New insights on growth trajectory in infants with complex congenital heart disease.

PURPOSE: We aimed to describe the weight-for-age Z-score growth trajectory (WAZ-GT) of infants with complex congenital heart disease (cCHD) after neonatal cardiac surgery in the first 4 months of life and assess potential risk factors. METHODS: We utilized data from a previously reported trial of the REACH telehealth home monitoring (NCT01941667) program which evaluated 178 infants with cCHD from 2012 to 2017. Over the first 4 months of life, weekly infant weights were converted to WAZ. WAZ-GT classes were identified using latent class growth modeling. Multinomial logistic regression models were used to examine the associations between potential risk factors and WAZ-GT classes. RESULTS: Four distinct classes of WAZ-GT were identified: maintaining WAZ > 0, 14%; stable around WAZ = 0, 35%; partially recovered, 28%; never recovered, 23%. Compared with reference group "stable around WAZ=0," we identified clinical and sociodemographic determinants of class membership for the three remaining groups. "Maintaining WAZ > 0" had greater odds of having biventricular physiology, borderline appetite, and a parent with at least a college education. "Partially recovered" had greater odds of hospital length of stay>14 days and being a single child in the household. "Never recovered" had greater odds hospital length of stay >14 and > 30 days, tube feeding at discharge, and low appetite. CONCLUSIONS: This study described distinct classes of WAZ-GT for infants with cCHD early in infancy and identified associated determinants. PRACTICE IMPLICATIONS: Findings from this study can be used in the identification of infants at risk of poor WAZ-GT and in the design of interventions to target growth in this vulnerable patient population.

Telehealth Home Monitoring and Postcardiac Surgery for Congenital Heart Disease.

OBJECTIVES: To test the effect of a 4-month telehealth home monitoring program (REACH), layered on usual care, on postdischarge outcomes in parents of infants recovering from cardiac surgery and their infants. METHODS: Randomized trial of infants discharged from the hospital after cardiac surgery for congenital heart disease. Consecutive infants with complex congenital heart disease undergoing cardiac surgery within 21 days of life were enrolled at 3 university-affiliated pediatric cardiac centers. RESULTS: From 2012 to 2016, 219 parent-infant dyads were enrolled; 109 were randomly assigned to the intervention group and 110 to the control group. At 4 months postdischarge, parenting stress was not significantly different between groups (total Parenting Stress Index in the intervention group was 220 and in the control group was 215; P = .61). The percentages of parents who met posttraumatic stress disorder (PTSD) criteria and parent quality of life inventory scores were also not significantly different between the 2 groups (PTSD in the intervention group was 18% and was 18% in the control group; P =.56; the mean Ulm Quality of Life Inventory for Parents in the intervention group was 71 andwas 70 in the control group; P = .88). Infant growth in both groups was suboptimal (the mean weight-for-age z scores were -1.1 in the intervention group and -1.2 in the control group; P = .56), and more infants in the intervention group were readmitted to the hospital (66% in the intervention group versus 57% in the control group; P < .001). CONCLUSIONS: When added to usual care, the REACH intervention was not associated with an improvement in parent or infant outcomes. Four months after neonatal heart surgery, ∼20% of parents demonstrate PTSD symptoms. Suboptimal infant growth and hospital readmissions were common.

Association of a Home Monitoring Program With Interstage and Stage 2 Outcomes.

Background In shunt-dependent, single-ventricle patients, mortality remains high in the interstage period between discharge after neonatal surgery and stage 2 operation. We sought to evaluate the impact of our infant single-ventricle management and monitoring program ( ISVMP ) on interstage mortality and stage 2 outcomes. Methods and Results This retrospective single-center cohort study compared patients enrolled in ISVMP at hospital discharge with historical controls. The relationship of ISVMP to interstage mortality was determined with a bivariate probit model for the joint modeling of both groups, using an instrumental variables approach. We included 166 ISVMP participants (December 1, 2010, to June 30, 2015) and 168 controls (January 1, 2007, to November 30, 2010). The groups did not differ by anatomy, gender, race, or genetic syndrome. Mortality was lower in the ISVMP group (5.4%) versus controls (13%). An ISVMP infant compared with a historical control had an average 29% lower predicted probability of interstage death (adjusted probability: -0.29; 95% CI , -0.52 to -0.057; P=0.015). On stratified analysis, mortality was lower in the hypoplastic left heart syndrome subgroup undergoing Norwood operation (4/84 [4.8%] versus 12/90 [14%], P=0.03) but not in those with initial palliation of shunt only ( P=0.90). ISVMP participants were younger at the time of the stage 2 operation (138 versus 160 days, P<0.001), with no difference in postoperative mortality or length of stay. Conclusions In this single-center study, we report significantly lower interstage mortality for participants with hypoplastic left heart syndrome enrolled in ISVMP . Younger age at stage 2 operation was not associated with postoperative mortality or longer length of stay.

The REACH protocol: an innovative strategy for home management of infants with complex CHD.

Approximately 32,000 infants are born with CHDs each year in the United States of America. Of every 1000 live births, 2.3 require surgical or transcatheter intervention in the first year of life. There are few more stressful times for parents than when their neonate receives a diagnosis of complex CHD requiring surgery. The stress of caring for these infants is often unrelenting and may last for weeks, months, and often years, placing parents at risk for developing post-traumatic stress disorder, as well as a drastic decrease in quality of life. Anxiety often peaks in the days and weeks after discharge from the hospital as families no longer have immediate access to nursing and medical staff. The purpose of this paper is to describe the methods of a randomised controlled trial that was designed to determine whether REACH would favourably affect parental and infant outcomes by decreasing parental stress, improve parental quality of life, increase infant stability, and decrease resource utilisation in infants with complex CHD.

What is the best measure of daytime sleepiness in adults with heart failure?

PURPOSE: To identify the best screening measure of daytime sleepiness in adults with heart failure (HF). DATA SOURCES: A total of 280 adults with HF completed the Epworth Sleepiness Scale, the Stanford Sleepiness Scale, and a single Likert item measuring daytime sleepiness. The sensitivity and specificity of these self-report measures were assessed in relation to a measure of daytime dysfunction from poor sleep quality. CONCLUSIONS: Only 16% of the sample reported significant daytime dysfunction because of poor sleep quality. Those reporting daytime dysfunction were likely to be younger (p < .001), to be unmarried (p = .002), to have New York Heart Association (NYHA) functional class IV HF (p = .015), and to report low income (p = .006) and fewer hours of sleep (p = .015). The measure of daytime sleepiness that was most sensitive to daytime dysfunction was a single Likert item measured on a 10-point (1-10) scale. Patients with a score ≥4 were 2.4 times more likely to have daytime dysfunction than those with a score <4. IMPLICATIONS FOR PRACTICE: Complaints of daytime dysfunction because of poor sleep are not common in adults with HF. Routine use of a single question about daytime sleepiness can help nurse practitioners to identify those HF patients with significant sleep issues that may require further screening.

Development of an international research agenda for adult congenital heart disease nursing.

BACKGROUND: Since the population of adults with congenital heart disease (CHD) is growing, the role of nurse specialists is expanding. In order to advance ACHD nursing, the establishment of an international nursing research agenda is recommended. We aimed to investigate research priorities as perceived by nurse specialists and researchers in ACHD. METHODS: We applied a sequential quan-qual design. In the quantitative phase, a two-round Delphi study was conducted, in which 37 nurse specialists and nurse researchers in ACHD care participated. Respondents assessed the level of priority of 21 research topics using a 9-point rating scale (1 = no priority at all; 9 = very high priority). In the qualitative phase, semi-structured interviews were performed with six selected Delphi panelists, to scrutinize pending research questions. RESULTS: This study revealed that priority should be given to studies investigating knowledge and education of patients, outcomes of Advanced Practice Nursing, quality of life, transfer and transition, and illness experiences and psychosocial issues in adults with CHD. A low priority was given to post-operative pain, sexual functioning, transplantation in ACHD, and health care costs and utilization. Agreement about the level of priority was obtained for 14 out of 21 research topics. CONCLUSION: Based on this study, we could develop an international research agenda for ACHD. Researchers ought to focus on these areas of highest priority, in order to expand and strengthen the body of knowledge in ACHD nursing.

Excessive daytime sleepiness is associated with poor medication adherence in adults with heart failure.

BACKGROUND: A relationship between excessive daytime sleepiness (EDS) and poor treatment adherence has been suspected but not confirmed. We hypothesized that medication adherence would be poorer in adults with heart failure (HF) and EDS and that cognitive status would be the mechanism of effect. METHODS AND RESULTS: A sample of 280 adults with chronic HF were enrolled into a prospective cohort comparison study. We identified a cohort with EDS and a control group without EDS and further divided both groups into those with and without mild cognitive decline. Data on medication adherence were obtained at baseline and 3 and 6 months by using the Basel Assessment of Adherence Scale. Regression analysis was used to clarify the contribution of EDS and cognition to medication adherence and to assess relationships over 6 months after adjusting for age, enrollment site, gender, race, functional class, depression, and premorbid intellect. At baseline, 62% of subjects were nonadherent to their medication regime. Nonadherence was significantly more common in those with EDS, regardless of cognitive status (P = .035). The odds of nonadherence increased by 11% for each unit increase in EDS (adjusted odds ratio 1.11; 95% confidence interval 1.05-1.19; P = .001). In longitudinal models, there was a 10% increase in the odds of nonadherence for each unit increase in EDS (P = .008). The only cognition measure significantly associated with medication adherence was attention (P = .047). CONCLUSIONS: Adults with HF and EDS are more likely to have problems adhering to their medication regimen than those without EDS, regardless of their cognitive status. Identifying and correcting factors that interfere with sleep may improve medication adherence.

What do cardiovascular nurses know about the hematological management of patients with Eisenmenger syndrome?

AIM: We investigated the level of knowledge of hematological management of patients with Eisenmenger syndrome among general cardiovascular nurses and nurses who specialize in congenital heart disease (CHD). METHODS: We conducted a survey at two international conferences attended by cardiovascular nurses. Nurses were asked to complete a questionnaire comprising two questions and three clinical case scenarios. Overall, 89 nurses participated (response rate 90.8%), 43 of whom specialized in CHD. RESULTS: The level of knowledge displayed among cardiovascular nurses is poor. About one-third of nurses not specialized in CHD recognized the definition of Eisenmenger syndrome and knew what normal hematocrit levels are. With respect to the cases presented, less than 10% of the nurses could give a correct answer. The level of knowledge of specialized nurses was significantly higher, but also here, important gaps in the level of knowledge could be observed. Less than two-thirds knew the reference values of hematocrit and knew the appropriate management in two cases. Less than half of the specialized nurses knew about the procedure of isovolumic phlebotomy. CONCLUSION: The level of knowledge displayed by cardiovascular nurses regarding the hematological management of patients with Eisenmenger syndrome is poor. Also the knowledge of nurses specialized in CHD could be improved.

Attitude toward and current practice of transfer and transition of adolescents with congenital heart disease in the United States of America and Europe.

The objective of this study was to explore the current practice and attitudes of pediatric cardiologists in the United States and Europe on the transfer and transition of children with congenital heart disease (CHD). A survey among pediatric cardiology programs in the United States and Europe was undertaken. Sixty-nine centers completed and returned the 61-item questionnaire that was specifically devised for this survey. Of 69 participating centers, 74% reported that they transfer their patients to adult-focused care. When a center transfers its patients, 80% transfer them to a formalized Adult Congenital Heart Disease Program. The median age of transfer is 18 years. Comorbidities, pregnancy, and patient/family request to leave pediatric cardiology were identified as initiators for transfer. Complexity of the heart defect was relatively less important when deciding whether to transfer patients. Only one-third of the centers that transfer their patients provide a structured preparation for patients and family. Development of a formal transition program is planned at 59% of the centers that transfer patients. In conclusion, timely transfer and a structured transition process of children with CHD are not implemented in all pediatric cardiology programs. Health-care providers working in pediatric cardiology should make their transfer policies explicit and transition programs ought to be developed.

Guidelines for the outpatient management of complex congenital heart disease.

An increasingly complex group of children is now being followed as outpatients after surgery for congenital heart disease. A variety of complications and physiologic perturbations, both expected and unexpected, may present during follow-up, and should be anticipated by the practitioner and discussed with the patient and family. The purpose of this position article is to provide a framework for outpatient follow-up of complex congenital heart disease, based on a review of current literature and the experience of the authors.