The Fontan Circulation: From Ideal to Failing Hemodynamics and Drug Therapies for Optimization |
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| Institution: | 1. Structural Heart Intervention Program, Université de Montréal, Montréal, Québec, Canada;2. Cardiology Division, Geneva University Hospitals, Geneva, Switzerland;3. Adult Congenital Heart Centre, Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada;4. Division of Congenital and Structural Cardiology, University Hospitals Leuven, Leuven, Belgium;5. Department of Cardiac Surgery, CHU Sainte Justine, Université de Montréal, Montréal, Québec, Canada;6. Division of Pediatric Cardiology, CHU Sainte Justine, Université de Montréal, Montréal, Québec, Canada;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. 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. Stollery Children’s Hospital, Edmonton, Alberta, Canada;2. The Hospital for Sick Children, Toronto, Ontario, Canada;3. Texas Children’s Hospital, Houston, Texas, USA;4. BC Children’s Hospital, Vancouver, British Columbia, Canada;5. Mazankowski Heart Institute, University of Alberta, Edmonton, Alberta, Canada;1. Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada;2. Joint Department of Medical Imaging, University Health Network, Toronto, Ontario, Canada;3. Peter Munk Cardiac Centre, University Health Network,University of Toronto, Toronto, Ontario, Canada |
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| Abstract: | Fontan palliation results in a hemodynamically complex circulation with multisystem consequences, which in the long term adversely affect many body processes. Systemic venous hypertension, nonpulsatile low-shear pulmonary blood flow, and low cardiac output are the 3 main characteristics of a Fontan circulation, leading to unavoidable slowly progressive failure. An appreciation of how the hemodynamics of a Fontan circulation change with time and relate to the various modes of Fontan circulatory failure is important. Accurate hemodynamic assessment aid this understanding and may permit early identification of potentially treatable drivers of decline. While no evidence-based or guideline-directed pharmacologic management strategy has been established in Fontan patients, understanding the hemodynamics of Fontan circulation failure will assist in the rational selection of potentially helpful drug therapies for individual patients. In this review, we present hemodynamic concepts of the optimal Fontan physiology and Fontan circulatory failure, review practical aspects of invasive hemodynamic assessment, and discuss the role of drug therapies in increasing systemic venous blood flow return and decreasing ventricular filling pressures in Fontan circulation. Often complementary to catheter-based or surgical interventions, pharmacologic management aims at preserving patency of the circuit, adequate systolic and diastolic ventricular function, atrioventricular valve function, an unobstructed ventricular outflow tract, and pulmonary vascular integrity in order to maintain an acceptable cardiac output. |
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