“How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns

doi:10.1016/j.ijcard.2018.01.098

International Journal of Cardiology

Volume 260, 1 June 2018, Pages 54-59

https://doi.org/10.1016/j.ijcard.2018.01.098 Get rights and content

Highlights

•
Fontan adults' greatest concerns are fear of death and life expectancy uncertainty.
•
Clear communication around outcomes could help alleviate these uncertainties.
•
Targeted psychological support around fear of death could be beneficial.
•
Lifestyle-integrated physical exercise could alleviate physical concerns.

Abstract

Background

Little is known about adults living with a Fontan circulation's concerns outside the scope of their clinical outcomes. We examined adults with a Fontan circulations' greatest concerns, as well as their concerns around anti-coagulation, pregnancy and finances.

Methods

Adults with a Fontan circulation in the Australian and New Zealand Fontan Registry were invited to complete an anonymous online survey, of which 57 participated. A qualitative method approach using thematic analyses was used.

Results

The greatest concerns for adults living with a Fontan circulation were fear of death/uncertainty around life expectancy which for many individuals colored their concerns around physical health, pregnancy and having children, quality of life and finances. Improving information about outcomes to patients with a Fontan circulation might alleviate uncertainties about their future.

Conclusions

Fear of death is the primary concern of adults with a Fontan circulation. It may require improved communication and targeted psychological interventions. Physical exercise incorporated as part of their lifestyle should be encouraged to alleviate physical concerns and also improve psychological well-being.

Introduction

Patients born with a single ventricle can now successfully be palliated to adulthood. Current estimates place the chances of surviving to adulthood at 85% for those born with a dominant left ventricle and around 65% for those born with a single right ventricle [1]. The population of patients surviving after the Fontan procedure, the last of the series of their palliative procedures, show that this population is growing. There are 1357 of these patients alive in Australia and New Zealand [2] captured in the Australian and New Zealand Fontan Registry [3], from a total population of 28,497,499 inhabitants [4]. We have predicted that this population will double within 18 years [5]. Some have estimated this population to be as large as 50,000 to 70,000 patients worldwide [6].

The majority of this population of survivors of the Fontan procedure are now comprised of young active adults [5]. While some information is available on the quality of life [7] and exercise performance [8] of this generation of patients, we concur with others [9,10] that there is a lack of understanding as to the daily concerns of patient group, particularly outside the scope of their clinical outcomes [7,10,11]. In Australia and New Zealand, following the development of a bi-National Registry [3], a consumer association was created: the Australian and New Zealand Fontan Advisory committee (ANZFAC). Their first action was to survey the greatest concerns of adults with a Fontan circulation and to use the results as a platform for developing further research agendas which are closely aligned with patient needs, and informing clinical practice.

Section snippets

Methods

Ethics approval for the study was obtained through the Royal Children's Hospital, Melbourne, Australia. An anonymous online survey was conducted assessing the needs of adults living with Fontan circulation through the Australian and New Zealand Fontan Registry in 2015. In addition to basic demographics, this purpose built questionnaire used both open-ended and closed questions (Table 1).

Results

A total of 57 adults with a Fontan circulation participated in the survey from the 298 Fontan Registry adults with email addresses (response rate 19%). Participants' geographical spread was focused on major cities in Australia and New Zealand. The majority of participants were either in married/de facto relationships (n = 33, 58%) or single (n = 20, 35%), female (n = 40, 70% — over representation of female responses), with an age range of 18 to 51 years (M = 33; SD = 9). No data around racial

Discussion

The growing population of young adults with a Fontan circulation is a testimony to the enormous efforts of our community to provide treatment to those born with the most extreme congenital cardiac conditions. Survival outcomes have steadily increased, but these patients are still facing an uncertain future: multi-systemic complications are expected as a result of their sustained elevated systemic venous pressures [15]. Up to a third will suffer from liver and renal complications [16] and many

Acknowledgments

The authors thank the participants of the study and our research assistants for their support in maintaining the Australian and New Zealand Fontan Registry. We also acknowledge the support provided to the Murdoch Children's Research Institute by the Victorian Government's Operational Infrastructure Support Program.

Funding

This work was supported by a National Health and Medical Research Council (NHMRC) Partnership grant (1076849). Prof. Yves d'Udekem is a Clinician Practitioner Fellow of the NHMRC (1082186).

Disclosures

Prof Yves d'Udekem is a consultant for the companies MSD and Actelion. The remaining authors report no relationships that could be construed as a conflict of interest.

References (28)

Y. d'Udekem et al.
Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance
J. Am. Coll. Cardiol.
(2012)
C. Schilling et al.
The Fontan epidemic: population projections from the Australia and New Zealand Fontan Registry
Int. J. Cardiol.
(2016)
J.P. Jacobs et al.
The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2017 update on outcomes and quality
Ann. Thorac. Surg.
(2017)
A.E. van den Bosch et al.
Long-term outcome and quality of life in adult patients after the Fontan operation
Am. J. Cardiol.
(2004)
M. Gurvitz et al.
Emerging research directions in adult congenital heart disease: a report from an NHLBI/ACHA working group
J. Am. Coll. Cardiol.
(2016)
J. Rychik
The relentless effects of the Fontan paradox
Semin. Thorac. Cardiovasc. Surg.
(2016)
A.M. Atz et al.
Longitudinal outcomes of patients with single ventricle after the Fontan procedure
J. Am. Coll. Cardiol.
(2017)
R. Cordina et al.
Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology
Int. J. Cardiol.
(2013)
D. Zentner et al.
Fertility and pregnancy in the Fontan population
Int. J. Cardiol.
(2016)
M. Gouton et al.
Maternal and fetal outcomes of pregnancy with Fontan circulation: a multicentric observational study
Int. J. Cardiol.
(2015)

Australian and New Zealand Fontan Registry Annual Report 2015. Australian and New Zealand Fontan Registry

(2016)

A.J. Iyengar et al.

The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry

Intern. Med. J.

(2014)

United Nations DoEaSA, Population Division

World Population Prospects: The 2015 Revision, Key Findings and Advance Tables

(2015)

K. Laohachai et al.

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

J. Am. Heart Assoc.

(2017)

Cited by (10)

Exploring the Promise of Telemedicine Exercise Interventions in Children and Adolescents With Congenital Heart Disease
2023, Canadian Journal of Cardiology
Youth with congenital heart disease (CHD) have reduced exercise capacity via various physical and psychosocial mechanisms. In addition to limited physiologic exercise capacity, these patients experience lower levels of physical activity, physical activity self-efficacy, health-related quality of life, and endothelial function. The study of exercise interventions and cardiac rehabilitation programs in pediatric CHD populations remains limited, particularly home-based interventions that incorporate real-time physiologic monitoring. Home-based interventions provide improved access and convenience to patients. This is principally important for patients from geographically disperse regions who receive their care at centralized subspecialty centres, as is the case for Canadian pediatric cardiac care. These programs, however, have traditionally not permitted the supervision of safety, technique, and adherence that are afforded by hospital/facility-based programs. As such, telemedicine is an important evolving area that combines the benefits of traditional home and facility-based cardiac rehabilitation. An additional key area lacking study surrounds the types of exercise interventions in youth with CHD. To date, interventions have often centred around moderate-intensity continuous exercise. High-intensity interval training might offer superior cardiorespiratory advantages but remains understudied in the CHD population. In this review, we highlight the existing evidence basis for exercise interventions in youth with CHD, explore the promise of incorporating telemedicine home-based solutions, and highlight key knowledge gaps. To address identified knowledge gaps, we are undertaking a 12-week randomized crossover trial of a home-based telemedicine high-intensity interval training intervention in youth with repaired moderate-severe CHD using a video game-linked cycle ergometer (known as the MedBIKE; https://spaces.facsci.ualberta.ca/ahci/projects/medical-projects/remote-rehab-bike-projects).
La capacité d’effort des jeunes atteints de cardiopathie congénitale est réduite par divers mécanismes physiques et psychosociaux. En plus de leur capacité d’effort physiologique limitée, ces patients ont des niveaux réduits d’activité physique, de connaissance de leurs propres capacités en matière d’activité physique, de qualité de vie liée à la santé et de fonction endothéliale. L’étude des interventions axées sur l’activité physique et des programmes de réadaptation cardiaque chez les populations pédiatriques atteintes de cardiopathie congénitale demeure limitée, particulièrement en ce qui a trait aux interventions à domicile qui intègrent une surveillance physiologique en temps réel. Les interventions à domicile offrent aux patients un accès et une commodité améliorés. C’est d’autant plus important pour les patients vivant dans des régions éloignées qui reçoivent leurs soins dans des établissements centralisés sous-spécialisés, comme c’est le cas pour les soins cardiaques des enfants au Canada. Ces programmes n’offrent toutefois pas la possibilité de surveiller l’innocuité, la technique et l’observance thérapeutiques, ce que permettent les programmes offerts dans un hôpital ou en établissement. À ce titre, la télémédecine est un important domaine en évolution qui associe les bienfaits de la réadaptation cardiaque classique à domicile à ceux offerts en établissement. Les types d’interventions axées sur l’activité physique chez les jeunes atteints d’une cardiopathie congénitale représentent un autre domaine pour lequel les études sont insuffisantes. Jusqu’à ce jour, les interventions sont souvent axées sur l’activité physique continue d’intensité modérée. L’entraînement par intervalles à haute intensité peut offrir des bienfaits cardiorespiratoires supérieurs, mais demeure sous-étudié dans la population des patients atteints de cardiopathie congénitale. Dans cet examen, nous indiquons les données probantes existantes sur lesquelles se fondent les interventions axées sur l’activité physique pour les jeunes atteints de cardiopathie congénitale, nous explorons les promesses de l’intégration de solutions à domicile offertes par la télémédecine et nous soulignons les lacunes en matière de connaissances. Afin de recenser ces lacunes, nous entreprenons un essai avec permutation d’une durée de 12 semaines d’une intervention à domicile d’entraînement par intervalles à haute intensité offerte par l’intermédiaire de la télémédecine aux jeunes atteints d’une cardiopathie congénitale modérée ou grave réparée, au moyen d’un ergocycle lié à un jeu vidéo (MedBike : https://spaces.facsci.ualberta.ca/ahci/projects/medical-projects/remote-rehab-bike-projects).
Quality of Life and Other Patient-Reported Outcomes Across the Life Span Among People With Fontan Palliation
2022, Canadian Journal of Cardiology
Citation Excerpt :
Primary patient concerns pertained to fear of death and uncertainty regarding life expectancy. This fear, in turn, influenced subthemes of physical health (eg, fatigue, fear of additional surgeries), decision-making about pregnancy and parenthood, finances (eg, ability to maintain employment), and QOL (eg, living independently and accessing psychological care).121 In a pilot study with a mixed-methods design, Dutch researchers explored QOL and sexual well-being in a sample of adults with Fontan physiology; their study included semistructured qualitative interviews with 8 patients.122
Traditional congenital heart disease (CHD) outcomes include mortality (survival to adulthood and life expectancy) as well as cardiac and noncardiac morbidity. Strategies to identify and manage sequelae have primarily focused on objective data obtained though invasive and noninvasive diagnostic approaches. In contrast, patient-reported outcomes (PROs) provide subjective information, using standardized measures, about patients’ health and well-being as reported directly by patients, without interpretation, interference, or assumptions made by clinicians or others. Selection of PRO measures entails thoughtful consideration of who the individuals being surveyed are, why assessment is occurring (eg, what are the domains of interest; clinical vs research), and what processes are in place for acquisition, administration, interpretation, and response. In this review, we focus on 3 domains of PROs for pediatric and adult patients with Fontan physiology: physical health status, psychological functioning, and quality of life. Infants, children, adolescents, and adults with CHD face a spectrum of challenges that might influence PROs across the life span. In general, patients with Fontan palliation tend to have lower physical health status, experience more psychological distress, and have equivalent or reduced quality of life compared with healthy peers. Herein, we provide an overview of PROs among people with Fontan circulation as a group, yet simultaneously emphasize that the optimal way to understand the experiences of any individual patient is to ask and listen. We also offer clinical and research initiatives to improve the adoption and utility of PROs in CHD settings, which show commitment to capturing, understanding, and responding to the patient voice.
L’issue classique des cardiopathies congénitales comprend le décès (survie jusqu’à l’âge adulte et espérance de vie écourtées) ainsi que la morbidité cardiaque et non cardiaque. Les stratégies de dépistage et de prise en charge des séquelles s’articulent essentiellement autour de données objectives obtenues par le biais d’approches diagnostiques invasives et non invasives. En revanche, les données d’autodéclaration des patients fournissent des informations subjectives sur la santé et le bien-être de ces derniers, au moyen de mesures normalisées, de façon directe et sans interprétation, intervention ni hypothèses de la part de cliniciens ou d’autres personnes. La sélection des paramètres d’autodéclaration des patients doit être mûrie et nécessite que l’on sache qui sont les répondants, pourquoi l’évaluation est effectuée (notamment en ce qui concerne la nature des domaines abordés, dans une optique clinique ou de recherche) et quels processus sont en place en matière de collecte, d’administration, d’interprétation et de réaction. Dans le présent article de synthèse, nous nous concentrons sur trois domaines d’autodéclaration des patients pédiatriques et adultes ayant subi l’intervention de Fontan : état de santé physique, bien-être psychologique et qualité de vie. Les nourrissons, les enfants, les adolescents et les adultes atteints de cardiopathies congénitales doivent composer avec un large éventail d’enjeux susceptibles d’influer sur les données d’autodéclaration tout au long de leur vie. Généralement, les patients qui ont subi l’intervention de Fontan tendent à présenter un moins bon état de santé physique, à éprouver plus de détresse psychologique et à avoir une qualité de vie équivalente ou inférieure comparativement à des pairs en bonne santé. Nous donnons ici un aperçu des données d’autodéclaration de personnes ayant subi l’intervention de Fontan, tous cas confondus. Nous soulignons également que la façon optimale de comprendre ce que vit un patient consiste à interroger et à écouter ce dernier. Nous proposons aussi des initiatives à l’intention des cliniciens et des chercheurs en vue d’accroître l’adoption et l’utilité des méthodes d’autodéclaration touchant les cardiopathies congénitales, dans l’optique d’une volonté affichée d’écoute, de compréhension et de réactivité à l’endroit du patient.
Reproductive Issues in Patients With the Fontan Operation
2022, Canadian Journal of Cardiology
Patients with the Fontan operation have a unique circulation, with a limited ability to increase cardiac output, and high central venous pressure. They may have diastolic and/or systolic ventricular dysfunction, arrhythmias, thromboembolic complications, or multiorgan dysfunction. All of these factors contribute to reproductive issues, including menstrual irregularities, infertility, recurrent miscarriage, and complications during pregnancy. Although atrial arrhythmias are the most common cardiac complications during pregnancy, patients can develop heart failure and thromboembolic events. Obstetric bleeding, including postpartum hemorrhage, is common. In addition to maternal complications, adverse fetal and neonatal events, such as prematurity and low birthweight, are very common. Counselling about these reproductive issues should begin early. For those who become pregnant, care should be provided by a multidisciplinary cardio-obstetric team familiar with the specific issues and needs of the Fontan population. In this review, we discuss infertility, contraception, and pregnancy in patients with the Fontan operation.
L’intervention de Fontan crée un schéma circulatoire unique se traduisant par une capacité limitée d’augmentation du débit cardiaque et une pression veineuse centrale élevée. Les patients qui ont subi cette intervention peuvent présenter une dysfonction ventriculaire diastolique et/ou systolique, des arythmies, des complications thromboemboliques ou une dysfonction multiviscérale. Tous ces facteurs contribuent à des problèmes de reproduction, notamment des irrégularités menstruelles, l’infertilité, des fausses couches répétées et des complications obstétricales. Les arythmies atriales sont les complications cardiaques les plus courantes pendant la grossesse, mais une insuffisance cardiaque et des événements thromboemboliques peuvent aussi survenir. Les saignements obstétricaux, dont l’hémorragie post-partum, sont fréquents. Outre les complications chez la mère, on note une fréquence élevée d’événements indésirables touchant le fœtus et le nouveau-né, tels que la prématurité et l’insuffisance pondérale à la naissance. Le counseling sur ces questions touchant la reproduction devrait commencer tôt. Les femmes enceintes devraient être prises en charge par une équipe cardio-obstétricale bien au fait des difficultés et des besoins spécifiques des patientes qui ont subi l’intervention de Fontan. Dans cet article de synthèse, nous abordons l’infertilité, la contraception et la grossesse chez les patientes ayant subi l’intervention de Fontan.
Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement
2020, Heart Lung and Circulation
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.
- •
  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.
Bridging the psychological issues of living with the Fontan circulation
2018, International Journal of Cardiology
Towards improving the lived experience of adult congenital heart patients: Suggestions for health care practitioners
2023, Cardiology in the Young

View all citing articles on Scopus

¹: This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

View full text

“How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns

Highlights

Abstract

Background

Methods

Results

Conclusions

Introduction

Section snippets

Methods

Results

Discussion

Acknowledgments

Funding

Disclosures

J. Am. Coll. Cardiol.

Int. J. Cardiol.

Ann. Thorac. Surg.

Am. J. Cardiol.

J. Am. Coll. Cardiol.

Semin. Thorac. Cardiovasc. Surg.

J. Am. Coll. Cardiol.

Int. J. Cardiol.

Int. J. Cardiol.

Int. J. Cardiol.

Australian and New Zealand Fontan Registry Annual Report 2015. Australian and New Zealand Fontan Registry

The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry

Intern. Med. J.

World Population Prospects: The 2015 Revision, Key Findings and Advance Tables

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

J. Am. Heart Assoc.