Cardiac Implantable Electronic Devices in the Fontan Patient |
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| Institution: | 1. Adult Congenital Heart Disease Center, Montreal Heart Institute, Medicine Department, Université de Montréal, Montréal, Québec, Canada;2. Division of Cardiology, Department of Medicine, Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, California, USA;3. UCLA Cardiac Arrhythmia Center, UCLA Health System, Los Angeles, California, USA;4. Division of Cardiology, Department of Pediatrics, UCLA Health System, Los Angeles, California, USA;1. Department of Psychiatry and Behavioural Neurosciences, McMaster University, Hamilton, Ontario, Canada;2. School of Rehabilitation Science, McMaster University, Hamilton, Ontario, Canada;3. Child Health Evaluative Science, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada;4. Factor-Inwentash Faculty of Social Work, University of Toronto, Toronto, Ontario, Canada;5. Transplant and Regenerative Medicine Centre, The Hospital for Sick Children, Toronto, Ontario, Canada;1. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA;2. Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA;1. Jewish General Hospital, MAUDE Unit, McGill University, Montréal, Québec, Canada;2. Toronto Adult Congenital Heart Disease Program, Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada;3. University Health Network, Toronto, Ontario, Canada;4. Ohio State University Medical Center, Columbus, Ohio, USA;5. St. Boniface General Hospital, University of Manitoba, Winnipeg, Manitoba, Canada;6. Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada;7. McGill University Health Centre, Montréal, Québec, Canada;1. Department of Pediatrics (Neurology), The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada;2. Department of Pediatrics (Neurology), BC Children’s Hospital and University of British Columbia, Vancouver, British Columbia, Canada;3. Division of Neurology, Vancouver Stroke Program, University of British Columbia, Vancouver, British Columbia, Canada;1. Center for Heart Disease and Mental Health, Heart Institute and the Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA;2. Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA;3. Equilibria Psychological Health, Toronto, Ontario, Canada |
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| Abstract: | As a result of remarkable progress in operative techniques and cardiology care during childhood, Fontan patients continue to age and require team-based multidisciplinary expertise to manage complications encountered in adulthood. They face particular challenges in terms of altered hemodynamic stressors, cardiac and hepatic failure, and arrhythmias. Arrhythmias in Fontan patients are highly prevalent and associated with underlying anatomy, surgical technique, and postoperative sequelae. Diagnostic tools, treatments, and device strategies for arrhythmias in Fontan patients should be adapted to the specific anatomy, type of surgical repair, and clinical status. Great strides in our understanding of arrhythmia mechanisms, options and techniques to obtain access to relevant cardiac structures, and application of both old and new technologies have contributed to improving cardiac implantable electronic device (CIED) therapies for this unique population. In this state-of-the-art review, we discuss the various arrhythmias encountered in Fontan patients, their diagnosis, and options for treatment and prevention, with a focus on CIEDs. Throughout, access challenges particular to the Fontan circulation are considered. Recently developed technologies, such as the subcutaneous implantable cardioverter defibrillator, carry the potential to be transformative but require awareness of Fontan-specific issues. Moreover, new leadless pacing technology represents a promising strategy that may soon become applicable to Fontan patients with sinus-node dysfunction. CIEDs are essential tools in managing Fontan patients, but the complex clinical scenarios that arise in this patient population are among the most challenging for the electrophysiologist treating patients with congenital heart disease. |
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