Summary
Background
In recent decades, advances in surgery and therapeutic catheterization have steadily increased the life expectancy and prevalence of adults with congenital heart disease (CHD).
Aims
We assessed medical and psychosocial variables of adults with CHD, according to the disease complexity.
Methods
We included, from a single-centre observational cohort study, 135 consecutive adults with CHD (median age of 40 years, interquartile range: 28.0–51.0) followed in our cardiology unit, who answered a questionnaire assessing daily activity and psychosocial functioning. Disease complexity was classified according to the Bethesda conference.
Results
Cardiac malformation complexity was simple in 61 (45.2%), moderate in 50 (37.0%) and complex in 24 (17.8%) patients. Cardiac surgery had been performed in 86.5% of moderate and complex patients. Complications (such as heart failure, arrhythmia and pulmonary hypertension) were mainly observed in the complex group ( P = 0.003). Physical activity was lower in the complex group (no activity in 58.8%, but sport previously contraindicated in 50% of these; P = 0.03). Education level tended to be lower in the complex and moderate groups than in the simple group (respectively, 31.2% and 33.3% vs. 45.7% had passed the Baccalaureate; P = 0.47). The pass rate was lower in patients with complications ( P = 0.037) or more than one cardiac surgery ( P = 0.03). In the complex group, 56.3% of patients were unemployed ( P = 0.048).
Conclusions
Complexity of heart disease and medical history affect education level and employment of adults with CHD. Academic education of children with a complex defect and career counselling are important to prevent unemployment among adults with CHD.
Résumé
Contexte
Grâce aux progrès médico-chirurgicaux ces dernières décennies, l’espérance de vie et la prévalence des adultes ayant une cardiopathie congénitale (ACC) ont constamment augmenté.
Objectif
Nous avons évalué les paramètres médicaux et psychosociaux des ACC selon la complexité de la cardiopathie.
Méthodes
Nous avons inclus consécutivement à partir d’une cohorte observationnelle monocentrique, 135 ACC (40 ± 12 ans) qui ont répondu à un questionnaire évaluant les aspects psychosociaux et de la vie quotidienne. La complexité de la cardiopathie était classée selon la conférence de Bethesda.
Résultats
La complexité de la cardiopathie était simple pour 61 patients (45,2 %), modérée pour 50 (37,0 %) et complexe pour 24 (17,8 %). Une chirurgie cardiaque a été effectuée pour 86,5 % des patients ayant une cardiopathie complexe ou modérée. Les complications (insuffisance cardiaque [ n = 15/24], arythmie [ n = 14/24], hypertension pulmonaire [ n = 6/24]) ont été principalement observées dans le groupe complexe ( p = 0,002). L’activité physique était moins importante dans le groupe complexe ( p = 0,03, et aucune activité pour 58,8 % mais 33 % avaient été contre indiqués). Les patients des groupes complexe et modéré ont tendance à avoir un niveau d’étude inférieur aux patients du groupe simple (respectivement 31,2 %, 33,3 % versus 45,7 % ont le Baccalauréat ; p = 0,47). L’obtention du Baccalauréat était plus faible pour les patients ayant au moins une complication ( p = 0,038) et plus d’une chirurgie cardiaque ( p = 0,03). Dans le groupe complexe, 56,3 % des patients étaient au chômage ( p = 0,048).
Conclusion
La complexité de la cardiopathie et l’histoire médicale des patients impactent le niveau d’éducation et taux d’emploi des ACC. La scolarité de ces enfants et l’orientation professionnelle sont importantes pour prévenir le chômage.
Background
Improved imaging, catheterization, medical therapy and surgical techniques in recent decades have led to the increased survival of children with congenital heart diseases (CHDs). Around 90% of children with CHD now reach adulthood . By 2010, adults accounted for 66% of the entire CHD population ; many of them require further medical care and surgery or catheterization during adulthood to treat residual lesions or complications, such as heart failure, arrhythmia or pulmonary hypertension . Dedicated attention is also required to deal with daily life issues . However, loss of follow-up of adults with CHD is a critical issue, and occurs particularly at the time of transition from paediatric to adult care . Thus programmes for education and for the transition from paediatric to adult medical care have been recommended .
The World Health Organization (WHO) defined health as ‘a state of complete physical, mental and social wellbeing, and not merely the absence of disease or infirmity’. Thus, it is important to address not only physical health, but also emotional and social functioning, together with occupational performance.
Sociodemographic factors, such as educational level or employment status, influence quality of life in healthy people as well as in chronically ill individuals . Little is known about the rate of psychosocial issues in adults with CHD in France.
The French Health Ministry encourages educational programmes at transition for adolescents with chronic disease . We aimed to evaluate how heart disease influences schooling and employment in CHD patients, to help to address career issues in the transition programme.
Methods
Population
We prospectively included 135 consecutive adults with CHD (age ≥ 18 years) who were followed in the University Hospital of Toulouse between January 2013 and December 2013. Patients were included during consultations for the regular follow-up of CHD or during hospitalization. Patients with asymptomatic bicuspid aortic valve, mitral valve prolapse, Marfan syndrome, cardiomyopathy or congenital arrhythmia were not included.
The examination started with the provision of oral and written information about the aim of the study, and informed consent was signed. The medical examination lasted 30 minutes, including the completion of a questionnaire and a physical examination. The study was performed in accordance with the principles set out in the Declaration of Helsinki and was declared to the National commission for data processing and freedoms (No. 1673455v0).
From medical records and clinical interviews, we collected demographic data as well as data on medical and surgical history. Baseline characteristics included demographics (age, sex) and medical history. The complexity of CHD was classified as simple, moderate or complex, according to the Bethesda conference criteria .
Daily life symptomatic status was assessed according to the New York Heart Association (NYHA) classification. Complications during adulthood were recorded and defined by history of arrhythmias, heart failure, pulmonary hypertension, endocarditis, sudden death and stroke. Arrhythmia was defined as any type of ventricular or supraventricular arrhythmia or bradycardia requiring therapy. Heart failure was defined as the need for specific medication or systemic ventricular dysfunction with symptoms and/or elevated brain natriuretic peptide . Pulmonary hypertension was diagnosed according to the fifth World Symposium on Pulmonary Hypertension . Stroke included transient ischaemic attack, and was diagnosed by neurological examination combined with neurological imaging. Endocarditis was diagnosed according to the modified Duke criteria . To assess the burden of hospitalization for the study population, we collected all hospitalization indications during the year 2013.
Sociodemographic indicators
The assessment of sociodemographic factors was performed using a self-administered questionnaire in French, developed by the investigators and delivered to the patient during consultation or hospitalization.
We recorded medical follow-up, education level, employment and physical activity. To assess education level, the number of patients with the ‘Baccalaureate’ (French academic qualification at the end of secondary education, corresponding to British A level) was noted, as well as the number of years of postgraduate schooling. Patients declared employment and the part-time or full-time character of their job. Physical activity was investigated via a standardized validated questionnaire (WHO-MONICA Optional Study of Physical Activity Questionnaire) , and was categorized into four levels: no physical activity; mild physical activity (20 minutes no more than once a week); moderate physical activity (20 minutes once or twice a week); and intense physical activity (20 minutes, at least three times a week). Lifestyle habits were collected, including alcohol consumption, smoking and drug addiction. The presence of tattoos and piercing, including ear piercing, was noted.
According to the WHO, alcohol consumption was defined as a patient drinking one glass of alcohol less frequently than every day (low-risk consumption). Alcoholism was defined as a patient drinking every day (high-risk consumption).
Statistical analysis
Statistical analysis was performed using Stata ® software, version 11.2 (StataCorp, College Station, TX, USA). Quantitative measurements are expressed as medians (interquartile ranges [IQRs]). Qualitative data are expressed as counts and percentages. The distribution of quantitative variables was assessed using the Shapiro-Wilk test. A comparative analysis of variables among patients classified as having simple, moderate or complex disease was performed. Patients with genetic syndrome and mental disability were excluded from analysis related to socioprofessional status. To compare categorical data, the χ 2 test was used or a non-parametric Fisher’s exact test when prerequisites for the χ 2 test were not met. A non-parametric median test was used to compare quantitative data between the three groups. The Mann-Whitney test was used to compare quantitative data within two groups. A P value < 0.05 was considered statistically significant.
Results
Study population
Data on demographics and medical history are reported in Table 1 . The study included 135 patients with a median age of 40 years (IQR: 28.0–51.0). The distribution of CHD type is reported in Fig. 1 . The study population involved 61 (45.2%) patients with simple CHD, 50 (37.0%) with moderate CHD and 24 (17.8%) with complex CHD. Atrial septal defect was the most frequent CHD ( n = 49; 47 ostium secundum and two sinus venosus). Of the nine patients with genetic syndrome, three had Down syndrome, two had Williams syndrome and NKX2.5 syndrome, and one had Noonan syndrome and Di-George syndrome.
| All patients ( n = 135) | Simple CHD ( n = 61; 45.2%) | Moderate CHD ( n = 50; 37.0%) | Complex CHD ( n = 24; 17.8%) | P | |
|---|---|---|---|---|---|
| Age (years) | 40.0 (28.0–51.0) | 45.0 (33.0–59.0) | 35.5 (25.0–45.0) | 35.5 (23.0–41.5) | 0.006 a |
| Men | 52 (38.5) | 21 (34.4) | 18 (36.0) | 13 (54.2) | 0.22 |
| Diagnosis during childhood | 88 (65.2) | 22 (36.1) | 44 (88.0) | 22 (91.7) | < 0.001 b |
| Genetic syndrome | 9 (6.7) | 4 (6.6) | 5 (10.0) | 0 (0.0) | 0.35 b |
| Hospitalization | 70 (51.8) | 33 (54.1) | 22 (44.0) | 15 (62.5) | 0.29 |
| Catheterization | 43 (31.9) | 25 (41.0) | 11 (22.0) | 7 (29.2) | 0.10 |
| Electrophysiology | 11 (8.1) | 1 (1.7) | 4 (8.0) | 6 (25.0) | 0.002 b |
| Cardiac surgery | 11 (8.1) | 2 (3.3) | 7 (14.0) | 2 (8.3) | 0.106 b |
| History of cardiac surgery | 76 (56.3) | 12 (19.6) | 44 (88.0) | 20 (83.3) | < 0.001 b |
| One surgery | 49 (36.3) | 11 (18.0) | 27 (54.0) | 11 (45.8) | |
| Two surgeries | 15 (11.1) | 1 (1.6) | 10 (20.0) | 4 (16.7) | |
| Three surgeries | 10 (7.4) | 0 (0.0) | 7 (14.0) | 3 (12.5) | |
| Four surgeries | 2 (1.5) | 0 (0.0) | 0 (0.0) | 2 (8.3) | |
| History of electrophysiology intervention | 16 (11.9) | 6 (9.8) | 4 (8.0) | 6 (25.0) | 0.12 b |
| Prosthetic valve | 9 (6.7) | 1 (1.6) | 6 (12.0) | 2 (8.3) | 0.06 b |
| Emergency admission | 10 (7.4) | 0 (0.0) | 3 (6.0) | 7 (29.2) | < 0.001 b |
Patients with complex CHD were younger ( P = 0.006). Diagnosis of the CHD was made during childhood in 65.2% of patients. Moderate and complex CHD were mainly diagnosed during childhood ( P < 0.001).
Complications and co-morbidities
Complication rates for the whole population and according to the complexity of CHD are reported in Table 2 . The complication rate increased with the complexity of the CHD. At least one complication was observed in 88% (95% confidence interval [CI]: 67.6–97.3%) of complex patients versus 49.5% (95% CI: 39.9–59.2%) for other patients ( P = 0.003). When the CHD was diagnosed during childhood, 54.5% of patients (95% CI: 43.6–65.2%) had at least one complication and 86.4% (95% CI: 65.1–97.1%) had complex CHD. No patient had a history of resuscitated sudden death (95% CI: 0.0–2.7%).
