Anxiety and Depression

Early studies have shown that approximately one-third of adults with CHD experience difficulties with depression and/or anxiety. Furthermore, it has been found that half of the CHD population presents with at least one lifetime depressive or anxiety disorder. These prevalence rates are higher than those in the general population. However, these data are all from North America and are based on studies in small samples. European data demonstrate different psychological outcomes. Assessment of the longitudinal development of psychopathology in a Dutch cohort showed that the prevalence of psychopathology, including internalizing behaviors such as anxiety and depression, was similar or even better compared to reference norms. An Italian study similarly concluded that psychological well-being of patients was comparable to reference norms. In a large German study, the level of trait anxiety was similar to that of healthy controls, and the prevalence of depressive symptoms was lower in patients than in the general population. A recent Belgian study in young people with CHD found that patients scored significantly lower on depressive symptoms than community adolescents. Overall, it can be concluded that the majority of studies do not find a higher prevalence of mood and anxiety disorders in adults with CHD. Irrespective of whether the levels of anxiety and depression in patients are similar or lower, the importance of assessing persistent or chronic depressive symptoms has been emphasized. Indeed, patients with persisting or recurring depressive symptoms do worse in terms of QOL and patient-reported health than patients who are experiencing one or no depressive episodes. This reminds us of the need for regular and longitudinal assessments of anxiety and depression throughout clinical follow-up.

Psychological Resilience

The attention that is given to psychological resilience is relatively new in the area of CHD. It refers to a person’s ability to properly adapt to stress and adversity. Hence, it looks at individuals’ capacities for adaptive behaviors, rather than at limitations or maladjustment. A particularly interesting concept is “sense of coherence” (SOC). SOC represents an individual’s generalized worldview and expresses the extent to which the individual perceives (1) stimuli as structured and predictable (ie, comprehensibility); (2) that resources are available to meet the demands posed by these stimuli (ie, manageability); and (3) that these demands are challenges worthy of investment (ie, meaningfulness). The relation among SOC, psychosocial health, and QOL has been confirmed in diverse patient populations and individuals from the general population. Based on these prior findings, it was hypothesized that growing up with CHD may contribute to the development of a strong SOC, as patient feelings of comprehensibility, manageability, and meaningfulness may be reinforced by disease-related experiences from a young age. This hypothesis has been confirmed. Indeed, patients with CHD seem to have a stronger SOC than healthy counterparts. Clinicians ought to give more attention to aspects of resilience, because it gives notice to positive aspects of living with CHD.

Personality

The notion that personality might influence health is getting more attention. Numerous studies in acquired heart diseases have linked Type A (characterized by hostility, time-urgency, and competitiveness) and Type D personality (characterized by negative affect and social inhibition) to mortality and adverse health outcomes. However, in the area of CHD, personality research is sparse. To date, only two studies on personality in individuals with CHD have been published. Schoormans et al. found that one-fifth of the patients with CHD presented with a Type D personality. Patients with a Type D personality feel more functionally impaired, report poorer health status and a lower QOL, and use fewer health care resources. Rassart et al. investigated personality traits beyond Type D personality, using the Big Five model of personality in young persons with CHD. These investigators found that the distribution of personality dimensions in adolescents with CHD was similar to that of the general population, except for a lower score on Extraversion. Overall, Extraversion, Agreeableness, Conscientiousness, and Emotional Stability were associated with better QOL and several generic and disease-specific domains of patient-reported health.

These initial studies indicated that certain personality characteristics could enable patients to deal with the challenges of living with CHD, and contribute favorably to their disease adaptation. Conversely, other personality characteristics (eg, the tendency to experience negative affect) may put patients at risk for poor adaptation.

Social Support

Support from parents, peers, and the larger social environment is important for all individuals, and thus also for persons with CHD. The impact of social support in persons with CHD has been investigated in several studies. It has been found that adequate social support is associated with better QOL, the willingness to participate in exercise programs, and an active problem-solving coping style. Support received from parents is less scrutinized. Studies have shown, however, that parental support positively impacts QOL and patient-reported health, and negatively impacts depression and loneliness. A recent study showed the combined effects of parental and peer support in young people with CHD. Patients who received both parental and peer support had fewer depressive symptoms and a higher QOL.

Academic Achievement

Findings on the impact of CHD on the academic abilities of patients are contradictory. Numerous studies have reported that children with CHD can achieve an educational level equivalent to that of healthy peers, while others indicate that these children display significant learning disabilities. These observations largely depended on whether patients with mental retardation were included or not. Indeed, developmental delays are highly prevalent, and are associated with the complexity of the heart defect. A population-based study showed that children with CHD have a 50% higher likelihood of receiving special education than those born without birth defects. The learning disabilities in patients requiring special education are primarily associated with chromosomal or noncardiac syndromes or with neurologic deficits resulting from extended hypoxemic periods during infancy. Indeed, there is mounting evidence that CHD is associated with structural brain abnormalities or microcephaly. For instance, impaired brain volumes have been observed in fetuses with some forms of CHD. Furthermore, 8% to 33% of newborns with complex CHD that requires cardiac surgery are found to have microcephaly. This lower brain maturity means that these newborns are even prone to developing acquired brain injuries, such as stroke or periventricular leukomalacia. Altogether, this poses significant neurodevelopmental challenges to persons with CHD, which impact their academic abilities.

Employment

Inconsistent findings also exist regarding the employment status of patients with CHD. Nonetheless, it is clear that a significant proportion of these patients experience problems with employability. Even patients with high academic achievements may have poor job prospects. In particular, patients with complex or only partially repaired lesions may find themselves at a disadvantage in obtaining employment. Education and career counseling that matches the patient’s interests with his or her physical abilities can help to prevent or reduce these job-related problems.

A problem that particularly disadvantages individuals with CHD is the presence of attention deficit hyperactivity disorder (ADHD). In the broad population of patients with CHD, the prevalence of ADHD is approximately 12% to 29%. In patients with specific complex heart defects, the prevalence increases to 40% to 50%. Hence, the prevalence of ADHD in patients with CHD is substantially higher than the 3% to 5% that is found in healthy controls. Understandably, the presence of ADHD challenges the chances on the labor market in afflicted patients.

Insurability

CHD can also hinder an individual from obtaining life and health insurance. Obtaining life insurance can be very difficult for patients with CHD, even for patients whose health status is rated as “excellent” or “good” by a cardiologist. This issue is elaborated on in Chapter 28 , and therefore will not be specifically addressed in this chapter.

Marital Status

Data are limited with respect to social functioning and marital status. A few studies examined marital rates and generally found CHD to be comparable to the general population. It has been reported that young adults tend to live at home longer, but this difference disappeared as they got older. Similarly, patients with CHD tend to cohabitate and/or marry less often, or marry at a later age and have children later in their twenties as compared to their noncardiac peers. These differences might be explained in terms of their self-perception and body image, particularly among patients repaired later in life.

Quality of Life in Congenital Heart Disease

The first article on QOL in CHD was published in 1974. Since then, the number of QOL studies in patients with CHD published annually has grown exponentially. Over the past years, several systematic literature reviews on QOL in (young) adults with CHD have been published. In a first literature review, Fteropoulli et al. provided a comprehensive overview of all relevant quantitative studies on QOL in adults with CHD published until November 2011. These authors found that most (20 out of 26) studies reported a reduced QOL in patients with CHD in the physical domain. Conversely, the majority of studies evaluating psychosocial (21 out of 24 studies) and environmental or occupational domains of QOL (4 out of 6 studies) found no differences between patients and controls. In general, these authors concluded that available studies have contradictory findings, possibly resulting from limitations in study methodology.

A second review, performed by Apers et al., expanded the previous review by identifying articles that were published between the end of 2011 and the beginning of 2013. In this period, 11 studies on QOL in (young) adults with CHD were published, 8 of which compared QOL in patients with CHD to a control group. These studies also report inconsistent findings: three studies found that QOL is worse in patients with CHD as compared to control subjects; two studies concluded that QOL is similar; and three studies even found a better QOL in patients with CHD compared to a control group. Apers et al. attributed these inconsistent findings to methodological limitations and differences between these studies.

The first meta-analysis on QOL in CHD was published in 2015. Schroder et al. included studies in adolescents or young adults with mild, moderate, or complex CHD, in which QOL was reported as one index score ranging from 0 (worst QOL) to 100 (best QOL), and in which comparative data from healthy controls or a reference population were provided. Eighteen studies were included in this systematic review, six of which were withheld from the meta-analysis. The meta-analysis showed that the QOL in adolescents and young adults born with CHD is not lower than the QOL of healthy age-matched individuals.

Another meta-analysis published in 2015 specifically looked at studies in which the Medical Outcomes Study Short Form-36 (SF-36) was used. Kahr et al. conducted analyses for the different levels of heart defect complexity and performed their meta-analysis on data from 33 studies. They found that physical functioning and general health perception were significantly lower in patients with moderate or complex heart defects, whereas the scores of patients with mild defects were similar to those of the controls. Given that the majority of the patients with CHD have a mild heart defect, it is understandable that the performance of the entire CHD population is no worse than the general population, as found in Schroder’s meta-analysis.

The Assessment of Patterns of Patient-Reported Outcomes in Adults with CHD-International Study (APPROACH-IS) is a landmark study in QOL in CHD. This large-scale international study included more than 4000 adults with CHD from 24 centers in 15 countries, and found that overall, QOL in patients was generally good. Using a uniform methodology, variation between the countries was observed, with Australia having the highest QOL score and Japan the lowest.

Determinants of Quality of Life in Congenital Heart Disease

The following correlates of poorer QOL among adults with CHD have been identified: lower academic performance and education ; lower employment rates ; fewer daily activities ; worse functional class ; lower exercise capacity ; lower social support ; cardiac surgery ; implantable cardioverter defibrillator placement ; physical limitations ; and type D (distressed) personality. Inconsistent findings were observed regarding the relationship between QOL and age ; sex ; medication ; disease severity ; and severity of residual lesions. Finally, QOL appeared to be unrelated to CHD subtype, cyanosis, personal resources, and family environment.

Conceptual and Methodological Rigor

Despite the findings of QOL research over the past 40 years, substantial conceptual and methodological problems have been identified. Bratt and Moons reviewed all QOL studies that were published over the past 40 years ( n = 234), and looked for temporal trends in the conceptual and methodological rigor by applying 10 review criteria that reflect the quality of QOL studies. Temporal improvements were observed, mainly because more articles with higher quality scores were published over the past 10 years. Despite this upward trend, the majority of articles still have a low quality score. Indeed, between 2005–2014, 52.4% of articles still failed to meet any of the quality criteria. This means that major weaknesses in methodological rigor remain present in published QOL research. Therefore, it is argued that the scientific community should give more attention to conceptual and methodological aspects of QOL research, and doing so, advance the caliber of QOL studies.