Quality of life

Over the past decade, two reviews of the literature on quality of life in patients with congenital cardiac disease have been published.Reference Moons, Van Deyk, Budts and De Geest^3, Reference Latal, Helfricht, Fischer, Bauersfeld and Landolt⁴ The first review was published in 2004 and focused on the conceptual and methodological rigour of studies about quality of life in children, adolescents, and adults with congenital cardiac disease.Reference Moons, Van Deyk, Budts and De Geest³ Overall, 70 articles were reviewed that included 8206 patients. This review revealed the following information:

• • 24% of the articles reviewed did not measure quality of life, but drew conclusions in terms of quality of life;

• • 43% of the articles did not describe quality of life in the sections of the manuscript about methods or results, but merely in the abstract or discussion;

• • 1% of the articles provided a conceptual definition of quality of life;

• • 24% explicitly stated the domains measured as components of quality of life; and

• • 3% gave a reason for choosing the instruments used.Reference Moons, Van Deyk, Budts and De Geest³

The authors concluded that the poor conceptual and methodological basis used in these studies implies that many results of studies about quality of life in patients with congenital cardiac disease were inconclusive. The authors, therefore, plead for more conceptual and methodological rigour with respect to future studies about quality of life.Reference Moons, Van Deyk, Budts and De Geest³

The second review was published in 2009 and addressed psychological adjustment and quality of life in children and adolescents following open cardiac surgery for congenital cardiac disease.Reference Latal, Helfricht, Fischer, Bauersfeld and Landolt⁴ Latal et al identified 12 articles on quality of life in patients aged 2–17 years.

• • In four studies, quality of life was comparable to normative samples;

• • In four other studies, an impaired quality of life was observed;

• • One study found a normal quality of life in 90% of patients;

• • One study revealed that patients who underwent arterial switch operation for transposition of the great arteries had a better quality of life than patients after atrial switch repair;

• • One study found a low agreement in quality of life assessment between parents and patients; and

• • One study addressed the psychometric properties of a quality-of-life instrument.Reference Latal, Helfricht, Fischer, Bauersfeld and Landolt⁴

Direct comparison between the studies is, however, not possible.

Some studies compared the quality of life of adult-aged patients with that of the general population or with normative data.Reference Fekkes, Kamphuis and Ottenkamp^8–Reference Jefferies, Noonan, Keller, Wilson and Griffith²² In general, previous studies found that quality of life in adult-aged patients is equivalent to that of the general population. When quality of life is measured in terms of functional status, scores of groups of patients are found to be lower than those of normative groups.Reference Kamphuis, Ottenkamp and Vliegen^15–Reference Simko and McGinnis^18, Reference Rose, Kohler, Kohler, Sawitzky, Fliege and Klapp^20, Reference Jefferies, Noonan, Keller, Wilson and Griffith²² Conversely, one study found that patients with mild cardiac defects showed a better quality of life, if they did not present with social restrictions.Reference Fekkes, Kamphuis and Ottenkamp⁸ Another study, in which quality of life was operationalised in terms of satisfaction with life, found that adults with congenital cardiac disease had a better quality of life than did their healthy counterparts.Reference Moons, Van Deyk and De Bleser² In addition, descriptive studies reported an excellent quality of life in patients with congenital cardiac disease.Reference Moons, De Bleser and Budts²³

Taken together, it can be concluded that patients with congenital cardiac disease reported a good quality of life. However, subjective perceptions of patients do not always correspond with objective measurements. A recent study, for instance, found that most patients severely overestimate their physical functioning compared to actual results of exercise testing.Reference Gratz, Hess and Hager²⁴ Therefore, exercise tests and quality-of-life instruments should be used on a complementary basis,Reference Hager and Hess²⁵ and cannot be used interchangeably.

There exist two major approaches for measuring quality of life:

• • the “need approach” and

• • the “want approach”.Reference Häyry²⁶

According to the need approach, quality of life depends on fulfilment of basic needs, such as

• • good health,

• • sufficient mobility,

• • good physical performance,

• • adequate nutrition, and

• • favourable shelter.

In this approach, quality of life is measured using standardised and pre-defined questionnaires about components or determinants of quality of life. This approach assumes that the relative importance of all items is equal among all respondents. The majority of studies about quality of life in patients with congenital cardiac disease use this approach.

By contrast, the want approach assumes that quality of life can only be affected by factors important to an individual.Reference Häyry²⁶ For example, according to the want approach, quality of life depends on

• • lifestyle,

• • previous experiences,

• • ambitions, and

• • dreams.Reference Häyry²⁶

Hence, in this approach, quality of life must be measured with instruments that permit respondents to indicate and, respectively, rate domains that are specifically important for their quality of life, that is, individual quality of life. In the field of congenital cardiac disease, studies about quality of life using the want approach are very scant.Reference Moons, Van Deyk and Marquet²⁷

The term quality of life is often erroneously interchanged with “health status” or “functional status”,Reference Moons²⁸ and therefore we do not describe these issues separately as distinct elements of patient-reported outcomes in this paper.

Symptoms

Dedicated research about symptoms in congenital cardiac disease is limited. Despite numerous studies anecdotally reporting the prevalence of symptoms in selected groups of patients, to the best of our knowledge, only one study has specifically addressed the experience of symptoms in adults with congenital cardiac disease.Reference Moons, Van Deyk, Marquet, De Bleser, Budts and De Geest²⁹ This study was a secondary analysis of data that was collected in a large-scale study about quality of life that included 629 adult-aged patients. The version of the CHD-TAAQOLReference Kamphuis, Zwinderman and Vogels³⁰ containing 77 items was used to assess the disease-specific determinants of quality of life. This questionnaire includes 13 items referring to cardiac symptoms. For each item, both the perceived frequency and associated distress were scored. Adults with congenital cardiac disease, including both men and women, reported the following five most frequently occurring symptoms:

• • shortness of breath after strolling 1–5 kilometres,

• • excessive perspiration,

• • dizziness,

• • palpitations, and

• • getting up often at night to go to the toilet.

Men with congenital cardiac disease reported the following most distressing symptoms:

• • difficulty in breathing while lying down,

• • shortness of breath after strolling less than 100 metres,

• • excess fluid in ankles, legs, and/or abdomen,

• • palpitation, and

• • shortness of breath after strolling 1–5 kilometres.

Women with congenital cardiac disease reported the following most distressing symptoms:

• • fainting,

• • difficulty breathing while lying down,

• • excessive perspiration,

• • severe bleeding due to anticoagulants, and

• • palpitations.

Women reported significantly greater frequency of symptoms and greater distress associated with symptoms than men.Reference Moons, Van Deyk, Marquet, De Bleser, Budts and De Geest²⁹ In conclusion, this study showed that the most frequently occurring symptoms are not necessarily the most distressing ones, and that women with congenital cardiac disease report a higher frequency of symptoms and greater distress associated with symptoms than men with congenital cardiac disease.

Adherence to treatment

The World Health Organization has defined adherence as the extent to which the behaviour of a patient corresponds with the agreed recommendations from a provider of health care.Reference Sabate³¹ In 2009, a systematic review of the literature with respect to issues of adherence to treatment among children with congenital and acquired cardiac disease, including recipients of cardiac transplants, was published.Reference Ittenbach, Cassedy, Marino, Spicer and Drotar³² Only a few studies were identified. In these studies, rates of adherence ranged from 96% for an in-patient programme of exercise, to 33% among those who made all of their medical appointments.Reference Ittenbach, Cassedy, Marino, Spicer and Drotar³²

More recently, a study on adherence to oral therapy for anticoagulation after replacement of a cardiac valve with a mechanical prosthesis in 57 patients was published; 25 of these patients had congenital defects of the cardiac valve.Reference Van Damme, Van Deyk, Budts, Verhamme and Moons³³ This study reported the following findings:

• • 72.2% of patients indicated that they were 100% adherent,

• • 75% stated that they did not miss one dose of their medication during the last month,

• • 14.3% missed one dose during the last month,

• • 7.1% missed one dose during the last 14 days,

• • 3.6% missed one dose during the last week, and

• • no patients reported a drug holiday, which was defined as missing two consecutive doses.Reference Van Damme, Van Deyk, Budts, Verhamme and Moons³³

Satisfaction with treatment

As far as we know, two studies have been published on the experiences of patients with the care that they received for congenital cardiac disease.Reference Wray and Maynard^34, Reference Moons, Pinxten and Dedroog³⁵ Both studies investigated experiences with respect to the transfer from paediatric cardiology towards services for adults. The first study was conducted in a large programme for patients with congenital cardiac disease in the United Kingdom. In this study, 38 patients aged 17–20 years completed a semi-structured questionnaire including open-ended questions. This yielded both quantitative and qualitative data. Patients reported that they more frequently received explanations about treatments and were more involved in the making of decisions in the adult-oriented areas than in paediatric areas. The following issues were important to adolescents and young people in the transition from paediatric cardiology towards services for adults:

• • having family/network around,

• • being informed,

• • being prepared,

• • being involved in the making of decisions,

• • reassurance of the expertise of specialists,

• • gaining confidence in new doctors,

• • fitting in with the new team,

• • not feeling lost in adult-oriented care, and

• • keeping records safe.Reference Wray and Maynard³⁴

In the second study, 14 adolescents with congenital cardiac disease, aged 15–17 years, were interviewed about their expectations and experiences on being transferred from paediatric cardiology to the programme for adults with congenital cardiac disease at a large centre of tertiary care in Belgium.Reference Moons, Pinxten and Dedroog³⁵ Of the participating patients, four had been transferred recently and 10 were planned for transfer. Hence, only four patients could report on their actual experiences, whereas the other 10 reflected on their expectations. Adolescents who were already transferred highlighted some key differences between paediatric cardiology and the programme for adults with congenital cardiac disease. For example, these patients were unaware that the two outpatient clinic settings, paediatric and adult, had a different sequence of the stages, such as electrocardiogram, echocardiogram, visit with the nurse-specialist, visit with the cardiologist, and so on. Despite their acknowledging a need to adjust, they also wanted to be informed about these differences. Indeed, adolescents who had had their first visit at the programme for adults with congenital cardiac disease were satisfied with the treatment that they received, but stated in retrospect that they had been largely unaware of what the outpatient visit would entail. Both the adolescents and their parents were unsure of whether parents were allowed to be present during the contacts with the providers of health care.Reference Moons, Pinxten and Dedroog³⁵

Utility or preference-based measures

Utility refers to preference-based valuations of the state of health, which are frequently used in evaluations of cost in health care. Measures of utility provide a quantitative estimate of preferences for particular states of health, primarily obtained from a representative sample of the general population. For each state of health, a corresponding index value ranging from 0 to 1 is computed. An index of 0 corresponds to death, while an index of 1 corresponds to perfect health.Reference Dolan, Gudex, Kind and Williams³⁶ To date, no utility or preference-based studies in congenital cardiac disease have been reported.