Da John Marino Pediatrics in Charleston Sc Reviews
Congenit Heart Dis. Author manuscript; bachelor in PMC 2016 Jan ane.
Published in concluding edited class as:
PMCID: PMC4414014
NIHMSID: NIHMS649071
Survival Information and Predictors of Functional Outcome an Average of 15 Years later the Fontan Procedure: The Pediatric Heart Network Fontan Accomplice
Andrew M. Atz
*Department of Pediatrics, Medical University of South Carolina, Charleston, SC
Victor Zak
†New England Research Institutes, Watertown
Lynn Mahony
‡Department of Pediatrics, Academy of Texas Southwestern Medical Center, Dallas, Tex
Karen Uzark
§Department of Pediatrics, University of Michigan, Ann Arbor, Mich
Peter Shrader
†New England Inquiry Institutes, Watertown
Dianne Gallagher
†New England Research Institutes, Watertown
Stephen M. Paridon
¶Children's Infirmary of Philadelphia, Philadelphia, Pa
Richard V. Williams
**Department of Pediatrics, University of Utah, Table salt Lake City, Utah
Roger Due east. Breitbart
††Boston Children's Hospital, Boston, Mass, U.s.
Steven D. Colan
††Boston Children'southward Hospital, Boston, Mass, United states of america
Jonathan R. Kaltman
‡‡National Heart, Lung, and Claret Institute, Bethesda, Doc
Renee Margossian
††Boston Children's Hospital, Boston, Mass, The states
Sara M. Pasquali
§§Section of Pediatrics, Duke University Medical Centre, Durham, NC
Kerstin Allen
†New England Research Institutes, Watertown
Wyman W. Lai
¶¶Department of Pediatrics, Columbia Academy Medical Center, New York, NY
Rosalind Korsin
¶¶Section of Pediatrics, Columbia Academy Medical Center, New York, NY
Bradley S. Marino
***Cincinnati Children'southward Hospital Medical Center, Cincinnati, Ohio, USA
Nicole Mirarchi
¶Children's Hospital of Philadelphia, Philadelphia, Pa
Brian Due west. McCrindle
†††The Hospital for Sick Children, University of Toronto, Toronto, Canada
Abstruse
Objective
Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are defective. The aim of this report was to better understand gimmicky outcomes in Fontan survivors by collecting follow-up information in a previously well-characterized cohort.
Design
Baseline data from the Fontan Cross-Sectional Report (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.ix ± 3.4 years. We assessed current transplant-free survival status in all subjects six.8 ± 0.4 years later on the Fontan ane study. Anatomic, clinical, and surgical data were nerveless forth with socioeconomic status and access to wellness care.
Results
Xxx subjects (5%) died or underwent transplantation since Fontan i. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Wellness Questionnaire concrete summary score (<44) measured at Fontan 1 were significantly more than likely to dice or undergo transplant than the residual, with a hazard ratio of six.2 (two.9–thirteen.five). Amid 516 Fontan survivors, 427 (83%) enrolled in this follow-up study (Fontan 2) at 18.four ± 3.4 years of historic period. Although mean scores on functional health status questionnaires were lower than the general population, private scores were inside the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF-36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the acting between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (due north = 68) since Fontan 1.
Conclusions
We institute 95% interim transplant-costless survival for Fontan survivors over an average of vii years of follow-up. Continued longitudinal investigation into adulthood is necessary to better empathize the determinants of long-term outcomes and to meliorate functional health status.
Introduction
The Fontan procedure results in nearly-normal systemic oxygen saturation in those built-in with a functional single ventricle, reducing the demands on the systemic ventricle and extending the lives of patients with fifty-fifty the nearly complex forms of congenital heart disease. Nevertheless, the resulting abnormal hemodynamic state is associated with a variety of tardily complications including decreased practise functioning, abnormal ventricular function, intracardiac and extracardiac thrombosis and embolic phenomena, arrhythmias and conduction system damage, cirrhosis, and poly peptide-losing enteropathy.1–four Equally these patients age into adulthood, access to specialized health intendance may become limited as a issue of insufficient insurance, lack of employment, and lack of education virtually the demand for evaluation past adult congenital heart physicians.5,6 Therefore, although the survival of children and adolescents with the Fontan procedure continues to improve, survivors remain at increased risk for mortality and morbidities, as well as physiologic limitations that are likely to touch concrete, psychological, and socioeconomic status.7,8
The Pediatric Center Network Fontan Cross-Sectional Written report (Fontan ane) characterized a multi-institutional cohort of 546 survivors subsequently the Fontan procedure, with an historic period of 6 to 18 years at enrollment in 2003–2004.9 The design of Fontan ane could non make up one's mind if observed differences betwixt older and younger subjects were related to the length of time living with Fontan physiology or to changes in medical, catheter-based, or surgical management strategies. The primary objective of the current written report (Fontan two) was to better empathize contemporary outcomes in Fontan survivors by collecting follow-upwardly data involving assessment of vital status, repeat functional health status, collection of interim medical history, and access to wellness intendance at an boilerplate of 7 years later on enrollment in Fontan 1.
Methods
Study Design and Patient Population
Vital and cardiac transplant condition data were assessed in all 546 subjects enrolled in Fontan ane. From this original group, subjects who were alive with a Fontan circulation were approached for enrollment into the present study. Each center's institutional review lath approved the protocol. Written informed consent and assent were obtained according to local requirements. Anatomic, clinical, and surgical data were nerveless at enrollment (November 2009 to May 2011) using standardized forms. Structured interviews with the parent/guardian and/or discipline were used to assess current clinical country, socioeconomic condition, family functioning, and admission to health care. Vital condition was assessed by either contact or by search of the social security death index annually post-obit enrollment.
Measures of Functional Health Status and Quality of Life
The Child Health Questionnaire (CHQ), which includes questionnaires for both the child and the parent, was used for subjects ≤18 years. In subjects aged ≥xix years, functional condition was measured with the Short-Grade Health Survey version ii (SF-36).10 The Pediatric Quality of Life Inventory (Peds-QL) with a self-report and a parallel parent proxy report was used to assess quality of life.11,12 In addition, a cardiac disease-specific module of the Peds-QL was administered. Pediatric Quality of Life Inventory age group versions (8–12 years old, xiii–18 years erstwhile, and 19–25 years old) have almost identical questions with small differences in linguistic communication. To preserve sample size and after consultation with experts (personal communication with J. Varni, MD, July, 2013), we combined these versions for analytic purposes.
Statistical Methods
The relationship betwixt transplant-costless survival and baseline (assessed at Fontan ane) covariates was examined using Cox proportional hazards regression modeling. Follow-up time was defined every bit the time from Fontan 1 medical record review until either death/transplant or the latest available assessment of vital/transplant status (at or subsequently Fontan 2 enrollment). We written report hazard ratios with 95% confidence intervals and P values associated with the Wald chi-square exam. Statistical significance was considered using a two-sided critical value of P = .05. The proportional hazards supposition was evaluated using a supremum test for nonproportionality based on Martingale residuals, and interaction terms with time were considered equally advisable. Key results are illustrated using Kaplan–Meier survival plots.
Subsequent analyses compared prespecified subgroups defined by ventricular morphology, Fontan procedure type, and age at enrollment. Differences in patient outcomes past subgroup levels were assessed using a regression model adjusted for age, gender (if gender mix differed), and clinical site (for medical history variables and medication use). For continuous outcomes, hateful values by subgroup were compared using a parametric analysis of variance-based exam and an age- and gender-adapted analysis of covariance. Additionally, median values by subgroup were compared using a nonparametric (Wilcoxon or Kruskal–Wallis) test. If any of these three tests was significant, Bonferroni corrections were calculated. For binary outcomes, frequencies in the subgroups were compared using Fisher's exact test and a gender- and historic period-adjusted logistic regression model. For ordered categorical outcomes, the presence of a linear trend was assessed using Mantel-Haenszel test for trend, and a gender- and age-adapted cumulative or multinomial logistic regression was used as appropriate. For interim medical history events, mean incidence rates of the event were compared by subgroup using Poisson regression. To explore differences related to age, we divided the study population into two groups (younger vs. older). For continuous variables and instruments validated across all ages, e.g. medical history and Peds-QL, 18 years was selected as a cutoff value. The cutoff of 18 was selected equally this age is used oft to define an adult and this provided similar sized groups for comparing. For instruments validated only in children (e.k., CHQ-PF50 and CHQ-CF87), sixteen years was selected as a cutoff. Similarly, for forms validated only in adults (e.g., SF-36), the adult cohort (divers as ≥19 years) was divided into 2 roughly similar sized groups; those < or ≥22.5 years. All analyses were performed using SAS statistical software version 9.3 (SAS Institute, Inc., Cary, NC, USA).
Results
Patient Characteristics
From the original Fontan 1 accomplice of 546 subjects, 29 (5%) either died (due north = sixteen) or were transplanted (northward = 13) prior to Fontan 2 enrollment and 1 subject died after enrollment. Additionally, one subject underwent a conversion to a two-ventricle circulation prior to Fontan 2 enrollment. The 516 surviving subjects with a Fontan apportionment were considered eligible for the Fontan 2 study and 427 subjects (83%) enrolled. Thirteen subjects (ii.5%) were lost to follow-up and 76 subjects (15%) did not give consent. The enrolled subjects were compared with the eligible but non-consenting subjects who either were lost to follow-upwardly or refused consent. Enrolled subjects were younger (18.4 ± 3.iv vs. xix.6 ± 3.3 years, P = .002) and had a lower proportion of males (58% vs. 71%, P = .02). Later aligning for age and gender, enrollees came from households with a college income level and higher level of maternal educational activity (P < .001 for both). No differences were identified in whatever cardiac characteristics or laboratory measures from Fontan 1.
Died/Transplanted vs. Transplant-free Survivors
Vital condition cess data were collected on all of the original 546 subjects except the xiii who were lost to follow-up. The social security decease alphabetize was searched for the thirteen lost-to-follow-up subjects and none were reported to have died. The 502 transplant-free survivors through the end of follow-upwards were compared with the thirty who died or had heart transplants (1 subject died after enrolling in Fontan ii) (Effigy 1). There were no differences in the risk of expiry/transplant based on age, dominant ventricular morphology, or blazon of Fontan process. On univariate assay, the risk of death or transplant after Fontan 1 was significantly associated with the following measurements obtained at Fontan 1: a poorer CHQ-PF50 physical summary score (36.6 ± 13.6 vs. 45.8 ± 11.5, P < .01) (Figure 2A), increased end systolic and end diastolic volume z-scores (P < .02 for both), a higher brain natriuretic peptide (BNP) concentration value (P = .04) (Figure 2B), and inability to perform an exercise test (P < .05). The hazard of decease/transplant for subjects with both an elevated BNP (>21 pg/mL) and a low physical summary score (<44) was 6.2 (two.nine–thirteen.v) times higher than for the rest of the cohort (Effigy 2C).
Transplant-free survival since Fontan one (with 95% conviction intervals) in a accomplice of 532 subjects with complete follow-upwards data. For improved resolution, the scale on the y-axis is limited to 0.9–1.0.
Association of acting (between Fontan 1 and Fontan 2) transplant-free survival with CHQ-PF50 physical summary score (A); serum BNP level (B); and both (C). For improved resolution, the calibration on the y-axis is limited to 0.75–one.0. BNP, brain natriuretic peptide; CHQ, Kid Wellness Questionnaire.
Fontan 2 Cohort Characteristics
The 427 enrolled subjects were 18.4 ± 3.four years former at Fontan 2 enrollment. The mean follow-up time was 6.viii ± 0.4 years from Fontan i and xv.2 ± 3.4 years after Fontan surgery. Since Fontan surgery, 119 subjects (28%) had additional cardiac surgery, with 55% of these (n = 66) occurring in the interim between Fontan 1 and Fontan two. During the interim flow, 10 subjects underwent a Fontan conversion. The most common surgical process performed since Fontan 1 involved placement or replacement of implantable electronic devices, which were currently in place in 13% (n = 56). A cardiac catheter intervention occurred in 242 subjects (57%); 32% of these (northward = 78) took place afterward Fontan 1. Since the Fontan procedure, stroke was reported in 2%, seizures in 5%, thrombosis in 9%, cirrhosis in 4%, plastic bronchitis in 0.5%, and protein-losing enteropathy in vii%. Arrhythmia receiving handling or intervention since the Fontan surgery occurred in 118 (28%) subjects; 58% of these (north = 68) happened since Fontan one.
Hateful CHQ-PF50 summary scores for the 264 subjects <19 years were significantly lower than those of historical, healthy, control subjectsxiii (44 ± 12 vs. 53 ± 9 for physical, and 48 ± 12 vs. 51 ± 9 for psychosocial summary scores, each P < .01). However, individual scores were within the 95th percentile confidence interval of the mean of historical healthy controls in 78% of subjects for the physical summary score and in 88% for the psychosocial summary score. (Effigy three) Mean summary scores among the 153 developed subjects completing the SF-36 were not dissimilar (P > .1) from those for healthy controls: the aggregate physical score was l ± eight; 97% of Fontan 2 subjects scored within the 95th percentile confidence interval for the mean of good for you command subjects. The aggregate mental score was 49 ± 12 with 91% of Fontan two subjects scoring within the 95th percentile confidence interval of the expected mean score of l (Figure 4).
The distribution of Child Wellness Questionnaire-Parent Form (CHQ-PF50) concrete (A) and psychosocial (B) summary scores from 257 children enrolled in the Fontan 2 Written report. The line shows the normal distribution for the full general population. SD, standard departure.
The distribution of Short Class 36 (SF-36) physical (A) and mental amass (B) scores from 153 adults enrolled in the Fontan 2 Study. The line shows the normal distribution for the general population. SD, standard deviation.
Association with Ventricular Authority
The ventricular morphology was left dominant in 214, right dominant in 141, and mixed in 72 of the 427 enrolled subjects (Table 1). Subjects with a dominant correct ventricle (RV) used a greater number of medicines. Those with a dominant RV were prescribed angiotensin-converting enzyme (ACE) inhibitors more frequently compared with the others, even subsequently adjustments were made for differences by clinical middle. Parents of subjects with a dominant RV reported a lower physical functioning domain score on the CHQ-PF50, although at that place was no difference in the analogous domain reported by the subjects themselves on the CHQ-CF87. Similarly, only a single domain on either the parent or subject, Peds-QL differed by ventricular authorization. Subjects (but not their parents) with a dominant RV reported a better "treatment" score compared with those with mixed ventricular morphology. Differences were observed in 8 of the 10 scales of the SF-36 among adult subjects with those with left ventricular morphology scoring improve and those with mixed ventricular morphology scoring the poorest.
Table one
Patient Characteristics by Ventricular Dominance
| Total (northward = 427) | Left (n = 214) | Right (due north = 141) | Mixed (n = 72) | P value* | ||
|---|---|---|---|---|---|---|
| Historic period, years | Hateful ± SD | 18.4 ± 3.4 | 18.7 ± iii.3 | 17.9 ± 3.iii | xviii.5 ± 3.7 | .15† |
| Male | n (%) | 249 (58%) | 119 (56%) | 94 (67%) | 36 (50%) | .05 ‡ |
| Post-Fontan surgery medical review tape | n | 426 | 214 | 140 | 72 | |
| Age, years | Mean ± SD | 18.half-dozen ± iii.4 | 18.9 ± 3.4 | 18.1 ± 3.4 | eighteen.viii ± 3.7 | .14 |
| Follow-up time, years | Mean ± SD | 15.2 ± 3.iv | 15.five ± 3.iii | 14.8 ± 3.iv | 15.ane ± three.four | .38 |
| Cardiac surgical procedure | due north (%) | 119 (28%) | 70 (33%) | 36 (26%) | xiii (18%) | .04 |
| Cardiac cath intervention | n (%) | 242 (57%) | 118 (55%) | 84 (60%) | 40 (56%) | .51 |
| Stroke | n (%) | 10 (2%) | 5 (two%) | v (4%) | 0 (0%) | .07 |
| Seizure | n (%) | 23 (5%) | 11 (five%) | 11 (eight%) | 1 (1%) | .05 |
| Thrombosis | n (%) | 39 (9%) | 24 (eleven%) | xi (eight%) | 4 (vi%) | .47 |
| Arrhythmia | northward (%) | 118 (28%) | 62 (29%) | 32 (23%) | 24 (33%) | .68 |
| Other important complications | n (%) | 215 (50%) | 98 (46%) | 73 (52%) | 44 (61%) | .25 |
| Protein-losing enteropathy | n (%) | 30 (7%) | xi (5%) | 8 (half dozen%) | 11 (15%) | .15 |
| Cirrhosis diagnosis | n (%) | xv (4%) | 5 (2%) | seven (five%) | three (4%) | .24 |
| Plastic bronchitis diagnosis | n (%) | 2 (0.5%) | i (0.five%) | one (1%) | 0 (0%) | .99 |
| IED in place | northward (%) | 56 (13%) | 38 (18%) | 15 (11%) | 3 (4%) | .002 |
| Clinical cess | ||||||
| Percentile height for age | Hateful ± SD | 36.seven ± 30.9 | 37.5 ± 31.4 | 34.3 ± xxx.2 | 39.seven ± 31.5 | .sixty |
| Height-for-historic period z-score | Mean ± SD | −0.6 ± i.iv | −0.6 ± one.4 | −0.8 ± 1.5 | −0.4 ± 1.five | .37 |
| Percentile weight for historic period | Mean ± SD | 45.1 ± 33.eight | 44.v ± 33.5 | 44.4 ± 35.7 | 48.7 ± 30.5 | .81 |
| Weight-for-age z-score | Mean ± SD | −0.three ± 1.6 | −0.3 ± 1.5 | −0.4 ± one.vii | −0.1 ± 1.2 | .68 |
| BMI-for-age z-score | Mean ± SD | −0.0 ± i.three | 0.0 ± i.2 | −0.1 ± 1.four | 0.1 ± one.1 | .95 |
| NYHA heart failure classification | .14 | |||||
| Class I | n (%) | 234 (55%) | 125 (58%) | 74 (53%) | 35 (49%) | |
| Form Two | n (%) | 119 (28%) | 57 (27%) | 42 (30%) | 20 (28%) | |
| Class III | n (%) | 49 (12%) | 22 (10%) | fifteen (11%) | 12 (17%) | |
| Class IV | north (%) | 24 (6%) | 10 (v%) | 9 (six%) | 5 (vii%) | |
| Medications | ||||||
| Number of medications | n (%) | .002 | ||||
| 0 | n (%) | 45 (11%) | 27 (13%) | vii (5%) | xi (xv%) | |
| one | n (%) | 99 (23%) | 60 (28%) | 25 (xviii%) | 14 (19%) | |
| ≥2 | northward (%) | 282 (66%) | 127 (59%) | 108 (77%) | 47 (65%) | |
| Mean ± SD | 2.9 ± ii.half-dozen | 2.5 ± two.four | 3.iv ± 2.9 | 2.eight ± 2.5 | <.001 | |
| ACE Inhibitors | n (%) | 226 (53%) | 92 (43%) | 102 (73%) | 32 (44%) | <.001 |
| CHQ-PF50—parent report | due north | 264 | 124 | 98 | 42 | |
| Historic period, years | Mean ± SD | xvi.iii ± i.6 | sixteen.iii ± 1.6 | xvi.3 ± 1.8 | 16.0 ± i.four | .48 |
| Physical summary score | Mean ± SD | 44 ± 12 | 43 ± 13 | 44 ± 12 | 47 ± 9 | .11 |
| Psychosocial summary score | Hateful ± SD | 48 ± 12 | 49 ± 10 | 47 ± 12 | 48 ± thirteen | .61 |
| Global wellness scale | Hateful ± SD | 77 ± 21 | 77 ± 21 | 76 ± 21 | 83 ± twenty | .22 |
| Physical functioning scale | Mean ± SD | 81 ± 21 | 82 ± 21 | 78 ± 23 | 88 ± 13 | .01 |
| CHQ-CF87—child report | n | 255 | 121 | 93 | 41 | |
| Age, years | Mean ± SD | sixteen.3 ± i.6 | sixteen.iii ± 1.5 | 16.3 ± one.8 | xvi.0 ± one.4 | .46 |
| Global health calibration | Mean ± SD | 76 ± 21 | 77 ± 20 | 75 ± 24 | 76 ± 21 | .52 |
| Physical functioning scale | Hateful ± SD | 87 ± fourteen | 88 ± 14 | 86 ± 16 | 90 ± 10 | .18 |
| Peds-QL—parent written report | due north | 262 | 123 | 97 | 42 | |
| Age, years | Mean ± SD | 16.two ± 1.6 | xvi.iii ± one.6 | 16.2 ± i.8 | sixteen.0 ± 1.4 | .48 |
| iv.0 generic core scales | ||||||
| Concrete functioning score | Hateful ± SD | 72 ± 21 | 72 ± 21 | 71 ± 22 | 75 ± 20 | .45 |
| Emotional performance score | Mean ± SD | 72 ± 20 | 72 ± 20 | 71 ± 21 | 73 ± 19 | .lxx |
| Social functioning score | Hateful ± SD | 71 ± 25 | 73 ± 25 | 67 ± 25 | 74 ± 23 | .09 |
| Schoolhouse functioning score | Mean ± SD | 64 ± 21 | 64 ± 22 | 63 ± 21 | 67 ± 18 | .76 |
| Psychosocial wellness summary score | Mean ± SD | 69 ± eighteen | 70 ± 18 | 67 ± 19 | 72 ± 17 | .27 |
| Physical health summary score | Mean ± SD | 72 ± 21 | 72 ± 21 | 71 ± 22 | 75 ± 20 | .45 |
| Full generic score of pediatric quality of life | Hateful ± SD | lxx ± eighteen | 71 ± 18 | 68 ± 18 | 73 ± sixteen | .27 |
| 3.0 cardiac module | ||||||
| Heart issues and handling score | Mean ± SD | 72 ± 19 | seventy ± 20 | 73 ± xix | 75 ± twenty | .28 |
| Treatment score | Mean ± SD | 89 ± 13 | 90 ± 14 | 88 ± thirteen | xc ± 11 | .31 |
| Perceived physical appearance score | Mean ± SD | 78 ± 23 | 78 ± 23 | 77 ± 25 | 82 ± 19 | .31 |
| Treatment anxiety score | Mean ± SD | 76 ± 26 | 76 ± 27 | 76 ± 25 | 74 ± 28 | .99 |
| Cerebral problems score | Mean ± SD | 60 ± 26 | 62 ± 26 | 57 ± 27 | 64 ± 24 | .25 |
| Communication score | Mean ± SD | 71 ± 29 | 68 ± 32 | 73 ± 28 | 76 ± 23 | .22 |
| Peds-QL—child written report | n | 408 | 206 | 132 | 70 | |
| Age, years | Mean ± SD | eighteen.five ± three.four | 18.8 ± iii.iv | xviii.1 ± iii.4 | xviii.7 ± iii.7 | .23 |
| 4.0 generic cadre scales | ||||||
| Physical operation score | Hateful ± SD | 75 ± 18 | 76 ± 18 | 73 ± eighteen | 74 ± xviii | .22 |
| Emotional performance score | Mean ± SD | 74 ± 21 | 74 ± xx | 74 ± 21 | 72 ± 22 | .68 |
| Social functioning score | Hateful ± SD | 78 ± 20 | eighty ± 18 | 76 ± 23 | 78 ± 21 | .thirteen |
| Full (n = 427) | Left (n = 214) | Right (north = 141) | Mixed (due north = 72) | P value* | ||
| School functioning score | Hateful ± SD | lxx ± xx | 71 ± 19 | 71 ± 22 | 68 ± xix | .71 |
| Psychosocial wellness summary score | Mean ± SD | 74 ± 17 | 75 ± 15 | 73 ± 19 | 73 ± 17 | .46 |
| Physical health summary score | Mean ± SD | 75 ± 18 | 76 ± 18 | 73 ± 18 | 74 ± xviii | .22 |
| Full generic score of pediatric quality of life | Mean ± SD | 74 ± sixteen | 75 ± 15 | 73 ± 18 | 73 ± 17 | .29 |
| 3.0 cardiac module | ||||||
| Heart problems and treatment score | Mean ± SD | 68 ± 19 | 68 ± 19 | lxx ± 20 | 67 ± 21 | .97 |
| Treatment score | Mean ± SD | 89 ± 13 | 89 ± 14 | 92 ± ix | 84 ± 17 | .002 |
| Perceived concrete appearance score | Mean ± SD | 77 ± 24 | 77 ± 24 | 78 ± 24 | 77 ± 23 | .xc |
| Treatment feet score | Mean ± SD | 81 ± 24 | 81 ± 24 | 83 ± 24 | 78 ± 24 | .74 |
| Cognitive problems score | Mean ± SD | 67 ± 23 | 69 ± 22 | 65 ± 24 | 66 ± 22 | .25 |
| Advice score | Mean ± SD | 73 ± 25 | 72 ± 24 | 74 ± 27 | 73 ± 25 | .96 |
| SF-36—developed | north | 153 | 85 | 39 | 29 | |
| Historic period, years | Mean ± SD | 22.4 ± i.9 | 22.3 ± i.8 | 22.iv ± 2.3 | 22.vi ± 1.9 | .81 |
| Aggregated physical score, norm based | Hateful ± SD | l ± 8 | 51 ± 8 | 49 ± 9 | 48 ± 8 | .eleven |
| Aggregated mental score, norm based | Mean ± SD | 49 ± 12 | 51 ± 11 | 49 ± x | 41 ± 14 | .001 |
| Concrete functioning (transformed scale) | Mean ± SD | 80 ± twenty | 84 ± 19 | 77 ± 21 | 75 ± twenty | .04 |
| Function—physical (transformed scale) | Mean ± SD | 81 ± 24 | 86 ± 21 | 80 ± 25 | 68 ± 29 | .003 |
| Bodily pain (transformed calibration) | Mean ± SD | 77 ± 23 | 82 ± 19 | 78 ± 23 | 63 ± 26 | <.001 |
| General wellness (transformed scale) | Mean ± SD | 63 ± 22 | 64 ± 22 | 63 ± 24 | 61 ± 21 | .78 |
| Vitality (transformed calibration) | Mean ± SD | 62 ± 21 | 65 ± nineteen | 60 ± 25 | 54 ± nineteen | .03 |
| Social functioning (transformed scale) | Mean ± SD | 81 ± 24 | 86 ± 20 | 79 ± 22 | 66 ± 31 | <.001 |
| Role—emotional (transformed scale) | Mean ± SD | 82 ± 26 | 87 ± 23 | 84 ± 21 | 67 ± 33 | <.001 |
| Mental health (transformed scale) | Mean ± SD | 73 ± xx | 77 ± 18 | 73 ± 19 | 62 ± 22 | .002 |
| Socioeconomic status | n | 420 | 211 | 139 | 70 | |
| Hollingshead Score | Hateful ± SD | 44 ± 15 | 42 ± 14 | 45 ± fourteen | 47 ± 16 | .04 |
Clan with Historic period
Younger subjects (<eighteen years, n = 225) were less likely to have suffered from stroke both since their Fontan process and in the interim between Fontan 1 and Fontan ii. Interim development of thrombosis since Fontan one was more probable in the older (≥18 years, n = 202) cohort. Younger subjects were more likely to be prescribed ACE inhibitors. For the Peds-QL that included all ages, but the handling scale differed, beingness better amongst the younger accomplice.
Because the CHQ is validated only in children ≤19 years quondam, we compared results of these tests between a younger group of 120 subjects <16 years and an older group of 144 subjects xvi–nineteen years quondam. The mental health, cocky-esteem, and general health perception scales were higher (better) among the younger group equally reported by both the subjects and their parents. We also compared a younger developed group comprising 76 adult subjects xix to <22.5 years with an older group of 77 subjects ≥22.five years. No differences were seen in any of the ten reported scales of the SF-36 (Table ii).
Table ii
Associations with Age
| Younger (Age < 18) | Older (Age > xviii) | P Value* | ||
|---|---|---|---|---|
| Medical review from Fontan 1 to Fontan 2 | due north = 212 | n = 214 | ||
| Follow-up time, years | Hateful ± SD | half-dozen.8 ± 0.4 | 6.9 ± 0.5 | .32 |
| Cardiac surgical procedure | n (%) | 29 (14%) | 37 (17%) | .36 |
| Cardiac cath intervention | north (%) | 38 (xviii%) | xl (19%) | .88 |
| Stroke | n (%) | 0 (0%) | 3 (ane%) | .04 |
| Seizure | n (%) | iv (2%) | 11 (5%) | .07 |
| Thrombosis | n (%) | 2 (ane%) | 12 (six%) | .005 |
| Arrhythmia | due north (%) | 26 (12%) | 42 (twenty%) | .06 |
| Other important complications | northward (%) | 74 (35%) | 84 (39%) | .49 |
| On center transplant waiting list | n (%) | 0 (0%) | 1 (ane%) | .99 |
| IED in identify | northward (%) | 22 (10%) | 34 (16%) | .xi |
| Medical review since Fontan surgery | n = 212 | north = 214 | ||
| Follow-up time, years | Mean ± SD | 12.8 ± 1.seven | 17.viii ± 2.viii | <.001 |
| PLE | n (%) | 13 (6%) | 17 (8%) | .85 |
| Cirrhosis | north (%) | iii (one%) | 12 (6%) | .07 |
| Plastic bronchitis | due north (%) | 1 (0.v%) | i (0.5%) | .81 |
| Clinical assessment | n = 216 | n = 210 | ||
| Percentile height for historic period | Mean ± SD | 36 ± 31 | 40 ± thirty | .32 |
| Tiptop-for-age z-score | Mean ± SD | −0.seven ± 1.5 | −0.5 ± 1.two | .27 |
| Percentile weight for age | Mean ± SD | 45 ± 34 | 46 ± 34 | .72 |
| Weight-for-age z-score | Mean ± SD | −0.4 ± 1.6 | −0.2 ± 1.6 | .55 |
| BMI-for-age z-score | Hateful ± SD | 0.0 ± 1.3 | 0.0 ± 1.iii | .94 |
| NYHA heart failure nomenclature | n (%) | .25 | ||
| Class I | 123 (57%) | 111 (53%) | ||
| Class 2 | 63 (xxx%) | 56 (27%) | ||
| Class III | 22 (ten%) | 27 (13%) | ||
| Class IV | eight (4%) | xvi (seven%) | ||
| Number of medications | n (%) | .22 | ||
| 0 | 18 (8%) | 27 (13%) | ||
| 1 | 55 (25%) | 44 (21%) | ||
| ≥2 | 145 (67%) | 137 (66%) | ||
| ACE inhibitors | north (%) | 127 (58%) | 99 (48%) | .03 |
| Peds-QL—subject area report | n = 207 | n = 201 | ||
| four.0 generic core scales | ||||
| Physical functioning score | Hateful ± SD | 76 ± 17 | 73 ± xx | .16 |
| Emotional operation score | Mean ± SD | 74 ± 20 | 73 ± 21 | .51 |
| Social functioning score | Mean ± SD | 78 ± 20 | 78 ± 21 | .94 |
| Schoolhouse functioning score | Mean ± SD | 70 ± xix | 71 ± 21 | .85 |
| Psychosocial health summary score | Mean ± SD | 74 ± 16 | 74 ± 18 | .74 |
| Concrete wellness summary score | Hateful ± SD | 76 ± 17 | 73 ± 20 | .16 |
| Total generic score of pediatric quality of life | Hateful ± SD | 75 ± xv | 74 ± 17 | .41 |
| 3.0 cardiac module | ||||
| Heart issues and treatment score | Mean ± SD | 70 ± 19 | 66 ± 20 | .06 |
| Treatment score | Hateful ± SD | 91 ± 11 | 87 ± fifteen | .001 |
| Perceived concrete appearance score | Mean ± SD | 79 ± 22 | 75 ± 26 | .xiii |
| Treatment anxiety score | Mean ± SD | 81 ± 24 | 81 ± 24 | .86 |
| Cognitive problems score | Mean ± SD | 69 ± 22 | 65 ± 24 | .08 |
| Advice score | Mean ± SD | 73 ± 24 | 72 ± 26 | .65 |
| Socioeconomic condition | n = 214 | n = 206 | ||
| Hollingshead score | Mean ± SD | 45 ± xiv | 43 ± xv | .19 |
| Median (IQR) | 47 (35, 56) | 45 (33, 55) | .19 | |
Association with Fontan Blazon
Fontan procedure types included atriopulmonary connection (APC, north = 54), total cavopulmonary connection intracardiac lateral tunnel (LT, n = 251), and total cavopulmonary connection extracardiac conduit (ECC, due north = 111). Afterwards aligning for historic period, subjects with an APC were less likely to take had a cardiac catheter based intervention since the Fontan process (31% APC vs. 59% LT vs. 65% ECC), P = .004. However, catheter interventions since Fontan 1 were not different between groups, P = .iv. The incidence of arrhythmia since the Fontan process was higher in APC subjects (44% APC vs. 28% LT vs. 19% ECC), but this was not meaning after bookkeeping for age, P = .four. However, the development of new arrhythmia requiring handling in the interim period between Fontan ane and Fontan 2 was significantly higher in APC subjects even after accounting for age (thirty% APC vs. 16% LT vs. 10% ECC), P = .049.
Characteristics of Health Care
There was a significant departure between sites for the frequency of a secondary source of insurance (P < .001). The rate was much college for Canadian subjects (79%) than for those from Us sites (10–26%). The vast majority of subjects (96%) take visited a cardiologist within the past ii years. Nosotros compared 295 subjects with a visit frequency of ≤1 visit per year with 125 subjects with ≥2 visits per year. In multivariable modeling, the only associated factor for more frequent visits was clinical site (P < .001, R two = 0.10). The well-nigh prevalent medical services reported by subjects were dental intendance (92%), ophthalmology (62%), educational back up (36%), gynecology (32% of the females), mental health (19%), and dermatology and neurology (15% each) (Tabular array 3).
Table 3
Characteristics of Health Care
| n (%) | |
|---|---|
| Health insurance (due north = 420) | |
| Yes | 406 (97%) |
| No | 14 (iii%) |
| Principal insurance type (north = 406) | |
| Private | 214 (53%) |
| Public | 192 (47%) |
| Secondary source of health insurance (north = 405) | |
| Yes | 134 (33%) |
| No | 271 (67%) |
| Visited a cardiologist in the by 2 years (n = 420) | |
| Yes | 404 (96%) |
| No | 16 (4%) |
| Frequency of cardiologist visits (n = 404) | |
| Less than once a yr | 41 (10%) |
| One time a year | 238 (59%) |
| Twice a year | 85 (21%) |
| More than than twice a yr | 40 (10%) |
| Visited an adult cardiologist in the by two years* (north = 284) | |
| Yes | 86 (30%) |
| No | 198 (seventy%) |
| Medical services received in the past 2 years (n = 420) | |
| Dental care | 385 (92%) |
| Ophthalmology | 261 (62%) |
| Education support | 150 (36%) |
| Obstetrician/gynecologist (n = 203)† | 64 (32%) |
| Mental health | 80 (19%) |
| Dermatology | 64 (15%) |
| Neurology | 61 (15%) |
| Orthopedic | 59 (14%) |
| Rehabilitation/physical therapy | 56 (13%) |
| Otolaryngology | 43 (10%) |
| Pulmonology | 42 (ten%) |
| Occupational therapy | 37 (9%) |
| Speech therapy | 37 (9%) |
| Endocrine | 27 (vi%) |
| Diet | 27 (6%) |
| Allergy services | 26 (6%) |
| Renal | 18 (4%) |
| Whatever other | 70 (17%) |
Discussion
Longitudinal studies that involve children with chronic diseases transitioning to adulthood are uncommon. This is the largest report of a prospectively followed multicenter accomplice of children with univentricular physiology palliated with a Fontan process. We establish that only two% were lost to follow-up and 83% of the original accomplice were willing to participate. Older patients and males were less likely to agree to participate. This is consequent with challenges that are faced in the transition of clinical care from pediatric cardiologists to developed congenital center illness specialists.fourteen
We plant 95% interim transplant-free survival over an average of 7 years of follow-up since enrollment in the Fontan one study. Those with poorer results during the baseline cess at Fontan i were more likely to have died or be transplanted. Specifically, we plant death or transplant to be associated with worse performance on previously administered CHQ-PF50 physical summary score, a more dilated ventricle as measured by echocardiography, and higher measured BNP assessed at Fontan 1. Previous studies examining vital status over fourth dimension for Fontan survivors and predictors of expiry or transplant have been express to single center reports that bridge big time periods (east.chiliad., 25 years)15 or accept included a much shorter length of follow-up (eastward.g., <2years).16 Our information show a slightly lower incidence of decease and transplant than a previous study of 321 surviving Fontan subjects with a hateful age of 21 years.sixteen
At that place have been concerns that patients with a Fontan circulation are destined to take an increasing take a chance over time for death or middle transplantation17 every bit well as a decline in ventricular performance and health status.xviii Rates of decline have been speculated to be greater among those with dominant RV morphology.xix,20 We did not find RV ascendant subjects to take a greater likelihood of death or transplant over the period of written report. This is in contrast to a cohort of earlier era Fontan subjects born before 1985 and followed for a median of 12 years in whom a ten-fold chance of heart failure death was seen in those with a morphologic RV.15 Nosotros did find an increase in the use of ACE inhibitors in both younger subjects and those with a dominant RV. Nosotros speculate this is a departure in do patterns that exists despite the lack of prove to suggest benefit.21 Although no of import differences in quality of life or functional wellness status were related to ventricular dominance among the children in our study, we did detect college (better) scores in 8 of 10 scales from the SF-36 among the adults with left ventricular authorisation.
The strength of this electric current report is its compatible median follow-up period for all subjects. This immune united states of america to compare outcomes since the Fontan completion (median xv ± three.4 years) as well as in the acting since Fontan 1 (6.viii ± 0.iv years). Subjects with an APC type of Fontan had fewer interventional catheter procedures betwixt Fontan completion and Fontan 1 cess compared with other Fontan types, a difference that was not observed in the interim menstruum since assessment at Fontan 1. This is likely due to a much higher rate of surgical fenestration in non-APC Fontan types with planned procedures to close these in the outset year or and then afterwards Fontan surgery.22 We were able to confirm previous reports that ongoing take a chance of development of arrhythmia is greater amidst subjects with an APC compared with other Fontan types.23
Quality-of-life studies complement mortality and morbidity data past taking physical, psychological, and social factors into consideration.24 Although the hateful CHQ-PF50 physical summary score was lower than those of salubrious control subjects, a large majority of boyish subjects (every bit reported past a parent) proceed to score within the normal range. Comparisons amid adults in our cohort evaluated by the SF-36 reveal even fewer differences compared with self-reported historic norms. These results may exist explained by coping mechanisms in the adult subjects resulting in a higher appreciation of health condition, which is exemplified by higher scores.25 This may too reflect the known discordance when comparing parent-completed surveys to discipline-completed surveys.26
Because of the nature of this observational cohort, we cannot exclude the possibility that the everyman performing subjects are less likely to survive with a Fontan circulation into adulthood.
A significant limitation illustrated in our report is that instruments to study functional wellness outcomes are validated only for specific age groups. There is not a unmarried musical instrument that includes the total age range of subjects who are transitioning from the teenage years to adulthood. The CHQ produces scores for 14 domains and two summary scores (physical operation and psychosocial functioning). Our results for the mean concrete summary score for Fontan subjects (44 ± 12) are similar to those subjects with asthma (48 ± 7), juvenile rheumatoid arthritis (42 ± 14), and epilepsy (48 ± 14). The same is truthful for the psychosocial summary scores, 48 ± 12 in our Fontan group, asthma (52 ± 8), epilepsy (47 ± 12), and attention deficit disorder (37 ± eleven).13 The SF-36 is a multipurpose, 36-item adult health survey that yields an 8-calibration profile of functional health and well-being scores as well as psychometrically based physical and mental health summary measures. The results of our cohort of adult subjects showed like scores when compared with a recent study involving 54 Danish subjects with a Fontan circulation.27 A recent systematic review of quality-of-life studies in adult congenital heart disease patients suggests that, in general, quality of life is compromised in the concrete domains compared with norms and less so in relation to psychosocial and environmental/occupational domains.28 These findings are supported by our results. Correlations are needed betwixt individual instruments that assess functional health status and quality of life across the transition from child to adult.
The results of this study must be viewed in light of certain limitations. The original Fontan 1 cohort was past necessity a subset of Fontan survivors. The time frame between Fontan i and 2 may have been a period of relative clinical stability and too short to observe meaningful changes in functional health condition and medical weather condition. As stated previously, in that location are no measurements of functional health status, which are validated across the full historic period range of the subjects being studied. Comparisons between younger and older groups of subjects divided the cohort into similar-sized groups. If this separation was made in different ways, our findings related to age might be unlike. The nowadays study was limited to assessment of functional wellness status and acting medical history. Our assessment was likewise restricted to the 85% of eligible Fontan i survivors who agreed to participate in this follow-upwards written report. Repeat laboratory testing and comparisons of these measures over time, which were not assessed at Fontan ii, volition be the subject of time to come research.
This is the largest report of a prospectively followed multicenter accomplice of children with univentricular physiology palliated with a Fontan process and provides a gimmicky overview of vital condition, functional health condition, and acting medical history in electric current survivors of the Fontan process. Incidence of death or transplantation is low and associated with prior poor functional wellness status and sure measures of ventricular performance. Functional health status was inside normal range in the majority of subjects. Information from this study will permit useful comparisons with the information from single ventricle subjects being followed prospectively from nascency in other studies. Continued longitudinal investigation into adulthood is necessary to meliorate understand these patients and improve their long-term outcomes and functional health status.
Acknowledgments
The authors would similar to thank the post-obit:
National Centre, Lung, and Blood Institute: Gail Pearson, Mario Stylianou, Jonathan Kaltman, Victoria Pemberton
Data Analogous Center: New England Research Institutes, Lynn Sleeper, Steven Colan, Dianne Gallagher, Victor Zak, Peter Shrader
Protocol Chair: Lynn Mahony, University of Texas Southwestern Medical Center
Clinical Site Investigators: Children'southward Hospital Boston, Jane Newburger (Chief Investigator), Roger Breitbart, Carolyn Dunbar-Masterson, Jill Handisides, Lisa-Jean Buckley, Bethany Trainor; Children'south Hospital of New York, Wyman W. Lai (Principal Investigator), Rosalind Korsin; Children's Infirmary of Philadelphia, Robert Shaddy, J William Gaynor, Stephen M Paridon, (Master Investigators), Kaitlyn Daniels, Tonia Morrison, Nicole Mirarchi * ;Duke University, Jennifer S. Li (Principal Investigator), Piers Barker, Mingfen Xu; Medical University of South Carolina, J. Philip Saul (Master Investigator), Andrew Grand. Atz, Patricia Infinger, Ann Harvey Frampton; Primary Children'due south Medical Center, Common salt Lake City, Utah, LuAnn Minich (Principal Investigator), Richard Williams, Linda Lambert; Hospital for Sick Children, Toronto, Brian McCrindle (Chief Investigator), Elizabeth Radojewski, Svetlana Khaikin, Patricia Walter; Cincinnati Children's Medical Center, Bradley Marino, Karen Uzark * .
Protocol Review Committee: Michael Artman, Chair; Timothy Feltes, Julie Johnson * , Jeffrey Krischer, G. Paul Matherne, Nakela Cook *
Data and Safety Monitoring Board: John Kugler, Chair; David Gordon, David J. Driscoll, Mark Galantowicz, Emerge A. Hunsberger, Holly Taylor, Thomas J. Knight, Catherine L. Webb * .
Funding: Supported by U01 grants from the National Middle, Lung, and Blood Establish (HL068269, HL068270, HL068279, HL068281, HL068285, HL068292, HL068290, HL068288).
Footnotes
*No longer at the establishment listed.
Authors' Contributions
Andrew M. Atz, Dr.: Concept/design, data analysis/ estimation, drafting article, disquisitional revision of article, approval of article, data collection.
Victor Zak, PhD: Concept/pattern, data analysis/ interpretation, drafting article, statistics, critical revision of article, blessing of article.
Lynn Mahony, Medico: Concept/design, critical revision of article, approval of article.
Karen Uzark, PhD: Concept/design, data interpretation, critical revision of article, approval of commodity.
Peter Shrader, MA: Data analysis/estimation, drafting article, statistics, critical revision of article, approval of article.
Dianne Gallagher, MS: Concept/design, data analysis/ interpretation, data collection, critical revision of article, blessing of commodity.
Stephen M. Paridon, MD: Concept/design, data estimation, disquisitional revision of article, approval of article.
Richard Five. Williams, Physician: Concept/design, information estimation, critical revision of commodity, approval of article.
Roger E. Breitbart, MD: Concept/blueprint, information estimation, critical revision of article, approval of article.
Steven D. Colan, Medico: Concept/design, data analysis/ interpretation, critical revision of article, approval of article.
Jonathan R. Kaltman, MD: Concept/design, data analysis/ interpretation, disquisitional revision of article, blessing of article.
Renee Margossian, Doc: Concept/design, data analysis/ interpretation, critical revision of article, approval of article.
Sara Yard. Pasquali, MD, MHS: Data analysis/interpretation, critical revision of article, approving of article.
Kerstin Allen, MS: Concept/design, data analysis/ interpretation, critical revision of article, blessing of commodity.
Wyman W. Lai, Physician, MPH: Concept/design, information interpretation, critical revision of article, approval of article.
Rosalind Korsin, RN: Concept/design, critical revision of article, information drove, blessing of article.
Bradley S. Marino, Doc: Concept/blueprint, data analysis/ estimation, critical revision of article, approving of article.
Nicole Mirarchi, RN: Concept/pattern, information collection, critical revision of article, approval of article.
Brian W. McCrindle, Doc, MPH: Concept/pattern, data analysis/interpretation, drafting article, disquisitional revision of article, approval of article.
Disharmonize of involvement: None.
This work is solely the responsibility of the authors and does non necessarily stand for the official views of the National Institutes of Health/ National Heart, Lung, and Blood Plant.
Clinical Trials Registration number: {"type":"clinical-trial","attrs":{"text":"NCT00132782","term_id":"NCT00132782"}}NCT00132782
No human relationship with industry exists.
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Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4414014/
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