Editorial
Pediatric & Congenital Electrophysiology Society: building an international paediatric electrophysiology organisation
- Mitchell Cohen, Shubhayan Sanatani, Elizabeth Stephenson, Jon Skinner, Fabrizio Drago, Andrew Davis, Jan Janousek, Eric Rosenthal, Kathryn K. Collins, John Triedman
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- Published online by Cambridge University Press:
- 14 April 2016, pp. 1039-1043
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The Pediatric and Congenital Electrophysiology Society (PACES) is a non-profit organisation comprised of individuals dedicated to improving the care of children and young adults with cardiac rhythm disturbances. Although PACES is a predominantly North American-centric organisation, international members have been a part of PACES for the last two decades. This year, PACES expanded its North American framework into a broadly expansive international role. On 12 May, 2015, paediatric electrophysiology leaders from within the United States of America and Canada met with over 30 international paediatric electrophysiologists from 17 countries and five continents discussing measures to (1) expand PACES’ global vision, (2) address ongoing challenges such as limited resource allocation that may be present in developing countries, (3) expand PACES’ governance to include international representation, (4) promote joint international sessions at future paediatric EP meetings, and (5) facilitate a global multi-centre research consortium. This meeting marked the inception of a formal international collaborative spirit in PACES. This editorial addresses some solutions to breakdown the continental silos paediatric electrophysiologists have practiced within; however, there remain ongoing limitations, and future discussions will be needed to continue to move the PACES global international vision forward.
Review Article
Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management
- M. Cecilia Gonzalez Corcia, Carlo de Asmundis, Gian-Battista Chierchia, Pedro Brugada
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- Published online by Cambridge University Press:
- 06 May 2016, pp. 1044-1055
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Brugada syndrome is an inherited arrhythmogenic disorder, characterised by coved-type ST-segment elevation in the right precordial leads, and is associated with increased risk of sudden death. It is genetically and clinically heterogeneous, presenting typically in the fourth or fifth decade of life. The prevalence of Brugada syndrome in the paediatric population is low compared with the adult population. Interestingly, over the last several years, there has been growing evidence in the literature of onset of the disease during childhood. Most of the paediatric cases reported in the literature consist of asymptomatic Brugada syndrome; however, some patients manifest the disease at different regions of the cardiac conduction system at a young age. Early expression of the disease can be affected by multiple factors, including genetic substrate, hormonal changes, and still unknown environmental exposures. The initial manifestation of Brugada syndrome in children can include sinus node dysfunction and atrial arrhythmias. Brugada syndrome can also manifest as ventricular arrhythmias leading to sudden death at an early age. In symptomatic children, performance of the ajmaline test by an experienced team can be safely used as a diagnostic tool to unmask latent Brugada syndrome. Defining indications for an implantable cardioverter defibrillator in children with the diagnosis of Brugada syndrome remains challenging. Given the rarity of the syndrome in children, most paediatric cardiologists will only rarely see a young patient with Brugada syndrome and there is still no universal consensus regarding the optimal management approach. Care should be individualised according to the specific clinical presentation, taking into account the family history, genetic data, and the family’s specific preferences.
Original Articles
Is pre-discharge echocardiography indicated for asymptomatic neonates with a heart murmur? A retrospective analysis
- Iyad Al-Ammouri, Fares Ayoub, Rima Dababneh
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- Published online by Cambridge University Press:
- 10 September 2015, pp. 1056-1059
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Objective
The purpose of this study was to determine whether a murmur detected on routine pre-discharge examination of asymptomatic newborn children in the first 48 hours of life warrants further investigation with echocardiography.
MethodsWe conducted a retrospective review of all echocardiography studies of neonates born at Jordan University Hospital between August, 2007 and June, 2014. Findings on physical examination as well as the indication of the echocardiographic studies were reviewed. We included asymptomatic neonates for whom echocardiographic studies were carried out due to the sole indication of a heart murmur on routine pre-discharge neonatal physical examination.
ResultsOf 309 asymptomatic newborns with murmurs on pre-discharge examination, echocardiography revealed 68 (22%) cases of CHD, with 18 (6%) designated as significant heart disease with anticipated intervention during infancy or childhood. The most common abnormality was ventricular septal defect occurring in 36 cases. Critical heart diseases detected included hypoplastic left heart syndrome in two and aortic valve stenosis in four newborns.
ConclusionsAlthough most asymptomatic neonates with heart murmurs have normal hearts, a small percentage may have significant heart disease. The decision to refer an asymptomatic newborn with a murmur for echocardiography before discharge from the hospital remains controversial and must be supported by other evidence such as murmur characteristics and local trends in parental compliance with follow-up well-baby visits.
Comparison of echocardiographic findings with laboratory parameters in obese children
- Ozlem Korkmaz, Hazım A. Gursu, Barbaros S. Karagun
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- Published online by Cambridge University Press:
- 14 September 2015, pp. 1060-1065
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Aim
The purpose of our study was to evaluate the association between insulin resistance and left ventricular size and function in obese children.
Material and methodsA total of 79 cases aged 10–16 years and diagnosed with obesity and 79 healthy and non-obese cases as controls were included in the study. Patient and control groups were divided into three groups in terms of age as group 1 (10–12 years), group 2 (12–14 years), and group 3 (14–16 years). Fasting blood glucose, lipid profile, and fasting insulin levels of the cases were assessed. Mitral valve E and A waves, left ventricular ejection fraction, fractional shortening, end-diastolic and end-systolic diameters, left atrium diameter, and septal wall thickness were measured using echocardiography.
ResultsMeasurements of septal diastolic thicknesses, left atrium diameter, and left ventricular end-systolic diameter of all the three groups obtained by echocardiography were statistically higher compared with the controls. In all the patient groups, the mitral valve E/A ratio was >1. In groups 2 and 3, there was a positive correlation between fasting insulin levels and HOMA-IR and left ventricular end-systolic diameter, end-diastolic diameter, and septal systolic and diastolic wall thicknesses.
ConclusionIn paediatric obesity, identification of early cardiac changes will be significant in allowing early diagnosis and treatment of cardiovascular diseases.
Exercise performance in young patients with complete atrioventricular block: the relevance of synchronous atrioventricular pacing
- M. Cecilia Gonzalez Corcia, Lorraine Saint Remy, Sebastien Marchandise, Stephane Moniotte
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- 22 January 2016, pp. 1066-1071
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At present, there are many pacing strategies for young patients with complete atrioventricular block. The most frequent policy is to attempt placing a dual-chamber system when possible; however, there is a group of patients that is functioning with a non-synchronous ventricular pacing, raising the question of the ideal timing to upgrade their systems. We investigated the exercise performance of a group of children and young adults with complete atrioventricular block and dual-chamber pacemakers in both single- and dual-chamber pacing modalities. A total of 15 patients performed maximal exercise stress testing after programming the VVIR or DDD modes with 2 hours of interval in a double-blind study protocol.
Compared with VVIR pacing, DDD pacing resulted in increase in the peak VO2, longer test duration, major increase in the heart rate achieved during peak exercise, decreased systemic non-invasive arterial blood pressure measured at maximal exercise, higher maximal workload, prolongation of the anaerobic threshold timing, and better self-rated performance perception in all the patients.
Synchronous atrioventricular pacing contributes to an increase in both the exercise performance and the performance perception in 100% of the patients. This difference contributes to create a sense of “fitness” with repercussions in the overall health, self-esteem, and life quality, as well as encourages youngster to practice sports. Our experience tends to favour upgrading patients’ systems to dual-chamber systems before reaching the adolescent years, even if the centre policy is to prolong as long as possible the epicardial site in order to avoid long years of right ventricular pacing.
Management of hypoplastic left heart syndrome with intact atrial septum: a two-centre experience
- Shyam K. Sathanandam, Ranjit Philip, David Gamboa, Andrew Van Bergen, Michel N. Ilbawi, Christopher Knott-Craig, B. Rush Waller, Alexander J. Javois, Bettina F. Cuneo
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- Published online by Cambridge University Press:
- 08 September 2015, pp. 1072-1081
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Introduction
Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes.
Materials and methodsA retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation.
ResultsThe incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58–1; specificity, 0.99, 95% CI, 0.96–1) was predictive of the need for emergent left atrial decompression.
ConclusionUsing a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.
Monitoring the health of the work environment with a daily assessment tool: the REAL – Relative Environment Assessment Lens – indicator
- Karen E. Hinsley, Audrey C. Marshall, Michelle H. Hurtig, Jason M. Thornton, Cheryl A. O’Connell, Courtney L. Porter, Jean A. Connor, Patricia A. Hickey
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- Published online by Cambridge University Press:
- 01 October 2015, pp. 1082-1089
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Background
Evidence shows that the health of the work environment impacts staff satisfaction, interdisciplinary communication, and patient outcomes. Utilising the American Association of Critical-Care Nurses’ Healthy Work Environment standards, we developed a daily assessment tool.
MethodsThe Relative Environment Assessment Lens (REAL) Indicator was developed using a consensus-based method to evaluate the health of the work environment and to identify opportunities for improvement from the front-line staff. A visual scale using images that resemble emoticons was linked with a written description of feelings about their work environment that day, with the highest number corresponding to the most positive experience. Face validity was established by seeking staff feedback and goals were set.
ResultsOver 10 months, results from the REAL Indicator in the cardiac catheterisation laboratory indicated an overall good work environment. The goal of 80% of the respondents reporting their work environment to be “Great”, “Good”, or “Satisfactory” was met each month. During the same time frame, this goal was met four times in the cardiovascular operating room. On average, 72.7% of cardiovascular operating room respondents reported their work environment to be “Satisfactory” or better.
ConclusionThe REAL Indicator has become a valuable tool in assessing the specific issues of the clinical area and identifying opportunities for improvement. Given the feasibility of and positive response to this tool in the cardiac catheterisation laboratory, it has been adopted in other patient-care areas where staff and leaders believe that they need to understand the health of the environment in a more specific and frequent time frame.
Data integrity of the Pediatric Cardiac Critical Care Consortium (PC4) clinical registry
- Michael Gaies, Janet E. Donohue, Gina M. Willis, Andrea T. Kennedy, John Butcher, Mark A. Scheurer, Jeffrey A. Alten, J. William Gaynor, Jennifer J. Schuette, David S. Cooper, Jeffrey P. Jacobs, Sara K. Pasquali, Sarah Tabbutt
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- 11 September 2015, pp. 1090-1096
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Background
Clinical databases in congenital and paediatric cardiac care provide a foundation for quality improvement, research, policy evaluations and public reporting. Structured audits verifying data integrity allow database users to be confident in these endeavours. We report on the initial audit of the Pediatric Cardiac Critical Care Consortium (PC4) clinical registry.
Materials and methodsParticipants reviewed the entire registry to determine key fields for audit, and defined major and minor discrepancies for the audited variables. In-person audits at the eight initial participating centres were conducted during a 12-month period. The data coordinating centre randomly selected intensive care encounters for review at each site. The audit consisted of source data verification and blinded chart abstraction, comparing findings by the auditors with those entered in the database. We also assessed completeness and timeliness of case submission. Quantitative evaluation of completeness, accuracy, and timeliness of case submission is reported.
ResultsWe audited 434 encounters and 29,476 data fields. The aggregate overall accuracy was 99.1%, and the major discrepancy rate was 0.62%. Across hospitals, the overall accuracy ranged from 96.3 to 99.5%, and the major discrepancy rate ranged from 0.3 to 0.9%; seven of the eight hospitals submitted >90% of cases within 1 month of hospital discharge. There was no evidence for selective case omission.
ConclusionsBased on a rigorous audit process, data submitted to the PC4 clinical registry appear complete, accurate, and timely. The collaborative will maintain ongoing efforts to verify the integrity of the data to promote science that advances quality improvement efforts.
Evaluation of a focussed protocol for hand-held echocardiography and computer-assisted auscultation in detecting latent rheumatic heart disease in scholars
- Liesl J. Zühlke, Mark E. Engel, Simpiwe Nkepu, Bongani M. Mayosi
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- 01 October 2015, pp. 1097-1106
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Introduction
Echocardiography is the diagnostic test of choice for latent rheumatic heart disease. The utility of echocardiography for large-scale screening is limited by high cost, complex diagnostic protocols, and time to acquire multiple images. We evaluated the performance of a brief hand-held echocardiography protocol and computer-assisted auscultation in detecting latent rheumatic heart disease with or without pathological murmur.
MethodsA total of 27 asymptomatic patients with latent rheumatic heart disease based on the World Heart Federation criteria and 66 healthy controls were examined by standard cardiac auscultation to detect pathological murmur. Hand-held echocardiography using a focussed protocol that utilises one view – that is, the parasternal long-axis view – and one measurement – that is, mitral regurgitant jet – and a computer-assisted auscultation utilising an automated decision tool were performed on all patients.
ResultsThe sensitivity and specificity of computer-assisted auscultation in latent rheumatic heart disease were 4% (95% CI 1.0–20.4%) and 93.7% (95% CI 84.5–98.3%), respectively. The sensitivity and specificity of the focussed hand-held echocardiography protocol for definite rheumatic heart disease were 92.3% (95% CI 63.9–99.8%) and 100%, respectively. The test reliability of hand-held echocardiography was 98.7% for definite and 94.7% for borderline disease, and the adjusted diagnostic odds ratios were 1041 and 263.9 for definite and borderline disease, respectively.
ConclusionComputer-assisted auscultation has extremely low sensitivity but high specificity for pathological murmur in latent rheumatic heart disease. Focussed hand-held echocardiography has fair sensitivity but high specificity and diagnostic utility for definite or borderline rheumatic heart disease in asymptomatic patients.
Surgical ligation of patent ductus arteriosus in premature infants: trends and practice variation
- Jacqueline G. Weinberg, Frank J. Evans, Kristin M. Burns, Gail D. Pearson, Jonathan R. Kaltman
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- 23 September 2015, pp. 1107-1114
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Objective
We sought to analyse the variation in the incidence of patent ductus arteriosus over three recent time points and characterise ductal ligation practices in preterm infants in the United States, adjusting for demographic and morbidity factors.
MethodsUsing the Kids’ Inpatient Database from 2003, 2006, and 2009, we identified infants born at ⩽32 weeks of gestation with International Classification of Diseases, Ninth Revision diagnosis of patent ductus arteriosus and ligation code. We examined patient and hospital characteristics and identified patient and hospital variables associated with ligation.
ResultsOf 182,610 preterm births, 30,714 discharges included a patent ductus arteriosus diagnosis. The rate of patent ductus arteriosus diagnosis increased from 14% in 2003 to 21% in 2009 (p<0.001). A total of 4181 ligations were performed, with an overall ligation rate of 14%. Ligation rate in infants born at ⩽28 weeks of gestation was 20% overall, increasing from 18% in 2003 to 21% in 2009 (p<0.001). The ligation rate varied by state (4–28%), and ligation was associated with earlier gestational age, associated diagnoses, hospital type, teaching hospital status, and region (p<0.001).
ConclusionThe rates of patent ductus arteriosus diagnosis and ligation have increased in the recent years. Variation exists in the practice of patent ductus arteriosus ligation and is influenced by patient and non-patient factors.
Development and persistence of depressive symptoms in adolescents with CHD
- Koen Luyckx, Jessica Rassart, Eva Goossens, Silke Apers, Leen Oris, Philip Moons
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- Published online by Cambridge University Press:
- 15 September 2015, pp. 1115-1122
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Patients with CHD are vulnerable to psychiatric disorders. The present study compared baseline depressive symptoms between adolescents with CHD and community adolescents, and also assessed the development and persistence of depressive symptoms in patients. We examined the implications of persistent depressive symptoms towards quality of life and patient-reported health. In total, 296 adolescents with CHD participated in a four-wave longitudinal study, with 9-month intervals, and completed measures of depressive symptoms – Center for Epidemiologic Studies Depression Scale (CES-D) – at time points one to four and of quality of life – linear analogue scale (LAS) – and patient-reported health – LAS and Pediatric Quality of Life Inventory – at T (time) 4. Information about diagnosis, disease complexity, and previous heart surgery was collected from medical records. At T1, 278 patients were matched 1:1 with community adolescents, based on sex and age. The findings of this study indicate that patients scored significantly lower on depressive symptoms compared with community adolescents. Depressive symptoms in the total patient sample were stable over time and were unrelated to disease complexity. Based on conventional cut-off scores of the CES-D, substantial individual differences existed in the extent to which depressive symptoms persisted over time: 12.2% of the patients reported elevated depressive symptoms at minimally three out of the four time points. Especially physical functioning, cardiac symptoms, and patient-reported health at T4 were predicted by persistent depressive symptoms, even when controlling for the level of depressive symptoms at T4. Our findings indicate that those involved in the care of adolescents with CHD should remain vigilant to persistent depressive symptoms and arrange timely referral to mental healthcare services.
Exercise and β-blocker therapy recommendations for inherited arrhythmogenic conditions
- Susan Christian, Martin Somerville, Sherry Taylor, Joseph Atallah
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- 23 September 2015, pp. 1123-1129
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Background
Management of individuals with long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy may involve exercise restriction and/or β-blocker therapy.
ObjectiveThis study assessed the practices of a group of paediatric electrophysiologists regarding the management of genotype-positive/phenotype-positive and genotype-positive/phenotype-negative individuals with these conditions.
MethodAn online survey was circulated to members of the Pediatric and Congenital Electrophysiology Society in May, 2014. The survey included questions addressing the respondents’ approach regarding exercise recommendations and prescription of β-blocker therapy.
ResultsA total of 45 cardiologists completed the survey. The majority of respondents restricted symptomatic patients from competitive sports; however, only approximately half restricted phenotype-negative mutation carriers from this level of activity. Recommendations were less consistent regarding other types of activities. A trend was identified regarding physician physical activity and exercise recommendations for phenotype-negative mutation carriers. Less-active physicians were more likely to restrict exercise. β-blocker therapy was discussed by the majority of respondents for symptomatic patients and a significant number of asymptomatic patients.
ConclusionExercise restriction for patients with long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy varies based on several factors including phenotype, type of exercise, guidelines referred to, and physicians’ own level of activity.
Systematic psychosocial screening in a paediatric cardiology clinic: clinical utility of the Pediatric Symptom Checklist 17
- Kari L. Struemph, Lydia R. Barhight, Deepika Thacker, Erica Sood
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- 01 October 2015, pp. 1130-1136
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Objective
To examine the clinical utility of the Pediatric Symptom Checklist 17 for identifying psychosocial concerns and improving access to psychology services within a paediatric cardiology clinic.
MethodParents of 561 children (aged 4–17 years) presenting for follow-up of CHD, acquired heart disease, or arrhythmia completed the Pediatric Symptom Checklist 17 as part of routine care; three items assessing parental (1) concern for learning/development, (2) questions about adjustment to cardiac diagnosis, and (3) interest in discussing concerns with a behavioural healthcare specialist were added to the questionnaire. A psychologist contacted the parents by phone if they indicated interest in speaking with a behavioural healthcare specialist.
ResultsPercentages of children scoring above clinical cut-offs for externalising (10.5%), attention (8.7%), and total (9.3%) problems were similar to a “normative” primary-care sample, whereas fewer children in this study scored above the cut-off for internalising problems (7.8%; p<0.01). Sociodemographic, but not clinical, characteristics were associated with Pediatric Symptom Checklist 17 scores. 17% of the parents endorsed concerns about learning/development, and 20% endorsed questions about adjustment to diagnosis. History of cardiac surgery was associated with increased concern about learning/development (p<0.01). Only 37% of the parents expressing psychosocial concerns reported interest in speaking with a psychologist.
ConclusionsThe Pediatric Symptom Checklist 17 may not be sensitive to specific difficulties experienced by this patient population. A questionnaire with greater focus on learning/development and adjustment to diagnosis may yield improved utility. Psychology integration in clinics serving high-risk cardiac patients may decrease barriers to behavioural healthcare services.
Medium-term follow-up of renal function in hypoxaemic congenital heart disease patients
- Efrén Martínez-Quintana, Fayna Rodríguez-González
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- 18 September 2015, pp. 1137-1143
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Introduction
Hypoxaemic congenital heart disease (CHD) patients are at higher risk of complications. The aim of this study was to compare and follow-up blood and 24-hour urine analytical data in hypoxaemic and non-hypoxaemic CHD patients.
MethodsThe inclusion criteria for this study were as follows: patients older than 14 years of age with a structural CHD with or without associated hypoxaemia.
ResultsIn total, 27 hypoxaemic and 48 non-hypoxaemic CHD patients were included in order to compare blood and 24-hour urine analytical data. Among hypoxaemic patients, 13 (48.1%) were male, two (7.4%) had diabetes mellitus, one of whom was a smoker, one (3.7%) had systemic arterial hypertension, and 11 (40.7%) showed pulmonary arterial hypertension. The mean follow-up time was 3.1±1.9 years. Hypoxaemic CHD patients showed higher proteinuria concentrations (g/24 hours) (0.09 (0.07; 0.46) versus 0.08 (0.07; 0.1), p=0.054) and 24-hour albumin excretion rate (µg/min) (16.5 (11.2; 143.5) versus 4.4 (0.0; 7.6), p<0.001) compared with non-hypoxaemic CHD patients; however, no significant differences were found in the proteinuria levels and in the 24-hour albumin excretion rate in CHD patients with associated hypoxaemia, both at baseline and at follow-up. When divided into groups, hypoxaemic patients with palliative shunts showed significantly higher proteinuria concentrations compared with hypoxaemic patients not operated on or with Fontan procedures (p=0.01). No significant differences were seen in 24-hour proteinuria and 24-hour albumin excretion rate during the follow-up of patients with palliative shunts.
ConclusionsHypoxaemic CHD patients have significant higher 24-hour proteinuria concentration and 24-hour albumin excretion rate compared with non-hypoxaemic CHD patients. Among hypoxaemic CHD patients, those with palliative shunts showed the highest 24-hour proteinuria concentrations.
Feasibility and safety of cardiopulmonary exercise testing in children with pulmonary hypertension
- Mohammad R. Abumehdi, Andrew J. Wardle, Rewa Nazzal, Athanasios Charalampopoulos, Ingram Schulze-Neick, Graham Derrick, Shahin Moledina, Alessandro Giardini
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- Published online by Cambridge University Press:
- 16 September 2015, pp. 1144-1150
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Background
Cardiopulmonary exercise testing helps prognosticate and guide treatment in adults with pulmonary hypertension. Concerns regarding its feasibility and safety limit its use in children with pulmonary hypertension. We aimed to assess the feasibility and safety of cardiopulmonary exercise testing in a large paediatric pulmonary hypertension cohort.
MethodsWe reviewed all consecutive cardiopulmonary exercise tests performed between March, 2004 and November, 2013. The exclusion criteria were as follows: height <120 cm, World Health Organization class IV, history of exercise-induced syncope, or significant ischaemia/arrhythmias. Significant events recorded were as follows: patient-reported symptoms, arrhythmias, electrocardiogram abnormalities, and abnormal responses of arterial O2 saturation.
ResultsA total of 98 children underwent 167 cardiopulmonary exercise tests. The median age was 14 years (inter-quartile range 10–15 years). Peak oxygen uptake was 20.4±7.3 ml/kg/minute, corresponding to 51.8±18.3% of the predicted value. Peak respiratory quotient was 1.08±0.16. All the tests except two were maximal, being terminated prematurely for clinical reasons. Baseline Oxygen saturation was 93.3±8.8% and was 81.2±19.5% at peak exercise. A drop in arterial O2 saturation >20% was observed in 23.5% of the patients. Moreover, five patients (3.0%) experienced dizziness, one requiring termination of cardiopulmonary exercise testing; five children (3.0%) experienced chest pain, with early cardiopulmonary exercise test termination in one patient. No significant arrhythmias or electrocardiogram changes were observed.
ConclusionExercise testing in non-severely symptomatic children with pulmonary hypertension is safe and practical, and can be performed in a large number of children with pulmonary hypertension in a controlled environment with an experienced team. Side-effects were not serious and were resolved promptly with test termination.
Predictive value of red blood cell distribution width for coronary artery lesions in patients with Kawasaki disease
- Haiyan Xu, Songling Fu, Wei Wang, Qing Zhang, Jian Hu, Lichao Gao, Weihua Zhu, Fangqi Gong
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- Published online by Cambridge University Press:
- 05 October 2015, pp. 1151-1157
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Recent studies have shown that elevated red blood cell distribution width is associated with poor outcome in cardiovascular diseases. In order to assess the predictive value of red blood cell distribution width, before treatment with intravenous immunoglobulins, for coronary artery lesions in patient with Kawasaki disease, we compared 83 patients with coronary artery lesions and 339 patients without coronary artery lesions before treatment with intravenous immunoglobulin. Clinical, echocardiographic, and biochemical values were evaluated along with red blood cell distribution width. A total of 422 consecutive patients with Kawasaki disease were enrolled into our study. According to receiver operating characteristic curve analysis, the optimal red blood cell distribution width cut-off value for predicting coronary artery lesions was 14.55% (area under the curve was 0.721; p=0.000); eighty-three patients (19.7%) had coronary artery lesions, and 70% of the patients with coronary artery lesions had red blood cell distribution width level >14.55%. Logistic regression analysis revealed that fever duration >14 days (odds ratio was 3.42, 95% confidence interval was 1.27–9.22; p=0.015), intravenous immunoglobulin resistance (odds ratio was 2.33, 95% confidence interval was 1.02–5.29; p=0.04), and red blood cell distribution width >14.55% (odds ratio was 3.49, 95% confidence interval was 2.01–6.05; p=0.000) were independent predictors of coronary artery lesions in patients with Kawasaki disease. In Conclusion, red blood cell distribution width may be helpful for predicting coronary artery lesions in patients with Kawasaki disease.
Parental perceptions of congenital cardiovascular malformations in their children
- Sameera Ezzat, Osamah Saeedi, Doa’a A. Saleh, Hala Hamzeh, Mohamed A. Hamid, Nancy Crowell, Camille Boostrom, Christopher A. Loffredo, Irene A. Jillson
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- Published online by Cambridge University Press:
- 12 November 2015, pp. 1158-1167
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We assessed parental attitudes towards congenital cardiovascular malformations in their children in a cross-sectional study in Egypt. Parents face many problems related to concerns about their child’s prognosis, but these associations with parental stress have never been evaluated in Egypt or examined in relation to religiosity in a predominantly Muslim society. Accordingly, we conducted interviews in Cairo with mothers of 99 sequential infants born with conotruncal heart malformations (cases) and 65 mothers of age-matched controls. The survey assessed healthcare access and usage, knowledge of congenital cardiovascular malformations, religiosity, the Locus of Control Scale, and the Parenting Stress Index. Results showed that 45% of the mothers of cases had correct knowledge about their child’s diagnosis; 85% were satisfied with the clinical care; and 79% reported that the cost of care was burdensome. Compared with parents of cases, parents of controls were more likely to report stress overall and all its subscales. Regarding belief about locus of control over health, God as a determining factor was given the highest endorsement. Mothers in the congenital cardiovascular malformations group reported a higher level of parental locus of control than did those in the control group. The correlations between stress and locus of control were stronger in the control than in the case group. Religiosity was related neither to stress nor to locus of control. Future studies can explore the roles that personal, familial, and societal factors play in exacerbating or reducing stress levels among parents of sick children, particularly in developing countries where economic pressures are acute.
Nurse and parent perceptions associated with the Parent Education Discharge Instruction Programme in southern India
- Sandra L. Staveski, V. P Parveen, Sai B. Madathil, Susan Kools, Linda S. Franck
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- Published online by Cambridge University Press:
- 12 October 2015, pp. 1168-1175
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Introduction
Parents of children with CHD require home care knowledge in order to ensure their child’s health and safety, but there has been no research on how to achieve this in a resource-constrained environment. The aim of this investigation was to compare parent and nurse perceptions of parent readiness for discharge after a structured nurse-led parent discharge teaching programme in India.
Materials and methodsA pre-post design was used to compare parent and nurse perceptions of parental uncertainty and readiness for hospital discharge before and after introduction of the parent education discharge instruction programme in a paediatric cardiac surgery unit.
ResultsParents (n=68) and nurses (n=63) participated in this study. After the discharge programme implementation, parents had less uncertainty (M=93.3 SD=10.7 versus M=83.6 SD=4.9, p=0.001) and ambiguity (M=40.8 SD=6.8 versus M=33.4 SD=3.7, p=0.001) about their child’s illness; however, they rated themselves as being less able to cope with the transition to home (M=24.3 SD=4.1 versus 23.1 SD=2.2, p=0.001) and as having less support at home than that required (M=31.5 SD=9.9 versus 30.9 SD=3.2, p=0.001). Parents’ and nurses’ perception of parental readiness for hospital discharge were more closely aligned after implementation of a nurse-led discharge programme (r=0.81, p=0.001).
ConclusionThe results of this study suggest that the discharge programme had positive and negative effects on parental perceptions of uncertainty and readiness for discharge. Further examination is warranted to delineate these influences and to design methods for supporting parents during the transition to home care.
Infective endocarditis prophylaxis: current practice trend among paediatric cardiologists: are we following the 2007 guidelines?
- Ronak J. Naik, Neil R. Patel, Ming Wang, Nishant C. Shah
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- Published online by Cambridge University Press:
- 30 December 2015, pp. 1176-1182
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Background
In 2007, the American Heart Association modified the infective endocarditis prophylaxis guidelines by limiting the use of antibiotics in patients with cardiac conditions associated with the highest risk of adverse outcomes after infective endocarditis. Our objective was to evaluate current practice for infective endocarditis prophylaxis among paediatric cardiologists.
MethodsA web-based survey focussing on current practice, describing the use of antibiotics for infective endocarditis prophylaxis in various congenital and acquired heart diseases, was distributed via e-mail to paediatric cardiologists. The survey was kept anonymous and was distributed twice.
ResultsData from 253 participants were analysed. Most paediatric cardiologists discontinued infective endocarditis prophylaxis in patients with simple lesions such as small ventricular septal defect, patent ductus arteriosus, and bicuspid aortic valve without stenosis or regurgitation; however, significant disagreement persists in prescribing infective endocarditis prophylaxis in certain conditions such as rheumatic heart disease, Fontan palliation without fenestration, and the Ross procedure. Use of antibiotic prophylaxis in certain selected conditions for which infective endocarditis prophylaxis has been indicated as per the current guidelines varies from 44 to 83%. Only 44% follow the current guidelines exclusively, and 34% regularly discuss the importance of oral hygiene with their patients at risk for infective endocarditis.
ConclusionSignificant heterogeneity still persists in recommending infective endocarditis prophylaxis for several cardiac lesions among paediatric cardiologists. More than half of the participants (56%) do not follow the current guidelines exclusively in their practice. Counselling for optimal oral health in patients at risk for infective endocarditis needs to be optimised in the current practice.
Accuracy of the Masimo SET® LNCS neo peripheral pulse oximeter in cyanotic congenital heart disease
- Michael J. Griksaitis, Gemma E. Scrimgeour, John V. Pappachan, Andrew J. Baldock
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- Published online by Cambridge University Press:
- 16 October 2015, pp. 1183-1186
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Introduction
Non-invasive peripheral pulse oximeters are routinely used to measure oxyhaemoglobin saturation (SpO2) in cyanotic congenital heart disease. These probes are calibrated in healthy adult volunteers between arterial saturations of ~75 and 100%, using the gold standard of co-oximetry on arterial blood samples. There are little data to attest their accuracy in cyanotic congenital heart disease.
AimsWe aimed to assess the accuracy of a commonly used probe in children with cyanotic congenital heart disease.
MethodsChildren with cyanotic congenital heart disease admitted to the Paediatric Intensive Care Unit with an arterial line in situ were included to our study. Prospective simultaneous recordings of SpO2, measured by the Masimo SET® LNCS Neo peripheral probe, and co-oximeter saturations (SaO2) measured by arterial blood gas analysis were recorded.
ResultsA total of 527 paired measurements of SpO2 and SaO2 (using an ABL800 FLEX analyser) in 25 children were obtained. The mean bias of the pulse oximeter for all SaO2 readings was +4.7±13.8%. The wide standard deviation indicates poor precision. This mean bias increased to +7.0±13.7% at SaO2 recordings <75%. The accuracy root mean square of the recordings was 3.30% across all saturation levels, and this increased to 4.98% at SaO2 <75%.
ConclusionsThe performance of the Masimo SET® LNCS Neo pulse oximeter is poor when arterial oxyhaemoglobin saturations are below 75%. It tends to overestimate saturations in children with cyanotic congenital heart disease. This may have serious implications for clinical decisions.