Family Stresses After Pediatric Heart Transplantation

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

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

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

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

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

Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: A multicenter crossâ sectional study

IntroductionWe sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors.MethodsThis was a crossâ sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL). Mixed effects regression modeling adjusting for clustering by center was used to evaluate factors associated with abnormal anthropometry and delayed puberty and associations with QoL.ResultsOf the 299 subjects, 42% were female. The median enrollment age was 13.9 years, and the median age at Fontan was 3 years. Fontan survivors had a higher prevalence of short stature relative to normative data (20% vs 5%, P 2 surgeries before Fontan was associated with delayed puberty. Lower family income (<$25 000) and hypoplastic left heart syndrome were associated with lower QoL.ConclusionCompared to the normal population, Fontan survivors have high prevalence of short stature, abnormal BMI and delayed puberty. Abnormal anthropometry, but not delayed puberty, was associated with lower overall QoL and perceived physical appearance scores. Routine screening for abnormal anthropometry, especially in HLHS and in lower socioeconomic status families, should be considered to allow interventions, which might ameliorate the negative psychosocial impact.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/144293/1/chd12597.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/144293/2/chd12597_am.pd

Measuring quality of life in muscular dystrophy

OBJECTIVES: The objectives of this study were to develop a conceptual model of quality of life (QOL) in muscular dystrophies (MDs) and review existing QOL measures for use in the MD population. METHODS: Our model for QOL among individuals with MD was developed based on a modified Delphi process, literature review, and input from patients and patient advocacy organizations. Scales that have been used to measure QOL among patients with MD were identified through a literature review and evaluated using the COSMIN (Consensus-Based Standards for the Selection of Health Measurement Instruments) checklist. RESULTS: The Comprehensive Model of QOL in MD (CMQM) captures 3 broad domains of QOL (physical, psychological, and social), includes factors influencing self-reported QOL (disease-related factors, support/resources, and expectations/aspirations), and places these concepts within the context of the life course. The literature review identified 15 QOL scales (9 adult and 6 pediatric) that have been applied to patients with MD. Very few studies reported reliability data, and none included data on responsiveness of the measures to change in disease progression, a necessary psychometric property for measures included in treatment and intervention studies. No scales captured all QOL domains identified in the CMQM model. CONCLUSIONS: Additional scale development research is needed to enhance assessment of QOL for individuals with MD. Item banking and computerized adaptive assessment would be particularly beneficial by allowing the scale to be tailored to each individual, thereby minimizing respondent burden