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Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, AustraliaMurdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Genomic Medicine, Royal Melbourne Hospital, Melbourne, Vic, AustraliaDepartment of Medicine, University of Melbourne, Melbourne, Vic, Australia
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, AustraliaFaculty of Medicine and Health, University of Sydney, Sydney, NSW, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Cardiac Surgery, Royal Children’s Hospital, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, Australia
Heart Centre for Children, Sydney Children’s Hospitals Network, Sydney, NSW, AustraliaSydney Medical School, University of Sydney, Sydney, NSW, Australia
Heart Centre for Children, Sydney Children’s Hospitals Network, Sydney, NSW, AustraliaSydney Medical School, University of Sydney, Sydney, NSW, Australia
Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, AustraliaDepartment of Medicine, University of Melbourne, Melbourne, Vic, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, AustraliaDepartment of Cardiology, Royal Children’s Hospital, Melbourne, Vic, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, AustraliaDepartment of Cardiology, Royal Children’s Hospital, Melbourne, Vic, Australia
Faculty of Medicine, University of Queensland, Brisbane, Qld, AustraliaQueensland Paediatric Cardiac Research, Children’s Health Queensland, Brisbane, Qld, Australia
Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, AustraliaDepartment of Medicine, University of Melbourne, Melbourne, Vic, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, AustraliaDepartment of Gastroenterology and Clinical Nutrition, Royal Children’s Hospital, Melbourne, Vic, Australia
Department of Paediatrics, University of Melbourne, Melbourne, Vic, AustraliaVictorian Paediatric Palliative Care Program, Royal Children’s Hospital, Melbourne, Vic, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Cardiac Surgery, Royal Children’s Hospital, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, Australia
Department of Genomic Medicine, Royal Melbourne Hospital, Melbourne, Vic, AustraliaDepartment of Pathology, University of Melbourne, Melbourne, Vic, Australia
Faculty of Medicine, University of Queensland, Brisbane, Qld, AustraliaQueensland Paediatric Cardiac Research, Children’s Health Queensland, Brisbane, Qld, Australia
Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, AustraliaDepartment of Medicine, University of Melbourne, Melbourne, Vic, Australia
Heart Centre for Children, Sydney Children’s Hospitals Network, Sydney, NSW, AustraliaDiscipline of Paediatrics, School of Women’s and Children’s Health, UNSW Medicine, University of New South Wales, Sydney, NSW, Australia
Heart Centre for Children, Sydney Children’s Hospitals Network, Sydney, NSW, AustraliaDiscipline of Paediatrics, School of Women’s and Children’s Health, UNSW Medicine, University of New South Wales, Sydney, NSW, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, AustraliaDepartment of Haematology, Royal Children’s Hospital, Melbourne, Vic, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, AustraliaDepartment of Cardiology, Royal Children’s Hospital, Melbourne, Vic, Australia
Heart Centre for Children, Sydney Children’s Hospitals Network, Sydney, NSW, AustraliaSydney Medical School, University of Sydney, Sydney, NSW, Australia
Department of Medicine, University of Melbourne, Melbourne, Vic, AustraliaDepartment of Gastroenterology and Hepatology, Royal Melbourne Hospital, Melbourne, Vic, Australia
Murdoch Children’s Research Institute, Melbourne, Vic, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, Vic, AustraliaDepartment of Cardiology, Royal Children’s Hospital, Melbourne, Vic, Australia
Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, AustraliaDepartment of Medicine, University of Melbourne, Melbourne, Vic, Australia
Faculty of Medicine and Health, University of Sydney, Sydney, NSW, AustraliaHeart Centre for Children, Sydney Children’s Hospitals Network, Sydney, NSW, Australia
4 Person or family member of a person with a Fontan circulation.
Angela Wood
Footnotes
3 Writing Committee. 4 Person or family member of a person with a Fontan circulation.
Affiliations
Department of Cardiology, Royal Children’s Hospital, Melbourne, Vic, Australiaongenital Heart Alliance of Australia and New Zealand Registry Board, Melbourne, Vic, Australia
The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival.
Keypoints
•
Lifelong quality medical care with access to multidisciplinary services, is of prime importance. Care includes regular tests for surveillance of health status.
•
Transition from paediatric to adult care is an active process that should commence during early adolescence and continue until successful engagement with adult congenital cardiology care.
•
Children and adults with a Fontan circulation often have reduced peak exercise capacity (on average, 60–65% of predicted values). Increasingly, evidence suggests exercise training may improve exercise capacity and cardiovascular function.
•
People with a Fontan circulation have higher rates of anxiety and behavioural disorders, and there needs to be a low threshold for the provision of mental health care.
•
Pregnancy has increased maternal and fetal risks, and pre-conception multidisciplinary assessment and counselling is essential.
•
Atrial arrhythmias are common, often late after Fontan surgical repair and due to intra-atrial re-entry or “flutter” mediated by atrial stretch and scarring. Some anti-arrhythmic agents, most classically the type IC drugs, may allow haemodynamically unstable, life-threatening 1:1 AV conduction.
•
Anticoagulation with warfarin is routine care in patients with atrial arrhythmias.
•
In patients with recurrent atrial arrhythmias, catheter ablation or surgical conversion may be considered.
•
The Fontan circulation is an ideal substrate for thrombus formation and may result in intracardiac or intravascular thrombosis, ischaemic stroke, or other embolic phenomena. Antiplatelet and anticoagulant agents are commonly prescribed for thromboprophylaxis in patients with a Fontan circulation. Evidence suggests that treatment with one of these agents is advantageous, but there is no consensus on which is optimal. Despite treatment, symptomatic thromboembolic events are associated with significant mortality.
•
Heart failure is the leading cause of morbidity and mortality. Diuretics provide symptomatic relief, however standard heart failure medical therapy is not of proven benefit.
•
Though not well understood, there is increasing concern regarding progressive liver disease with a long-term risk of hepatocellular carcinoma.
•
Despite early higher mortality post heart transplant, these individuals have better long-term survival outcomes compared with many other heart transplant recipients.
This position statement is a consensus undertaking that aims to provide expert opinion regarding the care of people with a Fontan circulation in Australia and New Zealand. Recognising that there is limited high-level evidence for the care of these people, this document has been developed as a position statement, rather than a guideline, and it does not provide ratings regarding the level of evidence for its statements. This model has been successfully applied for other chronic conditions [
]. In the absence of high-level evidence for this population, many sections of this document describe a common, rather than a universal, approach to management. The authors recognise that different centres across Australia and New Zealand have different levels of expertise and experience in certain aspects of management, which can make important contributions to local decision making.
The concept for this document was developed during a meeting of the Australian and New Zealand (ANZ) Fontan Registry Steering Committee in September 2017. This Steering Committee oversees the ANZ Fontan Registry. The registry was created in 2009 and at the latest census date (2017) comprised 1,574 participants [
Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand.
A group of clinicians (the Chair and three Co-Chairs) was established, and decisions regarding content and expert contributors were made by discussion. In early 2018, the Cardiac Society of Australia and New Zealand (CSANZ) was notified of the intention to create this document, through the Quality Standards Committee. Expert contributors were approached and asked to work on a specific aspect of the document. Representation was also sought from people with a Fontan circulation and their family members; these representatives reviewed the document as a whole, ensuring a patient- and family-centred approach. The document was additionally reviewed by a non-congenital heart disease (non-CHD) cardiologist.
Conflicts of Interest
All members of the writing group were asked to declare any potential conflict of interest. Conflicts of interest were considered broadly to include any relationship, whether financial or otherwise, between any contributing author and any other entity that either would or might be perceived to have influenced the author’s contribution to the content of this document. Declared conflicts of interest are noted at the end of the document.
Rationale for a Position Statement
As survival of people with a Fontan circulation increases, so too does recognition of the potential challenges in managing their health [
Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand.
]. Clinicians are often faced with meeting the needs of this population while not yet being able to appreciate a “whole-of-life” health trajectory. People with a Fontan circulation and their families expect and need education about their condition, assistance with self-management, professional care, and assessment of their capacity to interact with life in all its joys and challenges. People with a Fontan circulation, their families, and clinicians need to balance optimism with appropriate and timely care, aiming to allow these individuals to live life fully while incorporating the reality that aspects of their future wellbeing remain unknown.
This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. Congenital cardiology lacks the large evidence base that is available to many other cardiology subspecialties. Consequently, in producing this document, the input of local experts has been sought, in partnership with patient representatives, to create a consensus care statement that draws together evidence, where available, with experience and wisdom. To date, only a single guideline dedicated to the management of people with a Fontan circulation has been published [
], which is now more than 20 years old. In reviewing the literature, contributors were asked to concentrate on more recent publications, where available. This document aims to be both comprehensive and accessible but also realistic about care within the Australian and New Zealand health care environment.
As this document only considers the care of people with a Fontan circulation, the first part of an individual’s and his or her family’s journey—the pre-Fontan phase—has not been included. However, the authors recognise that the pre-Fontan period also has significant challenges, that people in this phase will benefit from expert multidisciplinary care, and that it likely creates the foundations for many of the subsequent challenges described herein.
1 Introduction
The Fontan circulation is a broad term for the circulatory state that results after one of several operations where systemic venous return is directed to the lungs without an intervening active pumping chamber. Named after Dr Francis Fontan [
], this type of surgery was first undertaken in New Zealand in 1975 and in Australia in 1980.
1.1 Challenges of Having a Chronic Health Condition
There is a significant body of work on chronic disease, but less attention has been paid to the multidimensional “whole-of-life” experience for individuals born with a critical illness, who have never been completely healthy. People with a Fontan circulation or other forms of congenital heart disease (CHD) tend to see themselves as more well and less limited than objective evidence would suggest [
]. A meta-analysis exploring chronic childhood diseases and emotional outcomes showed a small increased risk of emotional difficulties that persisted into early adulthood [
]. Although this document focusses largely on the physical disease and its treatment, this should not detract from an understanding that it may be more beneficial to speak with people with a Fontan circulation and their families in terms of achieving and maintaining wellbeing. Additionally, this document assumes that established health maintenance advice for the general population will occur alongside disease-specific treatment.
1.2 The Challenge of Maximising Opportunities While Faced With an Uncertain Future
For health care providers, the challenge is to maintain optimism [
]. Ideally, this necessitates a close therapeutic relationship that involves non-paternalistic and respectful information exchange, including, at times, respect for the individual’s wish to not know more detailed information. Honesty is required about what is unknown, with acknowledgement that uncertainty in illness may itself be a significant source of distress for people with a Fontan circulation and their carers [
]. Although perhaps self-evident, recognition of this should underpin heath care providers’ interactions with patients and their encouragement of patients’ active involvement and participation in life and medical decision making [
1.3 The Fontan Circulation and a Whole-of-Life Trajectory Overview
One of the greatest impacts for a child with a Fontan circulation is the potential for adverse interaction between medical events and participation in education. Physical limitations may prevent or reduce the child’s capacity to interact with peers through play and sporting activities. There are also impacts for parents and siblings, particularly in terms of parental stress and changes in family functioning. The effects of cardiopulmonary bypass and neonatal ill health on neurodevelopment remain active areas of research, and sequelae of this may further complicate school transition and participation.
Adolescence is a challenging life milestone for many people, regardless of their health status [
]. Looking after adolescents requires being comfortable with listening to and encouraging open conversations about sexual maturity and activity, contraception, and the use of alcohol, cigarettes, and illicit drugs. Adolescents with health problems may also seek participation in treatment decision making, which may challenge their parents or carers [
]. It is important to encourage this move to autonomy and independence, recognising this transition as a milestone in personal development, while maintaining a safety net of availability, information, and support [
]. This often occurs when commencing higher education or employment and while navigating the creation of significant personal relationships, parenthood, and other roles and responsibilities [
]. For adults with a Fontan circulation, employment opportunities may be affected by the need for medical appointments and interventions and, for some, will be limited by physical ability. These limitations may also affect participation in sport and leisure activities and contribute to personal concerns about future parenthood.
Life expectancy is likely to gain increasing focus, particularly for individuals who experience significant complications or deterioration, with recent research highlighting this as a primary concern for people with a Fontan circulation [
]. It is important to recognise that, although we do not know how long people with a Fontan circulation will live, the expectation is that their life expectancy will be lower than for an age- and sex-matched healthy comparison group. As such, access to specialised psychological and palliative care support services should be available, as the need arises.
2 A Consumer Perspective: Comments From People With a Fontan Circulation and Their Parents
For people with a Fontan circulation and their families, access to lifelong quality medical and mental health care is of prime importance. Quality care is recognised as being provided by trained specialists through central and regional services [
Quality care is based on a dynamic partnership between the person with a Fontan circulation and his or her family and caregivers. It is a partnership that values the individual by providing clear communication, patience in educating and explaining medical terminology frequently (potentially at every appointment), and active engagement with the patient and his or her family in decision making across the care trajectory. An actively engaged patient and family can better advocate for their needs and wishes and are more likely to remain engaged as the transition to independent adult care occurs [
]. As active agents in their care, people with a Fontan circulation and their families also value their right to participate in and contribute to research opportunities, should they arise, in consultation with their care team.
People with a Fontan circulation aim to live full and active lives outside their clinical appointments, and consideration of their wellbeing, mental health, and broader social and cultural context is important. The greatest concerns focus on uncertainty about life expectancy [
] and exposure to hardship and suffering. Referral to appropriate services and linkage with community-based supports are of benefit to patients and families. The value of the “lived experience” should be recognised, and mechanisms such as actively seeking the input of representatives within the ANZ Fontan Registry has been found to support optimal health care, research, and development that incorporates perspectives from both people with a Fontan circulation and their families [
Care of people with a Fontan circulation is recognised to be complex and best supported by access to multidisciplinary specialists, as required. This may include referral to other medical specialties (e.g. haematology, gastroenterology, neurology, and endocrinology), as well as mental health and allied health services. People born with functionally single ventricles should also receive repeated assessment for neurodevelopmental impairment, with referral to early intervention services that support optimal long-term outcomes [
Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association.
]. Fractured service provision can be frustrating from a patient’s perspective, and communication between the multiple care providers is essential. For logistical and quality care reasons, the preference is typically for a centralised integrated care model, with specialist clinics incorporating the multidisciplinary team (i.e. the “one-stop shop” approach).
3 Medical Review
3.1 Overview
Everyone with a Fontan circulation requires lifelong regular medical surveillance and care, which is provided by a paediatric cardiologist in childhood and transitions to an adult CHD cardiologist in the teenage years. American and European guidelines for adult CHD recommend annual medical review, unless more frequent assessment is clinically indicated [
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
]. Although location of medical review is not stipulated, the importance of adult CHD imaging occurring at a service with CHD expertise is recognised [
]. This recommendation therefore often determines the location of care provision, especially for major patient care decision making. Importantly, non-cardiac surgery should ideally be performed in the patient’s CHD service hospital, with a cardiac anaesthetist; or, at a minimum, after consultation with the patient’s usual CHD cardiologist [
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
The elevated systemic venous pressure and restricted cardiac output that physiologically characterise the Fontan circulation are increasingly recognised as affecting other organ systems, most notably the liver and kidneys [
]. Screening for end-organ dysfunction is recommended, but there is no clearly defined age at which to start screening, nor is there sufficient information to define an optimum frequency of testing [
Clinical practice statements for people with a Fontan circulation are in their infancy and will evolve as population-based research becomes available. Having a benchmark for standards of care is an important starting point. Surveillance suggestions based on an Australasian clinician survey and literature review [
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
], are shown in Table 1. A timeline to help guide proactive commencement of counselling on different life care aspects for people with a Fontan circulation is presented in Figure 1.
Table 1Consensus Suggestions for Surveillance of “Well” Patients With a Fontan Circulation
These suggestions are based on two sources: a survey of paediatric and adult congenital heart disease clinicians on care of patients with a Fontan circulation, conducted through the Australian and New Zealand Fontan Registry [42]; and the 2018 American Heart Association guidelines for the management of adults with congenital heart disease [30].
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
, including a change from “well” Fontan status necessitating extensive assessment
General advice
Same age as general population
Vaccination Cardiovascular risk factors (primary prevention) Early referral for any other health care concerns (e.g. anxiety and depression)
Abbreviations: CT, computed tomography; ECG, electrocardiogram; MR, magnetic resonance imaging.
* These suggestions are based on two sources: a survey of paediatric and adult congenital heart disease clinicians on care of patients with a Fontan circulation, conducted through the Australian and New Zealand Fontan Registry
The long-term management of children and adults with a Fontan circulation: a systematic review and survey of current practice in Australia and New Zealand.
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
This figure is intended as a general guide to age ranges at which to commence counselling discussions about different aspects of care for the patient with a Fontan circulation. Counselling is recognised to be an ongoing process and needs to be individualised to each situation. It is anticipated that potential issues identified may extend throughout the entire life experience, and the age grouping only identifies the age at which these issues should usually start to be addressed.
An electrocardiogram (ECG) is a simple clinical surveillance tool for sinus node dysfunction, heart block, and other arrhythmias at follow-up visits. This is particularly relevant for those with atriopulmonary Fontan connections [
] or those in whom the original anatomy suggests an increased risk of development of sinus node or conduction abnormalities. Holter monitoring is useful when clinically indicated in the assessment of chronotropic competency. Its use in regular surveillance has been recommended in the latest American guidelines, but without clear evidence to support routine use [
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
]. Along with event and implantable loop recorders, the Holter monitor provides an additional option when arrhythmia is suspected.
3.3 Exercise Testing
The impact of lifelong adjustment to a different functional normality may manifest in various ways in people with a Fontan circulation. Under-reporting of symptoms and a discrepancy between subjective and quantitative assessment of functional status are common [
]. Repeated cardiopulmonary exercise testing is therefore a useful tool for regular surveillance. Exercise capacity in people with a Fontan circulation declines over time, and the rate of decline may be a better predictor of future adverse events than exercise performance at any one point in time [
]. There is no clear evidence on when to start exercise testing, or its optimal frequency, for the asymptomatic or minimally symptomatic patient.
3.4 Echocardiography
Transthoracic echocardiography is the fundamental imaging tool used in people with a Fontan circulation, given its widespread availability and low invasiveness [
]. Nevertheless, there are significant limitations to its use in this population that arise from poor image quality, especially in older patients, and reliance on geometric indices of ventricular size and function, which are not designed for heterogeneous ventricular morphologies [
Two-dimensional global longitudinal strain rate is a preload independent index of systemic right ventricular contractility in hypoplastic left heart syndrome patients after Fontan operation.
A preliminary comparison of two-dimensional speckle tracking echocardiography and pressure-volume loop analysis in patients with Fontan physiology: the role of ventricular morphology.
]. Transoesophageal echocardiography requires a general anaesthetic in young patients or conscious sedation in adults, both with associated risks. Its use should be reserved for situations where it will assist surgical planning, as an imaging adjunct in catheter intervention, or to rule out a thrombus in the heart before electrical cardioversion or after an embolic event [
Cardiac magnetic resonance imaging (CMR) has advantages over echocardiography in assessment of ventricular size and function, particularly for systemic right ventricles, and in its ability to review Fontan flow dynamics. Guidelines had previously left its use in surveillance to individual assessment [
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
Comparison of echocardiographic and cardiac magnetic resonance imaging measurements of functional single ventricular volumes, mass, and ejection fraction (from the Pediatric Heart Network Fontan Cross-Sectional Study).
]. Use in paediatric settings is limited by the usual requirement for a general anaesthetic until patients reach 10 to 12 years of age, although faster imaging sequences are increasingly allowing younger children to be assessed. The development of both exercise CMR assessment [
] and CMR-compatible pacemakers may increase its utility.
3.6 Cardiac Computed Tomography and Cardiac Catheterisation
As cardiac computed tomography (CT) and cardiac catheterisation involve radiation exposure, this needs to be considered, noting prior (and likely future) radiation exposure. CT can provide functional analysis but does not provide comprehensive information on flow haemodynamics [
]. These procedures therefore do not form part of regular surveillance but are useful adjuncts where echocardiography or CMR either cannot provide the information required or are contraindicated (see section 11).
3.7 Antibiotic Prophylaxis
Recommendations for antibiotic prophylaxis are the same as those for the general CHD patient population. Thus, antibiotic prophylaxis is recommended for patients with a history of infective endocarditis; for those with a prosthetic valve (regardless of mode of implant and valve type); for a 6-month period after surgery with prosthetic material; for patients with residual intracardiac shunts at the site of, or adjacent to, previous repair with prosthetic material or a device; and for those with uncorrected cyanotic heart disease [
2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
Prognostic value of exercise variables in 335 patients after the Fontan operation: a 23-year single-center experience of cardiopulmonary exercise testing.
Prognostic value of exercise variables in 335 patients after the Fontan operation: a 23-year single-center experience of cardiopulmonary exercise testing.
]. Whether interventions that increase exercise capacity reduce morbidity and mortality is unproven, but current evidence, albeit in small numbers of patients, suggests exercise training may improve exercise capacity and cardiovascular function.
4.2 Fontan Physiology, the Peripheral Muscle Pump and Exercise Limitations
In the absence of a subpulmonary ventricle, systemic venous blood flow to the pulmonary arteries is dependent on gravitational forces, respiration, and the peripheral muscle pump, in addition to cardiac function [
]. The absence of a subpulmonary pump also restricts preload and reduces exercise-related stroke volume increase—the major factor contributing to exercise limitation [
]. Cardiac filling is further compromised by abnormal pulmonary vascular development and dysfunction, secondary to non-pulsatile pulmonary blood flow [
]. Although it is becoming clear that the pathophysiology of exercise limitation is multifactorial, the relative contribution of each component is not well understood.
4.3 Benefits of Regular Moderate to Vigorous Exercise
About 10% of adults with a Fontan circulation are “super-Fontan” individuals, with normal or supranormal exercise capacity. Regular participation (at least three times a week) in moderate to vigorous sporting activities is characteristic of this group [
Six-minute walking test to assess exercise tolerance and cardiorespiratory responses during training program in children with congenital heart disease.
Motor training of sixty minutes once per week improves motor ability in children with congenital heart disease and retarded motor development: a pilot study.
], two included a Fontan control group and all but one involved aerobic-based interventions with or without a light resistance component. A 20-week high intensity whole-body resistance training program in a group of 11 adults with a Fontan circulation increased skeletal muscle mass by 2 kg and peak exercise capacity by 10%. Stroke volume increased at rest and during exercise in the trained state, likely due to improved venous return [
]. Combined aerobic and light resistance exercise has also been shown to enhance function of skeletal muscle afferent nerves that control blood flow and autonomic responses [
], and this may be another benefit of particular relevance to those with Fontan physiology. For children with a Fontan circulation, exercise training may improve ventilatory efficiency [
Inspiratory muscle training is associated with improved inspiratory muscle strength, resting cardiac output, and the ventilatory efficiency of exercise in patients with a Fontan circulation.
]. Additional potential benefits of exercise, such as improvements in quality of life, body image, mental health, weight, cardiac function, and endothelial function, have been shown in patients with other conditions [
]. Maintaining a healthy weight is especially important for those with a Fontan circulation because of the profound respiratory dependence of the circulation.
4.4 Safety
In the past, people with complex CHD were often advised against vigorous exercise because of unproven safety concerns. Experience with maximal exercise testing and exercise training has shown that acute arrhythmic events are rare and, when they do occur, are usually not associated with exertion [
]. In general, screening before an exercise program is recommended, with clinical assessment and exercise testing with oximetry to characterise peak exercise capacity, heart rate response, degree of desaturation, and any arrhythmia. Unless reversible by appropriate intervention, patients with frequent arrhythmias, a right ventricular-dependent coronary circulation, unstable HF, severe aortic dilatation, moderate to severe valve regurgitation or stenosis, outflow obstruction, or ventricular impairment are probably not suitable for moderate to vigorous levels of physical activity and may need close supervision in a hospital setting, even with lighter exercise prescription.
For patients with (non-CHD) biventricular HF, exercise prescription is incorporated into clinical care guidelines [
2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines.
], no adverse clinical events were recorded in 4 years of follow-up. This is particularly significant because systemic venous pressure may rise considerably during periods of vigorous activity, in contrast to the normal circulation [
]. It is unclear whether transient increases in venous pressure for short periods predispose to end-organ damage or if exercise-induced flow improves pulmonary vascular physiology and overall reduces resting venous pressure, as suggested by recent data [
Choice of sporting activity may need to include consideration of an associated increased risk of bleeding, which will affect people receiving anticoagulation therapy.
4.5 Exercise Prescription
Exercise programs should include both aerobic and resistance exercises (Table 2) and aim for at least 30 minutes a day on most days of the week. For children, exercise through game-based activities should be promoted. At all ages, adherence is improved by enjoyment of the exercise program.
Table 2Training Intensity Based on Cardiac Abnormalities to Guide Exercise Prescription
Evidence shows that between 21% and 76% of patients experience a lapse in regular follow-up cardiology care after transfer from paediatric to adult care [
Prevalence and predictors of gaps in care among adult congenital heart disease patients: HEART-ACHD (The Health, Education, and Access Research Trial).
Prevalence and correlates of successful transfer from pediatric to adult health care among a cohort of young adults with complex congenital heart defects.
]. The transition of patients with a Fontan circulation from paediatric to adult cardiology care requires a multidisciplinary, holistic, individualised, flexible, and carefully planned approach, with equal emphasis on patients and their parents and carers [
Expectations and experiences of adolescents with congenital heart disease on being transferred from pediatric cardiology to an adult congenital heart disease program.
Transition should commence during early adolescence and continue into adulthood, until the patient is successfully engaged with adult congenital cardiology care [
Are two youth-focused interventions sufficient to empower youth with chronic health conditions in their transition to adult healthcare: a mixed-methods longitudinal prospective cohort study.
]. This process should be facilitated by paediatric and adult cardiology teams, including a dedicated transition lead (or leads), who will ideally be a congenital cardiology nurse [
Transition should encompass multiple developmentally appropriate educational, vocational, and psychosocial care sessions, with a focus on self-management, as appropriate, and documented transition plans [
], including a joint transfer process, where feasible, and individualised patient follow-up to ensure a successful transfer.
The patient, his or her parent or carer and the receiving adult congenital cardiology team should each receive a copy of the final diagnostic test results, clinical summaries, operation reports, information relevant to other care needs, and contact details. This referral pack should also include details of the patient’s recommended first appointment in adult cardiology care [
There should be clarity on how and when the transfer to adult cardiology care is completed, including where to seek emergency assistance if needed between the last scheduled paediatric appointment and the first adult appointment. The transition process must also ensure provision of appropriate community supports; for example, from a general practitioner.
Success is most often encountered when this process is supported by an institution-wide policy on transition that is integrated into the cardiology care framework at all sites [
Are two youth-focused interventions sufficient to empower youth with chronic health conditions in their transition to adult healthcare: a mixed-methods longitudinal prospective cohort study.
Children with a Fontan circulation may experience profound emotional, behavioural, neurodevelopmental, and social challenges in the early years of life. This can have lifelong consequences for them and their families, affecting their future health, wellbeing, and quality of life. Recognition and early intervention and support may prevent or minimise these effects [
Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association.
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