Referring Provider Opinions of Pediatric Cardiology Evaluations Performed by Nurse Practitioners.

In the setting of physician shortages, nurse practitioner (NP) roles have evolved, with increasing independence across most healthcare settings. We sought to characterize referring clinician perceptions of NP-performed outpatient pediatric cardiology consultations. We electronically distributed to pediatric and family medicine physicians and NPs in Arkansas our 11-item survey assessing the acceptability of pediatric cardiology consultations being completed by an NP under varying circumstances. Circumstances included seven common referral indications, and the scale offered five answer choices ranging from "definitely unacceptable" to "definitely acceptable". A total of 292 of 1756 (16.6% response rate) referring clinicians responded to the survey. Overall, 57% of responses indicated that NP-completed pediatric cardiology evaluations were either definitely or probably unacceptable. Acceptability was varied by referral indication and referring clinician characteristics. Unacceptability of NP-completed pediatric cardiology evaluations was greatest among family medicine physicians (81%), pediatricians (66%), and clinicians working in solo or two-physician practices (77%) or community hospitals/clinics (71%). If NP evaluation of a murmur included required review with a cardiologist, the unacceptability rate dropped from 50 to 24% (p < 0.0001). Unacceptability was higher in physicians who do not work with NPs (69%) compared to those who do (60%) (pp < 0.0001). Many referring physicians were willing to send patients ≥ 100 miles to ensure evaluation by a pediatric cardiologist. Most referring physicians find pediatric cardiology evaluations performed by NPs to be unacceptable. Requisite review with a cardiologist improved acceptability of NP evaluations. Many referring physicians would send patients much farther to guarantee evaluation by a cardiologist.

Heart Transplantation in Children With Down Syndrome.

Background Children with Down syndrome (DS) have a high risk of cardiac disease that may prompt consideration for heart transplantation (HTx). However, transplantation in patients with DS is rarely reported. This project aimed to collect and describe waitlist and post- HTx outcomes in children with DS. Methods and Results This is a retrospective case series of children with DS listed for HTx. Pediatric HTx centers were identified by their participation in 2 international registries with centers reporting HTx in a patient with DS providing detailed demographic, medical, surgical, and posttransplant outcome data for analysis. A total of 26 patients with DS were listed for HTx from 1992 to 2020 (median age, 8.5 years; 46% male). High-risk or failed repair of congenital heart disease was the most common indication for transplant (N=18, 69%). A total of 23 (88%) patients survived to transplant. All transplanted patients survived to hospital discharge with a median posttransplant length of stay of 22 days. At a median posttransplant follow-up of 2.8 years, 20 (87%) patients were alive, 2 (9%) developed posttransplant lymphoproliferative disorder, and 8 (35%) were hospitalized for infection within the first year. Waitlist and posttransplant outcomes were similar in patients with and without DS (P=non-significant for all). Conclusions Waitlist and post-HTx outcomes in children with DS selected for transplant listing are comparable to pediatric HTx recipients overall. Given acceptable outcomes, the presence of DS alone should not be considered an absolute contraindication to HTx.

Methodological Challenges Encountered in a Study of the Impact of Animal-assisted Intervention in Pediatric Heart Transplant Patients.

PURPOSE: The purpose of this pilot study was to examine the impact of animal-assisted intervention (AAI) on ambulation, physiologic stability, patient satisfaction, and perceived benefit in hospitalized pediatric heart transplant patients. DESIGN AND METHODS: This pilot study used a two-period, two-sequence cross-over design. Using a convenience sample of heart transplant patients between the ages six and nineteen, each subject participated in one AAI and one non-AAI study session over one week. All study sessions started with an aspect of walking. RESULTS: Three males and two females participated. Average age was 15 years. Subjects walked on average 1906 ft during the AAI session as compared to 1933 ft in the non-AAI session. Subjects spent a longer time (17 min) walking in the AAI session as compared to the non-AAI session (15 min). Blood pressure and respiratory rates remained stable. All subjects reported they liked working with the dog. Eighty percent of subjects actively engaged in physical contact and communication with the dog. No safety-related adverse events were reported. CONCLUSIONS: AAI may assist adolescent heart transplant patients to be more motivated to participate in therapeutic ambulation when walking with a dog. Further studies are needed to identify successful recruitment strategies in this highly vulnerable patient population. PRACTICE IMPLICATIONS: Many clinical challenges exist in implementing AAI research in the pediatric heart transplant population. A conceptual model is introduced to better understand the methodological challenges of conducting AAI research in the hospital setting. Key components include research, organizational, AAI, subject and time factors.

Catheter-Associated Blood Stream Infections in Intracardiac Lines.

Purpose Right atrial (RA) or intracardiac lines are commonly used for hemodynamic monitoring in children undergoing cardiac surgery. In some institutions, these lines are used as the preferred long-term access line due to concerns for catheter-associated blood stream infections (CABSI) and catheter-related venous thrombosis with central lines in other locations. However, the rates and risk factors for CABSI and other complications are not known for RA lines. We undertook this study to estimate CABSI rates for RA lines in comparison with central catheters of various types and locations and to evaluate the incidence of other complications associated with the use of RA lines. Methods After approval from the Institutional Review Board, a retrospective review of all patients undergoing cardiac surgery at Arkansas Children's Hospital between the dates of January 1, 2006 and December 31, 2011 was performed. Demographic data, clinical features, and outcomes were summarized on a per-patient level. Type, location of placement, and duration of all centrally placed catheters as well as associated complications were recorded. Central venous lines (CVL) used in our unit include peripherally inserted central catheters (PICC) lines, and antibiotic and heparin coated double or triple lumen lines placed in internal jugular (IJ), femoral (Fem), or RA positions. The data were analyzed using statistical software STATA/MP. Results A total of 2,736 central lines were used in 1,537 patients. Data on line duration, alteplase use, and percentage of lines developing CABSI are described in the study. Disease severity as assessed by risk-adjusted classification for congenital heart surgery (RACHS) score ( p < 0.046), year of placement ( p < 0.001), and line type adjusted for thrombolytic (alteplase) use are significantly associated with risk of any CABSI. Overall, IJ and RA lines had least risk of CABSI while PICC lines had the highest CABSI rates. RA lines are also associated with other medically significant complications. Conclusion The CABSI rates associated with RA lines are lower than those seen with PICC lines. However, RA line use is associated with other, potentially significant complications. RA lines may be used cautiously as long-term access lines in cardiac patients in whom it is important to preserve venous patency for future interventions.

Improved Survival While Waiting and Risk Factors for Death in Pediatric Patients Listed for Cardiac Transplantation.

Our aim is to determine (a) the effect of changes in pre-transplant management and era of listing on survival of children listed for HTx and (b) risk factors for death while waiting. This retrospective study included all children listed between 1/1993 and 12/2009 at our center. Survival was determined using survival analysis and competing outcomes modeling. There were 254 listed patients of whom 144 (57%) had congenital heart disease, 208 (82%) were status 1, 52 used ECMO (20%), and 28 used ventricular assist device support (VAD) (11%) beginning in 2005. Overall mortality while waiting was 17% at 6 months, and 69% underwent transplant. Seven of 95 patients (7%) died waiting after 2004 compared to 36 of 159 (23%) before. ECMO and earlier year of listing were significant risk factors (p < 0.001) for wait-list mortality, whereas mortality was significantly lower (p = 0.002) after availability of VADs. Race, gender, blood type, and congenital diagnosis were not significant risk factors for death. Survival in pediatric patients listed for HTx has improved significantly in the current era at our institution. The availability of pediatric VADs has had a significant impact on survival while waiting in children listed for transplantation.

Online Focus Groups with Parents And Adolescents with Heart Transplants: Challenges and Opportunities.

Pediatric heart transplant recipients are scarce and widely dispersed. Previous studies of adolescents in this population were limited to small homogenous samples. Although online focus groups are an emerging data collection method, its use in pediatric populations has not been fully realized. The purpose of this study was to explore the feasibility of using online focus groups with pediatric populations. Adolescents (aged 13 to 21 years) at least 6 months post-heart transplant and their parents were recruited from two children's hospitals. An online discussion forum (iTracks) was used to conduct asynchronous focus groups with separate parent and adolescent groups. Six parents and four adolescents participated in the discussions. iTracks provided a framework for conducting focus groups in dispersed populations. Access to the discussion transcripts enhanced data analysis and eliminated transcription costs. Overall, online discussion forums were a feasible and cost-effective option to conduct online focus groups in this pediatric population.

Heart Transplantation in the Short Term After Chemotherapy.

Anthracycline antibiotics are an effective therapy for a variety of neoplastic diseases. Dilated cardiomyopathy is a known risk of their use. Because of the risk of new or recurrent neoplasm with immunosuppression transplantation is often delayed. Our patient developed early cardiomyopathy with congestive heart failure 3 months after completion of chemotherapy. Given the severity of her cardiac symptoms the decision was made to proceed with heart transplantation in the short term after completion of her chemotherapy. We report the success to 1 year of this decision and discuss the implications of her genetic and oncologic diagnoses in this clinical scenario.

Transition to self-management after pediatric heart transplant.

CONTEXT: Little is known about adolescent transition to self-management after heart transplant. This gap in knowledge is critically important because the consequences of poor self-management are costly and life-threatening, often resulting in nonadherence, rejection, repeated hospitalizations, and poor quality of life. OBJECTIVE: To explore how adolescents and parents perceive their roles in self-management, and how adolescents integrate self-management into their daily lives and navigate the transition from parent-dominated to self-management. DESIGN: Qualitative descriptive design, using online focus groups. SETTING: Online focus groups using itracks, an online qualitative software program. PARTICIPANTS: A purposive sample of 4 adolescents, 13 to 21 years old, who were at least 6 months posttransplant, and of 6 parents of adolescent heart transplant recipients. RESULTS: Several parallel themes emerged from the parent and adolescent online focus groups. Managing medications was the predominant theme for both parents and adolescents. For the remaining themes, parents and adolescents expressed similar ideas that were categorized into parallel themes, which included staying on top of things/becoming independent, letting them be normal/being normal, and worries and stressors. CONCLUSIONS: The transition to self-management after heart transplant was a clear goal for both parents and adolescents. The transition is a shared responsibility between parents and adolescents with a gradual shift from parent-directed to self-management. The process of transition was not linear or smooth, and in several instances, parents described efforts to transfer responsibility to the adolescent only to take it back when complications arose. Additional research with a larger sample is needed in order to fully understand adolescent heart transplant recipients' transition to self-management.

Quantification, identification, and relevance of anti-human leukocyte antigen antibodies formed in association with the berlin heart ventricular assist device in children.

BACKGROUND: Ventricular assist devices (VADs) are increasingly being used in pediatric patients to provide long-term cardiac support. One potential complication of VAD therapy is the development of antibodies directed against human leukocyte antigens (HLA). This phenomenon has not been well described with the Berlin Heart EXCOR VAD, the most commonly used VAD in pediatric patients. METHODS: The records of all pediatric patients undergoing VAD support using the Berlin Heart device at our institution between April 2005 and August 2011 were reviewed retrospectively. Demographic and clinical data regarding the VAD course were collected. Assessment of anti-HLA antibodies was performed using Luminex, and antibodies were quantified using mean fluorescence intensity (MFI). Assessment for anti-HLA antibodies was performed before VAD implantation and in serial fashion after VAD implantation. Clinically significant anti-HLA antibodies (sensitization) were defined by an MFI of more than 1000. RESULTS: Thirty-six patients were supported with the Berlin Heart VAD; 13 met inclusion criteria. The majority (85%) carried the diagnosis of dilated cardiomyopathy. Evidence of sensitization pre-VAD was found in 69%; new-onset sensitization (the development of new antibodies on VAD) occurred in 69%. All patients survived to transplantation. In two patients, the retrospective crossmatch was positive, but only in one patient was the crossmatch positive for antibodies formed while on VAD. CONCLUSIONS: Using Luminex and MFI quantification, anti-HLA antibodies are common before VAD implantation in pediatric patients. While on VAD support, new anti-HLA antibodies formed in a majority, but the immediate impact of these antibodies appears to be limited.

Rapid reduction in donor-specific anti-human leukocyte antigen antibodies and reversal of antibody-mediated rejection with bortezomib in pediatric heart transplant patients.

BACKGROUND: High titer donor-specific antibodies (DSA) and positive crossmatch in cardiac transplant recipients is associated with increased mortality from antibody-mediated rejection (AMR). Although treatment to reduce anti-human leukocyte antigen antibodies using plasmapheresis, intravenous immunoglobulin, and rituximab has been reported to be beneficial, in practice these are often ineffective. Moreover, these interventions do not affect the mature antibody producing plasma cell. Bortezomib, a proteasome inhibitor active against plasma cells, has been shown to reduce DSA in renal transplant patients with AMR. We report here the first use of bortezomib for cardiac transplant recipients in four pediatric heart recipients with biopsy-proven AMR, hemodynamic compromise, positive crossmatch, and high titer class I DSA. METHODS: Patients received four intravenous dose of bortezomib (1.3 mg/m(2)) over 2 weeks with plasmapheresis and rituximab. DSA specificity and strength (mean fluorescence intensity) was determined with Luminex. All had received previous treatment with plasmapheresis, intravenous immunoglobulin, and rituximab that was ineffective. RESULTS: AMR resolved in all patients treated with bortezomib with improvement in systolic function, conversion of biopsy to C4d negative in three patients and IgG negative in one patient, and a prompt, precipitous reduction in DSAs. In three patients who received plasmapheresis before bortezomib, plasmapheresis failed to reduce DSA. In one case, DSA increased after bortezomib but decreased after retreatment. CONCLUSIONS: Bortezomib reduces DSA and may be an important adjunct to treatment of AMR in cardiac transplant recipients. Bortezomib may also be useful in desensitization protocols and in prevention of AMR in sensitized patients with positive crossmatch and elevated DSA.

Effectiveness of a simulation-based educational program in a pediatric cardiac intensive care unit.

Optimal staff performance of resuscitation skills is best achieved through regular effective training. However, providing this teaching in a busy high-acuity pediatric cardiac intensive care unit (ICU) had become a challenge due to time and logistical constraints. A program to effectively and efficiently teach ICU nurses the skills necessary in patient resuscitation was developed using simulation training to better meet staff learning needs. Training via simulation provides an ideal learning environment with hands-on experience with the roles required in patient resuscitation. A simulation training program incorporating simulation training was developed for ICU nursing staff. All staff nurses in the ICU were required to attend over a year's time. The program involved mock resuscitation scenarios in which participants performed various resuscitation roles, followed by video review and group debriefing. All participants completed a survey prior to and immediately following participation in the training and again at 1 year. Data collected included self-report of knowledge, skill, and comfort related to patient resuscitation. Data revealed statistically significant improvement in scores pre and post training and at 1 year for self-reported knowledge, skills, and comfort related to resuscitation. Nursing staff reported that simulation training in resuscitation skills was helpful and positively impacted their knowledge, comfort, and skills. Feedback from nursing staff continues to be very positive, and performance of actual resuscitations on the unit has improved anecdotally.

Professional development for an advanced practice nursing team.

Since its development in 1998, the Cardiology Advanced Practice Nurse team has been plagued by retention issues. The coordinator for the team developed this leadership project while participating in the 2008 to 2009 Maternal-Child Health Leadership Academy sponsored by Sigma Theta Tau International and Johnson & Johnson. The focus of this project was to develop, implement, and evaluate initiatives to empower the advanced practice nurse team, promote their professional development, and ultimately improve retention of team members. Although evaluation data show progress toward addressing work environment issues, retention remains an ongoing problem.

Parent education after newborn congenital heart surgery.

The moment an infant is diagnosed with a congenital heart defect, parents experience a mixture of shock, disbelief, fear, anger, and often a profound sense of sadness. In the midst of these emotions they must learn to provide for the special needs of their infant. Providing parents with the knowledge and skills to care for their infant during this stressful time requires the concerted effort of a multidisciplinary team who can provide clear, concise, and consistent communication. This article provides a step-by-step guide to the information needs of families who are preparing to take their infants home after cardiac surgery. Parents must understand the underlying cardiac defect and the goals and objectives of the surgical repair. In addition to normal newborn care, parents need to learn about care of the incision, nutritional support, and how to safely administer prescribed medications. Potential complications and when to call their health care provider or seek emergency care are an important focus of teaching. Information about infant development, challenges specific to their infant, and pragmatic strategies to support normal development are of prime interest to parents. Written reference materials, a Web resource list, and a family discharge teaching tool provide tangible resources for the family after discharge.

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.