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Quality of life in adolescents and young adults with CHD is not reduced: a systematic review and meta-analysis

Published online by Cambridge University Press:  12 November 2015

Morten Schrøder ,
Kirsten A. Boisen ,
Jesper Reimers ,
Grete Teilmann  and
Jesper Brok
Morten Schrøder*
Affiliation:
Department of Pediatric and Adolescent Medicine, Center of Adolescent Medicine, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
Kirsten A. Boisen
Affiliation:
Department of Pediatric and Adolescent Medicine, Center of Adolescent Medicine, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
Jesper Reimers
Affiliation:
Department of Pediatric and Adolescent Medicine, Section of Pediatric Cardiology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
Grete Teilmann
Affiliation:
Department of Pediartric and Adolescent Medicine, Nordsjællands Hospital, University of Copenhagen, Hillerød, Denmark
Jesper Brok
Affiliation:
Department of Paediatric and Adolescent Medicine, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
*
Correspondence to: M. Schrøder, MD, Department of Pediatric and Adolescent Medicine, Center of Adolescent Medicine, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, 2100-Denmark. Tel: +452 840 3244; Fax: +45 35454673; E-mail: mortenschroder@gmail.com
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Abstract

Purpose

We performed a systematic review and meta-analysis of observational studies assessing quality of life in adolescents and young adults born with CHD compared with age-matched controls.

Methods

We carried out a systematic search of the literature published in Medline, Embase, PsychINFO, and the Cochrane Library’s Database (1990–2013); two authors independently extracted data from the included studies. We used the Newcastle–Ottawa scale for quality assessment of studies. A random effects meta-analysis model was used. Heterogeneity was assessed using the I2-test.

Results

We included 18 studies with 1786 patients. The studies were of acceptable-to-good quality. The meta-analysis of six studies on quality of life showed no significant difference – mean difference: −1.31; 95% confidence intervals: −6.51 to +3.89, I2=90.9% – between adolescents and young adults with CHD and controls. Similar results were found in 10 studies not eligible for the meta-analysis. In subdomains, it seems that patients had reduced physical quality of life; however, social functioning was comparable or better compared with controls.

Conclusion

For the first time in a meta-analysis, we have shown that quality of life in adolescents and young adults with CHD is not reduced when compared with age-matched controls.

Keywords

CHDquality of lifeadolescentsyoung adultsoutcome
Type
Review Articles
Information
Cardiology in the Young , Volume 26 , Supplement 3 , March 2016 , pp. 415 - 425
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Copyright
© Cambridge University Press 2015 

Nearly 1% of all children are born with CHD.Reference Hoffman and Kaplan 1 Improved treatment for CHD over the past few decades has dramatically decreased the morbidity and mortality for these patients.Reference Greutmann and Tobler 2 – Reference Boneva, Botto, Moore, Yang, Correa and Erickson 4 As a result of this, ~85–90% of children with CHD currently survive to adulthood.Reference Somerville 5 – Reference Warnes, Liberthson and Danielson 7 The population of surviving patients with CHD is, thus, increasing by 5%/year. Should this trend continue, there will be more adults than children living with CHD 10 years from now.Reference Brickner, Hillis and Lange 6 , Reference Webb 8 , Reference Moons, Van Deyk, Marquet, De Bleser, De Geest and Budts 9

In examining long-term outcomes in children born with CHD, there has been an increasing focus on quality of life as a supplement to other outcome measures such as morbidity, mortality, and paraclinical and physiological measures.Reference Lane, Lip and Millane 10 – Reference Tong, Sparacino, Messias, Foote, Chesla and Gilliss 12 Quality of life is a multidimensional tool assessing an individual’s physical, mental/emotional, and social functioning,Reference Koot and Wallander 13 thereby encompassing dimensions that biomedical outcome measures do not.Reference Waters, Davis, Ronen, Rosenbaum, Livingston and Saigal 14

Earlier reviews assessing quality of life in adolescents and young adults with CHD concluded that quality of life was reduced compared with healthy peers.Reference Latal, Helfricht, Fischer, Bauersfeld and Landolt 15 , Reference Dahan-Oliel, Majnemer and Mazer 16 In this review, we have identified several studies not previously included in reviews, thus providing a more up-to-date assessment. Furthermore, this is the first time that a meta-analysis assessing quality of life in adolescents and young adults with CHD compared with age-matched controls has been carried out.

Materials and methods

This systematic review and meta-analysis was performed with guidance from the Cochrane Handbook of Systematic Reviews,Reference Higgins and Green 17 the PRISMA guidelines for meta-analysis and systematic reviews,Reference Liberati, Altman and Tetzlaff 18 and the MOOSE guidelines for meta-analyses of observational studiesReference Stroup, Berlin and Morton 19 (see Supplementary table 1). Before initiating the study process, the study protocol was registered in the PROSPERO database for systematic reviews (registration number CRD42013005699) (see Supplementary table 2).

Data sources and search strategies

In accordance with recommendations,Reference Harris, Quatman, Manring, Siston and Flanigan 20 we made a preliminary protocol with a pilot study as the first step. This allowed us to make the appropriate corrections to the study design and search strategy for the final study protocol.

In September, 2013, we conducted searches in the following electronic databases with the help of an experienced health science librarian: Medline, Embase, PsychINFO, and the Cochrane Central Register of Controlled Trials. Furthermore, we manually examined reference lists from all the selected articles and reviews to identify additional studies.

For this review, two independent researchers – M.S. and senior researchers G.T./K.B./J.B./J.R. – performed study and data collection individually. Search citations were screened based on the title and abstracts, and finally the full text was reviewed. Researchers settled disagreements by discussion until consensus. We used medical keywords as well as subject headings and text word combinations for the search. The study flow chart is displayed in Figure 1. An example of a full search string is given in Supplementary file 3.

Figure 1 Study flow selection diagram.

Study eligibility

In the systematic review, we included all quantitative studies that assessed quality of life in adolescents (12–18 years) and/or young adults (19–30 years) with CHD. Acquired heart disease and heart disease associated with genetic disorders were not included. Disease severity was categorised as mild, moderate, or severe according to the classification of Warnes et alReference Warnes, Williams and Bashore 21 For inclusion in the meta-analysis, studies had to present a quantitative assessment of quality of life preferably on a scale from 0 to 100 with higher scores indicating better quality of life.

Studies were included if >50% of the patients were between 12 and 30 years of age. We also included studies if it was possible to use subgroup data from this specific age group regardless of mean age.

The comparison groups in eligible studies had to consist of healthy age-matched controls from the background population. Studies were also included if they compared outcomes between groups with different types of CHD or if they compared patients with test norms from the background population. Studies were not included if no control group or comparative data were reported.

In studies assessing surgical patients with CHD, we included studies where patients had undergone surgery from 1990 to 2013. This period was chosen because we wanted to assess the current patient population and to ensure that time-dependent advances in surgical treatments were minimised.Reference Greutmann and Tobler 2 – Reference Boneva, Botto, Moore, Yang, Correa and Erickson 4

We excluded studies with parent and proxy reports, because several studies have shown that parent and proxy reports yield different resultsReference Latal, Helfricht, Fischer, Bauersfeld and Landolt 15 , Reference Landolt, Valsangiacomo Buechel and Latal 22 , Reference Spijkerboer, EMWJ, Bogers, Verhulst and Helbing 23 and because we believe that young people should and could answer for themselves. All the eligibility criteria are listed in Table 1.

Table 1 Eligibility criteria.

QOL=quality of life; RCT=randomised controlled trial

Quality assessment of included studies

The Newcastle–Ottawa scale was moderated to fit our study design and served to assess the quality of the eligible studies.Reference Higgins and Green 17 , Reference Wells, Shea and O’Connell 24 The Newcastle–Ottawa scale scores each study by assigning 0 to 9 stars and assesses the studies in three domains – selection of patients and controls, comparability between groups, and outcome and follow-up; 0 to 3 stars indicate poor study quality, 4 to 6 stars indicate acceptable study quality, and 7 to 9 stars indicate good study quality.

Statistical analysis

For the meta-analysis, a forest plot was calculated using weighted scores and a random effects model. We chose the random effects model over the fixed effects model because it accounts for the variations between studies,Reference DerSimonian and Laird 25 which we expected due to inclusion of different types of CHDs. Standardised mean difference was used in the meta-analysis for combining continuous data as we expected different scores to assess quality of life. The degree of heterogeneity across studies was determined using the I2-test, with I2 values of 25 or less, 50, and 75% or greater representing low, moderate, and high inconsistency, respectively; p<0.05 was considered to be statistically significant. All the statistical calculations were performed with the assistance of a statistician using R Statistical and Programming Software (version 2.9.0.) (R Foundation for Statistical Computing, Vienna, Austria).

Results

We identified 562 unique articles (see Fig 1). Of these, 18 studies (n=1746 patients) met the eligibility criteria,Reference Spijkerboer, EMWJ, Bogers, Verhulst and Helbing 23 , Reference Berkes, Varni, Pataki, Kardos, Kemény and Mogyorósy 26 – Reference Overgaard, Schrader and Lisby 42 and six studies (n=328 patients) were pooled in the meta-analysis.Reference Brothers, McBride and Marino 27 , Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 , Reference Kwon, Mussatto, Simpson, Brosig, Nugent and Samyn 31 , Reference Tahirović, Begić, Tahirović and Varni 39 – Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41 All the included studies were cross-sectional studies (n=14) or retrospective cohort studies (n=4). The included studies encompassed mild, moderate, or severe forms of CHD. Characteristics of the included studies are displayed in Table 2.

Table 2 Characteristics of studies included in systematic review and meta-analyses.

ASD=atrial septal defect; QOL=quality of life; VSD=ventricular septal defect

The mean Newcastle–Ottawa Score was 6.8 (range from four to eight) corresponding to acceptable study quality (see Table 2). The detailed scoring for each study is displayed in Supplementary table 4.

All six studies included in the meta-analysis used validated tools that assessed quality of life on a scale of 0 to 100 with higher scores indicating better quality of life. Table 3 displays the different tools for assessing quality of life.

Table 3 Characteristics and selected raw data of studies included in meta-analyses.

ASD=atrial septal defect; QOL=quality of life; VSD=ventricular septal defect

The meta-analysis showed no significant difference in quality of life between adolescents and young adults with CHD and age-matched controls. Standardised mean difference in average scores for quality of life between the two groups was −1.31 with 95% confidence intervals: −6.51 to +3.89. Heterogeneity (I2) was 90.9%, indicating a high degree of inconsistency (see Fig 2).

Figure 2 Forest plot of quality of life in adolescents and young adults compared with age-matched controls. Each horizontal line represents a study and the raw data of each study are displayed. In the graph, each study is represented by a box whose size correlates to the weight of the study, whereas the line through the box represents the 95% confidence intervals. Studies located on the left side of the mean difference line report worse quality of life in patients versus controls, whereas studies on the right side of the mean difference line report better quality of life in patients. The diamond at the bottom of the graph is the meta-analytic summary that shows no significant difference in quality of life between patients and controls as the confidence intervals cross the mean difference line. Study 1: Uzark et al,Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41 United States of America (2008); Study 2: Brothers et al,Reference Brothers, McBride and Marino 27 United States of America (2009); Study 3: Gierat-Haponuik et al,Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 Poland (2011); Study 4: Kwon et al,Reference Kwon, Mussatto, Simpson, Brosig, Nugent and Samyn 31 United States of America (2011); Study 5: Tahirović et al,Reference Tahirović, Begić, Tahirović and Varni 39 Bosnia Herzagovina (2011); Study 6: Teixeira et al,Reference Teixeira, Coelho and Proença 40 Portugal (2011).

Of the six studies included in the meta-analysis (n=328 patients), three studies assessed a mixed population of patients,Reference Tahirović, Begić, Tahirović and Varni 39 – Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41 whereas the other three studies assessed a smaller group of patients with specific forms of CHD.Reference Brothers, McBride and Marino 27 , Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 , Reference Kwon, Mussatto, Simpson, Brosig, Nugent and Samyn 31 Mild, moderate, and severe forms of CHD were represented in the meta-analysis, and interestingly the three studies encompassing severe CHDsReference Tahirović, Begić, Tahirović and Varni 39 – Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41 were found on both sides of the mean difference (Fig 2).

Meta-analyses could only be performed on sum scores for quality of life. It was not possible to perform a meta-analysis on subdomain scores due to large heterogeneity in reporting styles.

A total of 12 studies were not included in the meta-analysis because they lacked sufficient data, and therefore could not meaningfully be pooled with the other studies in the meta-analysis. Examples include insufficient reporting of quantitative raw dataReference Larsen, McCrindle, Jacobsen, Johnsen, Emmertsen and Hjortdal 32 , Reference Manlhiot, Knezevich, Radojewski, Cullen-Dean, Williams and McCrindle 35 and use of alternative quality-of-life scales.Reference Luyckx, Missotten, Goossens and Moons 34 , Reference Spijkerboer, Utens, De Koning, Bogers, Helbing and Verhulst 38 All 12 studies were cross-sectional studies or retrospective cohort studies that used validated tools based on self-reporting to assess quality of life. The 12 studies covered the whole spectrum of CHD from mild to severe forms, including both surgically corrected anomalies and anomalies that did not require surgical intervention. See Table 2 for details of the included studies.

A total of 8 out of 12 studies concluded that quality of life in adolescents and young adults with CHD was comparable with age-matched controls;Reference Berkes, Varni, Pataki, Kardos, Kemény and Mogyorósy 26 , Reference Cohen, Mansoor, Langut and Lorber 28 , Reference D’Udekem, Cheung and Setyapranata 29 , Reference Larsen, McCrindle, Jacobsen, Johnsen, Emmertsen and Hjortdal 32 , Reference Luyckx, Missotten, Goossens and Moons 34 , Reference Pike, Evangelista, Doering, Eastwood, Lewis and Child 36 , Reference Spijkerboer, Utens, De Koning, Bogers, Helbing and Verhulst 38 , Reference Overgaard, Schrader and Lisby 42 two other studies reported that quality of life was comparable or better in adolescents and young adults with CHD.Reference Spijkerboer, EMWJ, Bogers, Verhulst and Helbing 23 , Reference Loup, von Weissenfluh, Gahl, Schwerzmann, Carrel and Kadner 33 In contrast to these findings, one study reported that quality of life in adolescents and young adults with CHD was reduced compared with age-matched controls.Reference Manlhiot, Knezevich, Radojewski, Cullen-Dean, Williams and McCrindle 35

A total of seven studies reported that the severity of CHD was negatively associated with quality of life.Reference Berkes, Varni, Pataki, Kardos, Kemény and Mogyorósy 26 , Reference Cohen, Mansoor, Langut and Lorber 28 , Reference Larsen, McCrindle, Jacobsen, Johnsen, Emmertsen and Hjortdal 32 – Reference Luyckx, Missotten, Goossens and Moons 34 , Reference Simko and McGinnis 37 , Reference Overgaard, Schrader and Lisby 42 In contrast to this, four studies reported that the severity of CHD did not have an impact on quality of life.Reference Spijkerboer, EMWJ, Bogers, Verhulst and Helbing 23 , Reference Spijkerboer, Utens, De Koning, Bogers, Helbing and Verhulst 38 , Reference Teixeira, Coelho and Proença 40 , Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41

No studies specifically compared quality of life between adolescents (12–18 years) and young adults (>18 years), and adequate subgroup analyses were not possible due to heterogeneity in the study design. In nine studies encompassing adolescents, seven studiesReference Spijkerboer, EMWJ, Bogers, Verhulst and Helbing 23 , Reference Berkes, Varni, Pataki, Kardos, Kemény and Mogyorósy 26 , Reference Brothers, McBride and Marino 27 , Reference Kwon, Mussatto, Simpson, Brosig, Nugent and Samyn 31 , Reference Larsen, McCrindle, Jacobsen, Johnsen, Emmertsen and Hjortdal 32 , Reference Spijkerboer, Utens, De Koning, Bogers, Helbing and Verhulst 38 , Reference Tahirović, Begić, Tahirović and Varni 39 found better or comparable quality of life, whereas two studiesReference Manlhiot, Knezevich, Radojewski, Cullen-Dean, Williams and McCrindle 35 , Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41 found worse quality of life. In six studies encompassing young adults, the numbers were fiveReference D’Udekem, Cheung and Setyapranata 29 , Reference Loup, von Weissenfluh, Gahl, Schwerzmann, Carrel and Kadner 33 , Reference Pike, Evangelista, Doering, Eastwood, Lewis and Child 36 , Reference Teixeira, Coelho and Proença 40 , Reference Overgaard, Schrader and Lisby 42 and one,Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 respectively.

When looking at the different domains of quality of life, four studiesReference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 , Reference Manlhiot, Knezevich, Radojewski, Cullen-Dean, Williams and McCrindle 35 , Reference Pike, Evangelista, Doering, Eastwood, Lewis and Child 36 , Reference Teixeira, Coelho and Proença 40 reported that CHD affected physical domains, for example, resulting in diminished physical activity.Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 In contrast to these findings, two studies reported that there was no association between exercise capacity and quality of life.Reference Brothers, McBride and Marino 27 , Reference Loup, von Weissenfluh, Gahl, Schwerzmann, Carrel and Kadner 33

Social functioning in adolescents and young adults with CHD was comparable with or was even better compared with age-matched controls in five studies.Reference Larsen, McCrindle, Jacobsen, Johnsen, Emmertsen and Hjortdal 32 , Reference Luyckx, Missotten, Goossens and Moons 34 , Reference Manlhiot, Knezevich, Radojewski, Cullen-Dean, Williams and McCrindle 35 , Reference Tahirović, Begić, Tahirović and Varni 39 , Reference Teixeira, Coelho and Proença 40 In accordance with this, two studies highlighted that social support plays a positive and important role for the overall quality of life.Reference Luyckx, Missotten, Goossens and Moons 34 , Reference Teixeira, Coelho and Proença 40

A total of four studies reported that high scores of quality of life were positively associated with higher achievement in the educational system and higher levels of education in adolescents and young adults with CHD.Reference Cohen, Mansoor, Langut and Lorber 28 , Reference Tahirović, Begić, Tahirović and Varni 39 , Reference Teixeira, Coelho and Proença 40 , Reference Bisoi, Murala and Airan 43

Discussion

In the present meta-analysis, we found that quality of life in adolescents and young adults born with CHD is not reduced compared with healthy age-matched controls. To our knowledge, this is the first time that a meta-analysis assessing quality of life in adolescents and young adults has been carried out. We found it clinically meaningful and relevant to pool the six selected studies in the meta-analysis, because these studies included similar age groups, similar types of CHDs, and used a validated quality-of-life tool with identical or similar scales to assess quality of life. Owing to heterogeneity in study design and reporting methods, further quantitative subgroup analyses were not possible; however, we found that studies encompassing severe CHDs were found on both sides of the mean difference in the forest plot, indicating that severe CHD is not necessarily associated with worse quality of life.

The result from the meta-analysis is supported by similar findings in the systematic review of the remaining 12 studies, where 10 out of 12 studies reported that quality of life in adolescents and young adults with CHD was similar or better compared with age-matched controls. In contrast, only 1 out of the 12 studies reported that quality of life in adolescents and young adults with CHD was worse. We found no difference in quality of life when comparing adolescents and young adults with CHD.

When looking at the studies that found worse quality of life in patients with CHD, an explanation for these findings may be reduced physical activity compared with controls based on the conviction of having some sort of undefined disability.Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 This may be caused by being treated as handicapped/ill by their family and the society.Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 , Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41 Living with the conviction of having some sort of disability and being treated like an ill/handicapped person by the society might also explain a general feeling of insufficiency.Reference Gierat-Haponiuk, Haponiuk, Chojnicki, Jaworski and Bakuła 30 , Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni 41 There may be a small subgroup of patients with severe heart disease, and thus impaired level of functioning and/or quality of life – for example, young patients in palliative care.

When looking at the subdomains of quality of life, we found that a majority of studies reported that patients with CHD had worse quality of life in the physical domains compared with controls. On the other hand, a majority of studies found that adolescents and young adults with CHD had comparable or even better social functioning compared with age-matched controls.

Strengths and weaknesses

Our study includes an exhaustive and reproducible search strategy according to a registered protocol. In addition, the included studies comprised all types of CHDs and covered studies from a geographically large area, thereby making the results relevant for a mixed, general population of adolescents and adults with CHD. Finally, the present review included several studies published within the last few years, thus providing an up-to-date long-term assessment of quality of life in adolescents and young adults with CHD.

There are several limitations to our analysis. The overall estimate of the meta-analysis should be interpreted with caution, as the total number of included studies in the meta-analysis was relatively small and the degree of heterogeneity was high. In addition, the heterogeneity between studies may complicate direct comparisons across studies; however, we believe that our eligibility criteria resulted in a group of selected studies that in many ways were comparable, also seen from a clinical point of view. Another limitation was that almost all the studies were cross-sectional studies, ranking in the lower end of the evidence hierarchy; however, to our knowledge, no additional cohort or case–control studies currently exist in this field. The meta-analysis included studies using scales from 0 to 100. Therefore, some studies were not included in the quantitative analysis but were only included in the systematic review. Finally, the average quality of the included studies was acceptable when rated based on the Newcastle–Ottawa scale; however, there exists no official threshold for distinguishing between poor-, acceptable-, and good-quality studies.

Interestingly, our results are contradictory to previous reviews that reported a reduced quality of life in adolescents and young adults with CHD compared with age-matched peers.Reference Latal, Helfricht, Fischer, Bauersfeld and Landolt 15 , Reference Dahan-Oliel, Majnemer and Mazer 16 In comparison with the findings of Dahan-Oliel et al,Reference Dahan-Oliel, Majnemer and Mazer 16 the reason for our contradictory results may be that Dahan-Oliel et alReference Dahan-Oliel, Majnemer and Mazer 16 included studies with patients who had undergone surgery before 1990. Thus, the surgery and the postoperative care might not meet today’s standards and might result in a reduced long-term outcome reflected in the domains of quality of life.Reference Greutmann and Tobler 2 – Reference Boneva, Botto, Moore, Yang, Correa and Erickson 4 Latal et alReference Latal, Helfricht, Fischer, Bauersfeld and Landolt 15 only included patients who had undergone open-heart surgery with the expectation of more patients in the severe disease category. In contrast, we included mild, moderate, and severe types of CHD. Moreover, our study included 12 studies published between 2008 and 2013 that were not available to Latal et alReference Latal, Helfricht, Fischer, Bauersfeld and Landolt 15 who conducted their review in 2008. Finally, Latal et alReference Latal, Helfricht, Fischer, Bauersfeld and Landolt 15 allowed both self-reporting and proxy reporting for quality of life, whereas we included only studies with self-reporting of quality of life. In comparison with the findings in the systematic review by Fteropoulli et al,Reference Fteropoulli, Stygall, Cullen, Deanfield and Newman 44 we found similar results, as Fteropoulli et al concluded that the quality of life of adult CHD patients is compromised in the physical domain compared with their healthy counterparts. In contrast to this – and again in accordance with our findings – no differences were found in relation to the psychosocial and environmental/occupational domains.

An explanation for our somewhat surprising and counter-intuitive finding has been previously investigated in other studies,Reference Moons, Van Deyk, Marquet, De Bleser, De Geest and Budts 9 , Reference Moons, Van Deyk and De Bleser 45 finding three possible coping mechanisms: “The disability paradox”Reference Albrecht and Devlieger 46 referring to the idea that people living with a chronic condition may experience good quality of life if they accept their impairment; “Sense of coherence”, which can positively affect an individual’s perception of quality of life by feelings of high comprehensibility, manageability, and meaningfulness;Reference Antonovsky 47 and “Response shift”, which is the change in the meaning of one’s self-evaluation as a result of a change in internal standards and values.Reference Rapkin and Schwartz 48 Another explanation for our results is a possible under-representation of severe CHD in the meta-analysis, where four of the six studies predominantly included mild-to-moderate CHDs; however, these studies also included severe CHDs, whereas one other study included severe CHD only. In terms of generalisability of our results, it is important to stress that mild-to-moderate CHD is far more common than severe CHD.

Most likely, a smaller group of patients with severe forms of CHD will have impaired quality of life in adolescence and adulthood; however, some of these patients may still have a better quality of life than that previously considered. Therefore, the present review can assist physicians in giving a more up-to-date view regarding the future when counselling patients and their parents.

When comparing our results with previous reviews,Reference Latal, Helfricht, Fischer, Bauersfeld and Landolt 15 , Reference Dahan-Oliel, Majnemer and Mazer 16 , Reference Fteropoulli, Stygall, Cullen, Deanfield and Newman 44 we believe that there is a tendency towards improving quality of life in adolescents and adults with CHD over the last few decades; however, continuous follow-up and vigorous efforts should be made to further improve the quality of life for these patients, especially in the physical domains of quality of life, where the adolescents and adults with CHD scored lower than age-matched controls. Lower physical quality of life has definite health implications. It is, therefore, of great importance to promote physical activity in this population, especially to promote a healthy lifestyle during adulthood. In addition, previous studies have found that quality of life is positively correlated with a low level of anxiety and depression, a good knowledge of the cardiac condition, adequate social support, and a strong sense of coherence.Reference Wang, Hay, Clarke and Menahem 49 Furthermore, it has previously been stressed that participation in age-expected activities such as education, having a job, participating in recreational and social activities, developing intimate relationships, and living independently are important supplemental measures of how this group of patients is doing.Reference Imms 50 , Reference Mackie, Islam and Magill-Evans 51 It is, therefore, highly important that comprehensive transitional programmes are developed and implemented addressing these issues. Hopefully, the upcoming results from the APPROACH-IS study can help shed light over these important issues.Reference Apers, Kovacs and Luyckx 52

For the first time in a meta-analysis, we have shown that quality of life in adolescents and young adults with CHD is not reduced when compared with age-matched controls. The findings in the qualitative assessment where the majority of studies reported that quality of life in adolescents and young adults living with CHD was comparable with age-matched controls support this result. In subdomains, it seems that patients had reduced physical quality of life; however, social functioning was comparable or better compared with controls.

Acknowledgements

The authors thank Professor Jacob Rosenberg, Statistician Thomas Kallemose, and Neonatologist James Dodd.

Financial Support

There were no study sponsors for the present study, and none of the authors received any form of financial support. All the five authors wrote the first draft of the manuscript, and no honorarium, grant, or other forms of payment were given to anyone to produce the manuscript.

Conflicts of Interest

None of the authors have any potential, perceived, or real conflicts of interest.

Supplementary Material

To view supplementary material for this article, please visit http://dx.doi.org/10.1017/S104795111500181X

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Figure 0

Figure 1 Study flow selection diagram.

Figure 1

Table 1 Eligibility criteria.

Figure 2

Table 2 Characteristics of studies included in systematic review and meta-analyses.

Figure 3

Table 3 Characteristics and selected raw data of studies included in meta-analyses.

Figure 4

Figure 2 Forest plot of quality of life in adolescents and young adults compared with age-matched controls. Each horizontal line represents a study and the raw data of each study are displayed. In the graph, each study is represented by a box whose size correlates to the weight of the study, whereas the line through the box represents the 95% confidence intervals. Studies located on the left side of the mean difference line report worse quality of life in patients versus controls, whereas studies on the right side of the mean difference line report better quality of life in patients. The diamond at the bottom of the graph is the meta-analytic summary that shows no significant difference in quality of life between patients and controls as the confidence intervals cross the mean difference line. Study 1: Uzark et al,41 United States of America (2008); Study 2: Brothers et al,27 United States of America (2009); Study 3: Gierat-Haponuik et al,30 Poland (2011); Study 4: Kwon et al,31 United States of America (2011); Study 5: Tahirović et al,39 Bosnia Herzagovina (2011); Study 6: Teixeira et al,40 Portugal (2011).

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