Summary
Increasing survival rates of patients with congenital heart disease have resulted in a new and growing patient population of adults with operated congenital heart disease. Medical professionals face the specific medical needs of these patients but must also deal with their daily life issues. Adult patients with congenital heart disease report difficulties in several areas of daily life, such as sport, employment, insurability and travel or driving. Moreover, they must have a healthy lifestyle to prevent cardiovascular complications. All these issues can be addressed in a specific educational program. In this review, we discuss the different daily life issues of adults with congenital heart disease and the preventive measures that can be proposed to improve their quality of life.
Résumé
L’augmentation de la survie des patients ayant une cardiopathie congénitale a conduit à l’émergence d’une population sans cesse croissante d’adultes ayant été opérés d’une cardiopathie congénitale. Aujourd’hui, les professionnels de santé font face aux besoins médicaux spécifiques de ces patients, mais doivent également répondre aux questions qui concernent leur vie quotidienne. Ces patients rapportent en effet des difficultés dans plusieurs domaines de la vie quotidienne comme le sport, l’emploi, l’assurabilité, les voyages ou la conduite de véhicule. Par ailleurs, ils doivent maintenir une bonne hygiène de vie pour prévenir les complications cardiovasculaires communes. Toutes ces questions peuvent être abordées dans un programme éducatif spécifique. Dans cette revue, nous discutons ces différentes questions de la vie quotidienne des adultes ayant une cardiopathie congénitale et les mesures préventives pouvant être proposées pour améliorer leur qualité de vie.
Introduction
The improvement in surgical techniques over the last decades has led to a dramatic increase in survival rates of patients with congenital heart disease (CHD). This success has resulted in the emergence of a growing population of adults with operated CHD. Medical professionals are currently managing the specific medical needs of these patients but have to deal concomitantly with their daily life issues. A minority of adults with mild CHD but a majority of those with complex CHD report difficulties in their daily life , with an impact on quality of life . It is of note that a high proportion of these patients have an inadequate level of knowledge about their disease and their potential in a variety of areas . Patients with complex CHD frequently felt that the direct limitation to their participation in sport, employment or education was their heart disease. Knowledge about reproduction issues, ‘family planning’ and risk related to pregnancy was found to be poor among women with CHD . An additional difficulty is access of these patients to life insurance and loan applications . Together with these issues, adult patients with CHD should have preventive attitudes in supplementary domains to prevent common cardiovascular complications and endocarditis. Acquiring autonomy to tackle all these aspects of daily life with a CHD is a complex process for adolescents and young adults. The development of a specific transition/transfer program with counseling and educational content related to these issues is crucial for this population. Hopefully, developing these programs should lead to a reduction in complications and an improvement in quality of life.
In this review, we will discuss the key daily life issues for patients with adult congenital heart disease (ACHD). These issues are sport and more general physical activities, employment, family planning, risky behaviors, traveling and driving. We will also analyze the proposals that have been made in the literature for managing these difficulties.
Physical activity
Exercise capacity is a major issue in ACHD that has a significant impact on daily activity and quality of life. Additionally, physical activity has a direct link with cardiac status and a variety of risks, such as arrhythmias or syncope. A recent review by Kempny et al. aggregated the available data on exercise capacity in ACHD comparing cardiopulmonary exercise test results in ACHD in their institution with the data published by other centres. This study confirmed that exercise capacity differs significantly across the spectrum of ACHD. The practical clinical consequence of these findings is that improving exercise capacity through aerobic training is probably worthwhile in virtually all ACHD patients. Kempny et al. proposed different occupations according to peak oxygen uptake (VO 2 ) results derived from their study. These findings could help greatly in choosing/prescribing recreational activities when counseling a patient with ACHD.
Today, it is widely recognized that physical activity has long-term beneficial effects not only on quality of life but also on long-term morbidity and overall mortality . As an example, exercise training in chronic heart failure reduces heart failure-related events, including mortality and hospital admission for worsening of heart failure . Data remain scarce on the feasibility and efficacy of exercise training programs in ACHD patients . Still, all these programs were safe and they all demonstrated an increase in exercise capacity or an improvement in quality of life ( Table 1 ), despite the controversial link between quality of life and exercise capacity. Indeed, self-estimated physical functioning, evaluated using quality of life scales, poorly predicts actual exercise capacity . It is worthwhile noting that even patients with pulmonary hypertension, who have the poorest VO 2 , experienced improvement in exercise endurance, symptomatic status and quality of life after carefully designed exercise training . The limited compliance of the patients was the main limitation in these studies that prescribed physical training. Indeed, Swan et al. showed that only one third of ACHD patients regularly practiced at least moderate exercise and one third had no physical activity despite their mean age being 26 years . To be efficient on outcomes, these programs have to be developed over a long period of time. To be sustainable, the programs need patient education reinforcement and are therefore necessarily time consuming. Patient education on the short- and long-term benefits of exercise must be part of ACHD management. Recommendations regarding physical training, exercise and sport should begin in early adolescence ( Fig. 1 ). It is important that the patient and their family are informed that exercise restriction is very rarely indicated. Obviously, safety is a major concern when prescribing exercise and athletic activities for young adults with cardiac disease, especially those with hypertrophic cardiomyopathy, coronary artery anomalies, Marfan syndrome and aortic valve disease, which have all been associated with sudden cardiac death. For major ACHD groups, recommendations are provided by the American Heart Association . Certainly, these guidelines should be adapted to individual status. To guide the exercise prescription and the choice of recreational activities, a cardiopulmonary exercise test may provide valuable information on the level/type of activity that is appropriate for each patient. Self-monitoring of physical activity through diaries and the use of simple devices such as accelerometers may also enhance awareness and motivation.
Education, employment and career
A recent multicentre study compared a large sample of adult patients with various types of CHD with a reference group, with regard to sociodemographic characteristics and lifestyle. The study showed that young ACHD patients (aged < 40 years) had poorer outcomes in educational attainment, employment and relationships compared with the reference group. This difference was found not only for moderate-to-severe defects but also for mild defects. These poorer outcomes were more pronounced in men, as in former studies . Actually, there could be discrimination against job applicants when they declare they have a CHD. Certainly, complex CHDs have become more prevalent than in the past – when patients did not survive until adulthood – and are more often associated with disability; their impact on employment might be consequently more visible. While Kamphuis et al. and Ternestedt et al. found no differences in employment status for ACHD patients compared with a reference group, the latter study did indeed find a clear difference between mild and severe defects, with better employment status for those with mild defects. Another cause for these differences in employment might be educational attainment, which Van Rijen et al. reported to be lower in young ACHD patients compared with in a reference group. Absence from school because of illness, need for hospitalization, recovery from invasive procedures and learning disabilities/neurocognitive defects could explain these poorer outcomes in patients with CHD .
In 2005, Crossland et al. reported that 33% of ACHD patients were unemployed (16% in matched controls) and that 25% had been unemployed for more than 1 year (3% in matched controls). In this study, only one fifth of ACHD patients received counseling regarding their career, while this counseling was more frequent in controls (31%). In 42% of ACHD patients, this counseling aimed to exclude some inappropriate occupations. Receiving career counseling was significantly associated with increased employment in the ACHD population, with 73% of ACHD patients given advice being employed compared with 46% of those not given advice. This pattern was not seen in controls. ACHD patients are less likely to receive useful advice regarding potential careers and find the advice given less helpful than controls, although receiving suitable advice can be considered efficient in improving the proportion of ACHD patients who get a job. Many patients were advised not to apply for specific jobs. This advice was often given by medical staff not trained in this field or by career advisors not trained to give advice based on the medical condition of the patient.
A variety of solutions have been proposed to improve the employment status of ACHD patients; the most straightforward seems to be helpful, positive, medically appropriate advice concerning potential careers. Guidelines based on CHD diagnosis are far from being perfect. This is particularly true for those lesions considered to be significant, as there is a wide variation in cardiac status within each headline diagnosis . Kempny et al. compared the required peak VO 2 values for a professional activity with the distribution of oxygen uptake expected for a given CHD condition. These results could assist clinicians in deciding how likely it is that young patients with CHD may be able to cope with the physical demands of a job as a career. Individualized discussion with the patient, involving both the cardiologist and the career advisor, preferably at a single clinic, seems to be more appropriate. Counselling the patient and their family on this delicate issue during the patient’s early adolescence facilitates appropriate career planning and overcomes unrealistic expectations.
In the study by Crossland et al. , more than half of patients with complex ACHD experienced problems in their careers. Physical problems were frequently reported as a specific reason for quitting a job. The most important specific adaptations mentioned concerned working time and workload. Career counseling and use of job adjustment, taking into account physical abilities, are important in the ACHD population to prevent unemployment. In France, the ‘Labour Code’ recognizes the quality of disabled worker ( reconnaissance qualité travailleur handicapé [RQTH]). When a patient applies, there is a hearing at the maison départementale des personnes handicapées . This procedure can be followed by an ACHD patient who is seeking a job or by an employed patient who requires an RQTH or an RQTH renewal. This labelling can be useful in ACHD patients by improving access to work or keeping them in their job.
Family planning
Sexual activity is an important component of quality of life for men and women with cardiovascular disease and their partners. The American Heart Association provides guidelines and recommends that physicians and other healthcare professionals have dialogue with patients on this topic. Reported deaths or strokes during sexual activity in the ACHD population are rare. In a study by Vigl et al. , 9% of women with ACHD reported symptoms during sexual activity, which included dyspnoea, perceived arrhythmia, increased fatigue or syncope. Symptoms were more common in patients with severe lesions, worse functional status or cyanosis. In another survey of men with ACHD, 9% reported dyspnoea or subjective arrhythmias and 5% reported chest pain during sexual activity. Again, symptoms were more common in patients with greater functional impairment (New York Heart Association class III) . According to the American Heart Association guidelines, sexual activity is safe in most patients with ACHD . Patients in whom the safety of sexual activity is less certain or unclear are those with significant pulmonary hypertension, cyanotic heart disease, severe left-sided heart outflow obstruction, uncontrolled arrhythmias and anomalous coronary artery passing between the pulmonary artery and aorta .
Contraception and pregnancy have now become important issues in this population. Both can, however, be associated with increased risks in women with ACHD . Other issues, such as adverse foetal outcomes and recurrence of CHD in offspring, must be addressed. Current guidelines recommend proactive counseling about contraception and pregnancy . Of note, information regarding the risks of contraception and pregnancy is currently ignored by women with ACHD Overall, sexual activity in adolescents with CHD and ACHD is lower than in matched controls but women are more sexually active than men. This also emphasizes the importance of starting an education program on birth control and pregnancy-associated risks very early in adolescence in female patients with CHD. Poor family functioning seems to promote risky sexual behavior . In education programs on birth control, these risks must be detected in adolescents. Specialist cardiologists or gynecologists must insist on their prevention and check patient knowledge on this topic.
A high proportion of women with ACHD have limited knowledge of the appropriate contraceptive method they should use and the risk of pregnancy to their own health . In these studies , 43–49% of the women had not been counseled about contraception, 20% used contraceptive methods that were contraindicated for their specific cardiac condition and 28% in the group with high pregnancy-associated risks were not using contraception despite having a sexual relationship . For the group of women considered to be at intermediate or high risk of complications during pregnancy, 37–48% did not recall receiving this information. Further, only half of women with contraindications to pregnancy recalled having received this information . Accurate information on contraception and the implications of CHD on pregnancy should be given through an education program starting during adolescence ( Table 2 ). There are several models to address this issue, including a collaborative approach between ACHD and contraception clinics, or the addition of contraception and pregnancy counseling by advanced practice nurses within ACHD clinics.
