Study
Sample size
Type of heart defect
Design
Instrument to measure quality of life
Comparison with control group
Chen et al. [20]
n = 289
Various types of ConHD
Cross-sectional
WHOQOL-BREF
Yes: age-matched general population
Gierat-Haponiuk et al. [21]
n = 30
Patients who underwent surgery for simple left-to-right shunts
Cross-sectional
EuroQOL-5D
Yes: healthy volunteers
Görler et al. [22]
n = 98
Patients after surgical repair of transposition of the great arteries (atrial versus arterial switch operation)
Cross-sectional
The Short Form (36) Health Survey
Yes: standard population
Mokhles et al. [23]
n = 463
Patients with a right ventricular outflow tract reconstruction with an allograft conduit
Cross-sectional
The Short Form (36) Health Survey
Yes: age-adjusted Dutch population norms
Overgaard et al. [14]
n = 62
Adult survivors with single-ventricle physiology
Cross-sectional
Linear Analog Scale Quality of Life; Satisfaction with Life Scale
Yes: healthy controls
Silva et al. [24]
n = 40
Various types of ConHD
Cross-sectional
WHOQOL-BREF
Yes: general population
Tay et al. [25]
n = 25
Patients with cyanotic ConHD and/or the Eisenmenger syndrome
Longitudinal (2 measurements: baseline and after 3 months)
CAMPHOR
No
Tay et al. [26]
n = 12
Patients with various types of ConHD with Eisenmenger syndrome
Longitudinal (2 measurements: baseline and after 3 months)
CAMPHOR
No
Teixeira et al. [27]
n = 74
Various types of ConHD
Cross-sectional
WHOQOL-BREF
Yes: Portuguese population as a whole
Vigl et al. [28]
n = 676
Various types of ConHD
Cross-sectional
The Short Form (36) Health Survey; single-item self-assessment of life satisfaction (ordinal scale from 1 to 7, transformed arithmetically to scale from 0 to 100)
Yes: age- and sex-matched controls of a representative national survey
Cotts et al. [15]
n = 25
Patients with congenitally corrected transposition of the great arteries
Cross-sectional
Linear Analog Scale Quality of Life; Satisfaction with Life Scale
Yes: adults with mild, haemodynamically insignificant defects
Müller et al. [29]
n = 330
Various types of ConHD
Cross-sectional
The Short Form (36) Health Survey
No
Opić et al. [16]
n = 26
Patients with tetralogy of Fallot and an implantable cardioverter defibrillator
Cross-sectional
Linear Analog Scale Quality of Life; Satisfaction with Life Scale
Yes: patients with an implantable cardioverter defibrillator, without tetralogy of Fallot
Schoormans et al. [18]
n = 302
Various types of ConHD
Cross-sectional
Satisfaction with Life Scale; TNO/AZL Adult Quality of Life-ConHD
No
Bang et al. [30]
n = 85
Various types of ConHD
Cross-sectional
WHOQOL-BREF
No
Cha et al. [31]
n = 13
Patients with Eisenmenger syndrome
Longitudinal (2 measurements: baseline and after 6 months)
The Short Form (12) Health Survey
No
Enomoto et al. [32]
n = 72
Various types of ConHD
Cross-sectional
The Short Form (36) Health Survey
Yes: Japanese normative data
Eslami et al. [19]
n = 346
Various types of ConHD
Cross-sectional
WHOQOL-BREF; Satisfaction with Life Scale
Yes: non-ConHD participants, matched by sex and age
Kahya Eren et al. [33]
n = 69
Patients with atrial septal defect following percutaneous closure
Cross-sectional
The Short Form (36) Health Survey
Yes: healthy controls matched according to age, sex, educational level, and economic, marital, and employment status
Müller et al. [34]
n = 56
Patients that had undergone arterial switch operation and atrial redirection (Senning procedure) for transposition of the great arteries
Cross-sectional
The Short Form (36) Health Survey
No
Müller et al. [35]
n = 546
Various types of ConHD
Cross-sectional
The Short Form (36) Health Survey
No
Ruys et al. [36]
n = 18
Patients after arterial switch operation
Cross-sectional
The Short Form (36) Health Survey
Yes: age- and gender-specific Dutch population sample
Younge et al. [37]
n = 245
Patients who had undergone corrective open-heart surgery
Cross-sectional
The Short Form (36) Health Survey
No
Schoormans et al. [38]
n = 845
Various types of ConHD
Longitudinal (2 measurements: baseline and after 2 years)
The Short Form (36) Health Survey; TAAQOL-ConHD
No
7.2.2 Comparison with Controls
Fourteen of the 24 published studies compared QOL in adults with ConHD to a healthy [14, 21, 33] or community [19, 21–24, 27, 28, 32, 36] control group or to other patient populations without ConHD [15, 16]. Four studies found that QOL is worse in patients with ConHD as compared to control subjects [14, 15, 19, 21], whereas three studies concluded that QOL is similar in patients and controls [16, 22, 33]. Further, three studies even found a better QOL in patients with ConHD as compared to a control group [24, 27, 36]. Additionally, three studies examined the scores on different subscales of QOL (i.e. SF-36) and found both significantly lower and significantly higher scores in patients with ConHD as compared to controls on different subscales of QOL, as well as no differences between both groups for certain subscales [23, 28, 32]. Finally, one study found that women with ConHD had worse QOL than control subjects, while men with ConHD did not differ in terms of QOL from controls [20]. The fact that the results are inconsistent could be due to methodological limitations and differences among the studies.
7.2.3 Determinants of Quality of Life
Knowledge of the factors contributing to QOL is of utmost importance in order to improve the effects of therapy and treatment. Sixteen of the 24 studies published on QOL in ACHD examined potential determinants of QOL [14–16, 18–20, 24, 27–30, 33–35, 37, 38]. Table 7.2 provides an overview of the potential determinants of QOL that were investigated.
Table 7.2
Overview of potential determinants of quality of life in adults with congenital heart disease
Chen et al. [20] | Overgaard et al. [14] | Silva et al. [24] | Teixeira et al. [27] | Vigl et al. [28] | Cotts et al. [15] | Müller et al. [29] | Opic et al. (2012) | Schoormans et al. [47] | Bang et al. [30] | Eslami et al. [19] | Kahya Eren et al. [33] | Müller et al. [34] | Müller et al. [35] | Younge et al. [37] | Schoormans et al. [38] | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Demographic characteristics | ||||||||||||||||
Older age | ↗ | = | ↘ | = | ↘ | ↗/↘ | ||||||||||
Female sex | ↘ | = | = | ↘ | ↗ | ↘ | ||||||||||
Having children | ↗ | |||||||||||||||
Higher academic performance or education | ↗ | ↗ | ↗ | ↗ | ||||||||||||
Higher employment rate or being employed | ↗ | ↗ | ||||||||||||||
More daily activities | ↗ | |||||||||||||||
Absence of financial strain | ↗ | |||||||||||||||
Lower employment status | ↗ | |||||||||||||||
Annual income | = | |||||||||||||||
Clinical characteristics | ||||||||||||||||
Cardiac surgery | ↘ | ↘ | ↘ | |||||||||||||
Surgical strategy (arterial switch compared to Senning) | ↗ | |||||||||||||||
Cardiac catheterisation | ↗ | |||||||||||||||
ConHD hospitalisation | ↘ | |||||||||||||||
Medication | = | ↘ | = | |||||||||||||
Better functional class | ↗ | ↗ | ↗ | |||||||||||||
More severe heart defects | ↘ | = | = | ↘ | = | ↘/↗ | ||||||||||
More severe residua | ↘ | = | ||||||||||||||
ConHD subtype or diagnosis | = | = | ||||||||||||||
Cyanosis | = | |||||||||||||||
Implantable cardioverter defibrillator | ↘ | |||||||||||||||
Physical limitations | ↘ | |||||||||||||||
Better exercise capacity | ↗ | |||||||||||||||
Time of diagnosis | = | |||||||||||||||
ConHD illness course | = | |||||||||||||||
Higher BMI | ↗ | |||||||||||||||
Having somatic symptoms | ↘ | |||||||||||||||
Higher levels of N-terminal pro-brain natriuretic peptide | ↘
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