When looking at the research available on the psychological functioning of adults with ConHD, there is often a comparison of this group to a healthy comparison or other populations, and the exploration of the correlation of psychological distress with medical and/or other variables.

Although there are common trends, diverging results have also been obtained, and they can be attributed to the vast differences of instruments utilized, but also perhaps to the different health-care systems and health benefits available in the different countries. In this paragraph we especially refer to a recent review conducted in our group [5] in which the labels of psychopathology, anxiety, depression and hostility, neuroticism, and self-esteem were identified.

The studies by Utens et al. and Van Rijen et al. [7, 8] are examples in which there is a comparison between adults ConHD and the healthy peer group. In the study by Utens and colleagues [7], no differences were found between the mean problem scores of the congenital heart disease adults and reference peers. Only some minor, small, and limited differences were found in two specific problem areas: the “Somatic Complaints” and the “Strange” subscales in the study by Utens.

In the second study of Van Rijen et al. [8], it was outlined that young female patients with congenital heart disease aged from 20 to 27 showed higher levels of psychopathology when compared with the reference group; however, when the mean scores of all separate scales of the patient sample and the reference groups were compared, only small differences on the other informant reports and hardly any differences on the self-reports were found.

A study by Cox [9] also confirmed that adults with ConHD had more favorable scores when compared with the control group; however, it is possible that the control group utilized was inappropriate (a group of patients of the department of orthopedics).

In another study of Utens and colleagues [10], a better functioning of the patients with congenital heart disease was found with respect to the reference group, and it was also confirmed that the population had favorable outcomes on daily and leisure time activities. These results were also confirmed in a study by Van Rijen and colleagues [11] in which a better emotional functioning of the congenital heart disease sample is reported when compared to the reference group.

These results indicate that these patients could have a greater sense of coherence [12] with respect to the healthy population, as also outlined in the chapter on quality of life by Apers, Luyckx, and Moons in this book (see Chap.​ 7). It is indeed possible that having to face an illness these individuals were made to mobilize more resources as they are growing up when compared to the healthy population and search for solutions or ways of coping appropriate to tackle stress [12].

Another possibility that was suggested was that denial and high achievement motivation could inflate the scores on the self-report data [10, 11]. As we shall see further on in this chapter, appearing to be normal is very important for this population, and this further confirms this possibility.

A study which does not confirm this trend is the one by Brandhagen [13], in which it was specified that adults with ConHD fare worse than the healthy population, showing more psychological distress. It is noteworthy that only 168 out of 463 patients completed the questionnaire.

When it comes to anxiety and depression, in a study by Kovacs [14], the objective was to identify the predictors of anxiety and depression in adults with congenital heart disease. It was indicated that 34 % of the patients resulted as having elevated anxiety. Loneliness and fear of negative evaluation, and not variables relating to the medical conditions of the patient, resulted as being predictors of anxiety.

When it comes to depression, 78 % of the sample resulted as having minimal symptoms, 10 % mild symptoms, and 12 % moderate to severe depressive symptomatology. Loneliness, perceived health status, and fear of negative evaluation resulted as being predictors of depression, and the actual disease severity and functional class did not predict mood severity. Similarly, in a study by Rietveld [15], worse scores on psychosocial adjustment and quality of life were linked to having negative thoughts, irrespective of the medical condition of the patients.

Another interesting thing outlined by the study done by Kovacs [14] is that in the subset of their sample who participated in structured clinical interviews, 29 out of 58 patients met diagnostic criteria for at least one lifetime mood or anxiety disorder. Out of these 39 % had never received any mental health treatment.

In a study by Popelova and colleagues [16] on a small group of patients with persistent cyanosis, depression was associated with older age, worse functional state, and unemployment but independent from the severity of cyanosis. In this case 34 % of the sample resulted as being depressed.

Similarly to Kovacs, in the previous studies of Bromberg and Horner [17, 18], psychiatric interviews were utilized to asses adults with ConHD. In the study done by Horner [18] where 29 patients with complex congenital heart disease were evaluated psychiatrically by both interview and questionnaire, the authors confirm that most of the population were functional in day-to-day life; however, 5 were diagnosed as having panic disorder, and 4 patients met full diagnostic criteria for major depression and had received no psychiatric treatment for these conditions.

Also in the study by Bromberg [17], undertreatment seemed to occur as more than one third of the patients who were assumed to be well adjusted experienced a diagnosable psychiatric disorder. Two patients out of 22 met diagnostic criteria for a general anxiety disorder, and 6 out of 22 patients met diagnostic criteria for a depressive episode.

Most of the literature indicates that there is no relationship between diagnosis, physical fitness or presence of residual symptoms, and worse psychological functioning [7–11, 14, 15, 19], with only few studies suggesting a weak link between physical functional class and psychological functioning [16, 20]. For this reason it is particularly important to explore the possible predictors of psychological distress in this population, in order to incorporate them in clinical practice.

As specified in the previous paragraph, there are strong indications in the literature that the objective physical condition of these patients is not directly linked to their psychological status. In some studies, other possible variables that could influence psychological distress have been explored [5, 14, 15, 19, 20], and these resulted in:

Perceived health status – In our clinical experience, perceived health status is one of the most important factors which influences how the patients feel about themselves, also because it is an indication that there could be significant gaps in illness knowledge in this population [21]. In a recent study in our center, we specifically wanted to examine the effects of perceived health status by including a numerical rating scale assessing the perceived severity of the patient condition [19]. It was verified that the perception of severity of the patient and not the objective medical criteria, including functional class, resulted as being correlated to psychological distress.

Health perception is the result of the integration of the information and feelings related to the health and of one’s limits, the health-care system, the family, and the society. It is influenced by childhood experiences, family relationships, working status, and culture. A lot of information is derived from somatic perceptions (symptoms), from the beliefs and knowledge linked to the disease, and from the information arising from the social environment, in particular the ones given by doctors. All of these components entail both evaluations and information both at a conscious level and an unconscious level, together with the resulting emotional responses, and all these processes influence psychological reactions [22].

Low capacity for physical exercise and imposed limitations and restrictions – As specified in the previous paragraphs, although the general trend in the literature is that the more objective medical conditions are generally not related to psychological functioning, in some studies it was pointed out that physical functionality and the more serious conditions have an influence on how the patients feel about themselves. As we shall describe later, it could be that these restrictions and impositions cause problems in daily life activities, causing problems in the social and work spheres of these patients.

Imposed restrictions resulted as being strong predictors of psychological well-being [20, 23, 24], and these are related to physical activity, lifestyle, and to the possibility of pregnancy. These limits may be imposed by the doctor, on the basis of the functional status and the severity of cardiac disease, and they also may be defined by patients themselves on the basis of subjective experiences, or they may be the result of uncertainties and erroneous beliefs. In fact, from our clinical experience we have seen that it is possible that patients both greatly underestimate the severity of their condition but also that they feel that the severity of their condition is much greater than a more objective medical evaluation of their situation.

Fear of negative evaluation and social isolation – In a review on the biopsychosocial experiences of adults with congenital heart disease in 2005 [25], Kovacs and colleagues outline how congenital heart disease can impact the social sphere of these patients. It is also important to evaluate the qualitative data available [26, 27] in which there is a further indication of the difficulties of these patients, as we shall describe in more in-depth manner later on in this chapter. From our clinical experience and also the data available, there seems to be a struggle to maintain a “normal” life, and this can be hindered from being evaluated negatively by others and also from having the perception that they cannot participate to social and work activities like everybody else. The importance of these factors is highlighted in the study by Kovacs mentioned previously [14] as both fear of negative evaluation and social isolation are linked with depression and anxiety.

It is very difficult to generalize about the life experiences of this population, since the different conditions are very diverse. Having said this, there seem to be some patterns of experience and perspective in this broad diversity [28]. Having a complex congenital heart condition can be regarded as having a chronic condition because of the long-term nature of the condition, the uncertainty of its course and prognosis, the signs and symptoms of the condition, and also the restriction on the everyday life of these patients [29].

Qualitative studies which take into consideration the life experiences of this population can give valuable insight on how to further improve psychosocial services for these patients. Indications can also be given on how to improve patient compliance and to strengthen the patients’ coping strategies by taking into consideration the subjective life experiences of these patients.

In two studies conducted on the adolescent population [30, 31], it was outlined how adolescents with ConHD struggle with physical limitations and face social exclusion. The limitations the patients felt were linked to the severity of the condition, and as many as one fourth of adults with ConHD report their parents as being overprotective during their childhoods and adolescence [13, 30, 32]. This could explain why in general these patients tend to live longer with their parents as overprotection could lead to less autonomy [33, 34]. In both researches the theme of being different and the attempts to cope with this emerged strongly.

This central theme of being different from healthy peers also emerges in the qualitative literature pertaining to the life experiences of these patients in their adulthood [26–28, 35–37], and there are often attempts and the struggle to feel normal and to be perceived as being normal by others which can be followed by feelings of ambivalence and also denial of the condition and efforts to exceed their physical boundaries [35].

In one particular older study [37], the problems encountered by the female patients with ConHD were focused upon mainly the concerns regarding fertility, contraception, and pregnancy. Other concerns which the female patients outlined concerned their surgical scars and body size, and they resulted as being uninformed about aspects regarding their condition and reproductive matters. There is an indication that the perception of body image is quite varied in males and in females and that females experience the consequence of scarring and cyanosis more negatively than men, who seem to have difficulties related to their body image during adolescence [26, 27].

Very often the patients refer to their hospitalization experiences, and in a study [38] it was outlined that the patients, their families, and the nurses had different expectations about the management of the physical needs and about the way the patients and the patients’ families behaved during acute phases of the patients’ illness. These discrepancies were associated with interpersonal conflict, distrust, anxiety, and dissatisfaction with the health care provided.

As outlined in this manual, the involvement of associations relative to this condition is very important. In the chapters by Geier and Campioni Chaps. 12 and 13, the European experience related to this is outlined. In a very interesting article [28], quotations from patients are reported from the Adult Congenital Heart Association in an attempt to address the patients’ perspectives in order to outline the implications for care.

When it comes to feedback regarding their state of health, we find two extremities: on the one hand some were told they were “miracle babies” and also at the other end, lost causes, that they would not survive for long, often erroneously. When told that they were lost causes, this often caused an increase in engagement in highly risky behavior and also difficulties to move into adulthood, because of a perception that there was no future to be put at risk or to work to.

In the conditions of moderate and great complexity, it was reported that there was a risk of language-based misperception in understanding if the condition is completely cured or not. There are also often gaps in knowledge regarding what is required to safeguard and optimize their health [21, 39], and this could be related to the fact that there have not been enough efforts to educate patients regarding their condition, the risks associated with it, and the necessity for lifelong follow-up.

Similarly to our experience, it is common for this population to have difficulties when having to go to the “regular” medical system, where the sanitary personnel is not specialized in ConHD. Not only they need to be vigilant because they might be given medication or treatment which could be inappropriate for them, but they report that insisting for specialized care can result in hostility.

ConHD can be considered as an invisible disability, as quite often it is not evident to others that the person has this condition. The adults often report that friends and family do not really understand their restrictions and that sometimes they also believe that changes in various responsibilities which could be cumbersome are not really required.

Other difficult situations the patients have to face are making difficult decisions regarding their medical care, referral for surgery, and in those occasions when they are informed that there is nothing left to be done to improve their health [18]. End of life care in this population is a particularly sensitive topic which will be developed in detail by Kovacs and colleagues in this book (Chap.​ 8).