Introduction
Children born with complex childhood illnesses like CHD are now surviving into adulthood. In fact, the number of adults born with CHD is rising exponentially in the United States, with an estimate that there are now more adults living with CHD than children with CHD. Reference Gilboa, Salemi, Nembhard, Fixler and Correa1–Reference Marelli, Mackie, Ionescu-Ittu, Rahme and Pilote3,Reference Lee, Bailey, Cullen-Dean, Aiello, Morin and Oechslin4,Reference Cotts5 A retrospective review of death certificates found that mortality has been declining from CHD, with a decrease of 24% between 1999 and 2006.6The expectation is for these patients to successfully enter adulthood and lead normal and productive lives. However, these patients need to transition their care from paediatric to adult-centred care in a timely fashion for optimal long-term outcomes. Often in the absence of established transition of care programmes, there is delayed, inappropriate, or incomplete transition of care. This often leads to fragmented care as well as emotional and financial stress on patients, their families, and the healthcare system. Lapses in care due to lack of successful transition of care from paediatric to adult care put young adults with CHD at risk for increased morbidity and premature death. Reference Marelli, Ionescu-Ittu, Mackie, Guo, Dendukuri and Kaouache2 Hence, transition and eventual transfer of care from paediatric to adult care is a crucial process to ensure lifelong access to specialised care. Reference Marelli, Ionescu-Ittu, Mackie, Guo, Dendukuri and Kaouache2
The process of transition of care has been described and endorsed by the American Academy of Paediatrics which states that “the goal of transition in health care for young adults with special healthcare needs is to maximise lifelong functioning and potential through the provision of high-quality, developmentally appropriate healthcare services that continue uninterrupted as the individual moves from adolescence to adulthood.” 7 The American Academy of Paediatrics advises that parents and their doctors begin to plan for transition as early as age 12, and they also advise parents to plan for the move to an adult doctor between ages 14 and 18. 7 However, despite these suggestions, the process of transition of care across several chronic healthcare conditions including CHD remains poorly executed across the United States.
Transition of care refers to the coordination and continuity of health care during movement from one healthcare setting to another and/or between healthcare practitioners as care needs change during a chronic illness. Reference Sable, Foster and Uzark8 Transition encompasses the process of patient and family education and preparation for young patients to assume their own care as they enter adulthood. Reference Cotts5 It is important to differentiate transition from the process of “Transfer of Care” which refers to a timepoint when the care of a patient with chronic healthcare conditions is handed off to an appropriate adult care provider. For young patients in their adolescence with chronic illnesses, the focus of transition of care is typically on moving their “medical home” successfully from the paediatric to the adult healthcare setting, which includes bolstering education around the chronic medical condition and emphasising patient independence and autonomy. Reference Reid, Irvine and McCrindle9,Reference Gurvitz, Valente and Broberg10 As children mature into young adults, their medical and psychosocial needs evolve beyond the scope of practice of their paediatric doctors, necessitating the process of transition to the adult setting. Reference Sable, Foster and Uzark8
It is now well recognised that a significant proportion of patients with CHD are lost to follow-up during a vulnerable period in their lives as they transition from paediatric to adult healthcare settings. This highlights the importance of establishing formal transition programmes. Many barriers have been described that can hinder successful transition without appropriate transfer of care, with high proportions of adolescent patients with CHD experiencing long gaps in care after leaving paediatric cardiology, even among those with severe CHD lesions. Reference Lee, Bailey, Cullen-Dean, Aiello, Morin and Oechslin4,Reference Mackie, Ionescu-Ittu, Therrien, Pilote, Abrahamowicz and Marelli11 Most adolescents and adults with moderate and severely complex CHD are at significant risk for long-term complications such as heart failure, arrhythmia, need for additional interventional or surgical procedures, and premature mortality. Notably, patients who receive specialised and non-interrupted comprehensive care often have better outcomes. Reference Nicolarsen12 According to Mylotte et al., there is an independent association between specialised adult CHD care and reduced mortality driven primarily by patients with severe underlying CHD. Reference Mylotte, Pilote and Ionescu-Ittu13 Therefore, it is imperative to understand the scope of this problem in both urban and non-urban settings. In this study, we sought to describe social, demographic, and clinical factors related to successful transition in a tertiary urban care facility in patients with CHD.
Materials and methods
Patients were identified utilising the electronic medical record (Epic) generated data warehouse. Inclusion criteria were patients with CHDs based on the AHA/ACC Guidelines 2018 Reference Stout, Daniels and Aboulhosn14 and age ≥15 years seen in the ambulatory paediatric cardiology clinic between 2013 and 2014. The only exclusion criteria were deceased patients. Clinical and demographic variables were collected by retrospective chart review. Patient charts were reviewed for the years 2015–2021 to determine if patients who met inclusion criteria were a) continued in care with paediatric cardiology, b) transitioned to adult cardiology or adult CHD, or were c) lost to follow-up. Independent predictors for successful transition of care or lost to follow-up were analysed using chi-squared tests. Severity of CHD was determined via the ACC/AHA adult CHD anatomy and physiology classification system per the national guidelines. Reference Stout, Daniels and Aboulhosn14 The frequency of paediatric cardiology visits was stratified into the following: annually, biannually, quarterly, and less than annually. Insurance information for each patient was collected and stratified into uninsured versus insured. The number of comorbidities and medications was collected for each patient.
Patients were sub-stratified into the following three categories: lost to follow-up, CIC whether are with paediatric cardiology, and transition of care to either adult cardiology or adult CHd. Transition to adult care was defined as at least one visit with a member of the adult CHD or an adult cardiology team. Statistical analysis was performed using the Pearson chi-square test of independence for each association. Analysis of variance was used with the numeric variables to describe the averages across the three transition groups. A post hoc two-sided t-test of adjusted means was used to determine the statistically significant difference between the groups. The number of comorbidities as well as the number of medications were compared across the three groups separately by using an analysis of variance for each outcome. In addition, adjusted means from each model were compared via post hoc testing.
Results
A total of 322 patients were identified (Fig 1), with 46% (N = 149) being female, 53% (N = 172) being male, and 1 patient identifying as non-binary. The majority of patients had moderate CHD (41%, N = 132). Only 9% (N = 30) had simple CHD while 17% (N = 55) had severely complex CHD (Table 1). The most common defects by complexity were simple CHD lesions: ventricular septal defect (N = 21, 6%), moderate CHD: tetralogy of Fallot (N = 28, 9%) and severe CHD: transposition of the great arteries (N = 20, 6%) (Table 2). Of the 322 patients, the majority of patients (53%, N = 172) had public insurance, followed by 67 patients (21%) with private insurance, while 47 patients (15%) were uninsured (Table 1). Regarding transition of care, of the 322 patients, 93 pts (29%) were lost to follow-up, 159 patients (49%) were seen by adult cardiology/adult CHD (successful transition of care) and 70 patients (22%) CIC with paediatric cardiology (Fig 1).
Transition of care: breakdown into LTF, CIC, and TOC. Abbreviations: LTF: lost to follow-up, CIC: continued in care, TOC: transition of care.
Baseline demographics
CHD lesions by complexity
A total of 188 patients were categorised with simple, moderate, or complex CHD lesions based on the AHA/ACC 2018 guidelines (Fig 2). Of the patients who were lost to follow-up, most (N:30, 64%) had moderate lesions followed by simple (N;11, 23%) and complex (N:6, 13%). Of the patients who CIC with paediatric cardiology, most (N:33, 66%) had moderate lesions followed by complex lesions (N:9, 18%), and simple lesions (N:8, 16%). Finally, of patients who successfully transitioned care, the majority had moderate lesions (N:75, 82%), followed by complex (N:10, 11%) and simple lesions (N:6, 7%) (Fig 2).
Transition of care by CHD severity. Abbreviations: LTF: lost to follow-up, CIC: continued in care, TOC: transition of care.
A chi-square test of independence revealed that the severity of CHD diagnosis was significantly associated with successful transition of care (χ2 = 25.9779, 6 df, p = 0.0002), with more complex lesions leading to a higher rate of successful transition. Having any form of healthcare insurance was associated with successful transition of care (χ2 = 45.6969, 2 df, p < .0001), compared to those patients who were uninsured with a higher loss to follow-up rate. Presence of an implantable cardioverter defibrillator was significantly associated with successful transition of care (χ2 = 11.7605, 2 df, p = 0.0028). In addition, higher frequency of paediatric cardiology visits was significantly associated with successful transition of care (χ2 = 24.3249, 6 df, p = .0005).
The analysis of variance test provided for comorbidities showed a significant difference in average number of comorbidities among the different transition groups (F = 17.48, p < 0.0001). Post hoc comparisons showed a significant difference between the CIC (with paediatric cardiology) and lost to follow-up groups (p < 0.001) with those patients with more comorbidities being in the CIC group. Patients with fewer medical comorbidities were more likely to be lost to follow-up. No significant difference was found in comorbidities between the lost to follow-up and successful transition of care group.
The most prevalent medications in our patient population included aspirin, lisinopril, enalapril, furosemide, and beta-blockers. An analysis of the average number of medications found a significant difference in the number of medications between the transition of care groups (F = 7.33, p = .0008). Post hoc comparisons showed that pts with a higher number of medications had successful transition of care, when compared to those who CIC (p = 0.0034). Similarly, patients who were on fewer medications tended to have a higher rate of lost to follow-up when compared to those who had successful transition of care (p = 0.0011). The average number of medications was not significantly different between CIC and lost to follow-up groups (p = 0.9477).
Discussion
Despite the growth of adult CHD as a specialised field over the last few decades, many adolescents and young adults with CHD are often lost to follow-up or experience long gaps in care after leaving paediatric cardiology due to lack of appropriate transition of care. Reference Reid, Irvine and McCrindle9 The expanding adolescent CHD population in the United States continues to bring new challenges as these patients age into adulthood. This single-centre study at a large urban centre describes the rate and factors associated with transition of care, including lost to follow-up and CIC in adolescents with all forms of CHD. Transition of care is a crucial and critical process to provide access to specialised care and lifelong surveillance. Despite this, there remains a high proportion of young patients with CHD in the United States who are unable to successfully go through transition of care and are lost to follow-up. Most patients with CHD, especially those with moderate and severely complex lesions, will require periodic assessment as they transition into adulthood, owing to the potential need for re-interventions, screening for arrhythmias or congestive heart failure. Many patients as they enter their third and fourth decades of life can also develop non-cardiac comorbidities, including liver, renal, endocrine, and neurological conditions. It is imperative that these patients receive uninterrupted health care which should include the period spanning adolescence to adulthood.
Over the last decade, though several steps have been identified to improve transition, the outcomes for successful transition in CHD continue to be suboptimal. In 2004, Reid et al had reported that only 48% of adolescents with CHD underwent successful transition. Their data suggested that ongoing discussions during adolescence with a focus on the importance of transition was of significant value to successful transition of care. Reference Reid, Irvine and McCrindle9 In a 2015 study, fewer than 30% of adults with CHD were appropriately followed by specialised providers, and only 48% of adolescent patients with CHD underwent successful transfer to adult care. Reference Heery, Sheehan, While and Coyne15 A multi-country systematic review from 2021 found that “discontinuity of care” had a pooled estimate proportion of 26.1% among the 17 studies included and the proportions were significantly higher in studies from the United States and in patients with simple CHD lesions. Reference Moons, Skogby, Bratt, Zuhlke, Marelli and Goossens16 In 2022, Moore et al described that 33% of their cohort were “actively missing” from care meaning they had not engaged in cardiac care and when the study engaged these patients, only 3% of them successfully returned to care. Reference Moore, Sheth and Lam17 Despite ongoing efforts, the trends regarding transition of care have not significantly changed over time, necessitating the ongoing importance of determining underlying factors that prevent engagement in health care for this high-risk population.
Several barriers to successful transition have been described in the literature. In the United States of America healthcare system, healthcare insurance has proven to be a barrier to successful transition, since patients with CHD may lose access to their parents’ insurance as they get closer to the actual age of transfer of care. Reference Cotts5 On an individual level, there is often reluctance from the patient, the patient’s family, and the patient’s paediatric cardiologist due to a perceived lack of quality adult CHD providers, emotional/cognitive delay of an adolescent patient with CHD, and a lack of structured transition programmes. Furthermore, transition is often avoided due to a patient’s and parental’s anxiety about the transition process and misperceptions about a patient’s prognosis and long-term consequences of their actions. Reference Lee, Bailey, Cullen-Dean, Aiello, Morin and Oechslin4 It has also been shown that adolescents with CHD are unconcerned about transition, lack knowledge about their underlying cardiac condition and are inadequately prepared for transfer to the adult healthcare setting. Many adolescents desire continuity in the paediatric setting with youth-oriented facilities, a personalised approach, and prefer that their parents remain involved in their care, but only in a secondary, supportive capacity. Reference Heery, Sheehan, While and Coyne15 Moceri et al revealed loss to follow-up may be due to poor health literacy and inadequate discussion highlighting the importance of the transition process. Reference Reid, Irvine and McCrindle9,Reference Moceri, Goossens and Hascoet18
Factors that have been shown to protect against a lapse in care include the following: beliefs that specialised adult care was necessary; poorer health status; attendance at paediatric appointments without parents; and direct paediatric referral to an adult CHd centre or programme. Reference Warnes, Williams and Bashore19,Reference Webb and Williams20
There has been a wide variation in reported percentages of patients with CHD who had lapses in care or were lost to follow-up. However, several of these studies are limited by being small cohorts of patients with variable study populations and recruitment methods. Within our single centre, 93 of 322 patients (28.9%) were lost to follow-up which is similar to several prior studies. Reference Kollengode, Daniels and Zaidi21 The growing adult CHd population remains heterogeneous, ranging from those with mild defects requiring little or no intervention to moderate and severely complex CHD lesions. The distribution of CHD severity in our population is similar to previously published estimates. Reference Marelli, Mackie, Ionescu-Ittu, Rahme and Pilote3,Reference Kollengode, Daniels and Zaidi21,Reference Warnes, Liberthson and Danielson22
Our data suggest that the severity of CHD diagnosis was significantly associated with successful transition of care, with more complex lesions leading to a higher rate of successful transition. This is similar to prior studies. Reference Norris, Webb and Drotar23,Reference Valente, Lewis and Vaziri24 However, it does suggest that patients with simple CHD lesions were more likely to be lost to follow-up. It is postulated, though not studied, that patients with simple CHD may be more prone to lost to follow-up since their understanding is that they do not need long-term follow-up and thus do not pursue transition of care.
Though the process of transition has been described in the literature over the last few decades, successful implementation of a transition process requires purposeful movement of adolescents and young adults from child-centred to adult-oriented healthcare systems. This is integral to minimise morbidity and mortality; however, most CHD programmes still do not have formal transition programmes. Though our centre has adjoining adult and paediatric cardiology programmes within the same health system, there was no formal transition of care programme at the time of this study. This allowed significant variability in the transition process based on patient, family, and paediatric cardiac provider’s preferences as to how patients should continue their long-term care, resulting in nearly 29% of CHD patients being lost to follow-up. These patients should have benefited from long-term continuity of care but instead were lost to follow-up. This represents a significant portion of patients with suboptimal transition and is consistent with prior studies that report that nearly half of adult CHD patients experience significant gaps in cardiology care at some point in their lives. Reference Gurvitz, Valente and Broberg10,Reference Knauth, Verstappen, Reiss and Webb25 Our study also demonstrated that almost 22% (N = 70) of the patients CIC with paediatric cardiology, thus continuing to receive long-term care and were not lost to follow-up. Being positioned at a large urban care setting with both departments residing in the same healthcare system, we would have expected more patients to have successful transition, however only 49% of patients (N = 159) had successful transition of care. For the purposes of this study, successful transition was considered contact with an adult CHd or a general adult cardiologist.
Many patients with CHD may lose access to their parents’ insurance as they get closer to the actual age of transfer of care. Reference Cotts5 The Patient Protection and Affordable Care act, signed into law in 2010, ensured medical coverage for patients with pre-existing conditions and allowed young adults to remain on their parent’s insurance policy until age 26 years. However, despite widespread availability and access to health insurance, barriers to accessing high-quality adult cardiac care still exist and often patients are unable to procure healthcare insurance leading to loss of follow-up. Of the 322 patients, the majority of the patients (77%, N = 250) had some form of healthcare insurance, while 47 pts (15%) were uninsured. When stratified into insured and uninsured, having any form of healthcare insurance was associated with successful transition, suggesting that a lapse in healthcare insurance coverage was a predisposing factor to experiencing a gap in care.
Our study also showed that those patients taking multiple medications had improved chances of long-term continuity of care which has also been reported in prior studies. Reference Kollengode, Daniels and Zaidi21 Similarly, it has been reported that patients who had undergone particular cardiac interventions, such as an implantable defibrillator, were more likely to remain in care thus leading to successful transition. These findings suggest that patients with CHD seem to stay in care when they are on medications or have cardiac devices or interventions, since they need to have these medications refilled or have their cardiac devices followed over a longer period of time, thus preventing a gap in follow-up care.
Limitations
Limitations include the retrospective analysis of data and inability to assess continuity of care outside our healthcare system in those who could not be contacted. The collected and analysed data was extracted from an electronic medical record and is subject to coding variations of CHD lesions. In addition, our study did not include analysis of patient or family understanding of the need for lifelong care for patients with CHD, their impressions of transitioning to adult cardiology, or the effect of cognitive impairment on the rate of successful transition. Lastly, our study did not investigate the different practices of the individual paediatric cardiologists as to when and if they initiate the process of transition.
Conclusions
The process of transition of care is well recognised and endorsed by the American Academy of Pediatrics since 2002. 7 More recently, the American Academy of Pediatrics along with the American Academy of Family Physicians and the American College of Physicians updated their recommendations for transition of care in 2018. Reference White and Cooley26
Due to the increasing number of patients with CHD undergoing transition, multiple governing academic bodies have put forth statements regarding the importance of transition of care for the CHD population. In 2008 and then in 2018, the American College of Cardiology / American Heart Association guideline documents for the management of adult CHd placed significant emphasis on the successful transition of care for patients with CHD. Reference Warnes, Williams and Bashore19,Reference Stout, Daniels and Aboulhosn27 In 2011, best practices in managing transition of adolescents with CHD to adulthood was published by the American Heart Association. Reference Sable, Foster and Uzark8 Unfortunately, despite advances in the field, there continue to be lapses in care which appear to be predictors for morbidity and poor longer outcomes, particularly for minority populations. Reference Mylotte, Pilote and Ionescu-Ittu13,Reference Lotstein, Inkelas, Hays, Halfon and Brook28 Today, despite over two decades of national efforts, there are still unmet American Academy of Pediatrics needs for this high-risk population. It is now well established that the transition of care process with eventual transfer of care for children with CHD should include a referral or contact with a specialised adult CHd centre.
This single-centre study aimed to explore various factors associated with successful American Academy of Pediatrics or lost to follow-up from paediatric to adult cardiology or adult CHd for adolescents and adults with CHD in a tertiary urban care centre. Successful transition can be empowering for patients and their families, as they build a new therapeutic relationship with an appropriate adult provider.
In our single-centre experience, patients with greater complexity of CHD, those using medications, those with an implantable defibrillator, and those with healthcare insurance were more likely to stay in medical care and undergo successful transition. Patients with simple CHD represent a subgroup with higher rates of lapses in care and who may benefit from increased scrutiny and focused transition education. In the context of the increasing numbers of adult CHd patients in the United States that continues to rise, there is an immediate and urgent need to improve the process of transition in patients with CHD to improve the long-term outcomes and allow these young people to meet their full adult potential in the years to come.
Acknowledgements
None.
Financial support
None.
Competing interests
All authors have no conflict of interest.
Ethical standards
The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation (please name) and with the Helsinki Declaration of 1975, as revised in 2008, and have been approved by the institutional review board at Mount Sinai.
