Editorial Bridging the psychological issues of living with the Fontan circulation Margarita Brida a,b,c, ⁎, Stefania Grbcic d , Christina Holbein e , Gruschen R. Veldtman e a Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK b National Heart and Lung Institute, Imperial College, London, UK c Division for Adult Congenital Heart Disease, Department of Cardiovascular Medicine, University Hospital Centre Zagreb, Croatia d Outpatient Psychology Consultancy, Flower Associates International Ltd., London, UK e Department of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States article info Article history: Received 22 February 2018 Accepted 26 February 2018 Patient reported outcomes, despite their powerful ability to focus re- search on patient centered issues, are still relatively neglected in the congenital heart disease (CHD) published literature and research culture. The question of ‘how long will I continue to be normal?’ crosses all patients' minds, (and their families) at many stages of their condi- tion. There is an increase in research, particularly in heart disease, re- garding not only the somatic aspects of disease, but taking also into account the patient as a whole, the holistic approach, including quality of life, overall wellbeing, and the social and psychological aspect, with a common goal to better address patients' needs and care [1–3]. Univentricular heart physiology is among the most complex and le- thal congenital heart defects. The development of the Fontan operation, five decades ago, has given birth to a new generation of adolescents and adults living with this condition. Life saving and transforming Fontan procedures represent a clear milestone in the management of patients with univentricular hearts. Nevertheless, the Fontan operation is pallia- tive in its nature, associated with numerous late complications includ- ing decreased exercise tolerance, arrhythmia, thromboembolism, hepatic dysfunction, protein-losing enteropathy, plastic bronchitis, challenges such as pregnancy, and inevitably shorter life span [4]. Thus, adult patients may also have a psychological burden of living with their chronic/life-long condition. A group of Fontan experts recently established the International Fontan Interest Group (I-FIG) [5,6] with a clear mission to provide scien- tific evidence and promote research in the field. Drs du Plessis, d'Udekem and co-authors from I-FIG conducted clinical study regarding adult Fontan patients' greatest concerns. The study included 57 adult Fontan patients (mean age 33 ± 9 years) from Australia and New Zealand who completed an anonymous online self-report survey covering concerns of living with a Fontan circulation. Fear of death and uncertainty around life expectancy were the main issues [7]. Previous studies already indicated that patients with CHD, particu- larly those with complex underlying cardiac defects and previous surgi- cal procedures, have higher levels of psychosocial difficulties and lower quality of life compared to either to the general population, and or to pa- tients with simple congenital heart defects [1–3]. From a psychological perspective, Fontan patients have a sense of foreboding that they have a limited and unpredictable time left in their life, which may contribute to higher levels of anxiety, as well as de- pression. There is a known bidirectional link between mood disorders (mainly anxiety and depression) and many chronic medical illnesses [8]. The anxiety may arise at different levels, ranging from a fear of death, to anxiety specifically relating to their needs being different from their peer group. Timely and appropriate communication regard- ing their condition with realistic, but positive support from pediatric cardiologists and parents during childhood may impact on their quality of life, mindset, and anxiety level. Their developmental stage, their abil- ity to comprehend and cope with the information that is provided to them also needs to be taken into account. Patients' fear of death is rational and a normative reflection of living with a Fontan circulation. Patients may struggle to change their mindset to alleviate and normalize this fear, rather than expect a positive change in their physical condition. This becomes a particular problem when in- dividuals have a mindset that the only way they can change their mood disorder is by changing their physical attributes. Other important fac- tors that may contribute to patients' mental health are relevant reac- tions and behavior of their family members and friends. Medically ill patients in general, often suffer psychologically without receiving appropriate support and treatment. Physicians, patients, and their families may fail to appreciate the implications of mood disorders in the wrong belief that it is an expected and unavoidable consequence of their serious illness or that the medical condition supersedes concerns for mental illness. Specific targeted psychological interventions for this group of patients are crucial following anxiety and depression screening (including suicide ideation; using a variety of screening tools, such as the Beck Depression Inventory among others). These targeted interventions need to be carefully structured, depending on the psychological diffi- culty (anxiety and/or depression), level of severity (mild, moderate, se- vere), stage of life, as well as age and gender (Fig. 1). International Journal of Cardiology 260 (2018) 72–73 DOI of original article: https://doi.org/10.1016/j.ijcard.2018.01.098. ⁎ Corresponding author at: Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. E-mail address: margarita.brida@icloud.com (M. Brida). https://doi.org/10.1016/j.ijcard.2018.02.105 0167-5273/© 2017 Published by Elsevier B.V. Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard