Quality of life in adults with congenital heart disease.

The number ofadults with congenital heart disease is growing as a result of advances in medical and surgical treatment. Many patients with corrected or palliated congenital heart disease will suffer from medical complications. Mortality and morbidity are the conventional measures for the effects oftreatment and prevention in medicine. However in chronic disease, such as grown-up congenital heart disease, health-related quality oflife and psychosocialfsues are ofmajor importance. In the methodological literature, consensus about the definition of health-related quality of life is growing. Health-related quality oflife is considered to be a multidimensional construct, including domains ofphysical, social, cognitive and emotional functioning ofthe patient. Literature invariably shows considerable disagreement between patients and proxies (such as the parent, physician, nurse or others) in their ratings ofthe patient's health-related quality oflife. Therefore, it is commonly proposed that the perception ofthe patient is crucial to the concept ofhealthrelated quality of life. In addition, several authors have suggested a second level of subjectivity to the concept of health-related quality of life, i.e. the value that the patient assigns to the perception of functioning. In fact, we are not only interested in the patient's perception ofhis ability to climb the stairs (subjective health status), butwe also want to know his personal feelings about that ability or disability (health-related quality of life). Based on this concept, the Leiden Centre for Child Health and Paediatrics developed a dedicated questionnaire called the TNO-AZLAdult Quality of Life questionnaire (TAAQOL). 1 In November 2002, Dr. Mascha Kamphuis successfully defended her thesis: 'Quality oflife in adults with congenital heart disease.2 In this thesis, Dr. Kamphuis reported on the investigations, using amongst others the TAAQOL questionnaire, in patients with previously operated complex congenital heart disease, patients with mild congenital heart disease and reference groups. From a meta analysis on quality of life in adults it was concluded that health-related quality oflife in research with congenital heart disease patients is seldom defined clearly and measures often aim at different targets. Therefore, studies should be compared with great caution. Health-related quality of life outcome is surprisingly positive in most studies that measured congenital heart disease patients' own perceptions. This might be explained by coping mechanisms. In patients with previously operated complex congenital heart disease, investigated with the TAAQOL, the relation between these measures and physical indices were determined.3 The health-related quality oflife ofthe patients was significantly worse than that ofthe general population in the domains gross motor functioning and vitality. Correlations between healthrelated quality oflife and physical indices were poor. This result indicates that these patients need specific attention for their physical quality of life. Objectively determined physical indices are only weakly related to healthrelated quality oflife. Therefore, when evaluating quality oflife, dedicated questionnaires such as the TAAQOL should be used. In patients with haemodynamically insignificant congenital heart disease, of which only a minority were still under cardiac supervision, the health-related quality of life did not differ from that in the general population. Nevertheless, patients experienced unnecessary difficulties with