Original Articles
Thrombotic events in critically ill children with myocarditis
- Kimberly Y. Lin, Basavaraj Kerur, Char M. Witmer, Lauren A. Beslow, Daniel J. Licht, Rebecca N. Ichord, Beth D. Kaufman
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- Published online by Cambridge University Press:
- 09 September 2013, pp. 840-847
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Background: Children with myocarditis have multiple risk factors for thrombotic events, yet the role of antithrombotic therapy is unclear in this population. We hypothesised that thrombotic events in critically ill children with myocarditis are common and that children with myocarditis are at higher risk for thrombotic events than children with non-inflammatory dilated cardiomyopathy. Methods: This is a retrospective chart review of all children presenting to a single centre cardiac intensive care unit with myocarditis from 1995 to 2008. A comparison group of children with dilated cardiomyopathy was also examined. Antithrombotic regimens were recorded. The primary outcome of thrombotic events included intracardiac clots and any thromboembolic events. Results: Out of 45 cases with myocarditis, 40% were biopsy-proven, 24% viral polymerase chain reaction-supported, and 36% diagnosed based on high clinical suspicion. There were two (4.4%) thrombotic events in the myocarditis group and three (6.7%) in the dilated cardiomyopathy group (p = 1.0). Neither the use of any antiplatelet or anticoagulation therapy, use of intravenous immune globulin, presence of any arrhythmia, nor need for mechanical circulatory support were predictive of thrombotic events in the myocarditis, dilated cardiomyopathy, or combined groups. Conclusions: Thrombotic events in critically ill children with myocarditis and dilated cardiomyopathy occurred in 6% of the combined cohort. There was no difference in thrombotic events between inflammatory and non-inflammatory cardiomyopathy groups, suggesting that the decision to use antithrombotic prophylaxis should be based on factors other than the underlying aetiology of a child's acute decompensated heart failure.
Heart rate variability in β-thalassaemia major with or without cardiac siderosis
- A. Alp, O. Ozdogan, C.C. Guloglu, M. Turker, B. Atabay
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- Published online by Cambridge University Press:
- 12 February 2013, pp. 263-267
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Objectives
Iron cardiomyopathy remains the major cause of death in β-thalassaemia major. Excessive iron loading could lead to cardiac dysfunction and arrhythmias. Reduced heart rate variability is associated with a higher risk of arrhythmia and sudden death after myocardial infarction and heart failure. Previous data have reported on reduced heart rate variability in patients with marked cardiac iron accumulation. In this study, we compared heart rate variability among β-thalassaemia major (TM) patients with or without cardiac siderosis.
MethodsOut of 70 β-thalassaemia major patients with preserved ejection fractions, 38 patients with cardiac T2* magnetic resonance imaging assessment were included in our study. Time domain heart rate variability parameters were analysed from 24-hour recorded electrocardiograms and were compared with the control group.
ResultsThe mean T2* magnetic resonance imaging value was 22.9 ± 13.3 (4.7–47.5). In 21 patients with β-thalassaemia major, the T2* magnetic resonance imaging values were greater than 20 ms and these patients were considered to be in the early stage of the disease. When we compare these patients with control subjects, the standard deviation of all NN intervals was still significantly lower (133.0 ± 32.2 versus 162.8 ± 32.9, p = 0.001) in β-thalassaemia major patients despite normal T2* magnetic resonance imaging values. On the contrary, the standard deviation of all NN intervals was not correlated with haemoglobin levels in these patients (p > 0.05).
ConclusionsHeart rate variability parameters were reduced even in β-thalassaemia major patients without evident cardiac siderosis, as specified by magnetic resonance imaging data. The results of this study show that reduction of heart rate variability may start before cardiac iron loading is demonstrated by T2* magnetic resonance imaging in β-thalassaemia major.
Psychosocial risk in families of infants undergoing surgery for a serious congenital heart disease
- Simone J. Hearps, Maria C. McCarthy, Frank Muscara, Stephen J. C. Hearps, Kylie Burke, Bryn Jones, Vicki A. Anderson
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- Published online by Cambridge University Press:
- 27 June 2013, pp. 632-639
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Objective: The aim of this study was to explore the acute psychosocial risk in families with infants undergoing surgery for a congenital heart disease and, secondarily, to explore the psychosocial impact of antenatal versus post-natal diagnoses. Method: The study sample comprised 39 caregivers (28 mothers) of 29 children diagnosed with a congenital heart disease and requiring surgery within the first 4 weeks of life. Psychosocial risk was measured using the Psychosocial Assessment Tool, which was adapted to include four novel items examining infant risk factors, namely, sleeping, feeding, crying, and bonding difficulties. Parents’ psychosocial risk was measured within 4 weeks after their child's surgery and stratified into a three-tiered framework: Universal, Targeted, and Clinical risk. Results: Of the total sample, 61.5% of parents were classified as Universal, that is, at lowest risk; 35.9% as Targeted, and 2.6% as Clinical. The within-family parent total Psychosocial Assessment Tool score correlations were non-significant, and there were no differences between families of infants who received post-natal versus antenatal diagnosis or single ventricle versus biventricular repair. Linear regression found that a higher parent education significantly predicted a lower total Psychosocial Assessment Tool score. Conclusions: Findings indicate that, although the majority of parents adapt to the acute stress of surgery for a serious cardiac illness in their infant, the remaining 38.5% report an increased psychosocial risk associated with higher rates of emotional distress, which may impact on the parental quality of life and capacity for optimal parenting. The distribution of psychosocial risk in parents of children undergoing surgery for a congenital heart disease is consistent with that described for parents of children with other serious paediatric diagnoses.
Screening miRNA and their target genes related to tetralogy of Fallot with microarray
- Xian-min Wang, Kui Zhang, Yan Li, Kun Shi, Yi-ling Liu, Yan-feng Yang, Yu Fang, Meng Mao
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- Published online by Cambridge University Press:
- 17 May 2013, pp. 442-446
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Our aim is to screen miRNAs and genes related to tetralogy of Fallot and construct a co-expression network based on integrating miRNA and gene microarrays. We downloaded the gene expression profile GSE35490 (miRNA) and GSE35776 (mRNA) of tetralogy of Fallot from the Gene Expression Omnibus database, which includes eight normal and 15 disease samples from infants, and screened differentially expressed miRNAs and genes between normal and disease samples (cut-off: p < 0.05; FDR < 0.05; and log FC > 2 or log FC < −2); in addition, we downloaded human miRNA and their targets, which were collected in the miRNA targets prediction database TargetScan, and selected ones that also appeared in our differentially expressed miRNAs and their predicted targets (score >0.9) and then made a relationship of diff_miRNAs and diff_genes of our results. Finally, we uploaded all the diff_target genes into String, constructed a co-expression network regulated by diff_miRNAs, and performed functional analysis with the software DAVID. Comparing normal and disease lesion tissue, we got 32 and 875 differentially expressed miRNAs and genes, respectively, and found hsa-miR-124 with 34 diff_target genes and hsa-miR-138 with two diff_target genes. Then we constructed a co-expression network that contains 231 pairs of genes. Genes in the network were enriched into 14 function clusters, and the most significant one is protein localisation. We screened the tetralogy of Fallot-related hsa-miR-124 and hsa-miR-138 with their direct and indirect differentially expressed target genes, and found that protein localisation is the significant cause affecting tetralogy of Fallot. Our approach may provide the groundwork for a new therapy approach to treating tetralogy of Fallot.
A 10-year single-centre experience in percutaneous interventions for multi-stage treatment of hypoplastic left heart syndrome
- Tomasz Moszura, Pawel Dryzek, Sebastian Goreczny, Anna Mazurek-Kula, Jacek J. Moll, Andrzej Sysa, Waldemar Bobkowski, Jadwiga A. Moll, Shakeel A. Qureshi
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- 12 February 2013, pp. 54-63
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Objectives
The purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications.
BackgroundConstant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment.
MethodsBetween January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation.
ResultsPercutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2 saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries.
ConclusionsPercutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2 saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.
Original Article
Critical care of patients with paediatric valvar cardiac disease*
- Ryan A. Moore, Saul Flores, David S. Cooper
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- 29 December 2014, pp. 1071-1076
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Valvar cardiac disease accounts for a large percentage of heart lesions in the paediatric population. There are a variety of congenital and acquired valvar lesions produced by different mechanisms that produce unique physiologies. With the development of more advanced cardiac interventional procedures and congenital cardiac surgery techniques, most of these lesions can be repaired in a single or multi-stage approach. Given the potential for significant symptomatology before intervention, it is imperative to review the critical care management of paediatric valvar heart disease. The purpose of this article is to provide an overview of the current critical care management of valvar cardiac disease. We approach each cardiac valve individually in order to review the pathophysiology caused by valve dysfunction (obstruction and incompetence) and elaborate on the most frequent diagnoses, specific cardiac physiology, potential for other organ system associations, and perioperative management.
Foetal therapy: what works? Closed interatrial septum
- Ulrike Herberg, Christoph Berg, Annegret Geipel, Ulrich Gembruch, Johannes Breuer
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- 27 August 2014, pp. 47-54
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Hypoplastic left-heart syndrome and critical aortic stenosis with severely restricted or intact foramen ovale are associated with high neonatal mortality and poor long-term outcome. Despite accurate foetal diagnosis and successful postnatal catheter-based and surgical intervention, the 1-month survival rate is about 33%. Changes in pulmonary vascular architecture resulting in pulmonary hypertension result in important long-term morbidity. Prenatal relief of left atrial and pulmonary hypertension may promote normal pulmonary vascular and parenchymal development and improve short- and long-term outcomes. Foetal atrial balloon septostomy, laser perforation, and stenting of the foetal interatrial septum are the current options for foetal therapy. This paper provides an overview of foetal diagnosis, selection of patients for foetal intervention, and interventional techniques, and also reviews the current status of foetal and postnatal outcomes after intrauterine intervention.
Original Articles
Tissue motion annular displacement of the mitral valve using two-dimensional speckle tracking echocardiography predicts the left ventricular ejection fraction in normal children
- David E Black, Jen Bryant, Charles Peebles, Keith M Godfrey, Mark Hanson, Joseph J Vettukattil
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- Published online by Cambridge University Press:
- 27 June 2013, pp. 640-648
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Background: The gold standard for determining the left ventricular ejection fraction is cardiac magnetic resonance imaging. Other parameters for determining the ejection fraction such as M-mode echocardiography are operator-dependant and often inaccurate. Assessment of the displacement of the mitral valve annulus using two-dimensional speckle tracking echocardiography may provide an accurate and simple method of determining the left ventricular ejection fraction in children. Method: We retrospectively studied 70 healthy 9-year-old children with no history of cardiovascular disease who had been assessed using cardiac magnetic resonance imaging and two-dimensional transthoracic echocardiography. Mitral displacement was determined using the tissue motion annular displacement (TMAD) feature of Philips QLAB version 9. The midpoint displacement of the mitral valve was calculated, and the predicted left ventricular ejection fraction was compared with magnetic resonance imaging-derived and M-mode-derived ejection fractions. Results: The mean ejection fraction derived from magnetic resonance imaging (64.5 (4.6)) was similar to that derived from the TMAD midpoint (60.9 (2.7), p = 0.001) and the M-mode (61.9 (7), p = 0.012). The TMAD midpoint correlated strongly with the magnetic resonance imaging-derived ejection fraction (r = 0.69, p < 0.001), as did the predicted fraction (r = 0.67, p < 0.001). The M-mode ejection fraction showed a poor linear correlation with both magnetic resonance imaging-derived and TMAD-derived fractions (r = 0.33 and 0.04, respectively). Conclusion: TMAD of the mitral valve is a simple, effective, and highly reproducible method of assessing the ejection fraction in normal children. It shows a strong linear correlation with magnetic resonance imaging-derived ejection fraction and is superior to M-mode-derived ejection fractions.
Extubation failure in infants with shunt-dependent pulmonary blood flow and univentricular physiology
- Punkaj Gupta, Rachel McDonald, Sunali Goyal, Jeffrey M. Gossett, Michiaki Imamura, Amit Agarwal, Warwick Butt, Adnan T. Bhutta
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- 18 January 2013, pp. 64-72
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Objective
The objective of the study was to identify incidence, aetiology, and outcomes of extubation failure in infants with shunt-dependent pulmonary blood flow at a single tertiary care, academic children's hospital. The second objective of this study was to determine the haemodynamic effects of transition of positive pressure ventilation to spontaneous breathing in infants with extubation failure.
Patients and methodsExtubation failure for our study was defined as the need for positive pressure ventilation within 96 hours after extubation. We collected demographics, pre-operative, intra-operative, post-operative, and peri-extubation data in a retrospective, observational format in patients who underwent a modified Blalock–Taussig shunt between January, 2005 and March, 2011. Infants undergoing Norwood operation or Damus–Kaye–Stansel with modified Blalock–Taussig shunt were excluded from the study. The cardiorespiratory variables collected before extubation and immediately after extubation included heart rate, respiratory rate, mean arterial blood pressure, central venous pressures, near infrared spectroscopy, oxygen saturations, and lactate levels. Clinical outcomes evaluated included the success or failure of extubation, cardiovascular intensive care unit length of stay, hospital length of stay, and mortality. Descriptive and univariate statistics were utilised to compare groups with extubation failure and extubation success.
ResultsOf the 55 eligible patients during the study period, extubation failure occurred in 27% (15/55) of the patients. Of the 15 patients with extubation failure, 10 patients needed reintubation and five patients received continuous positive pressure ventilation without getting reintubated. There were three patients who had extubation failure in the first 2 hours after extubation, nine patients in the 2–24-hour period, and three patients in the 24–96-hour period. In all, eight patients were extubated in the second attempt after the first extubation failure, with a median duration of mechanical ventilation of 2 days (1 day, 6 days). The median age of patients at extubation was 19 days (12 days, 22 days) and median weight of patients was 3.6 kg (3.02 kg, 4.26 kg). In all, 38% (21/55) of the patients were intubated before surgery. The most common risk factors for failed extubation were lung disease in 46% (7/15), cardiac dysfunction in 26% (4/15), diaphragmatic paralysis in 13% (2/15), airway oedema in 6% (1/15), and vocal cord paralysis in 6% (1/15). The median duration of mechanical ventilation was 4 days (1 day, 10.5 days), median cardiovascular intensive care unit length of stay was 11 days (6.5 days, 23.5 days), and the median hospital length of stay was 30 days (14 days, 48 days). The overall mortality at the time of hospital discharge was 7%.
ConclusionsExtubation failure in infants with shunt-dependent pulmonary blood flow and univentricular physiology is high and aetiology is diverse. Cardiopulmonary effects of removal of positive pressure ventilation are more pronounced in children with extubation failure and include escalation in the need for oxygen requirement and increase in mean arterial blood pressure. The majority of extubation failures in this select patient population occurs in the first 24 hours. Extubation failure in these patients is not associated with increased hospital length of stay or mortality.
Original Article
Extracardiac malformations: associations and importance: consequences for perinatal management of foetal cardiac patients
- Dick Oepkes, Monique Haak
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- 27 August 2014, pp. 55-59
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Foetal echocardiography has become an established subspecialty in paediatric cardiology. The skills to perform detailed assessment of foetal cardiac anomalies in a tiny, moving foetus surrounded by sometimes challenging layers of maternal tissue are not easy to obtain, and require prolonged periods of specific training and supervised scanning in expert centres. The goal of this overview is to discuss the importance of the awareness of the fact that expert examination of the foetal heart and in-depth knowledge of newborn surgical interventions are only a part of the picture; many cardiac abnormal foetuses do not just have an isolated cardiac defect. The extracardiac malformations, when present, play a major role in determining the treatment and prognosis. The challenge of providing optimal prenatal care to the pregnant woman, who remains the primary patient at least until the umbilical cord is cut, carrying a foetus with a cardiac malformation is to organise rapid, effective, and complete evaluation of all possible foetal anomalies in a multidisciplinary team approach, followed by clear and honest non-directive counselling on all possible options. Only then, with the maximum involvement of the parents, decisions on perinatal management can be taken by the team. Controversies do exist in view of various obstetric, paediatric, and other specialists on how to best organise this multidisciplinary care programme. In this paper, we provide a practical, logical, and efficient path of care for the pregnant woman suspected to carry a foetus with a heart defect.
Original Articles
Mitro-aortic aneurysms in children: single-centre experience and review of the literature
- Manuel Magalhães, Luis Bakero, José Fragata, Filipa Paramés, Isabel Freitas, Mónica Rebelo, José D. Martins, Conceição Trigo, Fátima F. Pinto
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- 26 September 2013, pp. 447-452
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Objectives: This publication aims to report the cases of four children with pseudoaneurysm of the mitral-aortic intervalvular fibrosa and carry out a review of the literature. Background: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a very rare anomaly in children. It can be either congenital or acquired, namely, after bacterial endocarditis or cardiac trauma. This pathology does not usually cause specific symptoms but its outcome may be potentially fatal. Methods: We report the cases of four patients presenting with pseudoaneurysm of the mitral-aortic intervalvular fibrosa, referred for treatment in a paediatric cardiology clinic. Patient clinical notes were retrospectively reviewed for aetiology, clinical presentation, diagnostic work-up, surgical treatment, and follow-up. Literature on the subject was extensively reviewed. Results: In three patients, pseudoaneurysm of the mitral-aortic intervalvular fibrosa was acquired, being secondary to bacterial endocarditis in two cases and establishing after mitral surgery in another case. The remaining patient had a “congenital” aetiology – no other cause could be traced. The diagnosis was achieved by transthoracic echocardiography for all patients, and confirmed in all by trans-oesophageal echocardiography, to better define morphological details and to access flow into the aneurysmal formation. All patients were submitted to corrective cardiac surgery. Of the patients, three survived and were cured by surgery, staying asymptomatic, and one died after repeated interventions, for persistent endocarditis. Conclusions: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a rare but potentially fatal anomaly. In our experience, surgical cure was achieved for the majority of the cases, except for a case for which infection could not be locally eradicated, leading to multiple reinterventions.
Original Article
Repairing the tricuspid valve in congenital heart diseases other than Ebstein’s*
- Ali Dodge-Khatami, Constantine D. Mavroudis, Jennifer Frost, Jeffrey P. Jacobs, Constantine Mavroudis
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- 29 December 2014, pp. 1077-1087
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The tricuspid valve is being increasingly recognised as an important safeguard to the heart with congenital heart disease. Both structural anomalies of the valve and functional burdens from other malformations of the right heart can lead to major haemodynamic consequences both upstream and downstream. The indications to surgically intervene on the tricuspid valve are evolving and vary depending on the malformation. The extant surgical techniques and their applications to corresponding frequent congenital anomalies of the tricuspid valve are reviewed.
Original Articles
Are the children and adolescents with congenital heart disease living in Southwestern Ontario really overweight and obese?
- Eva Welisch, Ralf Rauch, Jamie A. Seabrook, MA Guido Filler, Kambiz Norozi
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- 25 September 2013, pp. 848-853
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Objective
To assess the prevalence of overweight and obesity in children with congenital heart disease and compare them with age-matched healthy children in Southwestern Ontario, Canada.
MethodsWe compared the Center of Disease Control weight and body mass index z-scores of 1080 children, aged 2 to 18 years, who presented to our paediatric cardiology outpatient clinic from 2008 to 2010 for congenital heart disease with 1083 healthy controls.
ResultsIn all, 18.2% of the children with congenital heart disease and 20.8% of healthy children were identified to be either overweight or obese. Overall, the weight category distribution had been similar between the congenital heart disease and healthy control groups, as well as between the congenital heart disease subgroups. There was no difference in normal weight and overweight/obese categories between children with congenital heart disease and healthy children. The underweight category, however, showed a significantly higher prevalence in congenital heart disease compared with healthy children (6.8 and 4.5%, respectively, p = 0.03).
ConclusionThe prevalence of overweight/obesity did not differ in children with congenital heart disease compared with age-matched healthy children; however, it is still high (18.2%). Obesity may represent an additional risk factor for the long-term cardiovascular health of congenital heart disease patients aside from the underlying heart defect.
Medical and surgical management of primary cardiac tumours in infants and children
- Massimo A. Padalino, Elena Reffo, Alessia Cerutti, Valentina Favero, Roberta Biffanti, Vladimiro Vida, Giovanni Stellin, Ornella Milanesi
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- 05 March 2013, pp. 268-274
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Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports. Therefore, we reviewed retrospectively our 27 years of clinical experience on the overall management of cardiac tumours among children in order to improve not only our knowledge but also to provide others with information about the incidence, clinical presentation, management, and long-term outcome of this rare disease. Between April, 1982 and April, 2009, 52 children were diagnosed with cardiac tumours at our Institution. Medical records and follow-up echocardiographic evaluations were studied. The diagnosis was prenatal in 35% of the patients. The most frequent tumour types were rhabdomyomas (61.5%), fibromas (15.4%), and myxomas (5.8%). There were no cases of primary malignant tumours. All diagnoses were achieved using two-dimensional echocardiography, and for 12 patients a pathological analysis was carried out. A total of 41 patients (79%) were managed medically, whereas 11 (21%) patients underwent surgical treatment. At a mean follow-up of 7.2 ± 5.4 years, two patients died of complications related to cardiac transplantation; all the remaining patients are in excellent clinical condition. In conclusion, cardiac tumours in paediatric practice are usually clinically and histologically benign. Only a few cases need surgery. Up to one-third of the cardiac masses are detectable prenatally. Rhabdomyoma is the most common histotype, followed by fibroma and myxoma. The long-term prognosis is generally good.
Original Article
Living with CHD: quality of life (QOL) in early adult life
- Maria E. G. Areias, Catarina I. Pinto, Patrícia F. Vieira, Marta Castro, Isabela Freitas, Sofia Sarmento, Samantha Matos, Victor Viana, José C. Areias
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- Published online by Cambridge University Press:
- 27 August 2014, pp. 60-65
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Aims
The aim of this study was to assess the quality of life, psychiatric morbidity, and the psychosocial adjustment of adolescents and young adults with CHD, and determine which variables play a role in buffering stress and promoting resilience and which ones have a detrimental effect; and to investigate the situation on school performance and failures, social and family support, physical limitations, and body image of these patients.
MethodsThe study enrolled 137 CHD patients (79 male), with age ranging from 12 to 26 years old (M=17.60±3.450 years). The patients were interviewed regarding social support, family educational style, self-image, demographic information, and physical limitations. They responded to questions in a standardised psychiatric interview (SADS-L) and completed self-reported questionnaires for the assessment of quality of life (WHOQOL-BREF) and psychosocial adjustment (YSR/ASR).
ResultsWe found a 19.7% lifetime prevalence of psychopathology in our patients (27.6% in female and 13.9% in male). Of them, 48% had retentions in school (M=1.61 year±0.82). The perception of quality of life in CHD patients is better compared with the Portuguese population in the social relationships and environmental dimensions. However, it is worse in complex forms of CHD than in moderate-to-mild ones, in cyanotic versus acyanotic patients, in moderate-to-severe versus mild residual lesions, in patients submitted versus those not submitted to surgery, in patients with versus without physical limitations, and patients who have need for medication versus those who do not. Social support is very important in improving quality of life of patients in all dimensions as well as academic performance.
ConclusionsFemale patients and patients with poor academic performance and poor social support have worse psychosocial adjustment and perception of quality of life.
Original Articles
The impact of actual and perceived disease severity on pre-operative psychological well-being and illness behaviour in adult congenital heart disease patients
- Edward Callus, Elisabeth M. W. J. Utens, Emilia Quadri, Cristian Ricci, Mario Carminati, Alessandro Giamberti, Massimo Chessa
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- Published online by Cambridge University Press:
- 27 March 2013, pp. 275-282
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Objectives: The purpose of this study was to investigate whether the objective medical parameters related to congenital heart disease and patients’ ratings of cardiac disease severity were related to psychological well-being and illness behaviour during the pre-operative period. Methods: A total of 143 patients (63 male patients; 16–73 years old) with congenital heart disease evaluated the severity of their cardiac condition using a numerical rating scale ranging from 0, indicating the least severe condition, to 100, indicating the most severe condition. Psychological well-being was assessed using the Psychological General Well-Being Index (total score ≤60 indicating severe distress) and illness behaviour using the Illness Behavior Questionnaire. Results: Pre-operative psychological well-being was not related to the objective medical parameters reflecting cardiac disease severity. In contrast, total psychological well-being scores correlated significantly with patients’ subjective ratings of disease severity (p < 0.001). When compared with the reference values from the Italian population, the mean scores of the patients on psychological well-being were similar. As regards the Illness Behavior Questionnaire, the scores on denial were higher and those on hypochondria were lower compared with other hospitalised patients. Conclusions: This study shows that the perception of cardiac disease severity, and not the medical parameters in congenital heart disease, is related to the patients’ pre-operative psychological state. Thus, more importance needs to be given to assessing the patients’ pre-operative perception and psychological state independently of cardiac severity. Targeted interventions with regard to the cardiac condition are recommended.
Left ventricular strain and strain rates are decreased in children with normal fractional shortening after exposure to anthracycline chemotherapy
- Thomas J. Moon, Shelley D. Miyamoto, Adel K. Younoszai, Bruce F. Landeck
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- 18 September 2013, pp. 854-865
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Background
Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiotoxicity. Previous methods to evaluate cardiac function are useful, but have significant limitations. We sought to determine the left ventricular strain and strain rate of paediatric cancer patients with normal fractional shortening treated with anthracyclines using the latest ultrasound feature-tracking technology.
Patients and methodsEchocardiograms on cancer patients before anthracycline exposure and following completion of treatment were retrospectively analysed using Velocity Vector Imaging software in the circumferential and longitudinal planes. The same analysis was performed on matched controls. Only patients with a fractional shortening ≥28% were included.
ResultsIn all, 71 patients were identified with an age at diagnosis of 10.5 ± 4.7 years. The time from diagnosis to follow-up was 3.9 ± 4.0 years and the cumulative anthracycline dose was 356 ± 106 mg/m2. Following anthracycline exposure, paediatric cancer patients had a higher heart rate and a lower longitudinal strain, longitudinal diastolic strain rate, circumferential strain, and circumferential systolic and diastolic strain rate when compared with controls. Diastolic strain rate showed the greatest percent difference following anthracycline exposure versus controls.
ConclusionDespite having a normal fractional shortening, children exposed to anthracyclines have subclinical derangement of their left ventricular deformation as measured by decreases in strain and strain rate in both the circumferential and longitudinal axis. In particular, there was a profound decrease in diastolic strain rate following anthracycline exposure compared with controls. Whether the decline of strain or strain rate can predict future risk of developing cardiomyopathy requires further investigation.
Atrial septal aneurysms in childhood: prevalence, classification, and concurrent abnormalities
- Andreas Giannopoulos, Christoforos Gavras, Stavroula Sarioglou, Fotini Agathagelou, Irene Kassapoglou, Fani Athanassiadou
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- Published online by Cambridge University Press:
- 07 June 2013, pp. 453-458
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Objectives: This study sought to investigate the prevalence of atrial septal aneurysms in the paediatric population and to define coexisting abnormalities and their incidence. Background: Few papers refer to the prevalence of atrial septal aneurysms in childhood. Methods: We enrolled a total of 4522 children aged more than 12 months who underwent a transthoracic echocardiography. Atrial septal aneurysm was defined as a protrusion of the interatrial septum or part of it >15 mm beyond the plane of the atrial septum or phasic excursion of the interatrial septum during the cardiorespiratory cycle of at least 15 mm in total amplitude and a diameter of the base of the aneurysm of at least 15 mm. Results: Atrial septal aneurysms were found in 47 children (1.04%). They involved almost the entire septum in 14 patients (28.89%) and were limited to the fossa ovalis in 33 (71.11%). An atrial septal aneurysm was an isolated structural defect in 17 (35.56%). In 30 (64.44%) patients, it was associated with interatrial shunting – atrial septal defect and patent foramen ovale. At the echo follow-up after a year, no changes were recorded. Conclusions: Prevalence of atrial septal aneurysms is almost 1%. The most common abnormalities associated are interatrial shunts, that is, a patent foramen ovale and an atrial septal defect. From a medical point of view, it is suggested that no action is to be taken during childhood, as a child with an atrial septal aneurysm is not at increased risk compared with a child without one. Follow-up is scheduled on an individual basis.
Original Article
Pulmonary valve preservation and restoration strategies for repair of tetralogy of Fallot*
- Constantine D. Mavroudis, Jennifer Frost, Constantine Mavroudis
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- Published online by Cambridge University Press:
- 29 December 2014, pp. 1088-1094
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Despite tremendous advances in surgical treatment of tetralogy of Fallot, augmenting the small right ventricular outflow tract remains a challenge. Transannular patch augmentation revolutionised surgical management, but did so at the expense of rendering patients with pulmonary insufficiency and the resulting problems associated therewith. Recent surgical efforts have focused on pulmonary valve preservation at initial correction and pulmonary valve restoration after transannular patching, with favourable results. In this manuscript, we review methods of pulmonary valve preservation and restoration.
Original Articles
Spinal surgery in the univentricular heart – is it viable?
- C. Pérez-Caballero Macarrón, E. Sobrino Ruiz, J. Burgos Flores, JL. Vázquez Martínez, A. Coca Pérez, E. Álvarez Rojas, JJ. Sánchez Ruas
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- Published online by Cambridge University Press:
- 07 February 2013, pp. 73-78
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Introduction
The management of patients with Fontan physiology who undergo scoliosis surgery is difficult. The purpose of this article was to describe our experience in the management of patients with Fontan circulation undergoing spinal surgery for correction of scoliosis.
Materials and methodsThis was a retrospective study including patients with Fontan physiology who underwent spinal orthopaedic surgery. Anaesthetic management, post-operative complications, paediatric intensive care unit and total hospital stay, and the need for blood transfusions were analysed.
ResultsWe identified eight children with Fontan physiology who had undergone spinal surgery from 2000 to 2010. All patients were receiving cardiac medications at the time of spinal surgery. The mean age at surgery was 14.8 years (range 12–21). In all, three patients needed inotropic support with dopamine (3, 5, and 8 μg/kg/min), which was started during surgery. During the immediate post-operative period, one patient died because of hypovolaemic shock caused by massive bleeding and dysrythmia. Mean blood loss during the post-operative period was 22.2 cc/kg (7.8–44.6). Surgical drainages were maintained for a mean time of 3 days (range 1–7). The mean hospital stay was 9.2 days (range 6–19). Pleural effusions developed in two patients. On follow-up, one patient presented with thoracic pseudarthrosis and another with a serohaematoma of the surgical wound.
ConclusionsSpinal surgery in patients with Fontan circulation is a high-risk operation. These patients must be managed by a specialised team.