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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.

We present a rare case of a two-week-old infant with tetralogy of Fallot , absent pulmonary valve syndrome , right aortic arch, and disconnected left pulmonary artery (LPA) whose origin was from ductal ligament adjacent to the left subclavian artery. One-stage surgical correction, including closure of ventricular septal defect (VSD), LPA reconstruction and reconnection to the pulmonary trunk, reduction in size of the right pulmonary artery (RPA), and right ventricular outflow tract reconstruction with valved conduit, was successfully performed with good clinical mid-term outcome.

Introduction Babies with cardiac anomalies are often asymptomatic at birth, and many remain undetected despite routine newborn examination. We retrospectively assessed the effect of routine pulse oximetry in detection of such anomalies from a hospital birth population of 31 946 babies born between 1 April 1999 and 31 March 2009. Method 29 925 babies who were not admitted to the neonatal unit at birth underwent postductal oxygen saturation measurement before discharge. If saturation was below 95% an examination was performed. If this was abnormal or saturation remained low, an echocardiogram was performed. All babies with cardiac anomaly diagnosed before 1-year were identified from the region9s fetal abnormality database. Results Critical anomalies affected 27 infants (1 in 1180); 10 identified prenatally, 2 after echocardiogram was performed because of other anomalies, 2 in preterm infants, 2 when symptomatic before screening, 5 by oximetry screening, 1 when symptomatic in hospital after a normal screen and 5 after discharge home. Serious anomalies affected 50 infants (1 in 640); 8 identified antenatally, 7 because of other anomalies, 3 in the neonatal unit, 5 by pulse oximetry screening, 11 by routine newborn examination, and 16 after discharge home. Conclusions Routine pulse oximetry aided detection of 5/27 of critical and 5/50 of serious anomalies in this sample, but did not prevent five babies with critical and 15 with serious anomalies being discharged undiagnosed. Results from screening over 250 000 babies have now been published, but this total includes only 49 babies with transposition, and even smaller numbers of rarer anomalies.

Objective The aim of this study was to examine the accuracy in predicting pathway location in children with Wolff–Parkinson–White syndrome for each of seven published algorithms. Patients and interventions ECGs from 100 consecutive children with Wolff–Parkinson–White syndrome undergoing electrophysiological study were analysed by six investigators using seven published algorithms, six of which had been developed in adult patients. Main outcome measures Accuracy and concordance of predictions were adjusted for the number of pathway locations. Results Accessory pathways were left-sided in 49, septal in 20 and right-sided in 31 children. Overall accuracy of prediction was 30–49% for the exact location and 61–68% including adjacent locations. Concordance between investigators varied between 41% and 86%. No algorithm was better at predicting septal pathways (accuracy 5–35%, improving to 40–78% including adjacent locations), but one was significantly worse. Predictive accuracy was 24–53% for the exact location of right-sided pathways (50–71% including adjacent locations) and 32–55% for the exact location of left-sided pathways (58–73% including adjacent locations). Conclusions All algorithms were less accurate in our hands than in other authors9 own assessment. None performed well in identifying midseptal or right anteroseptal accessory pathway locations.

Cardiovascular malformations are an important cause of infant death and the major cause of death due to malformation. Our aims were to analyse and categorise all deaths in infants with cardiovascular malformations, and to analyse trends in mortality over time and influences on mortality. We obtained details of infant deaths and cardiovascular malformations from the population of one health region for 1987–2006. We categorised deaths by cause and by presence of additional chromosomal or genetic abnormalities or non-cardiac malformations. In 676,927 live births the total infant mortality was 4,402 (6.5 per 1,000). A total of 4,437 infants had cardiovascular malformations (6.6 per 1000) of whom 458 (10.3%) died before 1 year of age. Of this number, 151 (33%) deaths had non-cardiac causes, 128 (28%) were cardiac without surgery and 179 (39%) occurred from cardiac causes after surgery. Death was unrelated to the cardiovascular malformation in 57% of infants with an additional chromosomal or genetic abnormality, in 76% of infants with a major non-cardiac malformation and in 16% of infants with an isolated cardiovascular malformation. Terminations of pregnancies affected by cardiovascular malformations increased from 20 per 100,000 registered births in the first 5 years to 78 per 100,000 in the last 5 years. A total of 2,067 infants (47%) underwent surgery and of these 216 (10%) died before 1 year of age. Conclusions: A total of 10.4% of infants who died had a cardiovascular malformation and two-thirds of deaths were due to the malformation or its treatment. Mortality declined due to increasing termination of pregnancy and improved survival after operation.

The sudden death of a child or teenager is a rare event with a profound impact on parents, family, friends and schoolmates. It often generates much publicity and instils fear in the parents of other healthy children. If the postmortem examination reveals a previously unsuspected cardiac abnormality, this raises the question of whether it could have been detected and death could have been prevented. If no cause is found at autopsy, and a cardiac arrhythmia is presumed to be responsible, there is no satisfactory resolution for the parents. Screening programmes for “young athletes” have been proposed in Europe and the USA based on the consensus views of experts and informed mainly by data from sudden deaths up to the age of 35 years.w1 This review will examine the feasibility of detection of potentially fatal latent heart defects and the possibility of screening in children and teenagers. Specific data on unexpected sudden death in children and teenagers are not easy to find. Corrado et al reported a rate of 1.0/105 in Italians up to the age of 35 years.1 The risk increased with age in this population so the proportion younger than 20 years was probably less than half. A report from an International Olympic Committee working group found that 40% of such deaths occurred under the age of 18 years.2 Maron reported a sudden death risk of 0.46/105 person years in high school athletes.3 Van Camp reported a rate of 0.4/105 in high school and college athletes, being 0.75/105 in males and 0.13/105 in females.w2 A study of cardiac arrest in schools estimated an annual incidence of 0.18/105 person-years among students.w3 It is reasonable to conclude that the overall average risk of sudden cardiac death in apparently normal …

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.