Eur J Pediatr DOI 10.1007/s00431-015-2557-x
REVIEW
From adolescents to adults with congenital heart disease: the role of transition Pamela Moceri 1,2 & Eva Goossens 3 & Sebastien Hascoet 4 & Carine Checler 1,2 & Béatrice Bonello 5 & Emile Ferrari 1 & Philippe Acar 4 & Alain Fraisse 6
Received: 31 July 2014 / Revised: 29 March 2015 / Accepted: 27 April 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Improved surgical care during the last decades, together with advances in medical management, led to a remarkable increase in survival of patients with congenital heart disease (CHD). However, aging of the CHD population brings new challenges, and loss of follow-up of adolescents and adults with CHD is a major concern. It is crucial to optimize the transfer of patients with CHD from paediatric to adult health care services to prevent loss to follow-up. The transition process plays a central role in the future health and follow-up of the patient. The aim of this review is to explain and discuss the clinical impact of the transition process in adolescents with CHD. We will also discuss specific CHD adolescents’ problems. Conclusion: Adolescence is a crucial phase for the formation of the personality. Understanding and acceptance of the responsibility for health at this stage through a transition pro-
cess with a multidisciplinary team will determine the quality of future medical follow-up and probably limit psychosocial issues in their adult life. What is known: • Aging of the congenital heart disease population brings new challenges to the organisation of care. • Loss of follow-up is a major concern for patients with congenital heart disease. What is new: • The quality of a formal transition process during adolescence will determine future outcomes in patients with congenital heart disease.
Keywords Congenital heart disease . Transition to adult care . Transfer of care
Communicated by Jaan Toelen Revisions received: 04 November 2014 / 29 March 2015 * Pamela Moceri [email protected] Eva Goossens [email protected] Sebastien Hascoet [email protected] Carine Checler [email protected] Béatrice Bonello [email protected] Emile Ferrari [email protected] Philippe Acar [email protected]
Alain Fraisse [email protected] 1
Cardiology Department, Pasteur University Hospital, Nice, France
2
Paediatric Cardiology, Hôpitaux Pédiatriques Universitaires de Nice, Nice, France
3
Center for Health Services and Nursing Research, KU Leuven, Leuven, Belgium
4
Paediatric Cardiology, Purpan University Hospital, Toulouse, France
5
Paediatric and Congenital Cardiology, La Timone University Hospital, Marseille, France
6
Paediatric Cardiology, Royal Brompton Hospital, London, UK
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Abbreviations ACHD Adult congenital heart disease CHD Congenital heart disease
Background Recent improvements in surgical, interventional and medical care have completely modified the epidemiologic profile of patients suffering from congenital heart disease (CHD). Improved care led to a remarkable decline in the mortality rates in children with CHD and therefore to a continuously growing number of adults with CHD, in particular those with complex lesions [27, 28]. In fact, approximately 85 to 90 % [30, 52] of babies born with cardiovascular anomalies reach adulthood. With continued improvement in surgical techniques and foetal diagnosis, this is expected to further increase in the next decades. Currently, the adult CHD population exceeds the paediatric CHD population and is estimated to be more than 1–2 million in the USA and 2–3 million in Europe [51]. Aging of the CHD population brings new challenges. In Europe, European Society of Cardiology guidelines for the management of grown-up congenital heart disease [3, 9] have been published in 2003 and updated in 2010. Congenital defects should be considered as Brepaired^ rather than Bcured^. Hence, even after successful surgery, patients will require lifelong cardiac surveillance by specialized adult congenital heart disease (ACHD) practitioners [21, 29]. As health care needs modifications when patients with CHD grow older, a transfer of care from paediatric cardiology to ACHD care is recommended [11]. A recent large cohort study [15] reported that 42 % of ACHD patients experienced significant gaps in cardiology care, usually occurring around the age of 19 years, at the time of transfer. This review article looks at the transition process from paediatric to adult care in CHD and focuses on the importance of transition in CHD adolescents, discussing the various issues of their future adult life with examples, in the light of recent literature.
Methods: database search Screening for relevant literature was performed using PubMed. A combination of Mesh terms and free text terms were used: transition to adult care, paediatric transition to adult care, congenital heart defect and congenital heart disease. A language limit was set, only English publications were selected for this review. The last search update was performed in August 2014.
What is the B transition process^ Transition is more than just crossing the roads; under normal conditions, it will help the adolescent being responsible for his own health and care. The primary objective of a formal transition program is to prepare the patient for the adult health care system, by providing him with support when learning. This includes a good understanding of the heart defect(s), the estimated prognosis, the performed and future interventions and the implications of the CHD on lifestyle choices such as family planning and work. The secondary aim is to prepare the patient for the transfer of care from paediatric to ACHD care, promoting self-care behaviours. Studies in Canada [25, 32], Germany [50], UK [8], and in the USA [53] have demonstrated that 21 to 76 % of adolescents with CHD are either lost to follow-up or experience lapses in care after leaving paediatric cardiology [13]. A recent Canadian survey [4] indicates that less than half of ACHD patients are receiving appropriate follow-up according to guidelines. We can consider that approximately 10 % of patients are lost to follow-up and 60 % experience lapses in care after the age of 18 years. The aim of the transition process is to obtain a high adherence of patients to their health care program after leaving their paediatric care provider. [1, 37]. A specific transition clinic is a possible organisation, with both a member of the paediatric and the adult health team discussing with the patient at the same time. Despite being an ideal situation, this is not the only option. The educational program can be developed during the routine outpatient visits, although it is important to have a transition coordinator, who is often a clinical nurse specialist. A good cohesion between the paediatric and adult cardiologist is of critical importance. According to the scientific statement from the American Heart Association [37], the coordinator can create the link between the paediatric and adult health care providers as well as reassure the patient and his family, as described by Viner R [48]. A nurse-led transition intervention usually results in improvement in knowledge scores for the adolescents [26]. No matter how transition care is provided, the program should include age-appropriate education about medical conditions and help to achieve skills in decision-making, self-care and self-advocacy manners [34, 35, 48]. Even patients who continue to see the same health care providers for CHD care should undergo a process of transition in order to develop the necessary skills to manage their health in an independent way. Data from Reid et al. [32] suggest that ongoing care during adolescence with continued discussion of the importance of education was very important for successful transition.
Timing of transition and transfer to adult care Adolescence has no absolute definition in time, and flexibility is therefore fundamental. Healthy behaviour attitude usually
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develops during late adolescence with, for example, the adoption of good food practices and leisure sports. However, adolescence is also the period in which risky behaviours can be manifested for the first time, such as drug and alcohol use. Thus, appropriate education about heart-healthy behaviours and CHD-specific issues should occur during this crucial period. However, periods of discussions and explanations about health-related issues and the need for heart-healthy behaviour should be introduced early in childhood. This period is part of the educational process of the family and the patient and helps introducing important concepts such as the requirement of lifelong surveillance, the concept of repaired but not cured heart disease and the need of specialized ACHD follow-up. It also helps modifying progressively the profile of the medical discussion during the consultation, from a doctor-parent discussion to doctor-patient assisted by his parents. It will fully involve the young adolescent in his care plan, being considered as the main partner. The timing of transition and transfer is depending on the maturity, the psychosocial status as well as other medical issues [38, 45]. About 20 % of adolescents with CHD suffer from psychiatric disorders, mostly depression and especially in women, thus potentially leading to inappropriate psychosocial adjustment [12]. Ideally, transfer to ACHD care will occur after accomplishment of the transition process and outside phases of psychological disequilibrium or any medical complications [49]. To help establishing the appropriate timing for transition, the use of a specific questionnaire assessing the patient’s skills can be interesting [39]. The Transition Readiness Assessment Questionnaire (TRAQ), a validated 33-item self-administered tool measuring skills needed to progress towards independence and skills needed for a successful transition, is currently considered as the best-validated tool to assess transition readiness [54]. Both families and patients should be informed on the aim, principles and planned timing of the transition process. Because paediatric and adult care substantially differ from each other, there should not be any transfer without transition in order to avoid confusion and inappropriate follow-up. Fig. 1 Timeline for transition. Pretransition occurs in early childhood, whereas transition usually begins around the age of 12 years, and patients are transferred to adult care at the end of the transition process. This process should be flexible, tailored to each patient
Duration of the transition period should be tailored to patients and flexible; for example, it can be longer for a young person with learning disabilities and/or very complex congenital heart disease. Subsequent transfer to the adult care can occur at an approximate age of 16–18 years (Fig. 1). Transfer at a higher age is associated with better transition outcomes [32], but based on national regulations, it is sometimes difficult to follow CHD patients >18 years in paediatric facilities. For example in France, follow-up in paediatric centres is possible until 18 years old; however, there is no strict limit of age. Each paediatric cardiac unit should establish a coordinated process to link with a specialist centre for ACHD. Before transfer, the adolescent should demonstrate the ability to feel responsible and manage his health independently of his family, as the adult health care system usually requires a higher level of personal responsibility and autonomy compared to the paediatric system. For the success of the transition process, both adult and paediatric health care providers should identify and communicate on how their management of CHD will differ, in order to prepare the patient for his transfer.
Family and the transition process From initial diagnosis, parents are completely involved in the health care of their child. The transition process will help parents reduce their anxiety, prepare and adjust to this shift in responsibility. Many children reach adolescence with little understanding of their condition, because of the lack of direct discussion and probably some degree of parental overprotection [42, 44]. Parents often find the transition difficult, and they may need support to allow the adolescent to become independent. Many studies suggest that parents of children with CHD are more likely to experience elevated symptoms of depression, anxiety, stress and anger [2, 22], and in consequence, they are more likely to divorce in comparison with parents of children suffering from other conditions [17]. We definitely need to involve the family in the transition process.
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Beyond being beneficial for the family to understand the disease, it is now proved that parents’ acceptance of the disease and transition has a positive effect on adolescent psychological well-being [7].
Table 1
Educational topics
Haemodynamic considerations
Therapeutic management
Need for a multidisciplinary team involved in transition In a recent study, assessing the level of disease-specific knowledge in a nationwide cohort of CHD patients from 10 to 30 years, it appears that patients are particularly keen to discuss psychosocial topics but the doctors did not meet these information preferences [23]. Previous studies also underlined doctor-patient communication problems, revealing an obvious need for multidisciplinary education programmes, including clear information on psychosocial topics such as leisure sports, family planning and work/career. Given the high rate of psychosocial disorders in adolescents with CHD [12], specific psychosocial care during this phase of fragility is important. The positive impact of a consultation with a special nurse has recently been demonstrated, resulting in a significant improvement in cardiac knowledge scores and self-management skills of CHD adolescents involved [26]. The use of internet applications for education and transition planning needs to be explored, internet being one of the most important sources of information in as many as 37.5 % of CHD young patients [23].
Problems addressed during the transition process in CHD patients A specialized educational curriculum is highly important and should be standardized as part of an organized transition process. Topics that should be discussed are listed in Table 1. This list of topics should help cover all future health issues that patients will encounter. This process can be accomplished by a variety of modalities, such as individual or group education sessions, books, dedicated websites, but always tailored to individual patients’ needs. The educational practitioner can either be a specialist nurse, a paediatric cardiologist or an ACHD care provider. The patient should be prepared for self-care and deeply involved in the process from early adolescence, to allow the development of the necessary self-care skills [36]. Youngsters should become increasingly involved in direct discussions about the diagnosis, medications, prognosis and lifestyle choices like specific recommendations for physical exercise with or without restrictions. Meeting alone with the adolescent is a simple way to involve directly the patient. A recent study reported that only 50 % of the studied ACHD cohort reported regular exercise and 30 % of patients were obese [5]. Exercise
Risk of endocarditis Arrhythmias
Contraception and pregnancy planning
Lifestyle/psychosocial issues
Haemodynamic future issues Symptoms and how to respond Investigations in follow-up (MRI, …) Management options Medications Side effects and interactions of drugs Sign, symptoms, risks and prevention Risks, signs and symptoms Diagnostic tools, further investigations Management options (ablation) Contraceptive options and risks Risk of pregnancy (mother and foetus) Pregnancy plan Risk of recurrence of the CHD Peer interactions Employment, life and health insurance Education/learning disabilities Anxiety and depression Healthy diet, physical activity Use of tobacco, alcohol and drugs Self-care Family issues
may improve functional capacity and quality of life in CHD young patients, including peak oxygen consumption and chronotropic incompetence [41] that have been associated to survival in a large cohort of adult CHD patients [10]. Despite these benefits, exercise is rarely discussed in routine clinic visit. Discussions are generally more focused on activity limitations instead of promotion of exercise [43]. Efforts should be made to encourage exercise and a healthier lifestyle. On the basis of published studies, including more than 4000 adult CHD patients with cardiopulmonary exercise test results, age-, gender- and CHD-specific Bnormal^ data on peak oxygen consumption levels are available [19]. These data can help the physician to advise adolescents on activities of daily life, sports and choice of profession based on their exercise test results. Education during the transition process should not only focus on the CHD but also discuss cardiovascular risk factors and promote heart-healthy behaviours. Risky sexual behaviours, birth control and pregnancy issues should also be discussed as promptly as possible. Data from the USA showed that about 70 % of adolescents sexually active were involved in one or more types of sexually risky behaviour [33]; therefore, it is extremely important to discuss sexual health with adolescents, particularly with women. Family planning and risk of recurrence of the CHD [31] should be detailed. The recurrence risk of the CHD is higher when the mother is affected (3–5 %) [6], but the estimation of this risk should be individualized to the type of defect and family history. Table 2 describes the problem of contraception and pregnancy in women with CHD. A recent German study concludes that the current counselling practice for family planning is
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Family planning in CHD women Current problems
Special care
Sexual activity [24–33]
- 28 %: inadequate birth control method - 20 %: contraindicated method - 43 % of women without any counselling on contraception - Comparable to mild to moderate physical activity - Symptoms ↗ with NYHA class
Pregnancy
- 72 % of sexually active CHD adolescents engaged in one or more types of risky sexual behaviour - 48 % of women not informed about pregnancy-related risks
-Adequate education for all patients - Barrier methods: annual failure rate up to t10 % - Oral progesterone contraception is useful With caution in patients with pulmonary hypertension, obstructive left heart disease, cyanotic CHD, uncontrolled arrhythmias and anomalous coronary artery passing between the pulmonary artery and aorta Education regarding risky sexual behaviours
Contraception [47]
inadequate [18]. In an adult cohort of CHD women, on a scale from 1 (very poor) to 10 (very well), the median rate for the level of information regarding sexuality was only 3.5 and 5 regarding the use of contraceptives as well as the reported level of knowledge regarding pregnancy and related risks. An unplanned pregnancy in an ACHD adolescent can have devastating effects on both the mother and the foetus. For example, in women with a Fontan circulation, intrauterine growth restriction and preterm delivery are common and may affect the baby. Regarding the maternal risks in Fontanpregnant women, the thromboembolic risk is particularly high, arrhythmias are more frequent, ventricular function can be impaired in relation with the volume overload and heart failure can occur. In sum, pregnancy in CHD women needs to be prepared, and this notion shall be introduced during adolescence. Recommendations about the risk of endocarditis should be clearly given, including general prevention measures (good oral hygiene and regular dental review). Antibiotic prophylaxis of endocarditis (amoxicillin or clindamycin) is recommended for dental procedures requiring manipulation of the gingival or periapical region of the teeth or/and perforation of the oral mucosa, only in higher risk patients: CHD patients with history of valve replacement; previous infective endocarditis; cyanotic CHD unrepaired or with residual defect, palliative shunt or conduit; in case of prosthetic material up to 6 months after the procedure; in case of persisting residual defect at the site of implantation of prosthetic material or device [16]. This transition process should be developmentally appropriate and flexible, especially in patients with cognitive disorders. Therapeutic education and understanding of the congenital heart condition is an ongoing process that should begin early in adolescence. It has recently been described [46] that the level of knowledge about the individual congenital heart defect in adolescents who were recently transferred to adult care is poor: Less than 50 % were able to describe their heart disease. Patient education is of critical importance, and efforts should be made during childhood and adolescence to improve knowledge of their condition. Patients should be taught to
- Adequate education/information
recognize important warning signs and symptoms specific to their congenital heart disease such as chest pain, syncope or breathlessness. The role of nurse specialist or advanced practice nurse is fundamental, especially in the setting of education and care coordination between adult and paediatric health care providers. The transition process is also the perfect time for a complete cardiac evaluation: before transfer to adult care. Any ongoing medical issue should be addressed. A transfer-ofcare letter (written care plan) should be provided at the end of the transition process, addressed to both patient and ACHD care providers [37], to avoid any loss of information. It will punctuate the end of the paediatric care. Once the transfer is realized, having a fresh look on the congenital patient is an opportunity. However, Brevolutions^ in the medical management after the first contact with ACHD care provider could be a mistake possibly leading to follow-up rupture. A trust relationship between the patient, his family and the adult care provider should be established. Ideally, feedback on the impact of the transition process should be given to the paediatric care specialist, in order to improve our transition programs.
Specific CHD transition issues In comparison with other chronic paediatric conditions, some aspects are specific to CHD and might impact the transition process. Gaps in care are probably more frequent in the field of congenital heart disease compared to chronic conditions such as cystic fibrosis or diabetes. Lost to follow-up in diabetes lead to increased risk of acute glycaemia complications responsible for long-term damage to end organs. However, these episodes are usually responsible for symptoms that can lead the young adults to their physician, while young patients with stable CHD are usually not experiencing any symptoms, leading them to feel free from complications and not requiring any medical follow-up.
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Patients with stable CHD may not require long-term medications; thus, they are less Bexposed^ to the health system. CHD young adult patients may not benefit from the pharmacist assistance to help them in the health care pathway and deliver therapeutic education. This might partly explain the high percentage of loss of follow-up in CHD. Therapeutic education in CHD patients is centred on the evolution of residual defects and surgical Bscars^. The major problem in CHD transition discussion regarding the haemodynamic point of view is that it needs to be individualized to the patient’s CHD, previous history of surgery and complications. Even if group discussions are interesting, especially within a group sharing the same CHD, the discussion should be tailored to each patient. However, the main goals of transition are the same in all paediatric conditions, and a major common objective shared by the different specialties is the need to assess the outcomes of transition in terms of morbidity.
Impact of a structured transition programme Generally, young adult patients reporting worsening (or lack of improvement) of their health status (compared to 6 years before) were less likely involved in discussions about the transition process and how their health care needs may change over time at the time of adolescence [40]. A structured education program (multidisciplinary planned intervention that provides support during the transition period) has been associated with a higher level of knowledge [14]. Patients achieving a successful transition are less likely to present care planning or insurability problems [40]. However, none of these studies about the impact of transition programs demonstrate a clear relationship between the educational program and medical outcomes. This is not really surprising, given that adolescents who were not involved in a transition process are more likely to become lost to specialized follow-up. Lapse of care in a cohort of adult CHD patients was associated with a 3.1-fold increase in urgent cardiac intervention [53]. In the absence of structured programs to guide this transition, there is often delayed or inappropriate care, improper timing of the transfer of care and emotional stress on the patients and their families [32], who are already frequently prone to anxiety [20]. Although successful transfer to adult care has not been clearly related to gender, educational level or diagnosis, it was associated with history of multiple paediatric cardiac surgeries and education about future follow-up in an ACHD clinic [40].
crucial phase for the formation of the personality, understanding and acceptance of the responsibility for health at this stage will determine the quality of future follow-up. Despite awareness of the medical community through a growing number of studies, patients lost to follow-up are still a crucial problem; therefore, the first of many challenges in ACHD patients is to optimize the transition from paediatric to adult cardiology to prevent lost to follow-up. Efforts should be made to develop specific transition programs in CHD adolescents with multidisciplinary teams and to develop research on large prospective cohorts of ACHD patients, in order to assess the impact of these educational programs on morbidity and quality of life.
Conflict of interest None Author’s contributions Pamela Moceri takes primary responsibility for communication with the journal. She has participated in the design of the work, literature search and analysis for the work. She has written the draft and has participated to the revisions. She approved the final version of the draft and agrees to be accountable for all aspects of this work. Eva Goossens has participated in the analysis of the articles, in writing the manuscript and revising it for its intellectual content. She has approved the final version of the work and agrees to be accountable for all the aspects of this work. Sebastien Hascoet, Carine Checler and Béatrice Bonello: They have participated to the literature analysis and data search. They have participated to important revisions of the work (for intellectual content). They approved the final version of the draft and agree to be accountable for this work. Emile Ferrari and Philippe Acar: They have participated to the concept of the study, have revised the draft critically for important intellectual content, have approved the final manuscript and agree to be accountable for all aspects of the work. Alain Fraisse: He has first imagined the work and has participated to the concept of the study. He has participated in writing the draft, interpreting data from the literature, revising our manuscript for important intellectual content. He has approved the final version of the work and agrees to be accountable for all the aspects of this work.
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Sawicki GS, Lukens-Bull K, Yin X et al (2011) Measuring the transition readiness of youth with special healthcare needs: validation of the TRAQ—Transition Readiness Assessment Questionnaire. J Pediatr Psychol 36:160–171. doi:10.1093/jpepsy/ jsp128 Sawicki GS, Whitworth R, Gunn L, Butterfield R, Lukens-Bull K, Wood D (2011) Receipt of health care transition counseling in the national survey of adult transition and health. Pediatrics 128:e521– e529. doi:10.1542/peds.2010-3017 Singh TP, Curran TJ, Rhodes J (2007) Cardiac rehabilitation improves heart rate recovery following peak exercise in children with repaired con- genital heart disease. Pediatr Cardiol 28:276–279. doi:10.1007/s00246-006-0114-0 Sparacino PS, Tong EM, Messias DK, Foote D, Chesla CA, Gilliss CL (1997) The dilemmas of parents of adolescents and young adults with congenital heart disease. Heart Lung 26:187–195 Swan L, Hillis WS (2000) Exercise prescription in adults with congenital heart disease: a long way to go. Heart 83:685–687. doi:10. 1136/heart.83.6.685 Tong EM, Kools S (2004) Health care transitions for adolescents with congenital heart disease: patient and family perspectives. Nurs Clin N Am 39(4):727–740. doi:10.1016/j.cnur.2004.07.011 Van Deyk K, Moons P, Gewillig M, Budts W (2004) Educational and behavioral issues in transitioning from pediatric cardiology to adult- centered health care. Nurs Clin N Am 39:755–768. doi:10. 1016/j.cnur.2004.07.010 Van Deyk K, Pelgrims E, Troost E et al (2010) Adolescents’ understanding of their congenital heart disease on transfer to adultfocused care. Am J Cardiol 106:1803–1807. doi:10.1016/j. amjcard.2010.08.020
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Vigl M, Kaemmerer M, Seifert-Klauss V et al (2010) Contraception in women with congenital heart disease. Am J Cardiol 106:1317– 1321. doi:10.1016/j.amjcard.2010.06.060 Viner R (2001) Barriers and good practice in transition from paediatric to adult care. J R Soc Med 94(Suppl 40):2–4 Viner R (2003) Bridging the gaps: transition for young people with cancer. Eur J Cancer 39:2684–2687. doi:10.1016/j.ejca.2003.08. 004 Wacker A, Kaemmerer H, Hollweck R et al (2005) Outcome of operated and unoperated adults with congenital cardiac disease lost to follow-up for more than five years. Am J Cardiol 95:776–779. doi:10.1016/j.amjcard.2004.11.036 Warnes CA, Liberthson R, Danielson GK et al (2001) Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 37:1170–1175. doi:10.1016/S0735-1097(01)01272-4 Warnes CA, Williams RG, Bashore TM et al (2008) ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). J Am Coll Cardiol 52:e143–e263. doi:10.1016/j.jacc.2008.10.001 Yeung E, Kay J, Roosevelt GE, Brandon M, Yetman AT (2008) Lapse of care as a predictor for morbidity in adults with congenital heart disease. Int J Cardiol 125:62–65. doi:10.1016/j.ijcard.2007. 02.023 Zhang LF, Ho JSW, Kennedy SE (2014) A systematic review of the psychometric properties of transition readiness assessment tools in adolescents with chronic disease. BMC Pediatr 14:4. doi:10.1186/ 1471-2431-14-4
REVIEW
From adolescents to adults with congenital heart disease: the role of transition Pamela Moceri 1,2 & Eva Goossens 3 & Sebastien Hascoet 4 & Carine Checler 1,2 & Béatrice Bonello 5 & Emile Ferrari 1 & Philippe Acar 4 & Alain Fraisse 6
Received: 31 July 2014 / Revised: 29 March 2015 / Accepted: 27 April 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Improved surgical care during the last decades, together with advances in medical management, led to a remarkable increase in survival of patients with congenital heart disease (CHD). However, aging of the CHD population brings new challenges, and loss of follow-up of adolescents and adults with CHD is a major concern. It is crucial to optimize the transfer of patients with CHD from paediatric to adult health care services to prevent loss to follow-up. The transition process plays a central role in the future health and follow-up of the patient. The aim of this review is to explain and discuss the clinical impact of the transition process in adolescents with CHD. We will also discuss specific CHD adolescents’ problems. Conclusion: Adolescence is a crucial phase for the formation of the personality. Understanding and acceptance of the responsibility for health at this stage through a transition pro-
cess with a multidisciplinary team will determine the quality of future medical follow-up and probably limit psychosocial issues in their adult life. What is known: • Aging of the congenital heart disease population brings new challenges to the organisation of care. • Loss of follow-up is a major concern for patients with congenital heart disease. What is new: • The quality of a formal transition process during adolescence will determine future outcomes in patients with congenital heart disease.
Keywords Congenital heart disease . Transition to adult care . Transfer of care
Communicated by Jaan Toelen Revisions received: 04 November 2014 / 29 March 2015 * Pamela Moceri [email protected] Eva Goossens [email protected] Sebastien Hascoet [email protected] Carine Checler [email protected] Béatrice Bonello [email protected] Emile Ferrari [email protected] Philippe Acar [email protected]
Alain Fraisse [email protected] 1
Cardiology Department, Pasteur University Hospital, Nice, France
2
Paediatric Cardiology, Hôpitaux Pédiatriques Universitaires de Nice, Nice, France
3
Center for Health Services and Nursing Research, KU Leuven, Leuven, Belgium
4
Paediatric Cardiology, Purpan University Hospital, Toulouse, France
5
Paediatric and Congenital Cardiology, La Timone University Hospital, Marseille, France
6
Paediatric Cardiology, Royal Brompton Hospital, London, UK
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Abbreviations ACHD Adult congenital heart disease CHD Congenital heart disease
Background Recent improvements in surgical, interventional and medical care have completely modified the epidemiologic profile of patients suffering from congenital heart disease (CHD). Improved care led to a remarkable decline in the mortality rates in children with CHD and therefore to a continuously growing number of adults with CHD, in particular those with complex lesions [27, 28]. In fact, approximately 85 to 90 % [30, 52] of babies born with cardiovascular anomalies reach adulthood. With continued improvement in surgical techniques and foetal diagnosis, this is expected to further increase in the next decades. Currently, the adult CHD population exceeds the paediatric CHD population and is estimated to be more than 1–2 million in the USA and 2–3 million in Europe [51]. Aging of the CHD population brings new challenges. In Europe, European Society of Cardiology guidelines for the management of grown-up congenital heart disease [3, 9] have been published in 2003 and updated in 2010. Congenital defects should be considered as Brepaired^ rather than Bcured^. Hence, even after successful surgery, patients will require lifelong cardiac surveillance by specialized adult congenital heart disease (ACHD) practitioners [21, 29]. As health care needs modifications when patients with CHD grow older, a transfer of care from paediatric cardiology to ACHD care is recommended [11]. A recent large cohort study [15] reported that 42 % of ACHD patients experienced significant gaps in cardiology care, usually occurring around the age of 19 years, at the time of transfer. This review article looks at the transition process from paediatric to adult care in CHD and focuses on the importance of transition in CHD adolescents, discussing the various issues of their future adult life with examples, in the light of recent literature.
Methods: database search Screening for relevant literature was performed using PubMed. A combination of Mesh terms and free text terms were used: transition to adult care, paediatric transition to adult care, congenital heart defect and congenital heart disease. A language limit was set, only English publications were selected for this review. The last search update was performed in August 2014.
What is the B transition process^ Transition is more than just crossing the roads; under normal conditions, it will help the adolescent being responsible for his own health and care. The primary objective of a formal transition program is to prepare the patient for the adult health care system, by providing him with support when learning. This includes a good understanding of the heart defect(s), the estimated prognosis, the performed and future interventions and the implications of the CHD on lifestyle choices such as family planning and work. The secondary aim is to prepare the patient for the transfer of care from paediatric to ACHD care, promoting self-care behaviours. Studies in Canada [25, 32], Germany [50], UK [8], and in the USA [53] have demonstrated that 21 to 76 % of adolescents with CHD are either lost to follow-up or experience lapses in care after leaving paediatric cardiology [13]. A recent Canadian survey [4] indicates that less than half of ACHD patients are receiving appropriate follow-up according to guidelines. We can consider that approximately 10 % of patients are lost to follow-up and 60 % experience lapses in care after the age of 18 years. The aim of the transition process is to obtain a high adherence of patients to their health care program after leaving their paediatric care provider. [1, 37]. A specific transition clinic is a possible organisation, with both a member of the paediatric and the adult health team discussing with the patient at the same time. Despite being an ideal situation, this is not the only option. The educational program can be developed during the routine outpatient visits, although it is important to have a transition coordinator, who is often a clinical nurse specialist. A good cohesion between the paediatric and adult cardiologist is of critical importance. According to the scientific statement from the American Heart Association [37], the coordinator can create the link between the paediatric and adult health care providers as well as reassure the patient and his family, as described by Viner R [48]. A nurse-led transition intervention usually results in improvement in knowledge scores for the adolescents [26]. No matter how transition care is provided, the program should include age-appropriate education about medical conditions and help to achieve skills in decision-making, self-care and self-advocacy manners [34, 35, 48]. Even patients who continue to see the same health care providers for CHD care should undergo a process of transition in order to develop the necessary skills to manage their health in an independent way. Data from Reid et al. [32] suggest that ongoing care during adolescence with continued discussion of the importance of education was very important for successful transition.
Timing of transition and transfer to adult care Adolescence has no absolute definition in time, and flexibility is therefore fundamental. Healthy behaviour attitude usually
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develops during late adolescence with, for example, the adoption of good food practices and leisure sports. However, adolescence is also the period in which risky behaviours can be manifested for the first time, such as drug and alcohol use. Thus, appropriate education about heart-healthy behaviours and CHD-specific issues should occur during this crucial period. However, periods of discussions and explanations about health-related issues and the need for heart-healthy behaviour should be introduced early in childhood. This period is part of the educational process of the family and the patient and helps introducing important concepts such as the requirement of lifelong surveillance, the concept of repaired but not cured heart disease and the need of specialized ACHD follow-up. It also helps modifying progressively the profile of the medical discussion during the consultation, from a doctor-parent discussion to doctor-patient assisted by his parents. It will fully involve the young adolescent in his care plan, being considered as the main partner. The timing of transition and transfer is depending on the maturity, the psychosocial status as well as other medical issues [38, 45]. About 20 % of adolescents with CHD suffer from psychiatric disorders, mostly depression and especially in women, thus potentially leading to inappropriate psychosocial adjustment [12]. Ideally, transfer to ACHD care will occur after accomplishment of the transition process and outside phases of psychological disequilibrium or any medical complications [49]. To help establishing the appropriate timing for transition, the use of a specific questionnaire assessing the patient’s skills can be interesting [39]. The Transition Readiness Assessment Questionnaire (TRAQ), a validated 33-item self-administered tool measuring skills needed to progress towards independence and skills needed for a successful transition, is currently considered as the best-validated tool to assess transition readiness [54]. Both families and patients should be informed on the aim, principles and planned timing of the transition process. Because paediatric and adult care substantially differ from each other, there should not be any transfer without transition in order to avoid confusion and inappropriate follow-up. Fig. 1 Timeline for transition. Pretransition occurs in early childhood, whereas transition usually begins around the age of 12 years, and patients are transferred to adult care at the end of the transition process. This process should be flexible, tailored to each patient
Duration of the transition period should be tailored to patients and flexible; for example, it can be longer for a young person with learning disabilities and/or very complex congenital heart disease. Subsequent transfer to the adult care can occur at an approximate age of 16–18 years (Fig. 1). Transfer at a higher age is associated with better transition outcomes [32], but based on national regulations, it is sometimes difficult to follow CHD patients >18 years in paediatric facilities. For example in France, follow-up in paediatric centres is possible until 18 years old; however, there is no strict limit of age. Each paediatric cardiac unit should establish a coordinated process to link with a specialist centre for ACHD. Before transfer, the adolescent should demonstrate the ability to feel responsible and manage his health independently of his family, as the adult health care system usually requires a higher level of personal responsibility and autonomy compared to the paediatric system. For the success of the transition process, both adult and paediatric health care providers should identify and communicate on how their management of CHD will differ, in order to prepare the patient for his transfer.
Family and the transition process From initial diagnosis, parents are completely involved in the health care of their child. The transition process will help parents reduce their anxiety, prepare and adjust to this shift in responsibility. Many children reach adolescence with little understanding of their condition, because of the lack of direct discussion and probably some degree of parental overprotection [42, 44]. Parents often find the transition difficult, and they may need support to allow the adolescent to become independent. Many studies suggest that parents of children with CHD are more likely to experience elevated symptoms of depression, anxiety, stress and anger [2, 22], and in consequence, they are more likely to divorce in comparison with parents of children suffering from other conditions [17]. We definitely need to involve the family in the transition process.
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Beyond being beneficial for the family to understand the disease, it is now proved that parents’ acceptance of the disease and transition has a positive effect on adolescent psychological well-being [7].
Table 1
Educational topics
Haemodynamic considerations
Therapeutic management
Need for a multidisciplinary team involved in transition In a recent study, assessing the level of disease-specific knowledge in a nationwide cohort of CHD patients from 10 to 30 years, it appears that patients are particularly keen to discuss psychosocial topics but the doctors did not meet these information preferences [23]. Previous studies also underlined doctor-patient communication problems, revealing an obvious need for multidisciplinary education programmes, including clear information on psychosocial topics such as leisure sports, family planning and work/career. Given the high rate of psychosocial disorders in adolescents with CHD [12], specific psychosocial care during this phase of fragility is important. The positive impact of a consultation with a special nurse has recently been demonstrated, resulting in a significant improvement in cardiac knowledge scores and self-management skills of CHD adolescents involved [26]. The use of internet applications for education and transition planning needs to be explored, internet being one of the most important sources of information in as many as 37.5 % of CHD young patients [23].
Problems addressed during the transition process in CHD patients A specialized educational curriculum is highly important and should be standardized as part of an organized transition process. Topics that should be discussed are listed in Table 1. This list of topics should help cover all future health issues that patients will encounter. This process can be accomplished by a variety of modalities, such as individual or group education sessions, books, dedicated websites, but always tailored to individual patients’ needs. The educational practitioner can either be a specialist nurse, a paediatric cardiologist or an ACHD care provider. The patient should be prepared for self-care and deeply involved in the process from early adolescence, to allow the development of the necessary self-care skills [36]. Youngsters should become increasingly involved in direct discussions about the diagnosis, medications, prognosis and lifestyle choices like specific recommendations for physical exercise with or without restrictions. Meeting alone with the adolescent is a simple way to involve directly the patient. A recent study reported that only 50 % of the studied ACHD cohort reported regular exercise and 30 % of patients were obese [5]. Exercise
Risk of endocarditis Arrhythmias
Contraception and pregnancy planning
Lifestyle/psychosocial issues
Haemodynamic future issues Symptoms and how to respond Investigations in follow-up (MRI, …) Management options Medications Side effects and interactions of drugs Sign, symptoms, risks and prevention Risks, signs and symptoms Diagnostic tools, further investigations Management options (ablation) Contraceptive options and risks Risk of pregnancy (mother and foetus) Pregnancy plan Risk of recurrence of the CHD Peer interactions Employment, life and health insurance Education/learning disabilities Anxiety and depression Healthy diet, physical activity Use of tobacco, alcohol and drugs Self-care Family issues
may improve functional capacity and quality of life in CHD young patients, including peak oxygen consumption and chronotropic incompetence [41] that have been associated to survival in a large cohort of adult CHD patients [10]. Despite these benefits, exercise is rarely discussed in routine clinic visit. Discussions are generally more focused on activity limitations instead of promotion of exercise [43]. Efforts should be made to encourage exercise and a healthier lifestyle. On the basis of published studies, including more than 4000 adult CHD patients with cardiopulmonary exercise test results, age-, gender- and CHD-specific Bnormal^ data on peak oxygen consumption levels are available [19]. These data can help the physician to advise adolescents on activities of daily life, sports and choice of profession based on their exercise test results. Education during the transition process should not only focus on the CHD but also discuss cardiovascular risk factors and promote heart-healthy behaviours. Risky sexual behaviours, birth control and pregnancy issues should also be discussed as promptly as possible. Data from the USA showed that about 70 % of adolescents sexually active were involved in one or more types of sexually risky behaviour [33]; therefore, it is extremely important to discuss sexual health with adolescents, particularly with women. Family planning and risk of recurrence of the CHD [31] should be detailed. The recurrence risk of the CHD is higher when the mother is affected (3–5 %) [6], but the estimation of this risk should be individualized to the type of defect and family history. Table 2 describes the problem of contraception and pregnancy in women with CHD. A recent German study concludes that the current counselling practice for family planning is
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Family planning in CHD women Current problems
Special care
Sexual activity [24–33]
- 28 %: inadequate birth control method - 20 %: contraindicated method - 43 % of women without any counselling on contraception - Comparable to mild to moderate physical activity - Symptoms ↗ with NYHA class
Pregnancy
- 72 % of sexually active CHD adolescents engaged in one or more types of risky sexual behaviour - 48 % of women not informed about pregnancy-related risks
-Adequate education for all patients - Barrier methods: annual failure rate up to t10 % - Oral progesterone contraception is useful With caution in patients with pulmonary hypertension, obstructive left heart disease, cyanotic CHD, uncontrolled arrhythmias and anomalous coronary artery passing between the pulmonary artery and aorta Education regarding risky sexual behaviours
Contraception [47]
inadequate [18]. In an adult cohort of CHD women, on a scale from 1 (very poor) to 10 (very well), the median rate for the level of information regarding sexuality was only 3.5 and 5 regarding the use of contraceptives as well as the reported level of knowledge regarding pregnancy and related risks. An unplanned pregnancy in an ACHD adolescent can have devastating effects on both the mother and the foetus. For example, in women with a Fontan circulation, intrauterine growth restriction and preterm delivery are common and may affect the baby. Regarding the maternal risks in Fontanpregnant women, the thromboembolic risk is particularly high, arrhythmias are more frequent, ventricular function can be impaired in relation with the volume overload and heart failure can occur. In sum, pregnancy in CHD women needs to be prepared, and this notion shall be introduced during adolescence. Recommendations about the risk of endocarditis should be clearly given, including general prevention measures (good oral hygiene and regular dental review). Antibiotic prophylaxis of endocarditis (amoxicillin or clindamycin) is recommended for dental procedures requiring manipulation of the gingival or periapical region of the teeth or/and perforation of the oral mucosa, only in higher risk patients: CHD patients with history of valve replacement; previous infective endocarditis; cyanotic CHD unrepaired or with residual defect, palliative shunt or conduit; in case of prosthetic material up to 6 months after the procedure; in case of persisting residual defect at the site of implantation of prosthetic material or device [16]. This transition process should be developmentally appropriate and flexible, especially in patients with cognitive disorders. Therapeutic education and understanding of the congenital heart condition is an ongoing process that should begin early in adolescence. It has recently been described [46] that the level of knowledge about the individual congenital heart defect in adolescents who were recently transferred to adult care is poor: Less than 50 % were able to describe their heart disease. Patient education is of critical importance, and efforts should be made during childhood and adolescence to improve knowledge of their condition. Patients should be taught to
- Adequate education/information
recognize important warning signs and symptoms specific to their congenital heart disease such as chest pain, syncope or breathlessness. The role of nurse specialist or advanced practice nurse is fundamental, especially in the setting of education and care coordination between adult and paediatric health care providers. The transition process is also the perfect time for a complete cardiac evaluation: before transfer to adult care. Any ongoing medical issue should be addressed. A transfer-ofcare letter (written care plan) should be provided at the end of the transition process, addressed to both patient and ACHD care providers [37], to avoid any loss of information. It will punctuate the end of the paediatric care. Once the transfer is realized, having a fresh look on the congenital patient is an opportunity. However, Brevolutions^ in the medical management after the first contact with ACHD care provider could be a mistake possibly leading to follow-up rupture. A trust relationship between the patient, his family and the adult care provider should be established. Ideally, feedback on the impact of the transition process should be given to the paediatric care specialist, in order to improve our transition programs.
Specific CHD transition issues In comparison with other chronic paediatric conditions, some aspects are specific to CHD and might impact the transition process. Gaps in care are probably more frequent in the field of congenital heart disease compared to chronic conditions such as cystic fibrosis or diabetes. Lost to follow-up in diabetes lead to increased risk of acute glycaemia complications responsible for long-term damage to end organs. However, these episodes are usually responsible for symptoms that can lead the young adults to their physician, while young patients with stable CHD are usually not experiencing any symptoms, leading them to feel free from complications and not requiring any medical follow-up.
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Patients with stable CHD may not require long-term medications; thus, they are less Bexposed^ to the health system. CHD young adult patients may not benefit from the pharmacist assistance to help them in the health care pathway and deliver therapeutic education. This might partly explain the high percentage of loss of follow-up in CHD. Therapeutic education in CHD patients is centred on the evolution of residual defects and surgical Bscars^. The major problem in CHD transition discussion regarding the haemodynamic point of view is that it needs to be individualized to the patient’s CHD, previous history of surgery and complications. Even if group discussions are interesting, especially within a group sharing the same CHD, the discussion should be tailored to each patient. However, the main goals of transition are the same in all paediatric conditions, and a major common objective shared by the different specialties is the need to assess the outcomes of transition in terms of morbidity.
Impact of a structured transition programme Generally, young adult patients reporting worsening (or lack of improvement) of their health status (compared to 6 years before) were less likely involved in discussions about the transition process and how their health care needs may change over time at the time of adolescence [40]. A structured education program (multidisciplinary planned intervention that provides support during the transition period) has been associated with a higher level of knowledge [14]. Patients achieving a successful transition are less likely to present care planning or insurability problems [40]. However, none of these studies about the impact of transition programs demonstrate a clear relationship between the educational program and medical outcomes. This is not really surprising, given that adolescents who were not involved in a transition process are more likely to become lost to specialized follow-up. Lapse of care in a cohort of adult CHD patients was associated with a 3.1-fold increase in urgent cardiac intervention [53]. In the absence of structured programs to guide this transition, there is often delayed or inappropriate care, improper timing of the transfer of care and emotional stress on the patients and their families [32], who are already frequently prone to anxiety [20]. Although successful transfer to adult care has not been clearly related to gender, educational level or diagnosis, it was associated with history of multiple paediatric cardiac surgeries and education about future follow-up in an ACHD clinic [40].
crucial phase for the formation of the personality, understanding and acceptance of the responsibility for health at this stage will determine the quality of future follow-up. Despite awareness of the medical community through a growing number of studies, patients lost to follow-up are still a crucial problem; therefore, the first of many challenges in ACHD patients is to optimize the transition from paediatric to adult cardiology to prevent lost to follow-up. Efforts should be made to develop specific transition programs in CHD adolescents with multidisciplinary teams and to develop research on large prospective cohorts of ACHD patients, in order to assess the impact of these educational programs on morbidity and quality of life.
Conflict of interest None Author’s contributions Pamela Moceri takes primary responsibility for communication with the journal. She has participated in the design of the work, literature search and analysis for the work. She has written the draft and has participated to the revisions. She approved the final version of the draft and agrees to be accountable for all aspects of this work. Eva Goossens has participated in the analysis of the articles, in writing the manuscript and revising it for its intellectual content. She has approved the final version of the work and agrees to be accountable for all the aspects of this work. Sebastien Hascoet, Carine Checler and Béatrice Bonello: They have participated to the literature analysis and data search. They have participated to important revisions of the work (for intellectual content). They approved the final version of the draft and agree to be accountable for this work. Emile Ferrari and Philippe Acar: They have participated to the concept of the study, have revised the draft critically for important intellectual content, have approved the final manuscript and agree to be accountable for all aspects of the work. Alain Fraisse: He has first imagined the work and has participated to the concept of the study. He has participated in writing the draft, interpreting data from the literature, revising our manuscript for important intellectual content. He has approved the final version of the work and agrees to be accountable for all the aspects of this work.
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