Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management: A Scientific Statement From the American Heart Association.

This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in-hospital readiness for discharge, caregiver support and education, healthcare teams and resources, surveillance strategies and practices, national quality improvement efforts, interstage outcomes, and future areas for research. The statement is directed toward pediatric cardiologists, primary care providers, subspecialists, advanced practice providers, nurses, and those caring for infants undergoing staged surgical palliation for single ventricle heart disease.

Transition experiences between hospital- and home-care for parents of children with hypoplastic left heart syndrome.

PURPOSE: Children with hypoplastic left heart syndrome (HLHS) experience numerous vulnerabilities during transitions from hospital to home during their first year of life. This paper examines the parents' responses to the situations they experience during the initial interstage transition as described through illness blogs. DESIGN AND METHODS: A qualitative descriptive design through inductive theme development using thematic analysis was performed for this study. Text data were used from publically available blogs written by parents of HLHS children on their feelings and experiences during transitions between hospital- and home-care during the interstage period. RESULTS: Six blogs were analyzed. Overall, the lack of difference in normalcy for these parents and their families was apparent. Major themes included: thoughts and feelings when discharged from the hospital, difficulties with hospital readmissions, protecting their child from infection, and developing a support system. CONCLUSIONS: Parents looked forward to going home and learned all they could about properly caring for their child before going home. They were disappointed and frustrated when returning to the hospital for either emergency or scheduled admissions and were not prepared to see their child in the intensive care unit environment again. Many parents isolated themselves and avoided the hospital as much as possible to avoid their child developing an infection. All parents had support systems through family and/or friends and found an unexpected sense of support through their blog families.

Grief and coping of parents whose child has a constant life-threatening disability, hypoplastic left heart syndrome with reference to the Dual-Process Model.

This paper reports on a study that examined the grief and coping of 29 parents whose child has hypoplastic left heart syndrome using the Dual Process Model. The study employed a secondary thematic analysis of interviews at key times of treatment and recovery for the child. After the diagnosis, parents experienced intense loss (LO), but focused upon restoration-orientated tasks (RO) to support their child. Over time, most parents employed a healthy oscillation between LO coping and RO coping, with waves of grief and with some grieving suppressed. There are some specific grief and coping and gender patterns employed by parents.

Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies.

Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period. A crucial focus of care is the comprehensive family training in the use of home surveillance monitoring of oxygen saturation, enteral intake, weight, and the early recognition of "red flag" symptoms indicating potential cardiopulmonary or nutritional decompensation. Beginning with admission to the intensive care unit of the newborn with hypoplastic left heart syndrome, nurses provide critical care and education to prepare the family for interstage home care. This article presents detailed nursing guidelines for educating families on the home care of their medically fragile infant with single-ventricle circulation.

Infant health after heart surgery.

Background The number of infants who survive initial surgery for complex congenital heart disease (CHD), such as hypoplastic left heart syndrome, is increasing, but they are often left with residual complex health needs.

Parents' preparedness for their infants' discharge following first-stage cardiac surgery: development of a parental early warning tool.

UNLABELLED: Aim The aim of this study was to explore parental preparedness for discharge and their experiences of going home with their infant after the first-stage surgery for a functionally univentricular heart. BACKGROUND: Technological advances worldwide have improved outcomes for infants with a functionally univentricular heart over the last 3 decades; however, concern remains regarding mortality in the period between the first and second stages of surgery. The implementation of home monitoring programmes for this group of infants has improved this initial inter-stage survival; however, little is known about parents' experiences of going home, their preparedness for discharge, and parents' recognition of deterioration in their fragile infant. METHOD: This study was conducted in 2011-2013; eight sets of parents were consulted in the research planning stage in September, 2011, and 22 parents with children aged 0-2 years responded to an online survey during November, 2012-March, 2013. Description of categorical data and deductive thematic analysis of the open-ended questions were undertaken. RESULTS: Not all parents were taught signs of deterioration or given written information specific to their baby. The following three themes emerged from the qualitative data: mixed emotions about going home, knowledge and preparedness, and support systems. CONCLUSIONS: Parents are not adequately prepared for discharge and are not well equipped to recognise deterioration in their child. There is a role for greater parental education through development of an early warning tool to address the gap in parents' understanding of signs of deterioration, enabling appropriate contact and earlier management by clinicians.

Parental Vigilance in Caring for Their Children with Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome (HLHS) is a complex heart malformation that requires life-saving treatments. Parents experience numerous challenges as they learn to parent a child with complex care requirements. The following research question guided this qualitative study: Is the parenting process among parents of a child with HLHS characterized by exaggerated vigilant parental action, and if so, how does this influence parental response? Situated within a larger program of pediatric cardiology research, this study included data from two grounded theory studies with parents of children with HLHS. This secondary analysis involved a thematic content analysis using sensitizing concepts of uncertainty, protectiveness, support, and mastery of complex care. Transcribed data from 55 interviews with 24 mothers and 17 fathers of young children with HLHS were analyzed for relevant and recurring themes. In mastering skills required to care for their child with HLHS, parents contrasted what was in their hands with what was out of their hands. Vigilant parental actions were evident as parents became skilled at providing complex care. Parents said they were sometimes excessive in their vigilant actions. In retrospect they viewed this vigilance as appropriate in some situations but exaggerated in other situations. Understanding parents' vigilant actions in response to their child's complex care can guide health care providers' interactions with families. Long-term follow up, both clinically and through research, is needed to assess the long-term consequences of exaggerated vigilant parental action on the child, parent, and family, and to determine and evaluate appropriate and timely intervention.

Feeding Protocols for Neonates With Hypoplastic Left Heart Syndrome: A Review.

Optimizing nutrition in neonates with hypoplastic left heart syndrome is essential, given the high rate of growth failure in this population. Infants with hypoplastic left heart syndrome are predisposed to nutritional deficiency as a result of their increased metabolic demand; however, early enteral feeding also increases the risk of serious gastrointestinal morbidity and mortality caused by poor intestinal perfusion. Consequently, providers have difficulty deciding when and how to safely feed these patients. A review of the literature found that implementation of a structured enteral feeding protocol may decrease the risk of gastrointestinal complications while also minimizing dependence on parenteral nutrition and decreasing length of hospital stay. As these studies were limited, further research is warranted to establish a best practice feeding protocol to decrease risk and optimize nutrition in this fragile population.

Supporting families in neonatal loss: relationship and faith key to comfort.

Around 20,000 neonatal deaths occur each year, many from congenital heart defects such as hypoplastic left heart syndrome. Nurses are on the frontline of caring for families experiencing neonatal loss. Careful spiritual and cultural assessment, attention to beliefs, focusing on relationship, and helping families create legacy can assist with grieving and making meaning out of loss.

Heart for bonding: a new protocol of care for hypoplastic left heart syndrome.

There are significant challenges involved in the perinatal and postnatal care of an infant with hypoplastic left heart syndrome (HLHS) and the infant's family. In the blink of an eye, the perfect child is lost, and a fragile infant is about to join the family. This case study and discussion is an overview of HLHS , a family's desire to make the birth of their infant normal, and how that desire initiated a change in philosophy and practice in our neonatal intensive care unit.

Ethical principles and parental choice: treatment options for neonates with hypoplastic left heart syndrome.

Nurses caring for children with congenital heart disease face unique challenges, especially when caring for neonates diagnosed with hypoplastic left heart syndrome (HLHS). The treatment options for these neonates present difficult choices for the child's decision makers and are not without significant life-altering consequences. In order to assist in the decision-making process, nurses as patient and family advocates should acknowledge the unique role they play in the informed consent process, while simultaneously identifying specific ethical principles that are components of this process. By incorporating the principles of autonomy, beneficence, and veracity into specific nursing interventions, nurses can assist families in making informed decisions regarding a treatment option that is best for the child as well as the family.

The physiologic basis for and nursing considerations in the use of subatmospheric concentrations of oxygen in HLHS.

Subatmospheric concentrations of oxygen are used in the preoperative and postoperative care of infants with hypoplastic left heart syndrome (HLHS). This technique increases the pulmonary vascular resistance (PVR) and thereby improves systemic blood flow. There have been no controlled studies of this therapy in humans. Changes in aortic blood flow patterns, suggesting improved systemic circulation after administration of nitrogen, have been shown by Doppler ultrasound. Video segments are included in the electronic version of this article to demonstrate the immediate circulatory effects of therapy. No significant long-term effects on PVR have been found. A clear understanding of the anatomy, physiology, and therapeutic maneuvers used to balance pulmonary blood flow and systemic blood flow is essential for caregivers of infants with HLHS. Infants receiving subatmospheric concentrations of oxygen require meticulous nursing care to detect early changes in the relative vascular resistance and to monitor for the hemodynamic effects of medical and nursing interventions.

The implications of pulmonary vascular resistance on the nursing care of an infant with hypoplastic left heart syndrome.

The pathophysiology of pulmonary vascular resistance is described Specific nursing interventions required to maintain stable haemodynamics are explored The major implications involved in suctioning infants post Norwood operation for hypoplastic left heart syndrome are discussed.

Family-centered care and the anticipated death of a newborn.

Providing support for a family whose infant is expected to die requires planning and coordinated intervention by members of the health care team. From diagnosis until the death of the infant, the family's choices and needs must be integrated into an acceptable plan of care. The role of the health care team in this process includes four principal phases: planning for the baby's birth, preparing the siblings, caring for the baby, and supporting the family both at the time of the death and afterward. Specific strategies used by physicians, nurses, and child life specialists can encourage the family's full participation in the birth and death of the infant. A description of one family's experiences highlights implications for family-centered practice.