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Health-Related Quality of Life in Adult Survivors after the Fontan Operation Manisha Kukreja MBBS, MPH, Ayesha S. Bryant MSPH, MD, David C. Cleveland MD, MBA, Robert Dabal MD, Neha Hingorani BS, James K. Kirklin MD
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Cite this article as: Manisha Kukreja MBBS, MPH, Ayesha S. Bryant MSPH, MD, David C. Cleveland MD, MBA, Robert Dabal MD, Neha Hingorani BS, James K. Kirklin MD, Health-Related Quality of Life in Adult Survivors after the Fontan Operation, Semin Thoracic Surg, http://dx.doi.org/10.1053/j.semtcvs.2015.08.007 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
HEALTH-RELATED QUALITY OF LIFE IN ADULT SURVIVORS AFTER THE FONTAN OPERATION
Manisha Kukreja, MBBS, MPH1 Ayesha S. Bryant, MSPH, MD1 David C. Cleveland, MD, MBA1 Robert Dabal, MD1 Neha Hingorani, BS 1 James K. Kirklin, MD1 University of Alabama at Birmingham, Alabama Department of Surgery, Division of Cardiothoracic Surgery Birmingham, AL 352941
There is no funding, no COI, and no potential conflicts for either of the authors. From the Department of Surgery, Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, AL Disclosures: Authors have nothing to disclose with regard to commercial support. Address for reprints: James K. Kirklin, MD, University of Alabama, Professor and Director, Division of Cardiothoracic Surgery, 760 THT, 1900 University Boulevard, Birmingham, AL 35294
[email protected] 205 934 5486 4009 Words Key words: Health-Related Quality of Life; Fontan procedures; Single-ventricle conditions.
Glossary of Abbreviations
HRQOL – Health related quality of life HT – Heart Transplant SF-36 – Short Form Survey
1
Abstract Objective: The primary objective of this study was to ascertain the long-term health-related quality of life (HRQOL) of adult patients who underwent a childhood Fontan operation for palliation of uni-ventricular cardiac anomalies.
The secondary objective was to compare the long-term
HRQOL of Fontan survivors to that of pediatric heart transplant recipients. Methods: This cross-sectional study examined adult survivors (>19 years) who underwent a Fontan operation during childhood (Fontan group) or a pediatric heart transplant (HT group) between 1988 and 2011 (23 year span). HRQOL was assessed using the Short Form-36 (SF-36) survey. The study group consisted of 49 Fontan group patients and 13 HT group patients who responded to the survey. Results: HRQOL Scores of the Fontan group were similar to an age-controlled healthy US population in social and mental functioning, energy/vitality, and overall mental component score (p ≥ 0.2). However, Fontan scores in physical functioning, bodily pain, general health, and overall by physical component were significantly below the age – controlled US population (p < 0.05). No differences were identified between Fontan and heart transplant patients. Conclusions: This favorable life-satisfaction period (average 18 years) should be considered when informing patients and families of expectations with the Fontan pathway versus certain higher risk procedures.
2
Central Message The long-term health-related quality of life in adult patients who underwent a childhood Fontan operation for single ventricle cardiac anomalies was similar to an age-controlled general population in mental components but somewhat less in physical component categories, with no differences from pediatric heart transplant recipients.
Introduction Several modifications of the Fontan operation coupled with medical and surgical advances in management during and after surgery have improved the surgical outcomes during the past two decades.1,2 Further it has been demonstrated that the current standardized Fontan operation offers excellent long-term survival, freedom from reoperations, good functional status, and absence of late increasing mortality.3,4 The short form 36 (SF-36) survey is a validated tool commonly used to assess four major areas of a person’s daily functioning, also known as health-related quality of life (HRQOL): physical health, emotional/mental
state, social functioning, and general health, often in
heterogeneous populations.5,6 The primary objective of this study was to ascertain the long-term HRQOL of adult patients (≥19 years) who underwent a childhood Fontan operation (Fontan group) for palliation of univentricular congenital cardiac anomalies at the University of Alabama at Birmingham (UAB). The secondary objectives were to compare the long term HRQOL of the Fontan group to that of heart transplant recipients (HT group) and also to a healthy comparison group derived from the general population.
3
Methods Patient population Eligibility criteria for the study were: patients who underwent either a childhood Fontan operation for a single ventricle congenital anomaly or a pediatric heart transplant between 1988 and 2011 at UAB, alive at the time of this study, and 19 years or older. The overall Fontan population included 207 patients who underwent the Fontan operation between 1988 and December 31, 2011. Eighty of these patients would potentially reach age 19 during 2014 or before. Twenty of these 80 patients died before 2014. Of the remaining 60 patients who received a QOL survey, the 49 respondents (82% response rate) formed the Study Group. SF-36 Quality of Life Instrument Health-related quality of life was assessed using the standard short form SF-36 (SF-36 V2) health survey. The SF-36 consists of 36 questions that measure eight domains of health: physical functioning (PF), role limitations due to physical health (RP), role limitations caused by emotional health problems (RE), social functioning (SF), bodily pain (BP), mental health (MH), vitality (VT), and general health (GH). It provides scores for each of these eight health domains, as well as two summary scores: the physical component summary (PCS) and the mental health component summary (MCS).
Additional definitions of these components are provided in
Appendix A. The HRQOL survey (SF-36) was used to assess the mental and physical component scores of these patients. The scores of the Fontan group were compared with the HRQOL scores of healthy patients (normative data derived from the general U.S. population), and also with scores of the HT group.7,8 For each domain, the scores range from 0 to 100, where a higher score indicates a better health state. This instrument is not designed to give an overall composite score. The SF-36 has 4
acceptable internal consistency, validity and test-retest reliability.9,10 The national normalized value (as validated by Quality Metric Inc, Lincoln, RI) is 50.0 with a standard deviation (SD) of 10.
Norms are available for the healthy general U.S. population as a whole and also for seven
age groups, male and females individually as well as combined as reported by the National Survey of Functional Health Status. 7,8
Overall, the SF-36 is a validated generic survey that allows assessment of health concepts that are not age, disease, or treatment specific. Therefore, it has been useful in assessing the health of general as well as specific populations, comparing the relative burden of diseases, differentiating the health benefits produced by different treatments, and screening individual patients. Study Protocol Patients were mailed a questionnaire SF-36 v2 health survey (standard short form SF-36) in May 2014. Patients were asked to return their survey within eight weeks. The study closing date was July 15th, 2014. This study was reviewed and approved by the UAB Institutional Review Board and all the participants provided informed consents. Statistical methods The QOL scores of each of the eight domains and the overall PCS and MCS were assessed. These data are presented as mean with standard deviation (±SD) and compared by independent t tests. A t-test was performed to assess the level of significance between the difference in means of sample population (Fontan group or HT group) and general population (normative data). We considered statistically significant differences with a p-value of ˂0.05. Cumulative frequency distributions were used to examine the response pattern of the overall sample populations. 5
The scores of each domain for the SF-36 v2 assessment were calculated using Health Outcomes Scoring software 4.5 “OptumInsight Smart Measurement System (SMS) Scoring Solution” (Quality Metric Incorporated, Lincoln, RI).11,12,13
This software uses an algorithm to
convert raw scores to standardized scores to allow comparison.
All statistics were performed
using SAS software v. 9.3 (SAS Institute, Cary, NC) in conjunction with custom software used for hazard analysis.14
Results Description of cohort Among the Fontan group, of the 60 surveys sent to eligible patients, 49 (82%) were completed and returned. Of the surveys not returned: one patient was in a mental rehabilitation center and unable to answer the survey questions, two were foreign patients residing overseas, four questionnaires were not returned, and four patients were lost to follow up. Among the Fontan group, the participants ranged in age from 19–55 years, with a median age of 25 years, a mean of 26 years; and 25 (52%) were male.
Mean follow-up for the Fontan group was 18 years
(range 3 to 24 years), of which 44 (90%) had greater than 10 years of follow-up All patients in the Fontan group received a PTFE tube connecting blood flow from the inferior vena cava to the pulmonary artery (“modern Fontan operation”). Demographics, anatomic features, and surgical details were compared between responders and non-responders to the QOL survey (Tables 1 and 2). Surgical and age-related characteristics were similar between groups. Among anatomic details, a diagnosis of tricuspid atresia was more common among non-responders. Among the heart transplant group, 14 questionnaires were sent to eligible patients and response rate was 93%. One patient did not respond due to personal reasons. Participants ranged in age from 19–32 years, with a median age of 22 years; and there were 7 (54%) women. For 6
the heart transplant group, mean follow up was 13 years (range 3 to 23 years). Thirty per cent of the transplant patients carried a diagnosis of congenital heart disease, and the remainder had cardiomyopathy. (Table 3). Comparison of scores among populations Because the distribution of ages in the Fontan (92% of patients between ages 19 and 34 years) and transplant patients (100% of patients between ages 19 and 32 years) did not follow the distribution of the normal population, the overall physical and mental component scores were subdivided by age groups (Figure 1). Since the normalized data for individual age groups was available for comparison, we used the normalized population for the age group 25 to 34 years as our age-controlled healthy U.S. population group for comparison. The HRQOL scores of the Fontan group with regard to social functioning, mental health, role-emotional, energy/vitally, as well as the overall mental component score were comparable to that of an age-controlled healthy US population (Table 4). However, the Fontan group reported a significantly diminished physical functioning, role-physical, freedom from bodily pain, general health perception, and overall physical component score compared to the healthy US population. A sub-analysis of the Fontan group by gender showed that among males, bodily pains, physical functioning, and role physical were significantly higher compared to the age-matched general US population (p ≤0.05) and females had significantly reduced general health perception (see again Appendix 1 for clarification) compared to the age-controlled general US population (p=0.02). The HRQOL scores of the HT group were not significantly different from the general age-controlled population in any domain except for social functioning, in which the HT group
7
exceeded the general population (Table 4). There were no significant differences in any of the eight subjective measures of HRQOL between the Fontan group and HT recipients group. Distribution of Scores within the Patient Population Comparing the Fontan population to the age-controlled general population in the Overall Physical Component Scores (Figure 2), 44% of the Fontan group scored with the lowest 25% of the general population, while only 10% of Fontan scores fell within the upper 25% of the population. Similarly, in the General Health assessment (Figure 3), 46% of Fontan patients scored with lowest 25% of the general population, while 18% matched with the upper 25% of the general population. The Fontan group fared better in the Overall Mental Component Scores (Figure 4), with 30% of Fontan patients scoring with the lowest 25% of the general age-matched population, while nearly 40% scored with the upper 25% of the general population.
Discussion Survival and HRQOL Over the past two decades, mortality attributed to univentricular heart repair has been reduced, and many patients now reach adulthood.
A recent publication by Dabal, et al,
demonstrated ongoing improvement in survival over time in the current era of pediatric cardiac surgery patients after Fontan surgery.4 The 1-, 10-, and 20- year survival with the use of a PTFE tube was 95%, 88%, and 77%, respectively. No late increasing phase of risk was identified out to 20 years. Most surviving patients were in NYHA class I or II at follow-up, even beyond 12 years. Less than 10% of patients were either in NYHA class III or unknown. However, many of these patients endure repeated hospital admissions, and some have repeat surgical procedures. The psychological impact of these experiences is particularly notable in adolescents and young adults. In addition, patients are often aware of the potential reduction in their life expectancy and
8
physical capabilities. Several studies have examined the intellectual, social, and emotional development and adjustment of children and adolescents with congenital heart disease, as well as mother–child relations and parental stress,15-20 with conflicting conclusions. Several investigators have reported negative psychosocial outcomes in these patients—for example, diminished self esteem, anxiety, depression, and poor emotional or social adjustment;21-24
while others have
reported more optimistic outcomes and a normal quality of life in selected subsets of congenital heart disease.25,26 Our findings demonstrate that patients after Fontan operations have similar long-term social/mental/emotional HRQOL outcomes compared to the healthy US general population and no significant difference from heart transplant recipients for nearly 2 decades.
However,
physical functioning and general health perception lag somewhat behind that of the normal population. Two previous studies in Fontan survivors support the findings of satisfactory QOL, which contradicts previous assumptions, parental proxy reports, or self-reports of decreased QOL.27-30 Some studies on physical functioning, general health, and mental health outcome in adults after Fontan operations have reported problems in these domains, whereas others show normal social behavior and comparable QOL with that of their healthy counterparts, thus supporting the generally favorable outcomes of our present study.31,32 To improve patients’ overall long term quality of life, physical activity must be encouraged and patients motivated to perform daily activities, avoiding excessive restrictions on themselves. Over time, most patients appear to adapt to their disease and maintain a positive outlook in day to day life. Impact of HRQOL on the Overall Calculus of “Outcomes” Despite the extensive validation of quality of life instruments, the very nature of this subject underscores its inherent subjectivity. What cannot be denied is the importance of how 9
the patient “feels” about the impact of alternative or competing therapies on his/her happiness and ability to function. This component of the “outcomes equation” becomes increasingly important as survival differences between therapies narrow.
Ultimately, a truly informed
decision between therapies such as transplantation or continued acceptance of a slowly failing Fontan; selection of a complex, imperfect 2- ventricular reconstruction vs. the known outcomes following the Fontan procedure; and many other therapeutic dilemmas will incorporate components of survival, likelihood and frequency of adverse events, probability of re-operations, and health-related quality of life. As we strive to refine this process, the focus to quantify these components gains importance in order to effectively explain what a patient and his/her family may “expect” with a given therapeutic choice. This study addresses one component of that “grand equation”, namely health-related quality of life. Our analysis is challenged by the limitations stated below, but the pursuit of such health-related quality of life analyses will increasingly become an important part of the longterm evaluation of many complex cardiac operations when alternative surgical or medical strategies are available.
Limitations of the study The primary limitation of this study was the cross sectional study design, which depends on patients self-reporting/evaluating and returning the questionnaires. We had only crosssectional data to analyze, we did not administer the survey both pre- and postoperatively, and thus we were unable to evaluate changes from baseline values and could not explore the temporal trends in health perceptions. Another limitation is our reliance on the use of published normative data rather than simultaneously studying a cohort of normal control subjects. Furthermore, the possibility than non-responders may have a different perception of their QOL
10
cannot be excluded. The number of responses from adults with pediatric heart transplants was likely too small to draw meaningful comparisons.
Conclusions Medical and surgical advances have led to a dramatic decrease in mortality among patients with congenital heart disease, with the vast majority surviving to adulthood. We aimed to evaluate the quality of life in surviving adults with one of the most severe forms of congenital heart disease. Despite the imponderables of the Fontan physiology and other disease related everyday stresses, a majority of these patients survive for a relatively long time with a good quality of life, particularly in the mental/social domains. This generally favorable extended lifesatisfaction period (average of 18 years) should be considered when informing patients and families of expectations with the Fontan pathway versus certain higher risk procedures when both the Fontan and 2-ventricle options are considered. HRQOL measurement is becoming an increasingly important aspect of all types of medical/surgical therapies. Future research is needed to encourage further prospective multicenter studies that evaluate routine QOL assessment in these adults to support this conclusion. Our ultimate goal is to learn more about the needs of these survivors in an effort to increase our understanding of ways to prevent or alleviate some of the problems that accompany this type of congenital heart disease and its treatment.
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Appendix A
Contents and explanations of each of domains of the QOL components evaluated by the SF-36 survey PCS – Physical component score (overall) •
PF- Physical Functioning: performance of physical activities such as self-care, walking,
and vigorous physical activities. •
RP- Role Physical: the degree to which a person’s typical role activities (e.g. Childcare,
job) are limited by physical health. •
BP- Bodily Pain: intensity, duration, and frequency of bodily pain and limitations in
usual activities due to pain. •
GH- General Health: the belief and evaluations of a person’s overall health.
MCS – Mental Health component score (overall) •
VT- Vitality: feeling of energy and absence of fatigue.
•
SF- Social Functioning: the degree to which a person develops and maintains social
relationships (ex with family and friends etc.). •
RE- Role Emotional: the degree to which a person’s typical role activities (e.g. child-
care, job) are limited by emotional problems. •
MH- Mental Health: a person’s emotional, cognitive, and intellectual status.
16
FIGURE LEGENDS Figure 1 Overall physical and mental component standardized scores by different age groups. The blue bars represent the mean score of Fontan patients in the stated age groups. The number of Fontan patients in each age category is designated in the row labelled “# in sample.” The mean score of the age-controlled general population is indicted in the row labelled “Norm” and by the horizontal bars. The p-values for differences with age groups are indicated. Figure 2 Cumulative frequency distribution for the standardized physical component scores among Fontan patients. The red solid and dashed lines indicate the 25th, 50th, and 75th percentiles in that domain for the general U.S. population aged 25-34 years. Figure 3 Cumulative frequency distribution for the standardized general health scores among Fontan patients. Depiction is as in Figure 2. Figure 4 Cumulative frequency distribution for the standardized overall mental component scores among Fontan patients. Depiction is as in Figure 2.
17
Table 1 COMPARISON OF SURGICAL AND AGE-RELATED DETAILS BETWEEN RESPONDERS AND NON-RESPONDERS TO THE QUALITY OF LIFE SURVERY N=49 (pts responded to survey) Bi-directional Glenn* Fenestration (yes)* Age-median years*at time of Fontan (range) Age distribution (yrs) 2.0 – 5.0 5.1 – 10.0 10.1 – 15.0 15.1 – 20.0 ≥ 20.1
23 (46.9%) 14 (28.6%) 6.2 yrs (2.5-32.7)
18 (36.7%) 15 (30.6%) 11 (22.4%) 3 (6.1%) 2 (4.1%)
N=11 (pts that did NOT respond to survey) 5 (45.4%) 1 (9%) 5.1 yrs (2.438.3) 5 (45.4%) 3 (27.3%) 2 (18.2%) 0 1 (9.0%)
TOTAL (N=60)
28 (46.7%) 15 (25%) 5.9 yrs (2.4-38.3)
23 (38.3%) 18 (30%) 13 (21.7%) 3 (5%) 3 (5%)
*No statistically significant difference between the N=49 and N=11 groups (p>0.05) for Bi-dir Glenn, Fenestration, or age at time of Fontan
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Table 2 COMPARISON OF ANATOMIC DETAILS BETWEEN RESPONDERS AND NONRESPONDERS TO THE QUALITY OF LIFE SURVEY Diagnosis
N=49 (pts responded to survey)
Tricuspid atresia DILV DORV, complex Heterotaxy/discordant atrioventricular connection Other single ventricle HLHS Single ventricle, mitral atresia DIRV Unbalanced AVSD Pulmonary atresia
TOTAL (N=60)
8 (16%) 8 (16%) 8 (16%) 6 (12%)
N=11 (pts that did NOT respond to survey) 5 (45.4%) 0 2 (18.2%) 0
6 (12%) 5 (10%) 3 (6%)
1 (9%) 1 (9%) 0
7 (12%) 6 (10%) 3 (5%)
3 (6%) 1 (2%) 1 (2%)
2 (18.2%) 0 0
5 (8%) 1 (2%) 1 (2%)
13 (22%) 8 (13%) 10 (17%) 6 (10%)
DILV, double inlet left ventricle; DORV, double outlet right ventricle; HLHS, hypoplastic left ventricle; AVSD, atrioventricular septal defect.
Table 3 Anatomic Diagnoses for 13 Heart Transplant Patients DIAGNOSIS Congenital (HLHS-2 Failed Fontan (DILV)-1, Other - 1 Cardiomyopathy -- Dilated N=2 -- Hypertrophic N=1 -- restrictive N=1 -- post-partum N=1 -- non-specified N=4
N 4
% 30.8%
9
69.2%
HLHS, hypoplastic left heart syndrome, DILV, double inlet left ventricle
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Table 4 Mean Standardized Quality of Life scores by cohort and of the general population (agecontrolled to 25 to 35 years using the SF-36v2 survey)
(C) Heart transplant patients (n=13) 50.7 ± 7.4
@ p value (AvsB)
49.6 ± 7.2
(B) General population (age: 2534 years) 53.8± 6.7
45.6 ± 12.7 48.8 ± 13.7 51.8 ± 8.1 50.1 ± 9.5 49.8 ± 9.6 56.4 ± 7.9 48.3 ± 10.3 50.1 ± 8.9 49.9 ± 12.1
52.7 ± 7.9 51.3 ±8.5 51 ± 8.8 49.6 ± 9.6 49.9 ± 9.5 52.3 ± 9.1 51.7 ± 9 53.5 ± 7.2 49.2 ± 9.4
45.4 ± 13.4 47.6 ± 13.3 54 ± 5.2 52.8 ± 6.1 50 ± 8.2 54.6 ± 10.8 47.7 ± 12 49.4 ± 8.9 51.6 ± 7.9
QOL Domains
(A) Fontan Patients (n=49)
1. Physical functioning 2. Role-physical 3. Role-emotional 4. Social functioning 5. Mental health 6.Energy/vitality 7. Bodily pain 8. General health 9. Overall PCS 10. Overall MCS
Health-Related Quality of Life in Adult Survivors after the Fontan Operation Manisha Kukreja MBBS, MPH, Ayesha S. Bryant MSPH, MD, David C. Cleveland MD, MBA, Robert Dabal MD, Neha Hingorani BS, James K. Kirklin MD
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PII: DOI: Reference:
S1043-0679(15)00119-7 http://dx.doi.org/10.1053/j.semtcvs.2015.08.007 YSTCS765
To appear in:
Semin Thoracic Surg
Cite this article as: Manisha Kukreja MBBS, MPH, Ayesha S. Bryant MSPH, MD, David C. Cleveland MD, MBA, Robert Dabal MD, Neha Hingorani BS, James K. Kirklin MD, Health-Related Quality of Life in Adult Survivors after the Fontan Operation, Semin Thoracic Surg, http://dx.doi.org/10.1053/j.semtcvs.2015.08.007 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
HEALTH-RELATED QUALITY OF LIFE IN ADULT SURVIVORS AFTER THE FONTAN OPERATION
Manisha Kukreja, MBBS, MPH1 Ayesha S. Bryant, MSPH, MD1 David C. Cleveland, MD, MBA1 Robert Dabal, MD1 Neha Hingorani, BS 1 James K. Kirklin, MD1 University of Alabama at Birmingham, Alabama Department of Surgery, Division of Cardiothoracic Surgery Birmingham, AL 352941
There is no funding, no COI, and no potential conflicts for either of the authors. From the Department of Surgery, Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, AL Disclosures: Authors have nothing to disclose with regard to commercial support. Address for reprints: James K. Kirklin, MD, University of Alabama, Professor and Director, Division of Cardiothoracic Surgery, 760 THT, 1900 University Boulevard, Birmingham, AL 35294
[email protected] 205 934 5486 4009 Words Key words: Health-Related Quality of Life; Fontan procedures; Single-ventricle conditions.
Glossary of Abbreviations
HRQOL – Health related quality of life HT – Heart Transplant SF-36 – Short Form Survey
1
Abstract Objective: The primary objective of this study was to ascertain the long-term health-related quality of life (HRQOL) of adult patients who underwent a childhood Fontan operation for palliation of uni-ventricular cardiac anomalies.
The secondary objective was to compare the long-term
HRQOL of Fontan survivors to that of pediatric heart transplant recipients. Methods: This cross-sectional study examined adult survivors (>19 years) who underwent a Fontan operation during childhood (Fontan group) or a pediatric heart transplant (HT group) between 1988 and 2011 (23 year span). HRQOL was assessed using the Short Form-36 (SF-36) survey. The study group consisted of 49 Fontan group patients and 13 HT group patients who responded to the survey. Results: HRQOL Scores of the Fontan group were similar to an age-controlled healthy US population in social and mental functioning, energy/vitality, and overall mental component score (p ≥ 0.2). However, Fontan scores in physical functioning, bodily pain, general health, and overall by physical component were significantly below the age – controlled US population (p < 0.05). No differences were identified between Fontan and heart transplant patients. Conclusions: This favorable life-satisfaction period (average 18 years) should be considered when informing patients and families of expectations with the Fontan pathway versus certain higher risk procedures.
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Central Message The long-term health-related quality of life in adult patients who underwent a childhood Fontan operation for single ventricle cardiac anomalies was similar to an age-controlled general population in mental components but somewhat less in physical component categories, with no differences from pediatric heart transplant recipients.
Introduction Several modifications of the Fontan operation coupled with medical and surgical advances in management during and after surgery have improved the surgical outcomes during the past two decades.1,2 Further it has been demonstrated that the current standardized Fontan operation offers excellent long-term survival, freedom from reoperations, good functional status, and absence of late increasing mortality.3,4 The short form 36 (SF-36) survey is a validated tool commonly used to assess four major areas of a person’s daily functioning, also known as health-related quality of life (HRQOL): physical health, emotional/mental
state, social functioning, and general health, often in
heterogeneous populations.5,6 The primary objective of this study was to ascertain the long-term HRQOL of adult patients (≥19 years) who underwent a childhood Fontan operation (Fontan group) for palliation of univentricular congenital cardiac anomalies at the University of Alabama at Birmingham (UAB). The secondary objectives were to compare the long term HRQOL of the Fontan group to that of heart transplant recipients (HT group) and also to a healthy comparison group derived from the general population.
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Methods Patient population Eligibility criteria for the study were: patients who underwent either a childhood Fontan operation for a single ventricle congenital anomaly or a pediatric heart transplant between 1988 and 2011 at UAB, alive at the time of this study, and 19 years or older. The overall Fontan population included 207 patients who underwent the Fontan operation between 1988 and December 31, 2011. Eighty of these patients would potentially reach age 19 during 2014 or before. Twenty of these 80 patients died before 2014. Of the remaining 60 patients who received a QOL survey, the 49 respondents (82% response rate) formed the Study Group. SF-36 Quality of Life Instrument Health-related quality of life was assessed using the standard short form SF-36 (SF-36 V2) health survey. The SF-36 consists of 36 questions that measure eight domains of health: physical functioning (PF), role limitations due to physical health (RP), role limitations caused by emotional health problems (RE), social functioning (SF), bodily pain (BP), mental health (MH), vitality (VT), and general health (GH). It provides scores for each of these eight health domains, as well as two summary scores: the physical component summary (PCS) and the mental health component summary (MCS).
Additional definitions of these components are provided in
Appendix A. The HRQOL survey (SF-36) was used to assess the mental and physical component scores of these patients. The scores of the Fontan group were compared with the HRQOL scores of healthy patients (normative data derived from the general U.S. population), and also with scores of the HT group.7,8 For each domain, the scores range from 0 to 100, where a higher score indicates a better health state. This instrument is not designed to give an overall composite score. The SF-36 has 4
acceptable internal consistency, validity and test-retest reliability.9,10 The national normalized value (as validated by Quality Metric Inc, Lincoln, RI) is 50.0 with a standard deviation (SD) of 10.
Norms are available for the healthy general U.S. population as a whole and also for seven
age groups, male and females individually as well as combined as reported by the National Survey of Functional Health Status. 7,8
Overall, the SF-36 is a validated generic survey that allows assessment of health concepts that are not age, disease, or treatment specific. Therefore, it has been useful in assessing the health of general as well as specific populations, comparing the relative burden of diseases, differentiating the health benefits produced by different treatments, and screening individual patients. Study Protocol Patients were mailed a questionnaire SF-36 v2 health survey (standard short form SF-36) in May 2014. Patients were asked to return their survey within eight weeks. The study closing date was July 15th, 2014. This study was reviewed and approved by the UAB Institutional Review Board and all the participants provided informed consents. Statistical methods The QOL scores of each of the eight domains and the overall PCS and MCS were assessed. These data are presented as mean with standard deviation (±SD) and compared by independent t tests. A t-test was performed to assess the level of significance between the difference in means of sample population (Fontan group or HT group) and general population (normative data). We considered statistically significant differences with a p-value of ˂0.05. Cumulative frequency distributions were used to examine the response pattern of the overall sample populations. 5
The scores of each domain for the SF-36 v2 assessment were calculated using Health Outcomes Scoring software 4.5 “OptumInsight Smart Measurement System (SMS) Scoring Solution” (Quality Metric Incorporated, Lincoln, RI).11,12,13
This software uses an algorithm to
convert raw scores to standardized scores to allow comparison.
All statistics were performed
using SAS software v. 9.3 (SAS Institute, Cary, NC) in conjunction with custom software used for hazard analysis.14
Results Description of cohort Among the Fontan group, of the 60 surveys sent to eligible patients, 49 (82%) were completed and returned. Of the surveys not returned: one patient was in a mental rehabilitation center and unable to answer the survey questions, two were foreign patients residing overseas, four questionnaires were not returned, and four patients were lost to follow up. Among the Fontan group, the participants ranged in age from 19–55 years, with a median age of 25 years, a mean of 26 years; and 25 (52%) were male.
Mean follow-up for the Fontan group was 18 years
(range 3 to 24 years), of which 44 (90%) had greater than 10 years of follow-up All patients in the Fontan group received a PTFE tube connecting blood flow from the inferior vena cava to the pulmonary artery (“modern Fontan operation”). Demographics, anatomic features, and surgical details were compared between responders and non-responders to the QOL survey (Tables 1 and 2). Surgical and age-related characteristics were similar between groups. Among anatomic details, a diagnosis of tricuspid atresia was more common among non-responders. Among the heart transplant group, 14 questionnaires were sent to eligible patients and response rate was 93%. One patient did not respond due to personal reasons. Participants ranged in age from 19–32 years, with a median age of 22 years; and there were 7 (54%) women. For 6
the heart transplant group, mean follow up was 13 years (range 3 to 23 years). Thirty per cent of the transplant patients carried a diagnosis of congenital heart disease, and the remainder had cardiomyopathy. (Table 3). Comparison of scores among populations Because the distribution of ages in the Fontan (92% of patients between ages 19 and 34 years) and transplant patients (100% of patients between ages 19 and 32 years) did not follow the distribution of the normal population, the overall physical and mental component scores were subdivided by age groups (Figure 1). Since the normalized data for individual age groups was available for comparison, we used the normalized population for the age group 25 to 34 years as our age-controlled healthy U.S. population group for comparison. The HRQOL scores of the Fontan group with regard to social functioning, mental health, role-emotional, energy/vitally, as well as the overall mental component score were comparable to that of an age-controlled healthy US population (Table 4). However, the Fontan group reported a significantly diminished physical functioning, role-physical, freedom from bodily pain, general health perception, and overall physical component score compared to the healthy US population. A sub-analysis of the Fontan group by gender showed that among males, bodily pains, physical functioning, and role physical were significantly higher compared to the age-matched general US population (p ≤0.05) and females had significantly reduced general health perception (see again Appendix 1 for clarification) compared to the age-controlled general US population (p=0.02). The HRQOL scores of the HT group were not significantly different from the general age-controlled population in any domain except for social functioning, in which the HT group
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exceeded the general population (Table 4). There were no significant differences in any of the eight subjective measures of HRQOL between the Fontan group and HT recipients group. Distribution of Scores within the Patient Population Comparing the Fontan population to the age-controlled general population in the Overall Physical Component Scores (Figure 2), 44% of the Fontan group scored with the lowest 25% of the general population, while only 10% of Fontan scores fell within the upper 25% of the population. Similarly, in the General Health assessment (Figure 3), 46% of Fontan patients scored with lowest 25% of the general population, while 18% matched with the upper 25% of the general population. The Fontan group fared better in the Overall Mental Component Scores (Figure 4), with 30% of Fontan patients scoring with the lowest 25% of the general age-matched population, while nearly 40% scored with the upper 25% of the general population.
Discussion Survival and HRQOL Over the past two decades, mortality attributed to univentricular heart repair has been reduced, and many patients now reach adulthood.
A recent publication by Dabal, et al,
demonstrated ongoing improvement in survival over time in the current era of pediatric cardiac surgery patients after Fontan surgery.4 The 1-, 10-, and 20- year survival with the use of a PTFE tube was 95%, 88%, and 77%, respectively. No late increasing phase of risk was identified out to 20 years. Most surviving patients were in NYHA class I or II at follow-up, even beyond 12 years. Less than 10% of patients were either in NYHA class III or unknown. However, many of these patients endure repeated hospital admissions, and some have repeat surgical procedures. The psychological impact of these experiences is particularly notable in adolescents and young adults. In addition, patients are often aware of the potential reduction in their life expectancy and
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physical capabilities. Several studies have examined the intellectual, social, and emotional development and adjustment of children and adolescents with congenital heart disease, as well as mother–child relations and parental stress,15-20 with conflicting conclusions. Several investigators have reported negative psychosocial outcomes in these patients—for example, diminished self esteem, anxiety, depression, and poor emotional or social adjustment;21-24
while others have
reported more optimistic outcomes and a normal quality of life in selected subsets of congenital heart disease.25,26 Our findings demonstrate that patients after Fontan operations have similar long-term social/mental/emotional HRQOL outcomes compared to the healthy US general population and no significant difference from heart transplant recipients for nearly 2 decades.
However,
physical functioning and general health perception lag somewhat behind that of the normal population. Two previous studies in Fontan survivors support the findings of satisfactory QOL, which contradicts previous assumptions, parental proxy reports, or self-reports of decreased QOL.27-30 Some studies on physical functioning, general health, and mental health outcome in adults after Fontan operations have reported problems in these domains, whereas others show normal social behavior and comparable QOL with that of their healthy counterparts, thus supporting the generally favorable outcomes of our present study.31,32 To improve patients’ overall long term quality of life, physical activity must be encouraged and patients motivated to perform daily activities, avoiding excessive restrictions on themselves. Over time, most patients appear to adapt to their disease and maintain a positive outlook in day to day life. Impact of HRQOL on the Overall Calculus of “Outcomes” Despite the extensive validation of quality of life instruments, the very nature of this subject underscores its inherent subjectivity. What cannot be denied is the importance of how 9
the patient “feels” about the impact of alternative or competing therapies on his/her happiness and ability to function. This component of the “outcomes equation” becomes increasingly important as survival differences between therapies narrow.
Ultimately, a truly informed
decision between therapies such as transplantation or continued acceptance of a slowly failing Fontan; selection of a complex, imperfect 2- ventricular reconstruction vs. the known outcomes following the Fontan procedure; and many other therapeutic dilemmas will incorporate components of survival, likelihood and frequency of adverse events, probability of re-operations, and health-related quality of life. As we strive to refine this process, the focus to quantify these components gains importance in order to effectively explain what a patient and his/her family may “expect” with a given therapeutic choice. This study addresses one component of that “grand equation”, namely health-related quality of life. Our analysis is challenged by the limitations stated below, but the pursuit of such health-related quality of life analyses will increasingly become an important part of the longterm evaluation of many complex cardiac operations when alternative surgical or medical strategies are available.
Limitations of the study The primary limitation of this study was the cross sectional study design, which depends on patients self-reporting/evaluating and returning the questionnaires. We had only crosssectional data to analyze, we did not administer the survey both pre- and postoperatively, and thus we were unable to evaluate changes from baseline values and could not explore the temporal trends in health perceptions. Another limitation is our reliance on the use of published normative data rather than simultaneously studying a cohort of normal control subjects. Furthermore, the possibility than non-responders may have a different perception of their QOL
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cannot be excluded. The number of responses from adults with pediatric heart transplants was likely too small to draw meaningful comparisons.
Conclusions Medical and surgical advances have led to a dramatic decrease in mortality among patients with congenital heart disease, with the vast majority surviving to adulthood. We aimed to evaluate the quality of life in surviving adults with one of the most severe forms of congenital heart disease. Despite the imponderables of the Fontan physiology and other disease related everyday stresses, a majority of these patients survive for a relatively long time with a good quality of life, particularly in the mental/social domains. This generally favorable extended lifesatisfaction period (average of 18 years) should be considered when informing patients and families of expectations with the Fontan pathway versus certain higher risk procedures when both the Fontan and 2-ventricle options are considered. HRQOL measurement is becoming an increasingly important aspect of all types of medical/surgical therapies. Future research is needed to encourage further prospective multicenter studies that evaluate routine QOL assessment in these adults to support this conclusion. Our ultimate goal is to learn more about the needs of these survivors in an effort to increase our understanding of ways to prevent or alleviate some of the problems that accompany this type of congenital heart disease and its treatment.
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Appendix A
Contents and explanations of each of domains of the QOL components evaluated by the SF-36 survey PCS – Physical component score (overall) •
PF- Physical Functioning: performance of physical activities such as self-care, walking,
and vigorous physical activities. •
RP- Role Physical: the degree to which a person’s typical role activities (e.g. Childcare,
job) are limited by physical health. •
BP- Bodily Pain: intensity, duration, and frequency of bodily pain and limitations in
usual activities due to pain. •
GH- General Health: the belief and evaluations of a person’s overall health.
MCS – Mental Health component score (overall) •
VT- Vitality: feeling of energy and absence of fatigue.
•
SF- Social Functioning: the degree to which a person develops and maintains social
relationships (ex with family and friends etc.). •
RE- Role Emotional: the degree to which a person’s typical role activities (e.g. child-
care, job) are limited by emotional problems. •
MH- Mental Health: a person’s emotional, cognitive, and intellectual status.
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FIGURE LEGENDS Figure 1 Overall physical and mental component standardized scores by different age groups. The blue bars represent the mean score of Fontan patients in the stated age groups. The number of Fontan patients in each age category is designated in the row labelled “# in sample.” The mean score of the age-controlled general population is indicted in the row labelled “Norm” and by the horizontal bars. The p-values for differences with age groups are indicated. Figure 2 Cumulative frequency distribution for the standardized physical component scores among Fontan patients. The red solid and dashed lines indicate the 25th, 50th, and 75th percentiles in that domain for the general U.S. population aged 25-34 years. Figure 3 Cumulative frequency distribution for the standardized general health scores among Fontan patients. Depiction is as in Figure 2. Figure 4 Cumulative frequency distribution for the standardized overall mental component scores among Fontan patients. Depiction is as in Figure 2.
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Table 1 COMPARISON OF SURGICAL AND AGE-RELATED DETAILS BETWEEN RESPONDERS AND NON-RESPONDERS TO THE QUALITY OF LIFE SURVERY N=49 (pts responded to survey) Bi-directional Glenn* Fenestration (yes)* Age-median years*at time of Fontan (range) Age distribution (yrs) 2.0 – 5.0 5.1 – 10.0 10.1 – 15.0 15.1 – 20.0 ≥ 20.1
23 (46.9%) 14 (28.6%) 6.2 yrs (2.5-32.7)
18 (36.7%) 15 (30.6%) 11 (22.4%) 3 (6.1%) 2 (4.1%)
N=11 (pts that did NOT respond to survey) 5 (45.4%) 1 (9%) 5.1 yrs (2.438.3) 5 (45.4%) 3 (27.3%) 2 (18.2%) 0 1 (9.0%)
TOTAL (N=60)
28 (46.7%) 15 (25%) 5.9 yrs (2.4-38.3)
23 (38.3%) 18 (30%) 13 (21.7%) 3 (5%) 3 (5%)
*No statistically significant difference between the N=49 and N=11 groups (p>0.05) for Bi-dir Glenn, Fenestration, or age at time of Fontan
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Table 2 COMPARISON OF ANATOMIC DETAILS BETWEEN RESPONDERS AND NONRESPONDERS TO THE QUALITY OF LIFE SURVEY Diagnosis
N=49 (pts responded to survey)
Tricuspid atresia DILV DORV, complex Heterotaxy/discordant atrioventricular connection Other single ventricle HLHS Single ventricle, mitral atresia DIRV Unbalanced AVSD Pulmonary atresia
TOTAL (N=60)
8 (16%) 8 (16%) 8 (16%) 6 (12%)
N=11 (pts that did NOT respond to survey) 5 (45.4%) 0 2 (18.2%) 0
6 (12%) 5 (10%) 3 (6%)
1 (9%) 1 (9%) 0
7 (12%) 6 (10%) 3 (5%)
3 (6%) 1 (2%) 1 (2%)
2 (18.2%) 0 0
5 (8%) 1 (2%) 1 (2%)
13 (22%) 8 (13%) 10 (17%) 6 (10%)
DILV, double inlet left ventricle; DORV, double outlet right ventricle; HLHS, hypoplastic left ventricle; AVSD, atrioventricular septal defect.
Table 3 Anatomic Diagnoses for 13 Heart Transplant Patients DIAGNOSIS Congenital (HLHS-2 Failed Fontan (DILV)-1, Other - 1 Cardiomyopathy -- Dilated N=2 -- Hypertrophic N=1 -- restrictive N=1 -- post-partum N=1 -- non-specified N=4
N 4
% 30.8%
9
69.2%
HLHS, hypoplastic left heart syndrome, DILV, double inlet left ventricle
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Table 4 Mean Standardized Quality of Life scores by cohort and of the general population (agecontrolled to 25 to 35 years using the SF-36v2 survey)
(C) Heart transplant patients (n=13) 50.7 ± 7.4
@ p value (AvsB)
49.6 ± 7.2
(B) General population (age: 2534 years) 53.8± 6.7
45.6 ± 12.7 48.8 ± 13.7 51.8 ± 8.1 50.1 ± 9.5 49.8 ± 9.6 56.4 ± 7.9 48.3 ± 10.3 50.1 ± 8.9 49.9 ± 12.1
52.7 ± 7.9 51.3 ±8.5 51 ± 8.8 49.6 ± 9.6 49.9 ± 9.5 52.3 ± 9.1 51.7 ± 9 53.5 ± 7.2 49.2 ± 9.4
45.4 ± 13.4 47.6 ± 13.3 54 ± 5.2 52.8 ± 6.1 50 ± 8.2 54.6 ± 10.8 47.7 ± 12 49.4 ± 8.9 51.6 ± 7.9
QOL Domains
(A) Fontan Patients (n=49)
1. Physical functioning 2. Role-physical 3. Role-emotional 4. Social functioning 5. Mental health 6.Energy/vitality 7. Bodily pain 8. General health 9. Overall PCS 10. Overall MCS
