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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        • Fontan F.
        • Baudet E.
        Surgical repair of tricuspid atresia.
        Thorax. 1971; 26: 240-248
        • Wald R.M.
        • Mertens L.L.
        Hypoplastic left heart syndrome across the lifespan: clinical considerations for care of the fetus, child, and adult.
        Can J Cardiol. 2022; 38: 930-945
        • Freud L.R.
        • Seed M.
        Prenatal diagnosis and management of single-ventricle heart disease.
        Can J Cardiol. 2022; 38: 897-908
        • Haller C.
        • Barron D.J.
        Surgical strategies in single ventricle management of neonates and infants.
        Can J Cardiol. 2022; 38: 909-920
        • Selvanathan T.
        • Smith J.M.C.
        • Miller S.P.
        • Field T.S.
        Neurodevelopment and cognition across the lifespan in patients with single-ventricle physiology: abnormal brain maturation and accumulation of brain injuries.
        Can J Cardiol. 2022; 38: 977-987
        • Khoury M.
        • Cordina R.
        Exercise training for people living with Fontan circulation: an underutilized intervention.
        Can J Cardiol. 2022; 38: 1012-1023
        • Kasparian N.A.
        • Kovacs A.H.
        Quality of life and other patient-reported outcomes across the lifespan among people with Fontan palliation.
        Can J Cardiol. 2022; 38: 963-976
        • Heidendael J.F.
        • Engele L.J.
        • Bouma B.J.
        • et al.
        Coagulation and anticoagulation in Fontan patients.
        Can J Cardiol. 2022; 38: 1024-1035
        • Rathgeber S.L.
        • Lam C.
        • Harris K.C.
        • Grewal J.
        Hepatic and renal consequences of single-ventricle physiology palliated with the Fontan operation.
        Can J Cardiol. 2022; 38: 1002-1011
        • Lam C.Z.
        • Nguyen E.T.
        • Yoo S.-J.
        • Wald R.M.
        Management of patients with single-ventricle physiology across the lifespan: contributions from magnetic resonance and computed tomography imaging.
        Can J Cardiol. 2022; 38: 946-962
        • Kamp A.N.
        • Nair K.
        • Fish F.A.
        • Khairy P.
        Catheter ablation of atrial arrhythmias in patients post-Fontan.
        Can J Cardiol. 2022; 38: 1036-1047
        • Mondesert B.
        • Moore J.P.
        • Khairy P.
        Cardiac implantable electronic devices in the Fontan patient.
        Can J Cardiol. 2022; 38: 1048-1058
        • Neethling E.
        • Heggie J.E.
        Considerations in critical-care and anesthetic management of adult patients living with Fontan circulation.
        Can J Cardiol. 2022; 38: 1100-1110
        • Wichert-Schmitt B.
        • d’Souza R.
        • Silversides C.K.
        Reproductive issues in patients with the Fontan operation.
        Can J Cardiol. 2022; 38: 921-929
        • Perrin N.
        • Dore A.
        • van de Bruaene A.
        • et al.
        The Fontan circulation: from ideal to failing hemodynamics and drug therapies for optimization.
        Can J Cardiol. 2022; 38: 1059-1071
        • Mackie A.S.
        • Veldtman G.R.
        • Thorup L.
        • Hjortdal V.E.
        • Dori Y.
        Plastic bronchitis and protein-losing enteropathy in the Fontan patient: evolving understanding and emerging therapies.
        Can J Cardiol. 2022; 38: 988-1001
        • Townsend M.
        • Jeewa A.
        • Adachi I.
        • et al.
        Ventricular assist device use in single-ventricle circulation.
        Can J Cardiol. 2022; 38: 1086-1099
        • Dipchand A.I.
        • Honjo O.
        • Alonso-Gonzalez R.
        • McDonald M.
        • Roche S.L.
        Heart transplant indications, considerations, and outcomes in Fontan patients: age-related nuances, transplant listing, and disease-specific indications.
        Can J Cardiol. 2022; 38: 1072-1085