A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease

AbstractChildren with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care – structured evaluation, psychosocial/spiritual, and communication support before surgery – or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (−7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.</jats:p

Family Stresses After Pediatric Heart Transplantation

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/73984/1/j.1751-7117.1989.tb00572.x.pd

Survival Data and Predictors of Functional Outcome an Average of 15 Years after the Fontan Procedure: The Pediatric Heart Network Fontan Cohort

ObjectiveMulticenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow‐up data in a previously well‐characterized cohort.DesignBaseline data from the Fontan Cross‐Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant‐free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care.ResultsThirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9–13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow‐up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within 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 interim 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 (n = 68) since Fontan 1.ConclusionsWe found 95% interim transplant‐free survival for Fontan survivors over an average of 7 years of follow‐up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long‐term outcomes and to improve functional health status.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/110738/1/chd12193.pd

Longitudinal Assessment of Growth in Hypoplastic Left Heart Syndrome: Results From the Single Ventricle Reconstruction Trial

Background: We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. Methods and Results: We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients 2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient‐ and stage‐specific targets. Clinical Trial Registration URL: http://clinicaltrials.gov/. Unique identifier: NCT00115934

Potential for reversibility of pulmonary vascular obstructive disease in children after cardiac transplantation

Patients with pulmonary vascular obstructive disease (PVOD) are usually not considered candidates for orthotopic cardiac transplantation, because the normal donor's right ventricle may be unable to function because of an acute increase in afterload, especially in the postischemic situation of the arrested and transported donar heart. The accepted guideline is that pulmonary vascular resistance (PVR) must be 8 Wood units (mm Hg/liters/min) or lower during maximal medical management.1,2 Patients whose PVR is between 4 and 8 Wood units are marginal candidates. Combined heart-lung transplantation or heterotopic cardiac transplantation is generally believed to be necessary when the PVR is 8 units. However, among children with a variety of congenital defects, PVOD is often reversible after correction, especially when correction is performed at a young age. This was the case in the patient described herein whose cardiac transplantation was successful despite a PVR of nearly 13 Wood units.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/25979/1/0000045.pd

Aquatic therapy for children with Duchenne muscular dystrophy: a pilot feasibility randomised controlled trial and mixed-methods process evaluation.

BACKGROUND: Duchenne muscular dystrophy (DMD) is a rare disease that causes the progressive loss of motor abilities such as walking. Standard treatment includes physiotherapy. No trial has evaluated whether or not adding aquatic therapy (AT) to land-based therapy (LBT) exercises helps to keep muscles strong and children independent. OBJECTIVES: To assess the feasibility of recruiting boys with DMD to a randomised trial evaluating AT (primary objective) and to collect data from them; to assess how, and how well, the intervention and trial procedures work. DESIGN: Parallel-group, single-blind, randomised pilot trial with nested qualitative research. SETTING: Six paediatric neuromuscular units. PARTICIPANTS: Children with DMD aged 7-16 years, established on corticosteroids, with a North Star Ambulatory Assessment (NSAA) score of 8-34 and able to complete a 10-m walk without aids/assistance. Exclusions: > 20% variation between baseline screens 4 weeks apart and contraindications. INTERVENTIONS: Participants were allocated on a 1 : 1 ratio to (1) optimised, manualised LBT (prescribed by specialist neuromuscular physiotherapists) or (2) the same plus manualised AT (30 minutes, twice weekly for 6 months: active assisted and/or passive stretching regime; simulated or real functional activities; submaximal exercise). Semistructured interviews with participants, parents (n = 8) and professionals (n = 8) were analysed using Framework analysis. An independent rater reviewed patient records to determine the extent to which treatment was optimised. A cost-impact analysis was performed. Quantitative and qualitative data were mixed using a triangulation exercise. MAIN OUTCOME MEASURES: Feasibility of recruiting 40 participants in 6 months, participant and therapist views on the acceptability of the intervention and research protocols, clinical outcomes including NSAA, independent assessment of treatment optimisation and intervention costs. RESULTS: Over 6 months, 348 children were screened - most lived too far from centres or were enrolled in other trials. Twelve (30% of target) were randomised to AT (n = 8) or control (n = 4). People in the AT (n = 8) and control (n = 2: attrition because of parental report) arms contributed outcome data. The mean change in NSAA score at 6 months was -5.5 [standard deviation (SD) 7.8] for LBT and -2.8 (SD 4.1) in the AT arm. One boy suffered pain and fatigue after AT, which resolved the same day. Physiotherapists and parents valued AT and believed that it should be delivered in community settings. The independent rater considered AT optimised for three out of eight children, with other children given programmes that were too extensive and insufficiently focused. The estimated NHS costs of 6-month service were between £1970 and £2734 per patient. LIMITATIONS: The focus on delivery in hospitals limits generalisability. CONCLUSIONS: Neither a full-scale frequentist randomised controlled trial (RCT) recruiting in the UK alone nor a twice-weekly open-ended AT course delivered at tertiary centres is feasible. Further intervention development research is needed to identify how community-based pools can be accessed, and how families can link with each other and community physiotherapists to access tailored AT programmes guided by highly specialised physiotherapists. Bayesian RCTs may be feasible; otherwise, time series designs are recommended. TRIAL REGISTRATION: Current Controlled Trials ISRCTN41002956. FUNDING: This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 21, No. 27. See the NIHR Journals Library website for further project information

Use of videotape to promote parenting of infants with serious congenital heart defects

Parents of infants with serious congenital heart defects have misconceptions and negative feelings which may threaten their ability to provide optimal care for their infant. In an effort to increase knowledge and promote a more positive, less anxious attitude among these parents, a videotape entitled `Your Baby with a Congenital Heart Defect' was developed and evaluated. Forty-three parents of thirty infants who required cardiac catherization in the first 8, weeks of life were randomly assigned to experimental (N = 23) or control (N = 20) groups. In the videotape viewed by the experimental parents, three families of cardiac infants related common feelings, problems and infant care experiences. The control group viewed a videotape describing the cardiac anatomy and functional changes associated with defects. Post-videotape questionnaires demonstrated significantly greater knowledge of the infant's behavior and needs in the experimental parents compared to controls (P P P P P = 0.02). We conclude that viewing the videotape on infant care improved parental attitude and their understanding of the behavior and needs of the neonate with congenital heart disease.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/25644/1/0000196.pd

Generalized and specific anxiety in adolescents following heart transplant

Mental health concerns are associated with worse outcomes after adult heart transplant. Illness‐specific anxiety is associated with worsened psychological well‐being after other solid organ transplants but has never been characterized after pediatric heart transplant. This single‐center cross‐sectional study aimed to evaluate illness‐specific and generalized anxiety after heart transplantation in adolescents. A novel 12‐item PHTF, GAD‐7, and the PedsQL were administered. Univariate associations of demographics, clinical features, and medication adherence as measured by immunosuppression standard deviation with the PHTF and GAD‐7 scores were evaluated. Internal consistency and validity of the PHTF were examined. In total, 30 patients participated. The most common illness‐specific fears were retransplantation, rejection, and more generally post‐transplant complications. The PHTF had good internal consistency (Cronbach α = .88). Construct validity was demonstrated between PHTF and GAD‐7 (r = .62) and PedsQL (r = −.54 to −.62). 23% endorsed moderate to severe generalized anxiety symptoms. More severe symptoms were associated with older age at survey (P = .03), older age at listing (P = .01) and having post‐transplant complications (P = .004). Patients with moderate or severe symptoms were more likely to report late immunosuppression doses (P = .004). Illness‐specific and generalized anxiety may be prevalent after pediatric heart transplant. Screening for anxiety in adolescents post‐transplant may identify those at risk for adverse outcomes including non‐adherence. The PHTF is a brief, valid, and reliable instrument identifying illness‐specific anxiety in this population.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/153669/1/petr13647.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/153669/2/petr13647_am.pd

Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation

ObjectiveTo develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).DesignUnder the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RANDâ UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an â open comment period.â Final approval of each QM was provided by a vote of the ACC ACPC Council.PatientsPatients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded.ResultsTwelve candidate QMs were generated. Seven metrics passed the RANDâ UCLA process. Four passed the â open comment periodâ and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age.ConclusionsApplication of the RANDâ UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/142334/1/chd12540_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/142334/2/chd12540.pd