The experience of fathers of children hospitalised with advanced heart disease.

BACKGROUND: There are little reported data on the perspectives of fathers caring for children with chronic conditions. Although survival of children with advanced heart disease has improved, long-term morbidity remains high. This study describes the experience and prognostic awareness of fathers of hospitalised children with advanced heart disease. METHODS: Cross-sectional survey study of parents caring for children hospitalised with advanced heart disease admitted for ≥ 7 days over a one-year period. One parent per patient completed surveys, resulting in 27 father surveys. Data were analysed using descriptive methods. RESULTS: Nearly all (96%) of the fathers reported understanding their child's prognosis "extremely well" or "well," and 59% felt they were "very prepared" for their child's medical problems. However, 58% of fathers wanted to know more about prognosis, and 22% thought their child's team knew something about prognosis that they did not. Forty-one per cent of fathers did not think that their child would have lifelong limitations, and 32% anticipated normal life expectancies. All 13 fathers who had a clinical discussion of what would happen if their child got sicker found this conversation helpful. Nearly half (43%) of the fathers receiving new prognostic information or changes to treatment course found it "somewhat" or "a little" confusing. CONCLUSIONS: Fathers report excellent understanding of their child's illness and a positive experience around expressing their hopes and fears. Despite this, there remain many opportunities to improve communication, prognostic awareness, and participation in informed decision-making of fathers of children hospitalised with advanced heart disease.

Assessing enrollment of eligible infants in the national pediatric cardiology quality improvement collaborative (NPC-QIC) through linkage to the pediatric cardiac critical care consortium (PC4) registry.

BACKGROUND: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) lacks a rigorous enrollment audit process, unlike other collaborative networks. Most centers require individual families to consent to participate. It is unknown whether there is variation across centers or biases in enrollment. METHODS: We used the Pediatric Cardiac Critical Care Consortium (PC4) registry to assess enrollment rates in NPC-QIC for those centers participating in both registries using indirect identifiers (date of birth, date of admission, gender, and center) to match patient records. All infants born 1/1/2018-12/31/2020 and admitted 30 days of life were eligible. In PC4, all infants with a fundamental diagnosis of hypoplastic left heart or variant or who underwent a surgical or hybrid Norwood or variant were eligible. Standard descriptive statistics were used to describe the cohort and center match rates were plotted on a funnel chart. RESULTS: Of 898 eligible NPC-QIC patients, 841 were linked to 1,114 eligible PC4 patients (match rate 75.5%) in 32 centers. Match rates were lower in patients of Hispanic/Latino ethnicity (66.1%, p = 0.005), and those with any specified chromosomal abnormality (57.4%, p = 0.002), noncardiac abnormality (67.8%, p = 0.005), or any specified syndrome (66.5%, p = 0.001). Match rates were lower for patients who transferred to another hospital or died prior to discharge. Match rates varied from 0 to 100% across centers. CONCLUSIONS: It is feasible to match patients between the NPC-QIC and PC4 registries. Variation in match rates suggests opportunities for improvement in NPC-QIC patient enrollment.

Evaluation of a rapid diagnostic test for measles IgM detection; accuracy and the reliability of visual reading using sera from the measles surveillance programme in Brazil, 2015.

Laboratory-based case confirmation is an integral part of measles surveillance programmes; however, logistical constraints can delay response. Use of RDTs during initial patient contact could enhance surveillance by real-time case confirmation and accelerating public health response. Here, we evaluate performance of a novel measles IgM RDT and assess accuracy of visual interpretation using a representative collection of 125 sera from the Brazilian measles surveillance programme. RDT results were interpreted visually by a panel of six independent observers, the consensus of three observers and by relative reflectance measurements using an ESEQuant Reader. Compared to the Siemens anti-measles IgM EIA, sensitivity and specificity of the RDT were 94.9% (74/78, 87.4-98.6%) and 95.7% (45/47, 85.5-99.5%) for consensus visual results, and 93.6% (73/78, 85.7-97.9%) and 95.7% (45/47, 85.5-99.5%), for ESEQuant measurement, respectively. Observer agreement, determined by comparison between individuals and visual consensus results, and between individuals and ESEQuant measurements, achieved average kappa scores of 0.97 and 0.93 respectively. The RDT has the sensitivity and specificity required of a field-based test for measles diagnosis, and high kappa scores indicate this can be accomplished accurately by visual interpretation alone. Detailed studies are needed to establish its role within the global measles control programme.

Living with Congenital Aortic Stenosis: Exercise Restriction, Patterns of Adherence, and Quality of Life.

Modern consensus panel guidelines recommend restriction from most organized sports for patients with moderate or severe aortic stenosis (AS). However, there is little published data on how frequently physicians deviate from guidelines, how well patients adhere to exercise restrictions, or the effect of restriction on patient-reported quality of life. In this study, we surveyed 93 subjects with AS and their cardiologists regarding participation in organized sports, physical activity, weightlifting, and exercise restriction. Subjects completed the pediatric quality of life inventory (PedsQL) and the pediatric cardiac quality of life inventory (PCQLI). We found that subjects with severe AS (n = 3) were commonly, but not universally, restricted from organized sports (n = 2, 66%). Subjects with moderate AS (n = 40) were rarely restricted from organized sports (n = 6, 17%). No physician-specific characteristics were associated with increased likelihood of recommending exercise restriction. Subjects were more likely to be restricted if they were older (16 years vs. 13 years, p 0.02) and had moderate versus mild AS (p 0.013). PCQLI scores for teens and young adults with AS (age 13-25) were lower than a comparison group of patients with mild congenital heart disease. For all age groups, the PedsQL social functioning score was lower for subjects with exercise restriction (p 0.052). In summary, cardiologists apply consensus guidelines leniently when restricting patients with moderate/severe AS from organized sports and weightlifting. Patients with AS routinely adhere to exercise restriction recommendations. Children and young adults with AS and exercise restriction have lower QOL scores in the social functioning domain.

Immunisation rates and predictors of undervaccination in infants with CHD.

Vaccination coverage for infants with CHD is unknown, yet these patients are at high risk for morbidity and mortality associated with vaccine-preventable illnesses. We determined vaccination rates for this population and identified predictors of undervaccination. We prospectively enrolled infants with CHD born between 1 January, 2012 and 31 December, 2015, seen in a single-centre cardiology clinic between 15 February, 2016 and 28 February, 2017. We assessed vaccination during the first year of life. Subjects who by age 1 year received all routine immunisations recommended during the first 6 months of life were considered fully vaccinated. We also evaluated influenza vaccination during subjects' first eligible influenza season. We obtained immunisation histories from primary care providers and collected demographic and clinical data via a parent survey and chart review. We used multivariable logistic regression to identify predictors of undervaccination. Among 260 subjects, only 60% were fully vaccinated. Vaccination rates were lowest for influenza (64.6%), rotavirus (71.1%), and Haemophilus influenzae type b (79.3%). Cardiac surgery with cardiopulmonary bypass during the first year of life was associated with undervaccination (51.5% versus 76.4% fully vaccinated, adjusted odds ratio 2.1 [95% confidence interval 1.1-3.9]). Other predictors of undervaccination were out-of-state primary care (adjusted odds ratio 2.7 [1.5-4.9]), multiple comorbidities (≥2 versus 0-1, adjusted odds ratio 2.0 [1.1-3.6]), and hospitalisation for >25% of the first year of life (>25% versus ≤25%, adjusted odds ratio 2.1 [1.1-3.9]). Targeted quality improvement initiatives focused on improving vaccination coverage for these infants, especially surrounding cardiac surgery, are needed.

The NEONATE score predicts freedom from interstage mortality or transplant in a modern cohort.

BACKGROUND: Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation. OBJECTIVES: We sought to validate the score in an external, modern cohort. METHODS: This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation. Points were allocated per the NEONATE score (Norwood type-Norwood/Blalock-Taussig shunt: 3, Hybrid: 12; extracorporeal membrane oxygenation post-op: 9, Opiates at discharge: 6, No Digoxin at discharge: 9, Arch Obstruction on discharge echo: 9, Tricuspid regurgitation ≥ moderate on discharge echo: 12; Extra oxygen plus ≥ moderate tricuspid regurgitation: 28). The composite primary endpoint was interstage mortality or heart transplant. RESULTS: In total, 1026 patients met inclusion criteria; 61 (6%) met the primary outcome. Interstage mortality occurred in 44 (4.3%) patients at a median of 129 (IQR 62,195) days, and 17 (1.7%) were referred for heart transplant at a 167 (114,199) days of life. The median NEONATE score was 0(0,9) in those who survived to Stage 2 palliation compared to 9(0,15) in those who experienced interstage mortality or heart transplant (p < 0.001). Applying a NEONATE score cut-off of 17 points that separated patients into low- and high-risk groups in the learning cohort provided 91% specificity, negative predictive value of 95%, and overall accuracy of 87% (85.4-89.5%). CONCLUSION: In a modern cohort of patients with single ventricle CHD and aortic arch hypoplasia, the NEONATE score remains useful at discharge post-Stage 1 palliation to predict freedom from interstage mortality or heart transplant.

Isometric Exercise Increases the Diameter of the Ascending Aorta in Youth with Bicuspid Aortic Valves.

Central arterial pressure rises to much higher levels during heavy isometric exercise compared to other forms of exercise. For this reason, patients with bicuspid aortic valves (BAV) are often restricted from heavy isometric exercise. Although this approach may be theoretically appealing, no data exist regarding the efficacy of this activity restriction.Patients between 12 and 21 years old with isolated BAV were enrolled if they had a previous echocardiogram at least 2 years prior to the current clinic visit. Patients were excluded if they had additional congenital heart disease, a diagnosed syndrome, or had undergone a procedure involving the aortic valve or ascending aorta. Patients completed a questionnaire regarding frequency and intensity of isometric exercise during the 3-month period prior to the visit. We compared aortic dimensions (Z-score), aortic stenosis, and aortic insufficiency between an echocardiogram performed as part of the current visit and one obtained 2-5 years previously using paired t tests and multivariable regression controlling for age, gender, degree of aortic stenosis, and the presence of isometric exercise. In this sample of 50 adolescents with isolated bicuspid aortic valve, 30 (60%) subjects did not participate in any isometric exercise. Over an average of 2.9 years (SD 0.9 years), we did not find a significant difference between changes in the Z-score diameters of the aortic root (0.9 vs 0.9, p = 0.913) and ascending aorta Z-score (2.9 vs 2.9, p = 0.757), between subjects engaging and not engaging in isometric exercise. Further, we did not find that changes in valve function (i.e., aortic stenosis and aortic insufficiency) differed between the two groups.In this sample of adolescents with isolated bicuspid aortic valve, there was no medium-term increase in aortic dilation or worsening valve function in those who engaged in isometric exercise versus those who refrained from isometric exercise.

Parent-Provider Communication in Hospitalized Children with Advanced Heart Disease.

Communication between parents and providers of children with cardiac disease is essential to parental decision-making. This study explored how parents of hospitalized children with advanced heart disease perceived communication with their child's providers. We performed a prospective survey study of parents and physicians of children with advanced heart disease age 30 days to 19 years admitted to the hospital for > 7 days over a 1-year period at a single institution (n = 160 parent-provider pairs). Descriptive statistics were primarily used and Fisher exact tests and kappa statistics were used to assess agreement. All parents rated communication with their child's care team as excellent, very good, or good, but 56% of parents reported having received conflicting information. Parental perception of "too many" people giving them information was associated with overall poorer communication and less preparedness for decision-making. One-third (32%) of parents felt unprepared for decision-making, despite 88% feeling supported. Parents and physicians showed poor agreement with respect to overall adequacy of communication, receipt of conflicting information, and evaluation of the most effective way for parents to receive information. Interventions involving physician communication training and proactive assessment of parent communication preferences may be beneficial.

A Systematic Evaluation of IgM and IgG Antibody Assay Accuracy in Diagnosing Acute Zika Virus Infection in Brazil: Lessons Relevant to Emerging Infections.

Accurate diagnostics underpin effective public health responses to emerging viruses. For viruses, such as Zika virus (ZIKV), where the viremia clears quickly, antibody-based (IgM or IgG) diagnostics are recommended for patients who present 7 days after symptom onset. However, cross-reactive antibody responses can complicate test interpretation among populations where closely related viruses circulate. We examined the accuracy (proportion of samples correctly categorized as Zika positive or negative) for antibody-based diagnostics among Brazilian residents (Rio de Janeiro) during the ZIKV outbreak. Four ZIKV enzyme-linked immunosorbent assays (ELISAs; IgM and IgG Euroimmun, IgM Novagnost, and CDC MAC), two dengue ELISAs (IgM and IgG Panbio), and the ZIKV plaque reduction neutralization test (PRNT) were evaluated. Positive samples were ZIKV PCR confirmed clinical cases collected in 2015-2016 (n = 169); negative samples (n = 236) were collected before ZIKV was present in Brazil (≤2013). Among serum samples collected ≥7 days from symptom onset, PRNT exhibited the highest accuracy (93.7%), followed by the Euroimmun IgG ELISA (77.9%). All IgM assays exhibited lower accuracy (<75%). IgG was detected more consistently than IgM among ZIKV cases using Euroimmun ELISAs (68% versus 22%). Anti-dengue virus IgM ELISA was positive in 41.1% of confirmed ZIKV samples tested. The Euroimmun IgG assay, although misdiagnosing 22% of samples, provided the most accurate ELISA. Anti-ZIKV IgG was detected more reliably than IgM among ZIKV patients, suggesting a secondary antibody response to assay antigens following ZIKV infection. Antibody ELISAs need careful evaluation in their target population to optimize use and minimize misdiagnosis, prior to widespread deployment, particularly where related viruses cocirculate.

Parent-Reported Symptoms and Perceived Effectiveness of Treatment in Children Hospitalized with Advanced Heart Disease.

OBJECTIVE: To characterize parent-reported symptom burden and effectiveness of symptom management in children hospitalized with advanced heart disease. STUDY DESIGN: Prospective survey study of 161 parents whose child was admitted to a single institution with advanced heart disease between March 2018 and February 2019 using the Survey about Caring for Children with Heart Disease. RESULTS: Of the 161 patients, 54% were under 2 years old with a diagnosis of single ventricle physiology (39%), pulmonary hypertension (12%), and other congenital heart disease (28%). Over one-half (56%) of parents reported that their child was experiencing a high degree ("a great deal"/"a lot") of symptoms. The most frequently reported symptoms were pain (68%), fatigue (63%), and breathing difficulties (60%). Of the symptoms that were treated, parents perceived successful treatment to be least likely for their child's sleep disturbance (24%), depression (29%), and fatigue (35%). Parents who reported their child's functional status as New York Heart Association class III/IV were more likely to report that their child was experiencing "a great deal" of symptoms, compared with those who reported class I/II (51% vs 19%, P < .001). Parents who reported their child was experiencing a high degree of suffering from fatigue were also more likely to report a high symptom burden (P < .001). CONCLUSIONS: Parents of children with advanced heart disease reported high symptom burden with a broad spectrum of symptoms. Parents reported fatigue and psychiatric symptoms frequently and rarely reported treatment as successful. Parents' view of their child's symptom burden was concordant with their perception of their child's functional status.

Characteristics of Interstage Death After Discharge from Stage I Palliation.

BACKGROUND: Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM. METHODS: This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015. Patients underwent S1P with right ventricle-pulmonary artery conduit (RVPAC), modified Blalock-Taussig-Thomas shunt (BTT), or Hybrid; transplants were excluded. RESULTS: IM occurred in 153 (7%) patients (median gestational age 38 weeks, 54% male, 77% white), at 88 (IQR 60,136) days of life, and 39 (IQR 17,84) days after hospital discharge; 13 (8.6%) occurred ≤ 30 days after S1P. The mortality rate for RVPAC was lower (5.2%; 59/1138) than BTT (9.1%; 65/712) and Hybrid (20.1%; 27/134). More than half of deaths occurred at home (20%) or in the emergency department (33%). The remainder occurred while inpatient at center of S1P (cardiac intensive care unit 36%, inpatient ward 5%) or at a different center (5%). Fussiness and breathing problems were most often cited as harbingers of death; distance to surgical center was the biggest barrier cited to seeking care. Cause of death was unknown in 44% of cases overall; in the subset of patients who underwent post-mortem autopsy, the cause of death remained unknown in 30% of patients, with the most common diagnosis being low cardiac output. CONCLUSIONS: Most IM occurred in the outpatient setting, with non-specific preceding symptoms and unknown cause of death. These data indicate the need for research to identify occult causes of death, including arrhythmia.

Researching, co-creating and testing innovations in paper-based health information systems (PHISICC) to support health workers' decision-making: protocol of a multi-country, transdisciplinary, mixed-methods research programme in three sub-Saharan countries.

BACKGROUND: Health information systems are crucial to provide data for decision-making and demand for data is constantly growing. However, the link between data and decisions is not always rational or linear and the management of data ends up overloading frontline health workers, which may compromise quality of healthcare delivery. Despite limited evidence, there is an increasing push for the digitalization of health information systems, which poses enormous challenges, particularly in remote, rural settings in low- and middle-income countries. Paper-based tools will continue to be used in combination with digital solutions and this calls for efforts to make them more responsive to local needs. Paper-based Health Information Systems in Comprehensive Care (PHISICC) is a transdisciplinary, multi-country research initiative to create and test innovative paper-based health information systems in three sub-Saharan African countries. METHODS/DESIGN: The PHISICC initiative is being carried out in remote, rural settings in Côte d'Ivoire, Mozambique and Nigeria through partnership with ministries of health and research institutions. We began with research syntheses to acquire the most up-to-date knowledge on health information systems. These were coupled with fieldwork in the three countries to understand the current design, patterns and contexts of use, and healthcare worker perspectives. Frontline health workers, with designers and researchers, used co-creation methods to produce the new PHISICC tools. This suite of tools is being tested in the three countries in three cluster-randomized controlled trials. Throughout the project, we have engaged with a wide range of stakeholders and have maintained the highest scientific standards to ensure that results are relevant to the realities in the three countries. DISCUSSION: We have deployed a comprehensive research approach to ensure the robustness and future policy uptake of findings. Besides the innovative PHISICC paper-based tools, our process is in itself innovative. Rather than emphasizing the technical dimensions of data management, we focused instead on frontline health workers' data use and decision-making. By tackling the whole scope of primary healthcare areas rather than a subset of them, we have developed an entirely new design and visual language for a suite of tools across healthcare areas. The initiative is being tested in remote, rural areas where the most vulnerable live.

Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

OBJECTIVES: Critical pulmonary stenosis (PS) and pulmonary atresia with intact ventricular septum (PAIVS) require urgent neonatal intervention. Since PS may be more insidious than PAIVS during gestation, we hypothesized that neonates with PS would have lower rates of prenatal detection than PAIVS. METHODS: We performed a retrospective chart review of all neonates who underwent diagnostic or interventional cardiac catheterization between 2000 and 2014 for critical PS or PAIVS. The rates of prenatal diagnosis were calculated for PS and PAIVS. Prenatal and postnatal echocardiographic data were reviewed. RESULTS: 178 patients met inclusion criteria: 91 with critical PS and 87 with PAIVS. The prenatal diagnosis rate for critical PS was lower than for PAIVS at 37% (34/91) vs 60% (52/87) (P = .003). At the time of diagnosis at a median gestational age of 25 weeks, the median TV z-score for patients with critical PS was larger than in PAIVS (-0.15 vs -3.0 P = .004). CONCLUSION: Critical PS had a lower prenatal detection rate than PAIVS, likely due to a relatively normal 4-chamber view at the time of routine second trimester screening in patients with PS. Color flow Doppler of the outflow tracts may improve detection, since outflow tracts may appear normal by 2D imaging.

Antibody-based assay discriminates Zika virus infection from other flaviviruses.

Zika virus (ZIKV) is a mosquito-borne flavivirus that emerged recently as a global health threat, causing a pandemic in the Americas. ZIKV infection mostly causes mild disease, but is linked to devastating congenital birth defects and Guillain-Barré syndrome in adults. The high level of cross-reactivity among flaviviruses and their cocirculation has complicated serological approaches to differentially detect ZIKV and dengue virus (DENV) infections, accentuating the urgent need for a specific and sensitive serological test. We previously generated a ZIKV nonstructural protein 1 (NS1)-specific human monoclonal antibody, which we used to develop an NS1-based competition ELISA. Well-characterized samples from RT-PCR-confirmed patients with Zika and individuals exposed to other flavivirus infections or vaccination were used in a comprehensive analysis to determine the sensitivity and specificity of the NS1 blockade-of-binding (BOB) assay, which was established in laboratories in five countries (Nicaragua, Brazil, Italy, United Kingdom, and Switzerland). Of 158 sera/plasma from RT-PCR-confirmed ZIKV infections, 145 (91.8%) yielded greater than 50% inhibition. Of 171 patients with primary or secondary DENV infections, 152 (88.9%) scored negative. When the control group was extended to patients infected by other flaviviruses, other viruses, or healthy donors (n = 540), the specificity was 95.9%. We also analyzed longitudinal samples from DENV-immune and DENV-naive ZIKV infections and found inhibition was achieved within 10 d postonset of illness and maintained over time. Thus, the Zika NS1 BOB assay is sensitive, specific, robust, simple, low-cost, and accessible, and can detect recent and past ZIKV infections for surveillance, seroprevalence studies, and intervention trials.

Fears and Stressors of Trainees Starting Fellowship in Pediatric Cardiology.

Post-graduate training for physicians involves a high level of stress. High stress during training has the potential to cause burnout, a well-studied phenomenon in medical trainees. Burnout has previously been shown to increase the risk of mental health problems and medical error in trainees. Little research has been done on the impact of stress on new fellows in general and pediatric cardiology fellows in particular; understanding common sources of stress offers the opportunity to design targeted interventions to support trainee wellness. New trainees in Boston Children's Hospital's Pediatric Cardiology Fellowship program were asked to answer to the following question at the beginning of their training: "What are you afraid of in the coming year?" A qualitative content analysis was done on their anonymous responses. Responses were coded and analyzed for common themes. The overall analysis found that 83% of fellows reported fear of "fellowship/career responsibilities." The second most common theme was "failure/disappointment" (78%) followed by "personal life" (74%), "emotional exhaustion" (61%); least common was "new hospital environment" (37%). The most common individual fear was "increased clinical responsibility" reported by 65% of the new fellows, while 62% reported fears of "imposter syndrome," and 58% about "burnout." We found that fellows commonly report fears about both clinical and personal responsibilities, similar to stressors found in studies on residency. It is important for pediatric cardiology fellowships to develop early and specific interventions designed to assist fellows in managing both their new clinical responsibilities and their other stressors.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era.

Scoliosis is common in children with congenital heart disease (CHD) and may have deleterious effects on quality of life and hemodynamics. Relatively little is known about the outcomes of spinal fusion for scoliosis repair in children with complex CHD. We reviewed all cases of children with CHD undergoing first time spinal fusion excluding those with minor CHD between 1995 and 2015. Seventy-eight patients were identified and included in the study. 97.4% of patients included had undergone prior cardiac surgery and sixteen patients had single ventricle circulations. 17.9% of patients experienced a significant perioperative event defined as an aggregate of the presence of any of the following: need for early unanticipated reoperation, neurologic deficit, postoperative bleeding requiring intervention, end organ dysfunction, or death. There were no deaths in our cohort. 38.5% of patients experienced any adverse event, the majority of which were related to perioperative fluid shifts. Larger preoperative Cobb angle and longer length of spinal fusion were associated with increased risk of significant perioperative event while larger preoperative Cobb angle and longer length of spinal fusion, older age at time of surgery, single ventricle circulation, cyanosis and patients taking cardiac medications at the time of surgery were more likely to experience any adverse event. Operative repair of scoliosis in children with complex CHD has been performed without mortality over a 20-year period in a single institution, albeit with a higher rate of perioperative complication than is seen in the general pediatric population. Patients with large preoperative Cobb angles and cyanotic single ventricle circulations appear to be at the highest risk for perioperative complications.

Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey.

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3-4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

Identifying best practices in interstage care: using a positive deviance approach within the National Pediatric Cardiology Quality Improvement Collaborative.

IntroductionTo identify interstage best practices associated with lower mortality, we studied National Pediatric Cardiology Quality Improvement Collaborative centres registry using a positive deviance approach. METHODS: Positive deviant and control centre team members were interviewed to identify potential interstage best practices. Subsequently, all collaborative centres were surveyed on the use of these practices to test their associations with centre mortality. Questionnaires were scored using Likert scales; the overall score was linearly transformed to a 0-100-point scale with higher scores indicating increased use of practices. Mortality was based on patients enrolled after a centre's first year in the collaborative. Centre mortality rates were divided into tertiles. Survey scores for the low mortality tertile were compared with the other tertiles. RESULTS: For this study, seven positive deviant and four control teams were interviewed. A total of 20 potential best practices were identified, including team composition, improvement practices, and parent involvement. Questionnaires were completed by 36/43 eligible centres, providing 1504 patients for analysis. Average survey score was 50.2 (SD 13.4). Average mortality was 6.1% (SD 4.1). There was no correlation between survey scores and mortality (r=0.14, p=0.41). The one practice associated with the low mortality tertile was frequency of discussion of interstage results: 58.3% of low mortality teams discussed results at least monthly versus 8.4% of the middle and high tertile centres (p=0.02). CONCLUSIONS: Low-mortality centres more frequently discuss interstage results than high-mortality centres. Heightened awareness of outcomes may influence practice; however, further study is needed to understand the variation in outcomes across centres.

Evaluation of left ventricular false tendons in children with idiopathic left ventricular tachycardia.

BACKGROUND: Left ventricular false tendons (FT) traverse the ventricular cavity and are thought to have some association with idiopathic left ventricular tachycardia (ILVT). However, reported prevalence of FT varies widely, making correlation difficult. Superior echocardiographic windows of pediatric patients may permit better analysis of FT in ILVT. Our study describes the relationship between FT and ILVT in young patients. METHODS: Retrospective case-control study of 30 ILVT patients with 98 controls compared for FT. Diagnosis of ILVT was made by electrocardiogram and clinical history, and for 25 patients was further confirmed by electrophysiology study (EPS). Presence of FT was identified by one blinded observer and verified by a second blinded observer. Presence of FT was then compared between ILVT patients and controls using Fisher's exact test. RESULTS: Presence of FT did not differ significantly between patients and controls (53% vs 43%, P  =  0.40). Twelve FT patients (19%) had multiple FTs detected, though the incidence of ILVT was no higher in the setting of multiple FTs. A total of 25 patients with ILVT underwent EPS for intended ablation therapy, with ultimate success in 22/25 (88%) after one or more ablation sessions. Of the 25 EPS patients, FTs were present in 11, but precise correlation between successful ablation location and FT location was not possible since intraprocedural echocardiography was not performed in this patient group. CONCLUSIONS: Presence of FTs did not differ between ILVT patients and controls. While FTs are not absolutely required for ILVT, they may still play a role in some cases.