Reduced health-related quality of life in older patients with congenital heart disease: A cross sectional study in 2360 patients

Abstract

Objective

Health-related quality of life questionnaires are frequently used to involve patients' impressions and feelings in the outcome evaluation. In patients with congenital heart disease (CHD) methodological heterogeneities, assessment in different age and diagnostic groups led to controversial findings. This study aims to give a comprehensive answer to the health-related quality of life in patients with CHD.

Patients and methods

From July 2001 to June 2013, 2360 patients (1058 female, 28.6 ± 11.2 years, range 14–75 years) with various kinds of CHD underwent a quality of life assessment with the SF-36 questionnaire and underwent a cardiopulmonary exercise test as part of their routine follow-up.

Results

Physical component summary score (PCS) develops from 96.1 %predicted in patients younger than 20 years, to 96.2 %predicted in patients aged 20 to 30 years, 92.3 %predicted in patients aged 30 to 40 years, and 92.6 %predicted in patients 40 years or older (r = − .114; p < .001). The decline was more prominent in the mental component summary score (MCS) declining from 104.1 %predicted in patients younger than 20 years, to 103.4 %predicted, 99.9 %predicted, and 97.5 %predicted (r = − .132; p < .001). Exercise capacity was impaired with 80.1 ± 23.0 %predicted and also declined slowly with age (r = − .084; p < .001).

Conclusions

Health-related quality of life in patients with CHD is progressively reduced in older age-groups. The decline is small, but more prominent in the mental components. This should be considered in the interpretation of studies, and strategies have to be developed to reduce this effect in future.

Introduction

Advances in diagnosis, surgical treatment and medical management have led to a dramatic increase in survival of infants and children with congenital heart disease (CHD). This resulted in a still increasing number of adolescents and adults with CHD [1]. As a result, the focus in long-term follow-up studies changed from a quantitative point of view on survival and redo procedures, to a more functional perspective involving hemodynamics, exercise performance and especially quality of life [2], [3].

However, the concept of quality of life is in debate. There are several questionnaires: for healthy people and for patients; as well as generic and disease specific ones. In general, the more disease specific they are, the better is the sensitivity in treatment studies, but the more they leave the primary goal to measure quality of life. The disease specific instruments mainly ask for typical symptoms and resemble more a perceived health status than real quality of life which is defined as “satisfaction with life” [4]. Generic multidimensional “health-related quality of life” (HRQoL) instruments are somewhere intermediate. They include domains that are a pure perceived health status, as well as domains closer to quality of life [5]. Therefore, we have chosen for such an instrument, the SF-36. In clinical outcome studies, it is an optimal addition to a cardiopulmonary exercise test (CPET). CPET objectively measures aerobic capacity which is considered the best substitute for cardiovascular fitness. On the other hand, the SF-36 covers physical, psychological, and social limitations as well as their impact on daily life. Whereas the domain of Physical Functioning as perceived health status correlates at least weakly with aerobic capacity [6], the self-reported domain of Mental Health closely resembles quality of life.

Assessing HRQoL in patients with CHD is important and frequently used to involve patients in the process of decision making especially before high risk procedures to evaluate how much the patient feels limited by his/her cardiac condition, as well as in the outcome evaluation [7], [8]. Also for prenatal counseling of future parents awaiting a child with a congenital heart defect, information regarding the patients short- and long-term HRQoL is of major interest for the decision whether or not to terminate the pregnancy [9].

Meanwhile, many studies have evaluated HRQoL of life in children, adolescents [10], [11], [12], [13] or adults [3], [14], [15], [16], [17], [18], [19], [20] in general, as well as with specific heart defects [11], [21], [22], [23], [24], [25], [26] or after a specific intervention [27], [28], [29]. They found coping strategies, denial mechanisms and pathways like the sense of coherence [2], [30] to mediate HRQoL. However, methodological heterogeneities, different age groups and specific congenital heart diseases led to controversial findings [7], [8]. Moreover, the majority of those studies evaluated younger patient groups. Data from older patients reaching an age beyond forty years were missing.

Therefore, this cross-sectional report aims to give a comprehensive answer to the HRQoL of patients with CHD with regard to their age and diagnosis.