Abstract
OBJECTIVE: To assess parental knowledge regarding lifelong congenital cardiac care (LLCCC).
BACKGROUND: National guidelines recommend that nearly 50% of adult survivors with congenital heart disease (CHD) receive LLCCC; the number of adults who receive such care seems far less. Inadequate parental knowledge of LLCCC might contribute to care interruption.
METHODS: In this multicenter study, we administered a questionnaire to parents of children with moderate and complex CHD to assess knowledge of LLCCC.
RESULTS: A total of 500 parents participated; the median age of their children was 10 years (range: 2–18 years). Most parents (81%) understood that their child would need LLCCC, but only 44% recognized that their child's cardiology care should be guided by an adult congenital heart specialist in adulthood. More than half (59%) of the parents stated that their current cardiology team had never spoken to them about LLCCC, but 96% wished to learn more. Variables associated with parental LLCCC knowledge included previous discussions regarding LLCCC, underlying cardiac surgical diagnosis, and level of parental education.
CONCLUSIONS: A substantial number of parents of children with moderate and complex CHD lack knowledge about LLCCC, but almost all of them have a desire to learn more about the care their child will need as an adult.
WHAT'S KNOWN ON THIS SUBJECT:
Although national guidelines recommend that adult survivors with congenital heart disease receive lifelong congenital cardiac care, fewer than half of them receive appropriate care. Inadequate parental knowledge about lifelong congenital cardiac care might contribute to care interruption.
WHAT THIS STUDY ADDS:
A substantial number of parents of children with moderate and complex congenital heart disease lack knowledge about lifelong congenital cardiac care, but almost all of them have a desire to learn more about the care their child will need as an adult.
Improved survival rates of patients with congenital heart disease (CHD) have led to a growing number of adults with CHD; population estimates exceed 1 million in the United States.1 National management guidelines recommend that all emerging adults with CHD receive at least 1 evaluation at an adult congenital heart disease (ACHD) center to determine the appropriate level of care. At least 50% of these patients will require active ACHD center management, and the remainder should be seen by a pediatric or adult cardiologist with a referral relationship with a regional ACHD center.2 Despite these recommendations, the number of adults with CHD who receive such specialized care is less than projected estimates.3,–,7 In addition, the majority of patients who currently receive specialized congenital cardiac services experience considerable gaps in care, including care interruptions that often begin in late adolescence and extend until the onset of an acute health care crisis.8,9 The reasons for such gaps are multifactorial, but likely include limitations in patients' and/or affected family members' understanding of lifelong congenital cardiac care (LLCCC) issues.10 Recognition of the need for LLCCC is the first step in preparation of the child and family for continuing care within an adult-oriented health care system. We therefore sought to determine if parents of children with moderate and complex CHD understood the need for LLCCC and the need to have this care guided by an ACHD specialist throughout adulthood.
METHODS
We conducted a multicenter cross-sectional survey of parents of children 2 to 18 years of age with repaired CHD. The study was limited to families of children with 4 diagnoses, for whom national guidelines recommend LLCCC guided by an ACHD specialist.2 These included repaired aortic coarctation, repaired tetralogy of Fallot (TOF), single ventricle physiology with Fontan palliation, and transposition of the great arteries (TGA) with an arterial switch operation. A total of 500 parents were targeted, including at least 90 parents from each diagnostic group. Parents were invited to participate at the time of their child's routine cardiology appointment and were provided a verbal overview of the study and a written information sheet. Consenting parents completed the survey before their child's cardiology visit. The survey was approved by the local institutional review board at each participating center. The study was initiated in January 2009 and completed in July 2010. The survey tool was developed over a 6-month timeframe through a working group of clinicians, individuals with congenital heart disease, and a survey methodologist. The final survey included a total of 17 questions within 4 categories: (1) current cardiology care; (2) need for LLCCC; (3) exposure to education regarding LLCCC; and (4) demographics.
Section 1 included 2 questions concerning the last time the child was seen by a congenital cardiologist or designee, and how often that child was typically seen. In section 2, parents were asked if they thought their child required LLCCC, whom they thought would provide such, and how often they felt that this care should be provided. Parents were asked in section 3 if they recollected their child's cardiologist or designee speaking to them about the type of cardiac care their child would need as an adult and how often they recalled receiving this information. The parent was then asked if they were interested in learning more about the kind of cardiac care that their child might need as an adult.
STATISTICAL ANALYSIS
Parental and patient characteristics were summarized by using medians and ranges for continuous variables and counts and proportions for categorical variables. We defined parental knowledge for LLCCC as the understanding that LLCCC should be guided by an ACHD specialist. Wilcoxon rank-sum tests, χ2 tests, or, in the event of small sample size, Fisher's exact tests were used to assess the associations between parental knowledge for LLCCC and other covariates. We used multivariable logistic regression to identify independent predictors of parental knowledge for LLCCC. Potential interactions between age and other covariates (ie, child's diagnosis, previous discussion by the cardiology team about LLCCC, and parental education) were considered for inclusion in the multivariable model. Because the proportion of missing values was <2% for most instrument items, adjustments for missing data were not performed. All tests were performed with a 2-sided α value of .05. Data analyses were performed by using SAS 9.2 (SAS Institute, Inc, Cary, NC).
RESULTS
A total of 500 of 510 (98%) parents approached agreed to study participation. Parental characteristics are summarized in Table 1. At the time of enrollment, the median age of these parents' children with CHD was 10 years (range: 2–18 years). The CHD diagnoses in affected children were repaired aortic coarctation in 126 (25%), Fontan operation for single ventricle in 141 (28%), repaired TOF in 134 (27%), and arterial switch operation for TGA in 99 (20%).
Parental Characteristics According to Center (N = 500)
The last cardiac visit before study enrollment was stated to be within 6 months by 49% of parents, between 6 and12 months by 41%, between 1 and 2 years by 7%, and more than 2 years by 3% of parents. Most parents (91%) stated that it was usually no more than 12 months between visits. However, 7% noted that their child was typically seen every other year, and 2% stated that the time interval between visits exceeded 2 years.
The majority of parents (81% [95% confidence interval: 77%–84%]) recognized their child required LLCCC, although 18% were unsure whether this was necessary, and 1% stated that they thought their child would not need this type of care. Previous discussion by the cardiology team about LLCCC and parental education were associated with parental knowledge of LLCCC (P < .0001 and P <.0001, respectively).
Of the parents who understood that their child would need LLCCC, most (93%) recognized that adult care should be provided by a cardiologist. The remainder were either unsure who should provide this care or thought it should be provided by the child's primary care physician (6% and 1%, respectively). Among the parents who recognized their child's cardiac care should be provided by a cardiologist, 62% stated this should be provided by an ACHD specialist, 12% thought it should be provided by a pediatric cardiologist, and 11% indicated that it should be provided by a general adult cardiologist. An additional 15% of the parents were unsure which type of cardiologist should provide their child's cardiac care in adulthood (see Fig 1).
Parental knowledge of LLCCC.
Overall, 56% (95% confidence interval: 52%–61%) had not known that LLCCC was recommended and that care during adulthood should be guided by an ACHD specialist. Factors associated with parental knowledge are listed in Table 2.
Association Between Parental Characteristics and Parental Knowledge That LLCCC Should be Guided by an ACHD Specialist
The child's CHD diagnosis was associated with parental understanding of LLCCC. For example, parents of children with repaired TOF and Fontan surgery showed a higher level of knowledge (48% and 50%, respectively) compared with parents of children with an arterial switch operation or repaired aortic coarctation (40% and 32%, respectively; P = .03).
The level of parental education was likewise associated with knowledge regarding LLCCC. The need for LLCCC provided by an ACHD specialist during adulthood was appropriately recognized by 29% of parents without a high school diploma, 41% of those with a high school diploma, 51% with a bachelors degree, and 51% with a master's or doctoral degree (P = .02).
In addition, a greater proportion of parents who recalled previous discussions with their cardiac team regarding LLCCC recognized the importance of LLCCC (P = .01). Overall, 41% of parents surveyed recalled having previously discussed LLCCC with their child's cardiology team. Recall of a single discussion was reported by 10% of parents, whereas 24% recalled a few discussions, and only 5% recalled frequent discussions. Parental recall of LLCCC discussions was not influenced by the age of the child, ie, 47% for ages 2 to 6 years, 35% for ages 7 to 11 years, 43% for ages 12 to 15 years, and 38% for ages 16 to 18 years (P not significant). The potential for interactions between discussion of LLCCC and other predictor variables was explored, but no significant interactions were found. The majority of parents (93%) stated they had never received written material regarding the cardiac care their child would need in adulthood. Of the small number (35 parents) who did receive written material, 27 stated that it had been distributed by their cardiology team, 7 by their child's primary care physician, and 1 parent each indicated that written information was received from either their medical insurance carrier, a patient advocacy group, or the Internet. Despite the limited number of parents who received information about LLCCC, the overwhelming majority (96%) were interested in learning more about the care their child would require in adulthood.
The parental relationship to the child, race and ethnicity, type of insurance, participating center, and timing and frequency of clinical visits were not associated with parental knowledge. The age of the child was also not associated with the parental level of knowledge (ie, 43% for ages 2–6 years, 43% for ages 7–11 years, 48% for ages 12–15 years, and 40% for ages 16–18 years; P not significant).
DISCUSSION
There are many perceived barriers to continuity of specialty cardiac services for patients with CHD; lack of parental knowledge regarding recommendation for children with specific CHD diagnoses to receive LLCCC is likely to be such an influence.11,–,15 In the current study, we demonstrate that a significant number of parents of children with moderate and complex CHD are unaware that national guidelines suggest LLCCC for their children. Such parents frequently stated that their health care team had not discussed this recommendation. Lack of parental knowledge likely contributes to lapse in health services for their children, which has been associated with increased morbidity in patients with CHD.16
The American College of Cardiology and the American Heart Association 2008 management guidelines recommend that the care of adults with moderate and complex CHD be guided by clinicians trained in ACHD.2 Despite this recommendation, the majority (51%) of parents in our study did not relate an understanding of this need. Parental awareness of the need for LLCCC was influenced by the child's underlying cardiac diagnosis, recollection of previous discussions with the health care team about LLCCC, and the parent's level of education.
Cardiac Diagnosis
We found that parents of children with an arterial switch operation and those with repaired aortic coarctation were less likely to be aware that LLCCC guided by an ACHD specialist in adulthood was recommended, in comparison to parents of children with TOF or Fontan palliation. All 4 of these diagnostic groups are at more than minimal risk for late complications and might require re-intervention. Patients in all diagnostic categories face potential long-term complications, including fibrosis at intracardiac and intravascular surgical sites, development of valvular disease, arrhythmia, thrombosis, aneurysmal formation, myocardial dysfunction, fluid retention, coronary artery disease, and multi-organ failure.2,17,–,30 Despite these shared potential sequelae, the perception of LLCCC needs differed according to underlying diagnosis. Although reasons remain unknown, parental knowledge might have been influenced by a greater perception of achieved normalcy for children with specific diagnoses, because of less frequent visits, testing, and interventions and less cardiovascular decompensation during childhood. Indeed, children with TGA and coarctation of the aorta generally have a single intervention during infancy with only outpatient visits for diagnostic tests subsequently, whereas patients with TOF repair or Fontan repair are more likely to receive active medical management and/or anticipating subsequent interventions and thus are more aware of the need for ongoing care.
LLCCC Discussion
Less than half of parents surveyed recalled a discussion with their health care team about LLCCC, and only a small proportion had been provided with written material. Previous discussions with health care professionals regarding the need for LLCCC was the single most important variable associated with parental knowledge of LLCCC. Even in the group of parents who stated they recalled a previous discussion about LLCCC, less than half understood the need for such care. Given that the overwhelming majority of parents expressed an interest in being better educated about LLCCC for their child, clinicians and institutions should provide a more structured approach to address these needs. In addition, patient advocacy groups might help increase public awareness and the availability of print material might help to improve knowledge regarding LLCCC.
Of interest, as institutions implement and sustain transitioning (self-care management) programs, one might expect parental knowledge to correlate with the increasing age of the affected child, because of repetition of education. Such an association was not identified in our study. Level of parental knowledge was not greater for patients aged 16 to 18 years compared with younger age groups (2–6, 7–11, and 12–15 years). We recognize, however, that transitioning (self-care management) programs and efforts are relatively new and that the effect of such programs on measurable improvements in parental education has not yet been demonstrated. It might be reasonably anticipated that, with continued commitment and growth of such programs, parental, and patient appreciation for LLCCC will likely improve.
The lack of parental knowledge of lifelong medical care is unlikely to be isolated to the CHD population. Additional study in other pediatric diseases that are likely to require lifelong medical care such as diabetes, sickle cell, childhood cancers, inflammatory bowel disease, and cystic fibrosis might be helpful. Given the shared need for transitioning (self-care management) skills for adolescent and adult patients with pediatric onset disease, institutions should consider providing resources for the development of such programs across subspecialty lines.
Parental Education
An important finding in this study is that the level of parental school education was strongly associated with knowledge for LLCCC for their child. The fact that 96% of parents expressed an interest in receiving additional information about the type of care their child will need in adulthood indicates the need for culturally appropriate educational materials directed toward the educational level of the parents. Although all parents might potentially benefit from such programs and educational tools, efforts should be intensified for the parental group most in need; ie, those without advanced education. Clinicians should inquire about the level of parental education, their native language, and their ability to read, and should adjust the educational plan accordingly.
Our study has several limitations. Despite the high response rate, the survey design inherently has the potential for response bias and the results reflect self-reporting, which might overestimate or underestimate knowledge and previous discussions regarding LLCCC. Desirability bias might overestimate the actual number of parents that understand the need for LLCCC for their child, because the survey tool itself suggests the need for such care. Results might not be generalizable to all parents of children with CHD because only 4 subgroups were studied. The survey tool has not been validated. In addition, indirect relationships or interactions might not have been detected with our statistical approach. Finally, all participating centers have an affiliated ACHD program, and 4 of the 9 centers have a formal transitioning program or process. These patients are often seen in the same clinic as the pediatric patients and their families. Our results, therefore, might overestimate the actual proportion of parents of children with CHD that understand the need for LLCCC.
CONCLUSIONS
The majority of parents of children with CHD have knowledge gaps regarding the need for LLCCC for their child and the need for this care to be guided by an ACHD specialist in adulthood. Discussion by the cardiology team regarding this need seems to improve parental knowledge. The overwhelming majority of parents of children with CHD are interested in learning more about the care their child will require in adulthood. This provides an opportunity within the different phases of care transition, to develop and implement educational programs designed to increase the knowledge of patients and their families regarding LLCCC. Additional study of child, parental, and caregiver influences on disease and care guidelines education, as well as of outcomes of specific programming regarding LLCCC, seems warranted.
ACKNOWLEDGMENTS
This work was supported in part by the Dunlieve Foundation.
We thank the American College of Cardiology's Adult Congenital and Pediatric Cardiology Section and the section's ACHD Work Group for support and guidance; Karin Coulter of Saving Little Hearts and members of the National Congenital Heart Coalition; and the numerous people who helped with this project, including Handan Titiz, EdM, Dionne Graham, PhD, Elizabeth Rodriguez-Heurtas, BS, Sarah Evans, BA, Erin E. Olson, BA, and Michelle J. Nickolaus, MSN, CRNP.
Footnotes
- Accepted August 20, 2011.
- Address correspondence to Susan M. Fernandes, MHP, PA-C, Department of Cardiology, Children's Hospital Boston, 300 Longwood Ave, Boston, MA 02115. E-mail: sue.fernandes{at}cardio.chboston.org
All of the authors made substantive intellectual contributions to either conception or design, acquisition of data or analysis and interpretation of the data, and provided critical input to the development of this article and approved the final version.
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
-
- CHD —
- congenital heart disease
- ACHD —
- adult congenital heart disease
- LLCCC —
- lifelong congenital cardiac care
- TOF —
- tetralogy of Fallot
- TGA —
- transposition of the great arteries
REFERENCES
- Copyright © 2011 by the American Academy of Pediatrics
