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PIK3CA-related overgrowth spectrum (PROS) conditions of the head and neck are treatment challenges. Traditionally, these conditions require multiple invasive interventions, with incomplete malformation removal, disfigurement, and possible dysfunction. Use of the PI3K inhibitor alpelisib, previously shown to be effective in PROS, has not been reported in PIK3CA-associated head and neck lymphatic malformations (HNLMs) or facial infiltrating lipomatosis (FIL). We describe prospective treatment of 5 children with PIK3CA-associated HNLMs or head and neck FIL with alpelisib monotherapy.A total of 5 children with PIK3CA-associated HNLMs (n = 4) or FIL (n = 1) received alpelisib monotherapy (aged 2-12 years). Treatment response was determined by parental report, clinical evaluation, diary/questionnaire, and standardized clinical photography, measuring facial volume through 3-dimensional photos and magnetic resonance imaging.All participants had reduction in the size of lesion, and all had improvement or resolution of malformation inflammation/pain/bleeding. Common invasive therapy was avoided (ie, tracheotomy). After 6 or more months of alpelisib therapy, facial volume was reduced (range 1%-20%) and magnetic resonance imaging anomaly volume (range 0%-23%) were reduced, and there was improvement in swallowing, upper airway patency, and speech clarity.Individuals with head and neck PROS treated with alpelisib had decreased malformation size and locoregional overgrowth, improved function and symptoms, and fewer invasive procedures.

Objective Although the right ventricle (RV) to pulmonary artery conduit in stage 1 Norwood operation results in improved interstage survival, the long-term effects of the ventriculotomy used in the traditional technique remain a concern. The periscopic technique (PT) of RV to pulmonary artery conduit placement has been described as an alternative technique to minimize RV injury. A retrospective study was performed to compare the effects of traditional technique and PT on ventricular function following Norwood operation. Methods A retrospective study of all patients who underwent Norwood operation from 2012 to 2019 was performed. Patients with baseline RV dysfunction and significant tricuspid valve regurgitation were excluded. Prestage 2 echocardiograms were reviewed by a blinded experienced imager for quantification of RV function (sinus and infundibular RV fractional area change) as well as for regional conduit site wall dysfunction (normal or abnormal, including hypokinesia, akinesia, or dyskinesia). Wilcoxon rank-sum tests were used to assess differences in RV infundibular and RV sinus ejection fraction and the Fisher exact test was used to assess differences in regional wall dysfunction. Results Twenty-two patients met inclusion criteria. Eight underwent traditional technique and 14 underwent PT. Median infundibular RV fractional area change was 49% and 37% (P = .02) and sinus RV fractional area change was 50% and 41% for PT and traditional technique (P = .007) respectively. Similarly qualitative regional RV wall function was better preserved in PT (P = .002). Conclusions The PT for RV to pulmonary artery conduit in Norwood operation results in better preservation of early RV global and regional systolic function. Whether or not this benefit translates to improved clinical outcome still needs to be studied.

Patients born with bilateral head and neck lymphatic malformations (BHNLMs) often require multiple invasive treatments, including tracheostomy. We hypothesized that primary targeted medical therapy (pTMT) with diagnostic needle aspiration reduces the need for invasive therapy such as surgical resection and/or sclerotherapy.Retrospective case review was performed of infants with BHNLMs (Grade 2 or De Serres stage IV and V) treated only at our institution from 2000 to 2021. Patients were divided into two cohorts: those managed with pTMT and those managed with observation, sclerotherapy, or surgical intervention (non-pTMT). Data regarding interventions, clinical outcomes, morbidity, and mortality were analyzed with descriptive statistics.Nine children with BHNLMs met inclusion criteria. Three (33%) were in the pTMT cohort and six (66%) were non-pTMT. Eight (89%) malformations were genotyped, and all demonstrated hotspot PIK3CA variants. All pTMT patients had sirolimus initiated in the first month of life and underwent needle aspiration of malformation cyst fluid for cell-free DNA samples. All pTMT patients tolerated medical therapy. For the non-pTMT cohort, primary treatment included none (deceased, n = 1, 17%), observation with needle aspiration (n = 1, 17%), surgical resection (n = 2, 33%), or combination surgery and sclerotherapy (n = 2, 33%). Intubation duration, intensive care and initial hospital length of stay were not different between cohorts. Four non-pTMT patients (67%) required tracheostomy, and two (33%) died prior to discharge. All pTMT patients survived and none required tracheostomy. Non-pTMT patients required a median of two invasive therapies prior to discharge (IQR 1-4) and a mean total of 13 over the course of their lifetime (IQR 1-16), compared to the pTMT group who did not require any lifetime invasive therapy, even after initial pTMT and discharge home.This study compares patients with BHNLMs (Grade 2) treated with pTMT versus those treated with observation or invasive therapy. Patients treated with pTMT required no surgical or invasive procedural treatment of their malformations, no tracheostomy placement, no unplanned readmissions after discharge, and had no mortalities. Needle aspiration was useful as a therapeutic adjunct for cell-free DNA diagnosis of PIK3CA variants, which guided TMT.

Background Alaska Native (AN) infants are at risk for severe disease due to respiratory syncytial virus (RSV) and influenza. Maternal immunization protects young infants through transplacental antibody transfer. RSV- and influenza-specific transplacental antibody transfer in mother–infant pairs has not previously been evaluated in the AN population. Methods Serum samples collected during pregnancy and at birth from AN mother–infant pairs in the Yukon-Kuskokwim Delta region (YKD) of Alaska (2000–2011; n = 75) and predominantly white pairs in Seattle, Washington (2014–2016; n = 57), were tested for RSV and influenza antibody using a microneutralization and hemagglutination inhibition assay, respectively, and compared between sites. Results Mean RSV antibody concentrations in pregnant women in YKD and Seattle were similar (log2 RSV antibody 10.6 vs 10.7, P = .86), but cord blood RSV antibody concentrations were significantly lower in infants born to mothers in YKD compared with Seattle (log2 RSV antibody 11.0 vs 12.2, P < .001). Maternal and cord blood influenza antibody concentrations were lower for women and infants in YKD compared with Seattle for all 4 influenza antigens tested (all P < .05). The mean cord to maternal RSV antibody transfer ratio was 1.15 (standard deviation [SD], 0.13) in mother–infant pairs in Seattle compared with 1.04 (SD, 0.08) in YKD. Mean cord blood to maternal antibody transfer ratios for influenza antigens ranged from 1.22 to 1.42 in Seattle and from 1.05 to 1.59 in YKD. Conclusions Though the transplacental antibody transfer ratio was high (>1.0) for both groups, transfer ratios for RSV antibody were significantly lower in AN mother–infant pairs. Further studies are needed to elucidate the impact of lower transplacental antibody transfer on infant disease risk in rural Alaska. Alaska Native and continental US mother-infant pairs have high transplacental antibody transfer ratios (>1.0) for influenza and respiratory syncytial virus, but anti-respiratory syncytial virus antibody levels are significantly lower in Alaska Native pairs than in those from the continental US.

PURPOSE OF REVIEW: This chapter will summarize the most recent literature regarding the current state of medical treatment for vascular anomalies. RECENT FINDINGS: Research into the biology of these anomalies has strengthened our understanding of each anomaly and has helped to pave the way for more tailored treatment options involving molecular and/or genetic targets. SUMMARY: While there is still a role for surgical intervention, medical therapies that target the etiology of vascular anomalies may represent an alternative or adjunctive approach in the management of these lesions.

ObjectiveTo describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales.MethodsThe UK’s national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code.ResultsThere were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (ConclusionTreatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.

AimsTo describe the ways in which parents recognise and make decisions about their child's symptoms following discharge home after congenital heart interventions in the first year of life and their experiences of seeking help.MethodsThis was a qualitative study involving semistructured interviews with parents. Twenty-one parents were recruited to the study. Parents all had a child who had congenital heart surgery in their first year of life between September 2009 and October 2013 at one of three UK cardiac centres; the children had either died or were readmitted as an emergency following initial discharge.ResultsSome parents were unable to identify any early warning signs. Others described symptoms of deterioration including changes in feeding and appearance, respiratory distress and subtle behavioural changes that may not be routinely highlighted to parents at discharge. Several barriers to accessing prompt medical assistance were identified including parents feeling that their concerns were not taken seriously, long wait times and lack of protocols at A&E.ConclusionsOur study highlights behavioural symptoms as being a potentially underemphasised sign of deterioration and identifies a number of barriers to parents accessing support when they are concerned. It is important that parents are encouraged to seek advice at the earliest opportunity and that those health professionals at the front line have access to the information they need in order to respond in an appropriate and timely way. A role for home monitoring was also noted as potentially useful in identifying at risk children who appear clinically well.

Among congenital heart diseases, none has undergone a more drastic change in diagnostic approach, management, and outcomes over the last 30 years than hypoplastic left heart syndrome (HLHS), with comfort care replaced as the predominant form of management by a palliative surgical treatment pathway.1 The outcomes of palliative procedures for HLHS have been well documented, but the longer-term picture is less clear. The UK National Congenital Heart Diseases Audit (NCHDA) captures every procedure undertaken for congenital heart disease and updated life status for patients resident in England and Wales from death certifications. From this data set, we identified patients born between 2000 and 2015 who fulfilled the criteria for HLHS: a small left ventricle, stenosis or atresia of the left-sided heart valves, normally aligned great arteries, and no common atrioventricular junction.2 All patients with HLHS require surgical palliation with a traditional Norwood or hybrid pathway or heart transplantation to survive. Hence, we were able to use procedure information in combination with diagnostic information to identify patients with HLHS within the NCHDA data set using the International Paediatric and Congenital Cardiac Codes. Because the NCHDA is a procedure-based data set, patients with HLHS who did not undergo any interventional treatment are not included. Because the hybrid procedure was introduced in the United Kingdom in 2006, we considered 2 eras for …

Objectives To describe the clinical presentation of lymphatic malformations (LM) and genotypically associated disorders and to summarize the recent literature regarding the genetic etiology of LM and provide a biologic correlation to medical and surgical management. Results LM are congenital lesions derived from a developmental abnormality of the lymphatic vessels. The severity of disease varies widely and complications can occur with higher staged disease and those associated with a known constellation of symptoms. Somatic mutations of the PIK3CA gene have been found to be an etiologic factor in the development of LM and associated overgrowth syndromes. Sirolimus is a mammalian target of rapamycin (mTOR) inhibitor that inhibits the pathway downstream of PIK3CA. Preliminary studies in select groups of patients suggest that sirolimus has a role in the medical management of certain aspects of this disease. Conclusions Discovery of LM molecular genetics has led to the possibility of targeted therapies and highlights the importance of precision medicine in rare diseases. Identifying genetic mutations in larger cohorts of patients with LM will lead to additional insights. Knowledge of the genetic basis for disease can then lead to discovery of directed medical therapy. A specific molecular diagnosis can also help families understand better why their child is different and provide accurate counseling for subsequent pregnancies. Level of Evidence 6

OBJECTIVES: Twenty years ago our institution published an analysis of a cluster of failures associated with the arterial switch operation (ASO). The concept of ‘near miss’ was explored to detect warning signs of suboptimal performance. The aim of the current study was to review the parameters best suited to monitoring early ASO outcomes in the modern setting and re-examine the concept of near misses as failure equivalents. METHODS: All ASOs performed in our institution between 1983 and 2012 were reviewed. The experience was divided into three eras (1983–92, 1993–2002 and 2003–12). The cumulative sum graphic for sequential monitoring was used for early mortality. The need to reestablish cardiopulmonary bypass (CPB), CPB time >240 min and extracorporeal membrane oxygenation (ECMO) were explored as variables of near misses. RESULTS: The cohort consisted of 606 patients. The 30-day mortality rate was 23% (n= 29) in Era 1, 6% (n= 14) in Era 2 and 1% (n=3) in Era 3. There were further 4, 8 and 6 deaths between 30 and 90 days in the three eras, respectively. In Era 3, the majority of deaths occurred between 30 and 90 days. In the current era, ECMO and CPB time >240 min as a marker of near miss was associated with an increased risk of death both within 30 days and 90 days after ASO. CONCLUSIONS: The 30-day outcomes of ASO have significantly improved over the last 30 years. As life-saving mechanical support after surgery has been implemented more often, an extended 90-day window of reporting can offer a more realistic outcome indicator of performance.

BackgroundBabies with CHDs are a particularly vulnerable population with significant mortality in their 1st year. Although most deaths occur in the hospital within the early postoperative period, around one-fifth of postoperative deaths in the 1st year of life may occur after hospital discharge in infants who have undergone apparently successful cardiac surgery.AimTo systematically review the published literature and identify risk factors for adverse outcomes, specifically deaths and unplanned re-admissions, following hospital discharge after infant surgery for life-threatening CHDs.MethodsA systematic search was conducted in MEDLINE, EMBASE, CINAHL, Cochrane Library, Web of Knowledge, and PsycINFO electronic databases, supplemented by manual searching of conference abstracts.ResultsA total of 15 studies were eligible for inclusion. Almost exclusively, studies were conducted in single US centres and focussed on children with complex single ventricle diagnoses. A wide range of risk factors were evaluated, and those more frequently identified as having a significant association with higher mortality or unplanned re-admission risk were non-Caucasian ethnicity, lower socio-economic status, co-morbid conditions, age at surgery, operative complexity and procedure type, and post-operative feeding difficulties.ConclusionsStudies investigating risk factors for adverse outcomes post-discharge following diverse congenital heart operations in infants are lacking. Further research is needed to systematically identify higher risk groups, and to develop interventions targeted at supporting the most vulnerable infants within an integrated primary and secondary care pathway.

AIM: To elicit the perceptions of helpline staff who talk to parents of children discharged after cardiac surgery in infancy about parents' key concerns. METHODS: A qualitative study involving semi-structured interviews with 10 staff at four heart charities. Interviews were recorded, transcribed and analysed using Framework analysis. RESULTS: Staff identified the knowledge, communication and support needs of parents which they described in terms of the impact of patient and family factors, sources of support and systems. Staff perceptions of helplines, in terms of the function of a helpline and the roles of its staff, together with staff's personal views based on their experience of multiple encounters with many families, influenced how they viewed families' needs and responded to their requests. CONCLUSION: Helpline staff provided important, previously un-captured evidence about the challenges faced by parents of children discharged after cardiac surgery in infancy. Staff have an important role in supporting communication, in terms of speaking to families about how to talk to professionals and talking to professionals directly to get or give information when parents are unable to do so. Capturing the perspective of helpline staff about communication issues has highlighted the need for interventions with professionals as well as parents. This article is protected by copyright. All rights reserved.

Funnel plots, which simultaneously display a sample statistic and the corresponding sample size for multiple cases, have a range of applications. In medicine, they are used to display treatment outcome rates and caseload volume by institution, which can inform strategic decisions about health care delivery. We investigated lay people's understanding of such plots and explored their suitability as an aid to individual treatment decisions. In two studies, 172 participants answered objective questions about funnel plots representing the surgical outcomes (survival or mortality rates) of institutions varying in caseload, and indicated their preferred institutions. Accuracy for extracting objective information was high, unless question phrasing was inconsistent with the plot's survival/mortality framing, or participants had low numeracy levels. Participants integrated caseload-volume and outcome-rate data when forming preferences, but were influenced by reference lines on the plot to make inappropriate discriminations between institutions with similar outcome rates. With careful choice of accompanying language, funnel plots can be readily understood and are therefore a useful tool for communicating risk. However, they are less effective as a decision aid for individual patient's treatment decisions, and we recommend refinements to the standard presentation of the plots if they are to be used for that purpose.

Objective To compare patient-reported, health-related quality of life (QoL) for children with serious congenital heart defects (CHDs) and unaffected classmates and to investigate the demographic and clinical factors influencing QoL. Design Retrospective cohort study. Setting UK National Health Service. Patients UK-wide cohort of children with serious CHDs aged 10–14 years requiring cardiac intervention in the first year of life in one of 17 UK paediatric cardiac surgical centres operating during 1992–1995. A comparison group of classmates of similar age and sex was recruited. Main outcome measures Child self-report of health-related QoL scores (Pediatric Quality of Life Inventory, PedsQL) and parental report of schooling and social activities. Results Questionnaires were completed by 477 children with CHDs (56% boys; mean age 12.1 (SD 1.0) years) and 464 classmates (55%; 12.0 (SD 1.1) years). Children with CHDs rated QoL significantly lower than classmates (CHDs : median 78.3 (IQR 65.0–88.6); classmates: 88.0 (80.2–94.6)) and scored lower on physical (CHDs: 84.4; classmates: 93.8; difference 9.4 (7.8 to 10.9)) and psychosocial functioning subscales (CHDs: 76.7, classmates: 85.0; difference 8.3 (6.0 to 10.6)). Cardiac interventions, school absence, regular medications and non-cardiac comorbidities were independently associated with reduced QoL. Participation in sport positively influenced QoL and was associated with higher psychosocial functioning scores. Conclusions Children with serious CHDs experience lower QoL than unaffected classmates. This appears related to the burden of clinical intervention rather than underlying cardiac diagnosis. Participation in sports activities is positively associated with increased emotional well-being. Child self-report measures of QoL would be a valuable addition to clinical outcome audit in this age group.

ObjectiveTo explore communication between clinicians and families of children undergoing heart surgery.DesignThis study was part of a larger study to select, define and measure the incidence of postoperative complications in children undergoing heart surgery. Parents of children recruited to a substudy between October 2015 and December 2017 were asked to complete a questionnaire about communication during their child’s inpatient stay. We explored all responses and then disaggregated by the following patient characteristics: presence of a complication, length of stay, hospital site, ethnicity and child’s age. This was a descriptive study only.SettingFour UK specialist hospitals.ResultsWe recruited 585 children to the substudy with 385 responses (response rate 66%).81% of parents reported that new members of staff always introduced themselves (18% sometimes, 1% no). Almost all parents said they were encouraged to be involved in decision-making, but often only to some extent (59% ‘yes, definitely’; 37% ‘to some extent’). Almost two-thirds of parents said they were told different things by different people which left them feeling confused (10% ‘a lot’; 53% ‘sometimes’). Two-thirds (66%) reported that staff were definitely aware of their child’s medical history (31% ‘to some extent’). 90% said the operation was definitely explained to them (9% ‘to some extent’) and 79% that they were definitely told what to do if they were worried after discharge (17% ‘to some extent’).Parents of children with a complication tended to give less positive responses for involvement in decision-making, consistent communication and staff awareness of their child’s medical history. Parents whose children had longer stays in hospital tended to report lower levels of consistent communication and involvement in decision-making.ConclusionsOur results emphasise the need for consistent communication with families, particularly where complications arise or for children who have longer stays in the hospital.

Human parainfluenza virus type 3 (HPIV3) is a common cause of upper and lower respiratory tract illness in infants and young children. Live-attenuated cold-adapted HPIV3 vaccines have been evaluated in infants but a suitable interval for administration of a second dose of vaccine has not been defined.HPIV3-seronegative children between the ages of 6 and 36 months were randomized 2:1 in a blinded study to receive two doses of 10⁵ TCID₅₀ (50% tissue culture infectious dose) of live-attenuated, recombinant cold-passaged human PIV3 vaccine (rHPIV3cp45) or placebo 6 months apart. Serum antibody levels were assessed prior to and approximately 4-6 weeks after each dose. Vaccine virus infectivity, defined as detection of vaccine-HPIV3 in nasal wash and/or a≥4-fold rise in serum antibody titer, and reactogenicity were assessed on days 3, 7, and 14 following immunization.Forty HPIV3-seronegative children (median age 13 months; range 6-35 months) were enrolled; 27 (68%) received vaccine and 13 (32%) received placebo. Infectivity was detected in 25 (96%) of 26 evaluable vaccinees following doses 1 and 9 of 26 subject (35%) following dose 2. Among those who shed virus, the median duration of viral shedding was 12 days (range 6-15 days) after dose 1 and 6 days (range 3-8 days) after dose 2, with a mean peak log₁₀ viral titer of 3.4 PFU/mL (SD: 1.0) after dose 1 compared to 1.5 PFU/mL (SD: 0.92) after dose 2. Overall, reactogenicity was mild, with no difference in rates of fever and upper respiratory infection symptoms between vaccine and placebo groups.rHPIV3cp45 was immunogenic and well-tolerated in seronegative young children. A second dose administered 6 months after the initial dose was restricted in those previously infected with vaccine virus; however, the second dose boosted antibody responses and induced antibody responses in two previously uninfected children.

Objective The study objective was to develop a risk model incorporating diagnostic information to adjust for case-mix severity during routine monitoring of outcomes for pediatric cardiac surgery. Methods Data from the Central Cardiac Audit Database for all pediatric cardiac surgery procedures performed in the United Kingdom between 2000 and 2010 were included: 70% for model development and 30% for validation. Units of analysis were 30-day episodes after the first surgical procedure. We used logistic regression for 30-day mortality. Risk factors considered included procedural information based on Central Cardiac Audit Database "specific procedures," diagnostic information defined by 24 "primary" cardiac diagnoses and "univentricular" status, and other patient characteristics. Results Of the 27,140 30-day episodes in the development set, 25,613 were survivals, 834 were deaths, and 693 were of unknown status (mortality, 3.2%). The risk model includes procedure, cardiac diagnosis, univentricular status, age band (neonate, infant, child), continuous age, continuous weight, presence of non–Down syndrome comorbidity, bypass, and year of operation 2007 or later (because of decreasing mortality). A risk score was calculated for 95% of cases in the validation set (weight missing in 5%). The model discriminated well; the C-index for validation set was 0.77 (0.81 for post-2007 data). Removal of all but procedural information gave a reduced C-index of 0.72. The model performed well across the spectrum of predicted risk, but there was evidence of underestimation of mortality risk in neonates undergoing operation from 2007. Conclusions The risk model performs well. Diagnostic information added useful discriminatory power. A future application is risk adjustment during routine monitoring of outcomes in the United Kingdom to assist quality assurance.

IntroductionPatients with repaired tetralogy of Fallot have good long-term survival but less is known about the subjectively assessed quality of life or objectively measured functional status of those who have not required subsequent pulmonary valve replacement. We assessed these parameters in a group of children and adults free from pulmonary valve replacement after tetralogy of Fallot repair.Methods and resultsA random sample of 50 subjects – 16 children and 34 adults, aged 4.1–56.7 years – who had undergone tetralogy of Fallot repair and were free from subsequent pulmonary valve replacement underwent cardiopulmonary exercise testing and completed standardised questionnaires assessing health-related quality of life and resilience. Patients were generally asymptomatic (median New York Heart Association class = I). Exercise capacity was within two standard deviations of normal for most children and adults (mean z VO2max: 0.20 ± 1.5; mean z VE/VCO2: −0.9 ± 1.3). Children reported a total health-related quality of life score similar to healthy norms (78 ± 10 versus 84 ± 1, p = 0.73). Adult survivors also reported quality of life scores comparable to healthy norms. Resilience was highly correlated with all domains of health-related quality of life (r = 0.713, p < 0.0001).ConclusionsPatients who have undergone tetralogy of Fallot repair in childhood and have not required pulmonary valve replacement have a good long-term health-related quality of life. The finding that patients with greater resilience had better health-related quality of life suggests that it may be beneficial to implement interventions to foster resilience.

As mortality after major cardiac surgery in early childhood falls, it becomes clearer that there is a high prevalence of ‘non-cardiac’ problems in survivors. Patient support groups for cardiac children have long lobbied for better access to general paediatric expertise and upcoming organizational changes underwrite this. Motor and cognitive deficits as well as emotional and behavioural difficulties may need addressing; all impact the child's quality of life. Participation in exercise-based activities and quality of life correlate poorly with disease severity, except at the worst end of the spectrum of heart disease. Cardiac function as measured objectively poorly predicts children's activity in daily life and some children are actively limited by personal habit or family concern, which is often inappropriate. Enough is now known about the prevalence of problems that survivors of childhood cardiac surgery experience in the education system that support may be justified, even when the original cardiac condition is no longer an ongoing problem.

Objective To evaluate the scale and clinical importance of loss to follow-up of past patients with serious congenital heart disease, using a common malformation as an example. To better understand the antecedents of loss to specialist follow-up and patients’ attitudes to returning. Design Cohort study using NHS number functionality. Content and thematic analysis of telephone interviews of subset contacted after loss to follow-up. Patients, intervention and setting Longitudinal follow-up of complete consecutive list of all 1085 UK patients with repair of tetralogy of Fallot from single institution 1964–2009. Main outcome measures Survival, freedom from late pulmonary valve replacement, loss to specialist followup, shortfall in late surgical revisions related to loss to follow-up. Patients’ narrative about loss to follow-up. Results 216 (24%) of patients known to be currently alive appear not to be registered with specialist clinics; some are seen in general cardiology clinics. Their median age is 32 years and median duration of loss to follow-up is 22 years; most had been lost before Adult Congenital services had been consolidated in their present form. 48% of the late deaths to date have occurred in patients not under specialist follow-up. None of those lost to specialist follow-up has had secondary pulmonary valve replacement while 188 patients under specialist care have. Patients lost to specialist follow-up who were contacted by telephone had no knowledge of its availability. Conclusions Loss to specialist follow-up, typically originating many years ago, impacts patient management.

OBJECTIVE: Meeting the needs of parents of critically ill children is increasingly being recognized as an important factor in the child's clinical outcome as well as the implications it has for future parenting. Little is specifically known about the experience of parents who have a child in the ICU for a prolonged period. Our objective was to understand the experiences of this group to assist in the identification of mechanisms for providing support. DESIGN: Qualitative study based on semistructured interviews. Data were analyzed using the Framework approach. SETTING: The study took place in a tertiary pediatric hospital containing three ICUs: PICU, cardiac ICU, and neonatal ICU (ICU will be used to encompass neonatal ICU, cardiac ICU, and PICU for the remainder of the article). SUBJECTS: Seventeen members of staff and 26 parents of a child who had a long stay in one of the three ICUs. INTERVENTIONS: Semistructured, tape-recorded interviews. MEASUREMENTS AND MAIN RESULTS: Analyses identified two overarching themes, "parent process" and "parent/staff interface," and six subthemes: adjustment, normalization/institutionalization, conflict, involvement in child's care, individualized care, and transition. Themes have been modeled to account for the parental process during a long stay in the ICU and the potential implications for the relationships and interactions between parents and staff. Findings are presented from the data related to the parent process and parent/staff interface. Inclusion of an element in the model related to staff indicates where support mechanisms should be focused. CONCLUSIONS: Over time, long-stay families will likely become more familiar with the environment of the ICU, more knowledgeable about their child's medical needs and more familiar with the staff looking after their child. This has a number of implications for staff working in the ICU.

Objective: Compared to shorter-stay patients, caring for long-stay patients in the ICU entails a disproportionate burden for staff. Our objective was to gain a deeper understanding of the impact on staff of caring for children who have a prolonged stay on the PICU. Design: Qualitative study based on semi-structured interviews. Data were analyzed using the Framework approach. Setting: Children’s tertiary hospital. Participants: Seventeen members of staff (7 psychosocial staff, 7 nurses, 3 consultants) working in the PICU, neonatal ICU, or cardiac ICU (PICU will be used to encompass neonatal ICU, cardiac ICU, and PICU for the remainder of this article). Interventions: Semi-structured, tape-recorded interviews. Measurements and Main Results: Staff reported both positive and challenging aspects of caring for long-stay patients in the PICU. Five key areas relating to the challenges of caring for long-stay patients were identified: staff expectations about their work, characteristics of the patient group, the impact on staff, the impact on the wider unit, and the availability of support. Staff views were often compounded by individual cases they had been involved with or had heard about which fell at either end of the spectrum of “good” and “bad”. Conclusions: Whilst there are reported benefits associated with caring for long-stay patients, there are a number of challenges reported that may have implications for staff and the wider unit. When caring for a particular sub-group of long-stay patients, staff may be more likely to experience negative impacts. A key priority for the PICU is to ensure that support mechanisms are timely, accessible, and allow staff to explore their own reactions to their work.

Background Improvements in hospital‐based care have reduced early mortality in congenital heart disease. Later adverse outcomes may be reducible by focusing on care at or after discharge. We aimed to identify risk factors for such events within 1 year of discharge after intervention in infancy and, separately, to identify subgroups that might benefit from different forms of intervention. Methods and Results Cardiac procedures performed in infants between 2005 and 2010 in England and Wales from the UK National Congenital Heart Disease Audit were linked to intensive care records. Among 7976 infants, 333 (4.2%) died before discharge. Of 7643 infants discharged alive, 246 (3.2%) died outside the hospital or after an unplanned readmission to intensive care (risk factors were age, weight‐for‐age, cardiac procedure, cardiac diagnosis, congenital anomaly, preprocedural clinical deterioration, prematurity, ethnicity, and duration of initial admission; c‐statistic 0.78 [0.75–0.82]). Of the 7643, 514 (6.7%) died outside the hospital or had an unplanned intensive care readmission (same risk factors but with neurodevelopmental condition and acquired cardiac diagnosis and without preprocedural deterioration; c‐statistic 0.78 [0.75–0.80]). Classification and regression tree analysis were used to identify 6 subgroups stratified by the level (3–24%) and nature of risk for death outside the hospital or unplanned intensive care readmission based on neurodevelopmental condition, cardiac diagnosis, congenital anomaly, and duration of initial admission. An additional 115 patients died after planned intensive care admission (typically following elective surgery). Conclusions Adverse outcomes in the year after discharge are of similar magnitude to in‐hospital mortality, warrant service improvements, and are not confined to diagnostic groups currently targeted with enhanced monitoring.

IntroductionEthnic differences in the birth prevalence of congenital heart defects (CHDs) have been reported; however, studies of the contemporary UK population are lacking. We investigated ethnic variations in incidence of serious CHDs requiring cardiac intervention before 1 year of age.MethodsAll infants who had a cardiac intervention in England and Wales between 1 January 2005 and 31 December 2010 were identified in the national congenital heart disease surgical audit and matched with paediatric intensive care admission records to create linked individual child records. Agreement in reporting of ethnic group by each audit was evaluated. For infants born 1 January 2006 to 31 December 2009, we calculated incidence rate ratios (IRRs) for CHDs by ethnicity and investigated age at intervention, antenatal diagnosis and area deprivation.ResultsWe identified 5350 infants (2940 (55.0%) boys). Overall CHD incidence was significantly higher in Asian and Black ethnic groups compared with the White reference population (incidence rate ratios (IRR) (95% CIs): Asian 1.5 (1.4 to 1.7); Black 1.4 (1.3 to 1.6)); incidence of specific CHDs varied by ethnicity. No significant differences in age at intervention or antenatal diagnosis rates were identified but affected children from non-White ethnic groups were more likely to be living in deprived areas than White children.ConclusionsSignificant ethnic variations exist in the incidence of CHDs, including for specific defects with high infant mortality. It is essential that healthcare provision mitigates ethnic disparity, including through timely identification of CHDs at screening, supporting parental choice and effective interventions. Future research should explore the factors underlying ethnic variation and impact on longer-term outcomes.

BackgroundWhile early outcomes of paediatric cardiac surgery have improved, less attention has been given to later outcomes including post-discharge mortality and emergency readmissions.ObjectivesOur objectives were to use a mixed-methods approach to build an evidenced-based guideline for postdischarge management of infants undergoing interventions for congenital heart disease (CHD).MethodsSystematic reviews of the literature – databases used: MEDLINE (1980 to 1 February 2013), EMBASE (1980 to 1 February 2013), Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1981 to 1 February 2013), The Cochrane Library (1999 to 1 February 2013), Web of Knowledge (1980 to 1 February 2013) and PsycINFO (1980 to 1 February 2013). Analysis of audit data from the National Congenital Heart Disease Audit and Paediatric Intensive Care Audit Network databases pertaining to records of infants undergoing interventions for CHD between 1 January 2005 and 31 December 2010. Qualitative analyses of online discussion posted by 73 parents, interviews with 10 helpline staff based at user groups, interviews with 20 families whose infant either died after discharge or was readmitted urgently to intensive care, and interviews with 25 professionals from tertiary care and 13 professionals from primary and secondary care. Iterative multidisciplinary review and discussion of evidence incorporating the views of parents on suggestions for improvement.ResultsDespite a wide search strategy, the studies identified for inclusion in reviews related only to patients with complex CHD, for whom adverse outcome was linked to non-white ethnicity, lower socioeconomic status, comorbidity, age, complexity and feeding difficulties. There was evidence to suggest that home monitoring programmes (HMPs) are beneficial. Of 7976 included infants, 333 (4.2%) died postoperatively, leaving 7634 infants, of whom 246 (3.2%) experienced outcome 1 (postdischarge death) and 514 (6.7%) experienced outcome 2 (postdischarge death plus emergency intensive care readmissions). Multiple logistic regression models for risk of outcomes 1 and 2 had areas under the receiver operator curve of 0.78 [95% confidence interval (CI) 0.75 to 0.82] and 0.78 (95% CI 0.75 to 0.80), respectively. Six patient groups were identified using classification and regression tree analysis to stratify by outcome 2 (range 3–24%), which were defined in terms of neurodevelopmental conditions, high-risk cardiac diagnosis (hypoplastic left heart, single ventricle or pulmonary atresia), congenital anomalies and length of stay (LOS) > 1 month. Deficiencies and national variability were noted for predischarge training and information, the process of discharge to non-specialist services including documentation, paediatric cardiology follow-up including HMP, psychosocial support post discharge and the processes for accessing help when an infant becomes unwell.ConclusionsNational standardisation may improve discharge documents, training and guidance on ‘what is normal’ and ‘signs and symptoms to look for’, including how to respond. Infants with high-risk cardiac diagnoses, neurodevelopmental conditions or LOS > 1 month may benefit from discharge via their local hospital. HMP is suggested for infants with hypoplastic left heart, single ventricle or pulmonary atresia. Discussion of postdischarge deaths for infant CHD should occur at a network-based multidisciplinary meeting. Audit is required of outcomes for this stage of the patient journey.Future workFurther research may determine the optimal protocol for HMPs, evaluate the use of traffic light tools for monitoring infants post discharge and develop the analytical steps and processes required for audit of postdischarge metrics.Study registrationThis study is registered as PROSPERO CRD42013003483 and CRD42013003484.FundingThe National Institute for Health Research Health Services and Delivery Research programme. The National Congenital Heart Diseases Audit (NCHDA) and Paediatric Intensive Care Audit Network (PICANet) are funded by the National Clinical Audit and Patient Outcomes Programme, administered by the Healthcare Quality Improvement Partnership (HQIP). PICAnet is also funded by Welsh Health Specialised Services Committee; NHS Lothian/National Service Division NHS Scotland, the Royal Belfast Hospital for Sick Children, National Office of Clinical Audit Ireland, and HCA International. The study was supported by the National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London. Sonya Crowe was supported by the Health Foundation, an independent charity working to continuously improve the quality of health care in the UK.

Background Improving integration and continuity of care across sectors within resource constraints is a priority in many health systems. Qualitative operational research methods of problem structuring have been used to address quality improvement in services involving multiple sectors but not in combination with quantitative operational research methods that enable targeting of interventions according to patient risk. We aimed to combine these methods to augment and inform an improvement initiative concerning infants with congenital heart disease (CHD) whose complex care pathway spans multiple sectors. Methods Soft systems methodology was used to consider systematically changes to services from the perspectives of community, primary, secondary and tertiary care professionals and a patient group, incorporating relevant evidence. Classification and regression tree (CART) analysis of national audit datasets was conducted along with data visualisation designed to inform service improvement within the context of limited resources. Results A ‘Rich Picture’ was developed capturing the main features of services for infants with CHD pertinent to service improvement. This was used, along with a graphical summary of the CART analysis, to guide discussions about targeting interventions at specific patient risk groups. Agreement was reached across representatives of relevant health professions and patients on a coherent set of targeted recommendations for quality improvement. These fed into national decisions about service provision and commissioning. Conclusions When tackling complex problems in service provision across multiple settings, it is important to acknowledge and work with multiple perspectives systematically and to consider targeting service improvements in response to confined resources. Our research demonstrates that applying a combination of qualitative and quantitative operational research methods is one approach to doing so that warrants further consideration.

Objective To explore the effect of research ethics, governance and consent requirements and recent reforms on UK-wide follow-up of children with congenital heart defects (CHD). Design Prospective cohort study. Setting UK National Health Service. Patients 3897 children with CHD requiring intervention, or resulting in death, before they were 1-year-old (1993–1995). Main outcomes Impact on study protocol, timeliness and findings of a multicentre study of survival and quality of life. Results The peer-reviewed study protocol was altered to accommodate ethics committee stipulations that researchers should not approach families directly with a request to participate and that the general practitioner9s (GP) permission be sought before the local clinician could do so. Individual consent was required to confirm the vital status of participants and for future tracing of public death registrations. Local study registration took a median of 40 weeks (IQR 25–57). 180 (24%) of 739 surviving children (five centres) could not be contacted because their GP was untraceable (32), had changed (128) or considered contact inappropriate (20). Invitations could not be sent to 31% from the most deprived compared with 17% from the least deprived areas. Conclusions Decision making concerning childhood interventions should be influenced by evidence on long-term outcomes. However, current UK research regulations hinder follow-up in multicentre studies. Stipulations preventing researchers contacting families directly with research invitations appear disproportionate to the risks, impede equitable access to research opportunities and introduce bias. The requirement for an individual9s consent to confirm whether they are alive and monitor survival precludes effective long-term follow-up.

Objectives: Congenital heart defects (CHD) are an important cause of death and morbidity in early childhood, but the effectiveness of alternative newborn screening strategies in preventing the collapse or death—before diagnosis—of infants with treatable but life-threatening defects is uncertain. We assessed their effectiveness and efficiency to inform policy and research priorities.Methods: We compared the effectiveness of clinical examination alone and clinical examination with either pulse oximetry or screening echocardiography in making a timely diagnosis of life-threatening CHD or in diagnosing clinically significant CHD. We contrasted their cost-effectiveness, using a decision-analytic model based on 100,000 live births, and assessed future research priorities using value of information analysis.Results: Clinical examination alone, pulse oximetry, and screening echocardiography achieved 34.0, 70.6, and 71.3 timely diagnoses per 100,000 live births, respectively. This finding represents an additional cost per additional timely diagnosis of £4,894 and £4,496,666 for pulse oximetry and for screening echocardiography. The equivalent costs for clinically significant CHD are £1,489 and £36,013, respectively. Key determinants of cost-effectiveness are detection rates and screening test costs. The false-positive rate is very high with screening echocardiography (5.4 percent), but lower with pulse oximetry (1.3 percent) or clinical examination alone (.5 percent).Conclusions: Adding pulse oximetry to clinical examination is likely to be a cost-effective newborn screening strategy for CHD, but further research is required before this policy can be recommended. Screening echocardiography is unlikely to be cost-effective, unless the detection of all clinically significant CHD is considered beneficial and a 5 percent false-positive rate acceptable.

ObjectiveTo qualitatively assess the discharge processes and postdischarge care in the community for infants discharged after congenital heart interventions in the first year of life.DesignQualitative study using semistructured interviews and Framework Analysis.SettingUK specialist cardiac centres and the services their patients are discharged to.SubjectsTwenty-five cardiologists and nurses from tertiary centres, 11 primary and secondary health professionals and 20 parents of children who had either died after discharge or had needed emergency readmission.ResultsParticipants indicated that going home with an infant after cardiac intervention represents a major challenge for parents and professionals. Although there were reported examples of good care, difficulties are exacerbated by inconsistent pathways and potential loss of information between the multiple teams involved. Written documentation from tertiary centres frequently lacks crucial contact information and contains too many specialist terms. Non-tertiary professionals and parents may not hold the information required to respond appropriately when an infant deteriorates, this contributing to the stressful experience of managing these infants at home. Where they exist, the content of formal ‘home monitoring pathways’ varies nationally, and families can find this onerous.ConclusionsService improvements are needed for infants going home after cardiac intervention in the UK, focusing especially on enhancing mechanisms for effective transfer of information outside the tertiary centre and processes to assist with monitoring and triage of vulnerable infants in the community by primary and secondary care professionals. At present there is no routine audit for this stage of the patient journey.

Surgical innovations are often introduced for their expected long-term benefits, but the decision to abandon the existing treatment must be based on the available short-term data and rational judgment. We present a framework for monitoring the introduction of a surgical intervention with long-term consequences and failure-time endpoints. The framework is based on Bayesian methods, and formally combines study data, clinical opinion, and external evidence to construct a posterior survival function from which intuitive summary statistics can be extracted to aid decision making. It incorporates learning effects and is adaptable to a wide variety of settings. The methods are illustrated on survival data from a cohort of 325 consecutive neonates treated for simple transposition of the great arteries with either the Senning or the Switch operation during the period 1978-1998. Udgivelsesdato: FEB 10 Surgical innovations are often introduced for their expected long-term benefits, but the decision to abandon the existing treatment must be based on the available short-term data and rational judgment. We present a framework for monitoring the introduction of a surgical intervention with long-term consequences and failure-time endpoints. The framework is based on Bayesian methods, and formally combines study data, clinical opinion, and external evidence to construct a posterior survival function from which intuitive summary statistics can be extracted to aid decision making. It incorporates learning effects and is adaptable to a wide variety of settings. The methods are illustrated on survival data from a cohort of 325 consecutive neonates treated for simple transposition of the great arteries with either the Senning or the Switch operation during the period 1978-1998.

BACKGROUND The objective of the current study was to examine the risk factors for progression in severity of anthracycline-induced cardiac dysfunction, thereby providing information that is useful in refining cancer treatment regimes and guiding follow-up. METHODS Serial echocardiograms were performed on 101 acute lymphoblastic leukemia survivors and 83 Wilms tumor survivors after a mean interval of 6.2 years and 6.7 years since last anthracycline dose, respectively, at first study, and after 10.3 years and 11.1 years, respectively, at second study. The paired data were contrasted with data from 100 normal subjects, and potential correlations with follow-up interval, cumulative dose, cancer diagnosis, gender, age at diagnosis, and growth were explored using univariate and multiple regression techniques. RESULTS The most important predictor of worsening cardiac performance was total anthracycline dose. As a group, patients receiving 10 years from the end of treatment. CONCLUSIONS Survivors who have received low-dose anthracycline require cardiac surveillance infrequently. In good prognosis tumors, cumulative anthracycline dose should be maintained at < 250 mg/m2. Cancer 2003;97:1991–8. © 2003 American Cancer Society. DOI 10.1002/cncr.11274

In this case report, we describe the use of the Aquadex™ system for ultrafiltration therapy in the pediatric cardiac intensive care setting in a patient with fluid overload and acute kidney injury after congenital heart surgery. The patient is an 11-year-old, 25 kg male with complex single ventricle anatomy who underwent a one and a half ventricle repair. The patient experienced multiple organ dysfunction syndrome including acute kidney injury in the early post-operative period secondary to low cardiac output syndrome and tachyarrhythmia. Ultrafiltration using the Aquadex™ system was utilized to treat fluid overload in the setting of acute kidney injury and hemodynamic instability. Negative fluid balance was safely achieved. It was subsequently possible to wean ventilatory and inotropic support. We conclude that the use of ultrafiltration therapy is feasible in hemodynamically unstable pediatric patients with significant fluid overload in the setting of acute kidney injury following congenital heart surgery.

Summary Background Assessment of the effect of fetal diagnosis on the prevalence of congenital heart disease at term requires national ascertainment because referral patterns are not rigorously structured. Methods Between 1993 and 1995, all 17 paediatric cardiac centres in the UK submitted to a database lists of all fetuses diagnosed, and all infants needing surgery or interventional catheterisation or dying in the first year of life because of structural heart disease; details included the postal area of residence. Findings There were 4799 affected pregnancies, 4165 babies born alive, 1124 fetal diagnoses, and 567 terminations of pregnancy because the fetus had structural heart disease. Thus, a fetal diagnosis was made in 23·4% of affected pregnancies (11·7% of all affected livebirths) with geographical variability in diagnostic rates. Interpretation Fetal cardiac screening has an effect on the prevalence and types of congenital heart disease seen at term because many affected pregnancies are terminated. If detection rates of affected fetuses rose nationally to those seen in the 15 postal areas where detection rates were significantly higher than the national average in 1993–95, we would expect about 218 fewer affected individuals to be born annually.

Objectives Many infants die in the year following discharge from hospital after surgical or catheter intervention for congenital heart disease (3–5% of discharged infants). There is considerable variability in the provision of care and support in this period, and some families experience barriers to care. We aimed to identify ways to improve discharge and postdischarge care for this patient group. Design A systematic evidence synthesis aligned with a process of eliciting the perspectives of families and professionals from community, primary, secondary and tertiary care. Setting UK. Results A set of evidence-informed recommendations for improving the discharge and postdischarge care of infants following intervention for congenital heart disease was produced. These address known challenges with current care processes and, recognising current resource constraints, are targeted at patient groups based on the number of patients affected and the level and nature of their risk of adverse 1-year outcome. The recommendations include: structured discharge documentation, discharging certain high-risk patients via their local hospital, enhanced surveillance for patients with certain (high-risk) cardiac diagnoses and an early warning tool for parents and community health professionals. Conclusions Our recommendations set out a comprehensive, system-wide approach for improving discharge and postdischarge services. This approach could be used to address challenges in delivering care for other patient populations that can fall through gaps between sectors and organisations.

Aims Whilst patient turnover in the Paediatric Intensive Care Unit (PICU) is high with an average stay of 2 days, improvements in life-sustaining treatments have resulted in increasing numbers of children who are hospitalised for long periods of time. A child’s admission to the PICU induces high levels of stress and anxiety in parents. A growing number of research studies have explored the needs of family members of critically ill children. However, little is known about the experience of parents who have a child in the PICU for a prolonged period of time. Methods Semi-structured interviews were conducted with 17 members of staff (3 consultants, 7 nursing staff and 7 allied-health professionals) and 27 parents whose child had a long-stay in one of three units providing intensive care at a tertiary paediatric hospital. Framework Analysis was used to analyse the data. Results Every family is different in how they cope and adapt to being in the PICU for a prolonged period of time. Despite differences, a number of things characterised long-stay parents’ experiences of being in the PICU. Parents adjusted to being in the PICU, developing routines which supported coping. Long-stay parents were grateful for any opportunity to be involved in their child’s care and relied upon the nursing staff to facilitate this. Their exposure to the PICU resulted in increased knowledge of their child’s condition and the environment. This knowledge led to heightened sensitivity to, and awareness of, the care provided by staff; both the good and bad. Moving on from the PICU can be particularly difficult for long-stay parents who have become accustomed to life in the PICU. Conclusion Long-stay families will likely become more familiar with the environment, more knowledgeable about their child’s medical needs and more familiar with the staff looking after their child than average-stay parents in the PICU. They are likely to seek a greater involvement in their child’s care and may be more critical of the care provided by staff. This has implications for staff working in the PICU.

Increasing numbers of operations in small infants with complex congenital heart disease are being carried out in the UK year on year, with more surviving the initial operation. However, even after successful surgery some of these infants remain fragile when they are discharged home. The aim of the study was to elicit parents' experiences of caring for a child with complex needs after major congenital heart surgery. We conducted a qualitative study involving semi-structured interviews with parents of 20 children (aged

ObjectiveTo categorise records according to primary cardiac diagnosis in the United Kingdom Central Cardiac Audit Database in order to add this information to a risk adjustment model for paediatric cardiac surgery.DesignCodes from the International Paediatric Congenital Cardiac Code were mapped to recognisable primary cardiac diagnosis groupings, allocated using a hierarchy and less refined diagnosis groups, based on the number of functional ventricles and presence of aortic obstruction.SettingA National Clinical Audit Database.PatientsChildren undergoing cardiac interventions: the proportions for each diagnosis scheme are presented for 13,551 first patient surgical episodes since 2004.ResultsIn Scheme 1, the most prevalent diagnoses nationally were ventricular septal defect (13%), patent ductus arteriosus (10.4%), and tetralogy of Fallot (9.5%). In Scheme 2, the prevalence of a biventricular heart without aortic obstruction was 64.2% and with aortic obstruction was 14.1%; the prevalence of a functionally univentricular heart without aortic obstruction was 4.3% and with aortic obstruction was 4.7%; the prevalence of unknown (ambiguous) number of ventricles was 8.4%; and the prevalence of acquired heart disease only was 2.2%. Diagnostic groups added to procedural information: of the 17% of all operations classed as “not a specific procedure”, 97.1% had a diagnosis identified in Scheme 1 and 97.2% in Scheme 2.ConclusionsDiagnostic information adds to surgical procedural data when the complexity of case mix is analysed in a national database. These diagnostic categorisation schemes may be used for future investigation of the frequency of conditions and evaluation of long-term outcome over a series of procedures.

Objective and Sample: This investigation assessed the comprehension of survival curves in a community sample of 88 young and middle-aged adults when several aspects of good practice for graphical communication were implemented, and it compared comprehension for alternative presentation formats. Design, Method, and Measurements: After reading worked examples of using survival curves that provided explanation and answers, participants answered questions on survival data for pairs of treatments. Study 1 compared presenting survival curves for both treatments on the same figure against presentation via 2 separate figures. Study 2 compared presenting data for 3 possible outcome states via a single “multistate” figure for each treatment against presenting each outcome on a separate figure (with both treatments on the same figure). Both studies compared alternative forms of questioning (e.g., “number alive” versus “number dead”). Numeracy levels (self-rated and objective measures) were also assessed. Results: Comprehension was generally good—exceeding 90% correct answers on half the questions—and was similar across alternative graphical formats. Lower accuracy was observed for questions requiring a calculation but was significantly lower only when the requirement for calculation was not explicit (13%–28% decrements in performance). In study 1, this effect was most acute for those with lower levels of numeracy. Subjective (self-rated) numeracy and objective (measured) numeracy were both moderate positive predictors of overall task accuracy (r ≈ 0.3). Conclusions: A high degree of accuracy in extracting information from survival curves is possible, as long as any calculations that are required are made explicit (e.g., finding differences between 2 survival rates). Therefore, practitioners need not avoid using survival curves in discussions with patients, although clear and explicit explanations are important

BackgroundTechnological advances in surgery, intensive care and medical support have led to substantial decrease in mortality for children with congenital heart defects (CHDs) over the last 50 years.MethodsUsing routinely-collected mortality and population data for England and Wales from 1959 to 2009, the authors investigated age, period and birth cohort trends in child mortality attributable to CHDs.ResultsThe total number of deaths with CHDs at all ages between 1959 and 2009 was 61 903 (33 929 (55%) males). Absolute numbers of CHD-related deaths in children (under age 15 years) fell from 1460 in 1959 to 154 in 2009. Infants (aged under 1 year) comprised over 60% of all deaths due to CHD during the 5-year period 1959–1963, but this fell to 22% by 2004–2008. Age-standardised death rates have declined for both sexes but, despite narrowing sex differences, males continue to have higher death rates. Successive birth cohorts have experienced improved death rates in the first year of life; however, declining mortality across all age-groups has only been observed for birth cohorts originating after 1989. Poisson regression modelling predicts continuing generational decline in mortality.ConclusionsDeath rates attributable to CHDs have fallen dramatically with advances in paediatric cardiac surgery and intensive care, largely due to decreased mortality in infants aged under 1 year. Initially, mortality in later childhood rose as infant deaths fell, suggesting death was delayed beyond infancy. Children born within the last 20 years experienced lower mortality throughout childhood.