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Canadian Journal of Cardiology

The Implications of Single-Ventricle Heart Disease Across the Lifespan

      Since the initial description of the Fontan operation in 1971
      • Fontan F.
      • Baudet E.
      Surgical repair of tricuspid atresia.
      for patients with single-ventricle physiology, our understanding of the modified natural history of this population has grown immensely. Initial advances in optimising surgical techniques based on the most common early complications were aimed at surviving childhood. Over time, some patients began to survive into early adulthood, which led to the focus on important sources of morbidity in early adulthood—specifically Fontan failure, ventricular dysfunction, and arrhythmias. In turn, this led paediatric and adult congenital cardiologists to concentrate on improving medical management and interventional approaches, including catheter ablation. The comorbidities associated with long-term venous hypertension are now becoming clearer. The failing Fontan remains a challenge for cardiologists across the lifespan, and medical therapies and advances in ventricular assist devices and heart transplantation have emerged as important options in the therapeutic armamentarium for this difficult problem. In this issue of Canadian Journal of Cardiology, we have assembled a collection of manuscripts that encapsulate current understanding of Fontan physiology, the clinical progression of these patients over the lifespan, and current approaches to deal with the complexities that arise in this circulatory state.
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