Analysis of associations between congenital heart defect complexity and health-related quality of life using a meta-analytic strategy
Introduction
In the current era, over 90% of children born with congenital heart disease (CHD) in developed countries survive to adulthood, most of them living relatively normal lives with good long-term survival prospects [1], [2]. With improving survival rates the focus of attention has shifted from mortality to morbidity, functional capacity and ultimately quality of life (QoL). Anticipating this move, Joseph Perloff argued in 1973 that “our ultimate goal … should focus on the quality of long-term survival … [and] to bring the individual to the peak of his[/her] physical capability” [3]. Testimony to this trend is a growing number of publications addressing functional aspects of patients' lives such as symptoms and objective exercise capacity. In addition, an increasing number of studies have been aiming to assess health-related QoL directly in CHD cohorts by applying patient-reported outcome measures (PROs), mostly questionnaires quantifying different aspects of QoL. As there is currently no consensus regarding the definition of QoL, many researchers have employed the World Health Organization's definition of health to investigate health-related QoL based on the five domains physical and mental health, level of independence, social relationships and environmental factors [4]. Unfortunately, the available primary evidence on health-related QoL in CHD is heterogeneous, sometimes even contradicting. This can partially be explained by differences in employed methods. After application of vigorous selection criteria, previous reviews have been able to describe these differences, [5], [6], [7], [8] but a quantitative meta-analysis of the existing data has not been achieved so far. Despite a large number of studies investigating QoL across the full spectrum of CHD patients, the effect of defect complexity on QoL is uncertain.
The aim of the current study was to systematically review the currently available literature on QoL in CHD with a particular focus on quantitative methods aiming to combine the results comparable with studies to overcome the limitations of small patient numbers and provide aggregate estimates on the association between CHD and CHD-sub-groups on different dimensions of QoL.
Section snippets
Search strategy
The PubMed online library was searched on April 25th 2014 for a combination of the phrases “quality of life” or “QoL” and a set of terms relating to all forms of congenital heart disease (Suppl. Table 1). After removal of duplicate results, studies were excluded based on language criteria (n = 66, Suppl. Fig. 1). All papers were screened by one author (PCK) and further papers were excluded if they met the following criteria: i) studies not including CHD patients (n = 213), ii) studies on relatives
Systematic search of the PubMed online library
Our systematic search resulted in a total list of 1015 publications, published between 1974 and 2014, that included the phrase “quality of life” in addition to a reference to CHD. After exclusion, 234 studies remained and were included in the further analyses (Suppl. Fig. 1). We found that the publication of studies that apply PROs in the setting of CHD showed an exponential increase over time (Fig. 1), with the maximum of published studies in 2013 (n = 45) and with ≈ 90% of studies being
Discussion
Using a systematic overview of the literature and meta-analytic methods we documented a growing interest in QoL in CHD patients over the last decades. Unfortunately, QoL-studies in this population are heterogeneous and difficult to compare due to the broad spectrum of CHD and the usage of more than 95 different QoL-instruments in the literature. Overall, however, we found significantly decreased QoL scores in the general health and physical functioning domains of the widely used SF36
Conclusions
Self-reported quality of life in CHD patients is — on aggregate — excellent and, in many domains of self-reported QoL as well as various CHD sub-populations indistinguishable from healthy individuals. Nevertheless, our findings indicate significant reductions of QoL in patients with moderate or high complexity CHD for the physical functioning and general health domains based on the most widely utilized PRO, the SF36 questionnaire.
The following are the supplementary data related to this article.
Funding sources
This study was supported by a research grant from the EMAH Stiftung Karla Voellm, Krefeld, Germany. Further funding was provided by the Cusanuswerk (to P.C.K.) and the German Research Foundation DFG (KA4018/1-1 to P.C.K.).
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