“How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns
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.
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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.
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2022, Canadian Journal of CardiologyCitation 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
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This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.