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Advance Care Planning in Adults with Congenital Heart Disease: A Patient Priority
International Journal of Cardiology 231 (2017) 105–109
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Advance Care Planning in Adults with Congenital Heart Disease: A Patient Priority☆ Lisa X. Deng a,1, Lacey P. Gleason a,1, Abigail M. Khan b,1, David Drajpuch a,1, Stephanie Fuller a,1, Leah A. Goldberg a,1, Christopher E. Mascio a,1, Sara L. Partington a,1, Lynda Tobin a,1, Yuli Y. Kim a,1, Adrienne H. Kovacs b,⁎,1 a b
Philadelphia Adult Congenital Heart Center, joint program of Hospital of the University of Pennsylvania and The Children's Hospital of Philadelphia, Philadelphia, PA, USA Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, USA
a r t i c l e
i n f o
Article history: Received 25 September 2016 Received in revised form 19 December 2016 Accepted 31 December 2016 Available online 4 January 2017 Keywords: Adult congenital heart disease Advance care planning Anxiety
a b s t r a c t Background: Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs. Methods: This study investigated ACHD patient preferences for ACP and factors that impact preferences. ACHD patients completed an ACP preferences questionnaire, the Hospital Anxiety and Depression Scale and a measure of attachment styles. Results: Of 152 ACHD patients (median age 33 years, 50% female), 13% reported previous ACP discussions with providers and 21% had completed advance directives. On a 0–10 scale, the median rating for the importance of discussing ACP with providers was 7; 18 years was identified as the most appropriate age to initiate this dialogue. Higher ratings for the importance of discussing ACP with providers was observed in patients who were female (p = 0.03), had lower disease complexity (p = 0.03), and had elevated anxiety symptoms (p = 0.001); elevated anxiety remained significant in a multivariable model. Interest in receiving information about life expectancy (61% overall) was greater among patients with lower disease complexity (p = 0.04) and a history of ≥2 cardiac surgeries (p = 0.01); disease complexity remained significant in a multivariable model. Conclusions: As a group, ACHD patients value the opportunity for ACP discussions and prefer earlier communication. Although some clinicians might avoid ACP discussions in patients who are generally more anxious or have less complex CHD, such avoidance does not appear to be warranted. © 2017 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Almost 90% of children born with congenital heart disease (CHD) will reach adulthood and adults now comprise almost 60% of the American CHD population [1,2]. However, adults with moderate or complex CHD continue to have substantial morbidity and mortality and the annual number of deaths within this group is projected to markedly increase [3,4,5,6]. As mortality has largely shifted to the
☆ This research was supported by a grant from Big Hearts to Little Hearts. ⁎ Corresponding author at: Knight Cardiovascular Institute, Oregon Health & Science University, 3181 Sam Jackson Park Rd, UHN-62, Portland, OR, 97239, United States. E-mail address: [email protected] (A.H. Kovacs). 1 This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.
http://dx.doi.org/10.1016/j.ijcard.2016.12.185 0167-5273/© 2017 Elsevier Ireland Ltd. All rights reserved.
adult setting, American guidelines for the management of adult CHD (ACHD) patients recommend that “all ACHD patients should be encouraged to complete an advance directive, ideally at a time during which they are not extremely ill or hospitalized” [7]. Advance care planning (ACP) is the process of planning for future medical care and typically includes the completion of advance directives and the appointment of a health care power of attorney. Canadian research documented that three-quarters of ACHD patients wanted to discuss end-of-life planning with health care providers, although only 1% recalled such discussions and only 5% had completed advance directives [8,9]. It is unknown whether Canadian findings are generalizable to an American setting. The objectives of this study were to (1) describe the ACP experiences and preferences in a sample of American ACHD patients, and (2) investigate whether patient preferences differ as a function of sociodemographic, medical or psychosocial factors.
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2. Methods 2.1. Study population This study was approved by our institutional ethics review board and written informed consent was obtained from all participants. Patients were consecutively enrolled at the outpatient clinic of an American northeastern tertiary ACHD center and inclusion criteria included age ≥ 18 years, documented history of CHD, and cognitive and language abilities to complete study questionnaires in English. 2.2. Study measures Patients completed a questionnaire focused on ACP and life expectancy that included questions used previously [9,10] and others written by our study team. ACP was defined as “the process of thinking about your preferences for future health care (including the kinds of treatment you would and would not want) and letting other people know your preferences should you get very sick and become incapable of speaking for yourself.” Patients were asked whether (and at what age) they had discussed ACP with a health care provider. Patients were also asked with whom, if anyone, they had talked about wishes for end-oflife care. In addition, patients were asked whether they had completed an advance directive or had identified a healthcare power of attorney who would make decisions about their health care should they be unable to do so for themselves. Patients were then asked the following questions: (1) On a scale of 0–10 (with 0 being least important and 10 being most important), how important is it to you to discuss advance care planning with your health care provider? (2) When do you think is the right age for a health care provider to start discussing advance care planning with a patient with congenital heart disease? (3) What is the best time to bring up ACP discussion? Response options to this third question included (a) Before getting sick, while healthy, (b) When first diagnosed with a life-threatening illness or complication from heart disease, (c) When first symptomatic with a life-threatening illness or complication from heart disease, (d) when first hospitalized with a life-threatening illness or complication from heart disease, and (e) If dying [9,10]. Next, patients were asked (yes or no) whether they wanted information about the estimated life expectancy of patients with their heart condition. They were also asked to rate how likely they were to have a shorter life expectancy compared to healthy people without CHD. Response options were: (a) a lot less likely, (b) slightly less likely, (c) about the same, (d) slightly more likely, and (e) a lot more likely. The Hospital Anxiety and Depression Scale (HADS), a 14-item measure, was used to measure symptoms of anxiety and depression [11]. Subscale scores for anxiety (HADS-A) and depression (HADS-D) range from 0 to 21 with scores ≥8 indicating clinically elevated symptoms of anxiety or depression [12]. Attachment style refers to the security of an individual's interpersonal relationships and was assessed using the Relationship Questionnaire, which is a self-report measure based on a 4-category model of attachment: secure, preoccupied, dismissing, and fearful [13]. People with a secure attachment style are comfortable with both intimacy and independence. Individuals with a preoccupied style are characterized as emotionally dependent on others. People with dismissing style are extremely self-reliant, and fearful individuals simultaneously desire and are afraid of emotional intimacy. Medical records were reviewed and CHD was categorized as being of simple, moderate, or great complexity [7]. Study data were collected and managed using REDCap (Research Electronic Data Capture), a secure web-based data capture application [14]. 2.3. Statistical analyses Data are summarized as medians with ranges or percentages as appropriate. With a univariate approach, we first evaluated whether
patient-reported importance of discussing ACP and interest in information about life expectancy varied as a function of sociodemographic factors, disease complexity, clinically elevated anxiety, clinically elevated depression, and attachment style. Correlation analyses, Chi-square tests and Mann Whitney tests were performed as appropriate. Effect sizes (eta-squared for importance of discussing ACP and phi and Cramér's V for interest in life expectancy) were also computed. Multivariable models were then performed to predict importance of discussing ACP and interest in information about life expectancy using variables selected on an a priori basis: age, sex, CHD complexity, HADS-A, HADS-D, and attachment style [15]. In multivariable models, HADS-A and HADS-D scores were entered as continuous variables and attachment style was dichotomized into secure vs. not secure. Missing values were excluded from analyses and given the exploratory nature of this research, significance was defined as p ≤ 0.05 for all analyses. Analyses were conducted using SPSS (Version 22.0. Armonk, NY: IBM Corp). 3. Results 3.1. Participants Of 321 patients invited to participate in the study, 165 consented, 26 declined, and 130 were unable to participate due to timing or scheduling issues. A total of 13 patients were excluded from the final analysis, thus generating a final study sample of 152. Table 1 summarizes the demographic and clinical characteristics of the study sample. The most common defects were tetralogy of Fallot (n = 29; 19%), d-transposition of the great arteries (n = 18; 12%), bicuspid aortic valve disease (n = 13; 9%), and coarctation of the aorta (n = 13; 9%). The median (range) HADS-A score was 7 (0–19), and the median (range) HADS-D score was 2 (0–14). The proportion of patients with elevated symptoms of depression, elevated symptoms of anxiety, and a secure attachment style did not differ between the three categories of defect complexity. 3.2. Advance care planning: Recalled discussions and communication preferences Nineteen patients (13%) recalled previous ACP discussions with their health care provider. The median (range) age of the first discussion occurred at 25 years (16–50). Most patients (n = 106, 70%) had discussed end-of-life wishes with other people, most commonly their spouse/partner (n = 61, 40%) and friends and family (n = 46, 51%). Thirty-two patients (21%) had completed advance directives and 28 (18%) had a healthcare power of attorney. Table 1 Demographic and clinical characteristics of study sample (N = 152). n (%) Age in years (mean & range) Female White Partnered College education or above Disease complexity Simple Moderate Great Total number of cardiac surgeries Elevated anxiety symptoms (HADS-A ≥ 8) Elevated depressive symptoms (HADS-A ≥ 8) Attachment Style Secure Dismissing Fearful Preoccupied Data are counts (percentages) or medians (range).
33 (19–67) 76 (50) 126 (83) 60 (40) 117 (77) 25 (16) 74 (49) 53 (35) 2 (0–8) 67 (46) 15 (11) 82 (57) 30 (21) 17 (12) 15 (10)
L.X. Deng et al. / International Journal of Cardiology 231 (2017) 105–109
On a scale of 0 to 10 regarding the importance of discussing ACP with health care providers, the median (range) score was 7 (0–10) and 83% (120/145) of patients reported a rating of 5 or higher (Fig. 1). Patients preferred providers to initiate the discussion at a median (range) age of 18 years (5–60). Most patients (n = 118; 78%) preferred ACP discussions to be initiated while healthy, before being diagnosed or hospitalized for a life threatening illness. Only 2 patients (1%) preferred to defer ACP discussions until they were dying. There were no significant correlations between age or education and importance of ACP discussions. As shown in Table 2, higher ratings for the importance of discussing ACP with providers were observed in patients who were female (p = 0.03), had lower disease complexity (p = 0.03), and had elevated anxiety symptoms (p = 0.001). Effect sizes were categorized as small for sex (eta-squared =0.025) and disease complexity (eta-squared =0.044) and medium for HADS-A score (eta-squared = 0.068). Although there was no statistically significant difference for relationship style, a small effect size (0.026) was revealed. In a multivariable model, the only factor associated with higher ratings was a higher HADS-A score (Beta =0.26; 95% confidence interval [CI] 0.03–0.30; p = 0.02). Though CHD complexity was associated with higher ratings for the importance of discussing ACP in the univariate analysis, it did not reach statistical significance on multivariable analyses (Beta = −0.16; CI -1.27–0.33; p = 0.06). 3.3. Patient preferences for information about life expectancy Ninety-three patients (61%) reported that they wanted information about the estimated life expectancy of patients with their heart condition; 47 (31%) did not want this information and 12 (8%) were unsure. As presented in Table 2, desire for information about life expectancy was greater among patients with lower disease complexity (p = 0.04) and a history of ≥ 2 cardiac surgeries (p = 0.01). Relationship style approached significance (p = 0.054). Education was not associated with interest in life expectancy information (less than college = 64% vs. college or above =67%, p = 0.76). Effect sizes were categorized as small for both disease complexity (phi =0.217) and number of cardiac surgeries (phi =0.207). Although a preference for discussing life expectancy did not differ significantly by HADS-A or relationship style, small effect sizes (0.108 and 0.239) were revealed. In the multivariable model, CHD complexity was the only factor associated with desire for information about life expectancy (odds ratio [OR] OR 0.55; 95% CI 0.31–0.99; p = 0.05). The following responses were elicited regarding how likely patients believed that they would have a shorter life expectancy than their peers without CHD: a lot less likely (n = 15; 10%), slightly less likely (n = 8;
50 45 40 35 30 25 20 15 10 5 0
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Table 2 Univariate analyses of factors associated with ACP preferences.
Sex Female Male Race White Non-white Education Less than college College or above Disease complexity Simple Moderate Great Number of cardiac surgeries b2 ≥2 HADS-A score b8 ≥8 HADS-D score b8 ≥8 Relationship style Secure Fearful Preoccupied Dismissing
Importance of discussing with provider (0–10 scale)
Want information about life expectancy
Median
P - value
n (%)
P - value
8 7
0.027
49 (71) 44 (62)
0.257
7 8
0.488
75 (65) 18 (72)
0.515
7 7
0.644
14 (64) 79 (67)
0.763
8 7 6
0.029
19 (86) 48 (68) 26 (55)
0.038
7 7
0.362
54 (76) 39 (57)
0.014
6 8
0.001
46 (61) 43 (72)
0.208
7 8
0.578
79 (67) 9 (60)
0.592
0.237
47 (61) 14 (82) 5 (42) 21 (78)
0.054
7 9 7 7
HADS-D, Hospital Anxiety and Depression Scale Depression subscale; HADS-A, Hospital Anxiety and Depression Scale Anxiety subscale.
6%), about the same (n = 28; 19%), slightly more likely (n = 58; 40%), a lot more likely (n = 37; 25%). Collapsed to create a dichotomous variable, 65% believed they were more likely to have a shorter life expectancy and this did not differ between patients according to depression and anxiety scores or categories of attachment style. Further, the percentage of patients who thought they had a shorter life expectancy did not differ by CHD complexity (simple = 63%; moderate = 65%; great =66%; p = 0.96) or education level (less than college =56%; college or above =67%, p = 0.30). 4. Discussion It was important to investigate whether American outcomes replicated Canadian results, given differences in the two health systems [16]. We observed that (i) ACP discussions rarely occur, (ii) as a group, patients with greater defect complexity deem ACP discussions to be of lower importance and are less likely to want information about life expectancy, and (iii) anxiety was positively correlated with greater interest in ACP discussions and life expectancy. 4.1. Low occurrence of ACP discussions despite high interest
<5
5-7 Patient Score
8 - 10
Fig. 1. Patient-reported importance of discussing ACP with health care providers on a 0 (least important) to 10 (most important) scale.
Approximately 1 in 10 patients recalled prior discussions with their providers and 1 in 5 had completed an advance directive or had a healthcare power of attorney. These findings are higher than the Canadian cohort [9] but nonetheless far below published recommendations [7]. Patients also recognized the importance of initiating ACP discussions prior to the diagnosis of a life-threatening illness [9]. The timing is critical because previous research has documented that many ACHD patients who die in the hospital experience aggressive life-sustaining measures without having discussed their preferences [17]. Known barriers include provider concerns about patient readiness for end-of-life discussions and challenges with prognostication [18]. The minority of patients in this study who discussed ACP with their
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providers did so several years later than they identified as the ideal age. Indeed, providers are known to be hesitant to initiate ACP discussions with younger patients [19,20]. It is of interest that the preferred age for the initiation of ACP planning is 18 years, which is the time when patients often transition from pediatric to adult health care. Discussion of long-term health expectations and the preparation of ACP documents have been recommended for transitioning adolescents and young adults with CHD [21,22] and raises the question of the optimal setting in which discussions should initially occur. Providers are encouraged to optimize skills in initiating and revisiting patient discussions regarding ACP and end-of-life care in order to provide the highest quality of care throughout patients' lifespans. Specific recommendations include normalizing these discussions, acknowledging prognostic uncertainty, and scheduling specific clinic visits [23]. 4.2. Lower interest in ACP among patients with more complex disease Although patients with more complex disease have the lowest life expectancy, we found that patients with complex CHD rated ACP discussions with health care providers as less important and were less likely to want information about the estimated life expectancy of patients with their heart condition. Further, only two-thirds of patients with CHD of great complexity thought they had a shorter life expectancy than their peers. As a group, patients with CHD are known to overestimate their life expectancy [24]. One hypothesis is that adults with CHD of greater complexity might exhibit avoidance toward discussions of ACP and/or life expectancy. A known patient-identified barrier to ACP discussion is patients' desire to focus on staying alive rather than talking about death [18]. A previous study of patients with complex CHD also revealed that denial might be used to help patients adapt [25]. Qualitative research is recommended to investigate why patients with complex CHD appear more reluctant to discuss ACP and life expectancy. A previous study observed that ACHD providers tend to raise end-oflife issues more often with patients of great rather than simple disease complexity [9]. Our findings suggest that clinicians ought to invite (although not impose) discussions of ACP and life expectancy with all patients, irrespective of disease complexity. It has been suggested that there is a subgroup of patients with CHD of simple complexity who anticipate a reduced life expectancy and for whom discussions of near-normal life expectancies may alleviate their concerns [18]. 4.3. Psychological correlates of ACP preferences Univariate analyses revealed that patients with elevated anxiety symptoms rated ACP discussions as more important and there was a small effect size for desire for information about life expectancy. There were also small effect sizes for patients with a fearful attachment style to report higher interest in ACP discussions and be more likely to want information about life expectancy. Attachment style is thought to influence health behaviors and the provider-patient relationship [26,27]. Among cancer patients, those with fearful attachment have been shown to report higher distress, symptom burden, and reduced wellbeing, but less likely to engage in healthcare [28]. Providers may be reluctant to initiate discussions with patients who appear anxious or fearful; however, our research supports that, as a group, it is these very patients who appear to most welcome discussions about ACP. Results should be interpreted with caution given the small to medium effect sizes; however, we can more confidently suggest that ACP discussions should not specifically be avoided with these patients. Furthermore, studies in non-cardiac populations show that individuals who have communicated their end-of-life preferences to providers report reduced levels of anxiety [29]. It would be interesting to investigate whether anxiety symptoms change in ACHD patients following ACP discussions. It is also important to emphasize that patient-reported interest in discussing ACP and/or life expectancy might not translate into
patients initiating these discussions or engaging in them with less distress. This highlights the importance of inviting discussions regarding ACP with all patients. Prior to initiating ACP conversations, however, clinicians should first elicit the patient's goals and preferences for information and decision-making to avoid contributing to an overwhelming experience [25] 4.4. Study Limitations The appropriateness of generalizing these findings to patients followed at other ACHD centers, as well as patients at our own center who declined study participation, is unknown. We note, however, that our results were consistent with those from a Canadian study that similarly revealed high interest in but low occurrence of ACP discussions. Participants were recruited from an outpatient clinic at one time point and thus we do not know how their responses might change over time. In addition, ACP experiences were elicited via self-report measures and may be subject to recall bias [8]. 5. Conclusions As a group, ACHD patients value the opportunity for ACP discussions and prefer early communication with their providers. Although some clinicians might avoid ACP discussions in patients who are generally more anxious or have less complex CHD, our results suggested that such avoidance is not warranted. Clinicians should strive to normalize ACP discussions and encourage timely completion of advance directives with all patients with the goal of optimizing the quality of life of patients at all phases during the course of their illness. Conflicts of interest None. References [1] P. Moons, L. Bovijn, W. Budts, A. Belmans, M. Gewillig, Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium, Circulation 122 (22) (2010) 2264–2272. [2] S.M. Gilboa, O.J. Devine, J.E. Kucik, M.E. Oster, T. Riehle-Colarusso, W.N. Nembhard, P. Xu, A. Correa, K. Jenkins, A.J. Marelli, Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010, Circulation 134 (2) (2016) 101–109. [3] C.L. Verheugt, C.S.P.M. Uiterwaal, E.T. Van Der Velde, et al., Mortality in adult congenital heart disease, Eur. Heart J. 31 (10) (2010) (1220-122). [4] P. Khairy, R. Ionescu-Ittu, A.S. Mackie, M. Abrahamowicz, L. Pilote, A.J. Marelli, Changing mortality in congenital heart disease, J. Am. Coll. Cardiol. 56 (2010) 1149–1157. [5] G.P. Diller, A. Kempny, R. Alonso-Gonzalez, et al., Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre, Circulation 132 (22) (2015) 2118–2125. [6] M. Greutmann, D. Tobler, A.H. Kovacs, et al., Increasing mortality burden among adults with complex congenital heart disease, Congenit. Heart Dis. 10 (2) (2015) 117–127. [7] C.A. Warnes, R.G. Williams, T.M. Bashore, et al., ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease), Circulation 118 (2008) 2395–2451. [8] D. Tobler, M. Greutmann, J.M. Colman, M. Greutmann-Yantiri, S.L. Librach, A.H. Kovacs, Knowledge of and preference for advance care planning by adults with congenital heart disease, Am. J. Cardiol. 109 (12) (2012) 1797–1800. [9] D. Tobler, M. Greutmann, J.M. Colman, M. Greutmann-Yantiri, L.S. Librach, A.H. Kovacs, End-of-life in adults with congenital heart disease: a call for early communication, Int. J. Cardiol. 155 (3) (22 2012) 383–387. [10] M.E. Lyon, M.A. McCabe, K.M. Patel, L.J. D'Angelo, What do adolescents want? An exploratory study regarding end-of-life decision-making, J. Adolesc. Health 35 (2004) e1–e6 (suppl). [11] A.S. Zigmond, R.P. Snaith, The hospital anxiety and depression scale, Acta Psychiatr. Scand. 67 (1983) 361–370. [12] I. Bjelland, A.A. Dahl, T.T. Haug, D. Neckelmann, The validity of the Hospital Anxiety and Depression Scale: an updated literature review, J. Psychosom. Res. 52 (2002) 69–77. [13] K. Bartholomew, L.M. Horowitz, Attachment styles among young adults: a test of a four-category model, J. Pers. Soc. Psychol. 61 (1991) 226–244.
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Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Advance Care Planning in Adults with Congenital Heart Disease: A Patient Priority☆ Lisa X. Deng a,1, Lacey P. Gleason a,1, Abigail M. Khan b,1, David Drajpuch a,1, Stephanie Fuller a,1, Leah A. Goldberg a,1, Christopher E. Mascio a,1, Sara L. Partington a,1, Lynda Tobin a,1, Yuli Y. Kim a,1, Adrienne H. Kovacs b,⁎,1 a b
Philadelphia Adult Congenital Heart Center, joint program of Hospital of the University of Pennsylvania and The Children's Hospital of Philadelphia, Philadelphia, PA, USA Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, USA
a r t i c l e
i n f o
Article history: Received 25 September 2016 Received in revised form 19 December 2016 Accepted 31 December 2016 Available online 4 January 2017 Keywords: Adult congenital heart disease Advance care planning Anxiety
a b s t r a c t Background: Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs. Methods: This study investigated ACHD patient preferences for ACP and factors that impact preferences. ACHD patients completed an ACP preferences questionnaire, the Hospital Anxiety and Depression Scale and a measure of attachment styles. Results: Of 152 ACHD patients (median age 33 years, 50% female), 13% reported previous ACP discussions with providers and 21% had completed advance directives. On a 0–10 scale, the median rating for the importance of discussing ACP with providers was 7; 18 years was identified as the most appropriate age to initiate this dialogue. Higher ratings for the importance of discussing ACP with providers was observed in patients who were female (p = 0.03), had lower disease complexity (p = 0.03), and had elevated anxiety symptoms (p = 0.001); elevated anxiety remained significant in a multivariable model. Interest in receiving information about life expectancy (61% overall) was greater among patients with lower disease complexity (p = 0.04) and a history of ≥2 cardiac surgeries (p = 0.01); disease complexity remained significant in a multivariable model. Conclusions: As a group, ACHD patients value the opportunity for ACP discussions and prefer earlier communication. Although some clinicians might avoid ACP discussions in patients who are generally more anxious or have less complex CHD, such avoidance does not appear to be warranted. © 2017 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Almost 90% of children born with congenital heart disease (CHD) will reach adulthood and adults now comprise almost 60% of the American CHD population [1,2]. However, adults with moderate or complex CHD continue to have substantial morbidity and mortality and the annual number of deaths within this group is projected to markedly increase [3,4,5,6]. As mortality has largely shifted to the
☆ This research was supported by a grant from Big Hearts to Little Hearts. ⁎ Corresponding author at: Knight Cardiovascular Institute, Oregon Health & Science University, 3181 Sam Jackson Park Rd, UHN-62, Portland, OR, 97239, United States. E-mail address: [email protected] (A.H. Kovacs). 1 This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.
http://dx.doi.org/10.1016/j.ijcard.2016.12.185 0167-5273/© 2017 Elsevier Ireland Ltd. All rights reserved.
adult setting, American guidelines for the management of adult CHD (ACHD) patients recommend that “all ACHD patients should be encouraged to complete an advance directive, ideally at a time during which they are not extremely ill or hospitalized” [7]. Advance care planning (ACP) is the process of planning for future medical care and typically includes the completion of advance directives and the appointment of a health care power of attorney. Canadian research documented that three-quarters of ACHD patients wanted to discuss end-of-life planning with health care providers, although only 1% recalled such discussions and only 5% had completed advance directives [8,9]. It is unknown whether Canadian findings are generalizable to an American setting. The objectives of this study were to (1) describe the ACP experiences and preferences in a sample of American ACHD patients, and (2) investigate whether patient preferences differ as a function of sociodemographic, medical or psychosocial factors.
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2. Methods 2.1. Study population This study was approved by our institutional ethics review board and written informed consent was obtained from all participants. Patients were consecutively enrolled at the outpatient clinic of an American northeastern tertiary ACHD center and inclusion criteria included age ≥ 18 years, documented history of CHD, and cognitive and language abilities to complete study questionnaires in English. 2.2. Study measures Patients completed a questionnaire focused on ACP and life expectancy that included questions used previously [9,10] and others written by our study team. ACP was defined as “the process of thinking about your preferences for future health care (including the kinds of treatment you would and would not want) and letting other people know your preferences should you get very sick and become incapable of speaking for yourself.” Patients were asked whether (and at what age) they had discussed ACP with a health care provider. Patients were also asked with whom, if anyone, they had talked about wishes for end-oflife care. In addition, patients were asked whether they had completed an advance directive or had identified a healthcare power of attorney who would make decisions about their health care should they be unable to do so for themselves. Patients were then asked the following questions: (1) On a scale of 0–10 (with 0 being least important and 10 being most important), how important is it to you to discuss advance care planning with your health care provider? (2) When do you think is the right age for a health care provider to start discussing advance care planning with a patient with congenital heart disease? (3) What is the best time to bring up ACP discussion? Response options to this third question included (a) Before getting sick, while healthy, (b) When first diagnosed with a life-threatening illness or complication from heart disease, (c) When first symptomatic with a life-threatening illness or complication from heart disease, (d) when first hospitalized with a life-threatening illness or complication from heart disease, and (e) If dying [9,10]. Next, patients were asked (yes or no) whether they wanted information about the estimated life expectancy of patients with their heart condition. They were also asked to rate how likely they were to have a shorter life expectancy compared to healthy people without CHD. Response options were: (a) a lot less likely, (b) slightly less likely, (c) about the same, (d) slightly more likely, and (e) a lot more likely. The Hospital Anxiety and Depression Scale (HADS), a 14-item measure, was used to measure symptoms of anxiety and depression [11]. Subscale scores for anxiety (HADS-A) and depression (HADS-D) range from 0 to 21 with scores ≥8 indicating clinically elevated symptoms of anxiety or depression [12]. Attachment style refers to the security of an individual's interpersonal relationships and was assessed using the Relationship Questionnaire, which is a self-report measure based on a 4-category model of attachment: secure, preoccupied, dismissing, and fearful [13]. People with a secure attachment style are comfortable with both intimacy and independence. Individuals with a preoccupied style are characterized as emotionally dependent on others. People with dismissing style are extremely self-reliant, and fearful individuals simultaneously desire and are afraid of emotional intimacy. Medical records were reviewed and CHD was categorized as being of simple, moderate, or great complexity [7]. Study data were collected and managed using REDCap (Research Electronic Data Capture), a secure web-based data capture application [14]. 2.3. Statistical analyses Data are summarized as medians with ranges or percentages as appropriate. With a univariate approach, we first evaluated whether
patient-reported importance of discussing ACP and interest in information about life expectancy varied as a function of sociodemographic factors, disease complexity, clinically elevated anxiety, clinically elevated depression, and attachment style. Correlation analyses, Chi-square tests and Mann Whitney tests were performed as appropriate. Effect sizes (eta-squared for importance of discussing ACP and phi and Cramér's V for interest in life expectancy) were also computed. Multivariable models were then performed to predict importance of discussing ACP and interest in information about life expectancy using variables selected on an a priori basis: age, sex, CHD complexity, HADS-A, HADS-D, and attachment style [15]. In multivariable models, HADS-A and HADS-D scores were entered as continuous variables and attachment style was dichotomized into secure vs. not secure. Missing values were excluded from analyses and given the exploratory nature of this research, significance was defined as p ≤ 0.05 for all analyses. Analyses were conducted using SPSS (Version 22.0. Armonk, NY: IBM Corp). 3. Results 3.1. Participants Of 321 patients invited to participate in the study, 165 consented, 26 declined, and 130 were unable to participate due to timing or scheduling issues. A total of 13 patients were excluded from the final analysis, thus generating a final study sample of 152. Table 1 summarizes the demographic and clinical characteristics of the study sample. The most common defects were tetralogy of Fallot (n = 29; 19%), d-transposition of the great arteries (n = 18; 12%), bicuspid aortic valve disease (n = 13; 9%), and coarctation of the aorta (n = 13; 9%). The median (range) HADS-A score was 7 (0–19), and the median (range) HADS-D score was 2 (0–14). The proportion of patients with elevated symptoms of depression, elevated symptoms of anxiety, and a secure attachment style did not differ between the three categories of defect complexity. 3.2. Advance care planning: Recalled discussions and communication preferences Nineteen patients (13%) recalled previous ACP discussions with their health care provider. The median (range) age of the first discussion occurred at 25 years (16–50). Most patients (n = 106, 70%) had discussed end-of-life wishes with other people, most commonly their spouse/partner (n = 61, 40%) and friends and family (n = 46, 51%). Thirty-two patients (21%) had completed advance directives and 28 (18%) had a healthcare power of attorney. Table 1 Demographic and clinical characteristics of study sample (N = 152). n (%) Age in years (mean & range) Female White Partnered College education or above Disease complexity Simple Moderate Great Total number of cardiac surgeries Elevated anxiety symptoms (HADS-A ≥ 8) Elevated depressive symptoms (HADS-A ≥ 8) Attachment Style Secure Dismissing Fearful Preoccupied Data are counts (percentages) or medians (range).
33 (19–67) 76 (50) 126 (83) 60 (40) 117 (77) 25 (16) 74 (49) 53 (35) 2 (0–8) 67 (46) 15 (11) 82 (57) 30 (21) 17 (12) 15 (10)
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On a scale of 0 to 10 regarding the importance of discussing ACP with health care providers, the median (range) score was 7 (0–10) and 83% (120/145) of patients reported a rating of 5 or higher (Fig. 1). Patients preferred providers to initiate the discussion at a median (range) age of 18 years (5–60). Most patients (n = 118; 78%) preferred ACP discussions to be initiated while healthy, before being diagnosed or hospitalized for a life threatening illness. Only 2 patients (1%) preferred to defer ACP discussions until they were dying. There were no significant correlations between age or education and importance of ACP discussions. As shown in Table 2, higher ratings for the importance of discussing ACP with providers were observed in patients who were female (p = 0.03), had lower disease complexity (p = 0.03), and had elevated anxiety symptoms (p = 0.001). Effect sizes were categorized as small for sex (eta-squared =0.025) and disease complexity (eta-squared =0.044) and medium for HADS-A score (eta-squared = 0.068). Although there was no statistically significant difference for relationship style, a small effect size (0.026) was revealed. In a multivariable model, the only factor associated with higher ratings was a higher HADS-A score (Beta =0.26; 95% confidence interval [CI] 0.03–0.30; p = 0.02). Though CHD complexity was associated with higher ratings for the importance of discussing ACP in the univariate analysis, it did not reach statistical significance on multivariable analyses (Beta = −0.16; CI -1.27–0.33; p = 0.06). 3.3. Patient preferences for information about life expectancy Ninety-three patients (61%) reported that they wanted information about the estimated life expectancy of patients with their heart condition; 47 (31%) did not want this information and 12 (8%) were unsure. As presented in Table 2, desire for information about life expectancy was greater among patients with lower disease complexity (p = 0.04) and a history of ≥ 2 cardiac surgeries (p = 0.01). Relationship style approached significance (p = 0.054). Education was not associated with interest in life expectancy information (less than college = 64% vs. college or above =67%, p = 0.76). Effect sizes were categorized as small for both disease complexity (phi =0.217) and number of cardiac surgeries (phi =0.207). Although a preference for discussing life expectancy did not differ significantly by HADS-A or relationship style, small effect sizes (0.108 and 0.239) were revealed. In the multivariable model, CHD complexity was the only factor associated with desire for information about life expectancy (odds ratio [OR] OR 0.55; 95% CI 0.31–0.99; p = 0.05). The following responses were elicited regarding how likely patients believed that they would have a shorter life expectancy than their peers without CHD: a lot less likely (n = 15; 10%), slightly less likely (n = 8;
50 45 40 35 30 25 20 15 10 5 0
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Table 2 Univariate analyses of factors associated with ACP preferences.
Sex Female Male Race White Non-white Education Less than college College or above Disease complexity Simple Moderate Great Number of cardiac surgeries b2 ≥2 HADS-A score b8 ≥8 HADS-D score b8 ≥8 Relationship style Secure Fearful Preoccupied Dismissing
Importance of discussing with provider (0–10 scale)
Want information about life expectancy
Median
P - value
n (%)
P - value
8 7
0.027
49 (71) 44 (62)
0.257
7 8
0.488
75 (65) 18 (72)
0.515
7 7
0.644
14 (64) 79 (67)
0.763
8 7 6
0.029
19 (86) 48 (68) 26 (55)
0.038
7 7
0.362
54 (76) 39 (57)
0.014
6 8
0.001
46 (61) 43 (72)
0.208
7 8
0.578
79 (67) 9 (60)
0.592
0.237
47 (61) 14 (82) 5 (42) 21 (78)
0.054
7 9 7 7
HADS-D, Hospital Anxiety and Depression Scale Depression subscale; HADS-A, Hospital Anxiety and Depression Scale Anxiety subscale.
6%), about the same (n = 28; 19%), slightly more likely (n = 58; 40%), a lot more likely (n = 37; 25%). Collapsed to create a dichotomous variable, 65% believed they were more likely to have a shorter life expectancy and this did not differ between patients according to depression and anxiety scores or categories of attachment style. Further, the percentage of patients who thought they had a shorter life expectancy did not differ by CHD complexity (simple = 63%; moderate = 65%; great =66%; p = 0.96) or education level (less than college =56%; college or above =67%, p = 0.30). 4. Discussion It was important to investigate whether American outcomes replicated Canadian results, given differences in the two health systems [16]. We observed that (i) ACP discussions rarely occur, (ii) as a group, patients with greater defect complexity deem ACP discussions to be of lower importance and are less likely to want information about life expectancy, and (iii) anxiety was positively correlated with greater interest in ACP discussions and life expectancy. 4.1. Low occurrence of ACP discussions despite high interest
<5
5-7 Patient Score
8 - 10
Fig. 1. Patient-reported importance of discussing ACP with health care providers on a 0 (least important) to 10 (most important) scale.
Approximately 1 in 10 patients recalled prior discussions with their providers and 1 in 5 had completed an advance directive or had a healthcare power of attorney. These findings are higher than the Canadian cohort [9] but nonetheless far below published recommendations [7]. Patients also recognized the importance of initiating ACP discussions prior to the diagnosis of a life-threatening illness [9]. The timing is critical because previous research has documented that many ACHD patients who die in the hospital experience aggressive life-sustaining measures without having discussed their preferences [17]. Known barriers include provider concerns about patient readiness for end-of-life discussions and challenges with prognostication [18]. The minority of patients in this study who discussed ACP with their
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providers did so several years later than they identified as the ideal age. Indeed, providers are known to be hesitant to initiate ACP discussions with younger patients [19,20]. It is of interest that the preferred age for the initiation of ACP planning is 18 years, which is the time when patients often transition from pediatric to adult health care. Discussion of long-term health expectations and the preparation of ACP documents have been recommended for transitioning adolescents and young adults with CHD [21,22] and raises the question of the optimal setting in which discussions should initially occur. Providers are encouraged to optimize skills in initiating and revisiting patient discussions regarding ACP and end-of-life care in order to provide the highest quality of care throughout patients' lifespans. Specific recommendations include normalizing these discussions, acknowledging prognostic uncertainty, and scheduling specific clinic visits [23]. 4.2. Lower interest in ACP among patients with more complex disease Although patients with more complex disease have the lowest life expectancy, we found that patients with complex CHD rated ACP discussions with health care providers as less important and were less likely to want information about the estimated life expectancy of patients with their heart condition. Further, only two-thirds of patients with CHD of great complexity thought they had a shorter life expectancy than their peers. As a group, patients with CHD are known to overestimate their life expectancy [24]. One hypothesis is that adults with CHD of greater complexity might exhibit avoidance toward discussions of ACP and/or life expectancy. A known patient-identified barrier to ACP discussion is patients' desire to focus on staying alive rather than talking about death [18]. A previous study of patients with complex CHD also revealed that denial might be used to help patients adapt [25]. Qualitative research is recommended to investigate why patients with complex CHD appear more reluctant to discuss ACP and life expectancy. A previous study observed that ACHD providers tend to raise end-oflife issues more often with patients of great rather than simple disease complexity [9]. Our findings suggest that clinicians ought to invite (although not impose) discussions of ACP and life expectancy with all patients, irrespective of disease complexity. It has been suggested that there is a subgroup of patients with CHD of simple complexity who anticipate a reduced life expectancy and for whom discussions of near-normal life expectancies may alleviate their concerns [18]. 4.3. Psychological correlates of ACP preferences Univariate analyses revealed that patients with elevated anxiety symptoms rated ACP discussions as more important and there was a small effect size for desire for information about life expectancy. There were also small effect sizes for patients with a fearful attachment style to report higher interest in ACP discussions and be more likely to want information about life expectancy. Attachment style is thought to influence health behaviors and the provider-patient relationship [26,27]. Among cancer patients, those with fearful attachment have been shown to report higher distress, symptom burden, and reduced wellbeing, but less likely to engage in healthcare [28]. Providers may be reluctant to initiate discussions with patients who appear anxious or fearful; however, our research supports that, as a group, it is these very patients who appear to most welcome discussions about ACP. Results should be interpreted with caution given the small to medium effect sizes; however, we can more confidently suggest that ACP discussions should not specifically be avoided with these patients. Furthermore, studies in non-cardiac populations show that individuals who have communicated their end-of-life preferences to providers report reduced levels of anxiety [29]. It would be interesting to investigate whether anxiety symptoms change in ACHD patients following ACP discussions. It is also important to emphasize that patient-reported interest in discussing ACP and/or life expectancy might not translate into
patients initiating these discussions or engaging in them with less distress. This highlights the importance of inviting discussions regarding ACP with all patients. Prior to initiating ACP conversations, however, clinicians should first elicit the patient's goals and preferences for information and decision-making to avoid contributing to an overwhelming experience [25] 4.4. Study Limitations The appropriateness of generalizing these findings to patients followed at other ACHD centers, as well as patients at our own center who declined study participation, is unknown. We note, however, that our results were consistent with those from a Canadian study that similarly revealed high interest in but low occurrence of ACP discussions. Participants were recruited from an outpatient clinic at one time point and thus we do not know how their responses might change over time. In addition, ACP experiences were elicited via self-report measures and may be subject to recall bias [8]. 5. Conclusions As a group, ACHD patients value the opportunity for ACP discussions and prefer early communication with their providers. Although some clinicians might avoid ACP discussions in patients who are generally more anxious or have less complex CHD, our results suggested that such avoidance is not warranted. Clinicians should strive to normalize ACP discussions and encourage timely completion of advance directives with all patients with the goal of optimizing the quality of life of patients at all phases during the course of their illness. Conflicts of interest None. References [1] P. Moons, L. Bovijn, W. Budts, A. Belmans, M. Gewillig, Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium, Circulation 122 (22) (2010) 2264–2272. [2] S.M. Gilboa, O.J. Devine, J.E. Kucik, M.E. Oster, T. Riehle-Colarusso, W.N. Nembhard, P. Xu, A. Correa, K. Jenkins, A.J. Marelli, Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010, Circulation 134 (2) (2016) 101–109. [3] C.L. Verheugt, C.S.P.M. Uiterwaal, E.T. Van Der Velde, et al., Mortality in adult congenital heart disease, Eur. Heart J. 31 (10) (2010) (1220-122). [4] P. Khairy, R. Ionescu-Ittu, A.S. Mackie, M. Abrahamowicz, L. Pilote, A.J. Marelli, Changing mortality in congenital heart disease, J. Am. Coll. Cardiol. 56 (2010) 1149–1157. [5] G.P. Diller, A. Kempny, R. Alonso-Gonzalez, et al., Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre, Circulation 132 (22) (2015) 2118–2125. [6] M. Greutmann, D. Tobler, A.H. Kovacs, et al., Increasing mortality burden among adults with complex congenital heart disease, Congenit. Heart Dis. 10 (2) (2015) 117–127. [7] C.A. Warnes, R.G. Williams, T.M. Bashore, et al., ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease), Circulation 118 (2008) 2395–2451. [8] D. Tobler, M. Greutmann, J.M. Colman, M. Greutmann-Yantiri, S.L. Librach, A.H. Kovacs, Knowledge of and preference for advance care planning by adults with congenital heart disease, Am. J. Cardiol. 109 (12) (2012) 1797–1800. [9] D. Tobler, M. Greutmann, J.M. Colman, M. Greutmann-Yantiri, L.S. Librach, A.H. Kovacs, End-of-life in adults with congenital heart disease: a call for early communication, Int. J. Cardiol. 155 (3) (22 2012) 383–387. [10] M.E. Lyon, M.A. McCabe, K.M. Patel, L.J. D'Angelo, What do adolescents want? An exploratory study regarding end-of-life decision-making, J. Adolesc. Health 35 (2004) e1–e6 (suppl). [11] A.S. Zigmond, R.P. Snaith, The hospital anxiety and depression scale, Acta Psychiatr. Scand. 67 (1983) 361–370. [12] I. Bjelland, A.A. Dahl, T.T. Haug, D. Neckelmann, The validity of the Hospital Anxiety and Depression Scale: an updated literature review, J. Psychosom. Res. 52 (2002) 69–77. [13] K. Bartholomew, L.M. Horowitz, Attachment styles among young adults: a test of a four-category model, J. Pers. Soc. Psychol. 61 (1991) 226–244.
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