Methods

Patients under follow-up with congenitally malformed hearts were selected at random from the departmental database, and sent a letter asking them if they would like to participate in the study. Patients were also recruited at our clinic for adults with congenital heart disease. In total, we approached 320 patients over the age of 16 years with congenital heart disease, and 299 of these agreed to participate. We excluded any patients known to have difficulties with learning. The structured questionnaire was posed in person by one of our research nurses, either in a quiet room in the outpatient department or in the home of the patient. Questions were asked under the categories of education, advice regarding potential careers, employment, and exercise ability. The majority of the questions required a “yes” or “no” answer to be ticked, such as “are you currently employed” and “have you ever been out of work for more than a year”? Questions that required more elaboration were left open. For example, the question “have you ever been advised against a specific occupation?”, which required the answer of “yes” or “no”, was followed by “who gave you this advice?” Including an explanation of the study, and the time required to obtain consent, the questionnaire took between 20 and 30 minutes to complete.

The patients with congenitally malformed hearts were divided into categories of mild, significant, and complex lesions, attempting to take into account the original diagnosis, previous surgery, and the need for future intervention. Mild disease was classified as those needing observation only, with no prospect of intervention in the foreseeable future. This included lesions such as mild aortic or mitral regurgitation, mild pulmonary stenosis, and those with atrial or ventricular septal defects subsequent to repair. Significant lesions included those in whom a correction has been attempted, but who are likely to require further intervention, or to have further medical problems related to their congenital cardiac malformation. Such patients included those with tetralogy of Fallot, those who had undergone a Ross procedure, those with repaired coarctation, and those with repaired atrioventricular septal defect with common valvar orifice. Complex lesions are those where corrective surgery could not be carried out, including those with the Eisenmenger reaction, those whose pulmonary circulations were shunt-dependant, those with Fontan circulations, and those with the morphologically right ventricle supplying the systemic circulation.

To obtain a control group of similar social background and expectations to the population of patients with congenitally malformed hearts, each adult with congenital heart disease was asked to give an identical questionnaire to a friend without congenital heart disease. Of the prospective controls, 177 posted back their questionnaire. The questionnaire included detailed questions about physical activity so that we were able to calculate accurately the standing within the classification of the New York Heart Association in both groups.

We used McNemar's test to compare matched pairs, and the Chi-squared test to compare proportions. If patients, or either of a matched pair, had not answered a question, they were excluded from the denominator for that question.

Ethics approval was obtained from the Joint Ethics Committee of Newcastle and North Tyneside Health Authority, University of Newcastle upon Tyne, and the University of Northumbria and Newcastle.

Results: all patients with congenital heart disease

Of the 299 patients, 137 were male (46 per cent), and the mean age was 30.9 years, with a range of 17–83 years. Within our categories, 109 (36 per cent) were deemed to have mild disease, 144 (48 per cent) significant disease, and 46 (16 per cent) severe disease. Excluding students, those on maternity leave, and those who had retired, 90 of 273 (33 per cent) were not working at the time of the questionnaire. Of this 273, 66 had been out of work at some point for more than a year. This high rate of unemployment is seen across the spectrum of congenital cardiac malformations, with no significant difference in rates of unemployment according to the severity of the different lesions (Table 1). The rate of employment of men and women with congenitally malformed hearts was the same, with 44 of 132 (33 per cent) men, and 46 of 141 (33 per cent) women, not working at the time of the questionnaire. Women were more likely to be working part time than men, with 31 of 95 (33 per cent) women compared to 7 of 60 (12 per cent) men employed part time, p equal to 0.0011. At the time of the study, the regional rate of unemployment was 8.3 per cent, and the national rate was 6.1 per cent.¹⁴

Table 1. Unemployment rates at different severities of congenital heart disease.

Results: comparison of matched pairs

Of those with congenitally malformed hearts whose control replied, 81 of 177 (46 per cent) were male, compared to 69 of 177 (39 per cent) controls. The mean age for the matched group with congenital heart disease was 32.4 years, with a range of 17–83 years, and the mean age of their matched controls was 32.9, with a range of 17–81 years.

Employment

Students, those on maternity leave, and those who had retired, were again excluded from the analysis regarding employment. When compared to matched controls, adults with congenital heart disease were significantly more likely to be out of work, and also more likely to have been out of work for greater than 1 year (Table 2). Although more of those with congenitally malformed hearts had limitations in their capacity to exercise, the difference in rates of employment remains significant when only cases and controls with the same tolerance as judged using the categorisation of the New York Heart Association are compared (Table 2). Among those who were employed, there was no significant difference in the proportions of each group working part time, with 17 of 87 (21 per cent) of those with congenitally malformed hearts working part time, compared to 26 of 87 (30 per cent) controls, p equal to 0.14.

Table 2. Unemployment rates in adults with congenital heart disease and their controls. “Exercise tolerance matched” includes only those matched pairs in which both the patient and control are in the same category within the system devised by the New York Heart Association.

Career advice and education

The mean age at leaving school was 16.4 years both for the adults with congenital heart disease and their controls, with a mean difference in age at leaving school between matched pairs of less than 0.1 years. Advice regarding potential careers, whether or not the advice was thought to be helpful, and participation in educational and vocational courses are shown in Table 3. Despite being less likely to consider the advice offered helpful, receipt of advice in the overall group of those with congenitally malformed hearts was associated with being employed, with 157 of 216 (73 per cent) patients who had received advice being employed, compared to 25 of 54 (46 per cent) who had not received advice being employed, p equal to 0.0002. This pattern was not significant in controls, with 122 of 146 (84 per cent) controls who had received advice being employed, compared to 17 of 21 (81 per cent) controls not receiving advice being employed, p equal to 0.09. A greater proportion of the patients with congenitally malformed hearts had been given advice against a specific career. A cardiologist had given this advice in 43 per cent of cases, a career advisor in 24 per cent, and a general practitioner in 11 per cent.

Table 3. Participation in further courses, and advice given concerning potential careers, in the patients with congenital heart disease and their matched controls. Numbers are patients answering “yes” to each question.

Discussion

To the best of our knowledge, ours is the first large study using matched controls to examine the state of employment of adults with congenital heart disease. We have shown that such adults are significantly more likely to be unemployed than their matched controls. It is of great concern that over a quarter of our patients has been out of work for more than 1 year. It is worth noting that both our patients and their matched controls have high rates of unemployment compared to the regional rate of unemployment. This is due in part to different definitions of unemployment, but it clearly illustrates the importance of having a properly controlled population. The reasons for this difference in unemployment are not straightforward, particularly given that unemployment was high across the whole spectrum of congenital cardiac malformations, and when taking into account limitations of exercise. The age at leaving school was comparable between the two groups, although we did not ask about academic achievement. Interestingly, more of the patients with congenitally malformed hearts proceeded to further education or a course, suggesting that motivation is not a problem. Given the ultimate outcome for employment, a likely explanation for this is that going onto further training is a reflection of difficulty in finding work, and that the courses were undertaken in an effort to gain employment. In the United States of America, it has been shown that patients with aortic stenosis, pulmonary stenosis, or a ventricular septal defect achieve a higher educational level than do the general population, and subsequently go on to have comparable rates of employment.¹⁰

The situation with advice regarding potential careers is unsatisfactory. As expected, similar numbers received advice, though fewer of those with congenital heart disease had found the advice helpful. Many were given advice against specific jobs, often by medical staff not trained to balance this with directed positive advice, or by career advisors who are not trained to give advice based on the medical condition of the patient. We have shown that, despite not finding the advice helpful, receiving advice was associated with being employed in those with congenitally malformed hearts. This may not be as a direct result of the advice given, as those who seek advice may also be more likely to obtain employment.

There are many possible ways of trying to improve the states of employment of adults with congenitally malformed hearts. The most obvious is helpful, positive, yet medically appropriate advice concerning potential careers. Guidelines based on each diagnosis, to which the advisor can refer, are far from perfect. This is particularly true for those lesions considered to be significant, as there is a wide variation in haemodynamic outcome within each headline diagnosis.^15–17 Individualised discussion with the patient, involving both the cardiologist and the career advisor, preferably at a single clinic, seems more appropriate. An adolescent or transition clinic, as advised by the intercollegiate working party of the United Kingdom on adolescent health, would seem an ideal forum for such discussion.¹⁸ We found disappointing rates of employment for all severities of disease, and did not see the worse rates of employment in those deemed to have more complex disease, as had been noted by others.^{12, 13} All our patients were chosen from a group of patients with congenitally malformed hearts under follow up, not a cohort followed from birth, and it may be that the label of follow-up is a disadvantage as far as application for employment is concerned. Educating potential employers about individual patients, seeking to prevent overestimation of the problems associated with mild disease, and underestimation of the difficulties created by complex disease, could help individual applications. Patients themselves may not be able to give a sufficiently accurate summary of their underlying condition, putting off potential employers even if the disease is mild.¹⁹

Limitations of our study

We have shown a strong correlation between having a congenitally malformed heart and being out of work. Further investigation is required to ascertain the exact reasons for this. We did not ask whether our patients who were unemployed were actively looking for work, whether any had taken early retirement, or how many of the patients, be they employed or unemployed, had told prospective employers about their congenital malformations. Although we assume that the congenital malformation was itself a significant factor in any difficulty in finding work, there may have been other comorbidities influencing the outcome with regard to employment. Further studies are needed to look into whether patients who are working are in the employment of their choice, and whether they experience difficulties with progression of their careers, or other aspects of work such a pension because of their congenitally malformed heart.

The population of adults with congenital heart disease is increasing. The majority does not require intervention, and is surgically cured or corrected. Irrespective of the severity of their disease, this population has a high rate of unemployment, potentially affecting social inclusion and the quality of their life. As a group, they find the advice given about potential careers to be unhelpful. We consider that it is important to discuss and address these issues at clinics designed specifically for adolescents and adults with congenital heart disease so as to make our patients aware of the potential difficulties, and help them find ways to overcome them.

Acknowledgements

Our study was supported by a grant from the Ann Coleman research fund. We thank Mark Pearce for his statistical advice and calculations.