit is most striking in patients with complex lesions, such as those born with a ‘single ventricle’.
Single-ventricle physiology, also called ‘univentricular circulation,’ ‘common ventricle’ or ‘functionally single ventricle’ encompasses several groups of lesions, characterised by the lack of two well-developed ventricles, one of which is typically hypoplastic or rudimentary. Therefore, lesions with two well-formed ventricles, which cannot be septated for other reasons, are not included in this definition.1 The hypoplastic left heart syndrome is included in the single-ventricle group, despite the different surgical strategy required.
Survival prospects and historical perspective
Survival prospects in patients with single ventricle have changed dramatically over the last four decades due to the advent of better and earlier diagnosis and advances in cardiac surgery. Surgical intervention is performed on one or several stages, and the final target is usually that of establishing a ‘Fontan circulation’ with systemic venous blood returning to the pulmonary arteries either through the right atrium or directly, but without the interposition of a subpulmonary ventricle. This approach was pioneered by Francis Fontan in Bordeaux, in 1968 and was first published in 1971.2 In its original version, the Fontan operation consisted of the superior vena cava being connected to the right pulmonary artery, …
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