Original Articles
Developmental screening in children with CHD: Ages and Stages Questionnaires
- Maia M. Noeder, Beth A. Logan, Kari L. Struemph, Nancy Condon, Isabel Mueller, Barbara Sands, Ryan R. Davies, Erica Sood
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- Published online by Cambridge University Press:
- 05 April 2017, pp. 1447-1454
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Objective
Standardised developmental screening tools are important for the evaluation and management of developmental disorders in children with CHD; however, psychometric properties and clinical utility of screening tools, such as the Ages & Stages Questionnaires, Third Edition (ASQ-3), have not been examined in the CHD population. We hypothesised that the ASQ-3 would be clinically useful for this population.
Study designASQ-3 developmental classifications for 163 children with CHD at 6, 12, 24, and/or 36 months of age were compared with those obtained from concurrent developmental testing with the Bayley Scales of Infant and Toddler Development, Third Edition.
ResultsWhen ASQ-3 screening failure was defined as ⩾1 SD below the normative mean, specificity (⩾81.9%) and negative predictive value (⩾81.0%) were high across ASQ-3 areas. Sensitivity was high for gross motor skills (79.6%), increased with age for communication (35.7–100%), and generally decreased with age for problem solving (73.1–50.0%). When ASQ-3 screening failure was defined as ⩾2 SD below the normative mean, specificity (⩾93.6%) and positive predictive value (⩾74.5%) were generally high across ASQ-3 areas, but sensitivity was low (31.1%) to fair (62.8%). The ASQ-3 showed improved accuracy in predicting delays over clinical risk factors alone.
ConclusionsThe ASQ-3 appears to be a clinically useful tool for screening development in children with CHD, although its utility varied on the basis of developmental area and time point. Clinicians are encouraged to refer children scoring ⩾1 SD below the normative mean on any ASQ-3 area for formal developmental evaluation.
Predictors of health-related quality of life in children with chronic heart disease
- Mandy Niemitz, Diana C. M. Gunst, Hedwig H. Hövels-Gürich, Michael Hofbeck, Renate Kaulitz, Christoph Galm, Felix Berger, Nicole Nagdyman, Brigitte Stiller, Thomas Borth-Bruhns, Ines Konzag, Christian Balmer, Lutz Goldbeck
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- Published online by Cambridge University Press:
- 18 May 2017, pp. 1455-1464
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Objective
Chronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease.
Patients and methodsThe Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers.
ResultsGreater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart–lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child’s health-related quality of life.
ConclusionsBeyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.
Initial assessment of a novel delivery system (NuDEL™ ®) for the covered Cheatham-Platinum stent
- Gareth J. Morgan, Damien Kenny, Christopher Duke, Kevin P. Walsh, Shakeel A. Qureshi
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- Published online by Cambridge University Press:
- 15 June 2017, pp. 1465-1469
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Aims
We sought to evaluate the first-in-man use of a new system for implantation of covered stents in patients with complex structural and CHD.
Methods and resultsRetrospective data were collected of the first 13 NuDEL™ delivery systems used in patients. The NuDEL™ comprises a covered Cheatham-Platinum stent mounted on a balloon-in-balloon and pre-loaded in a long delivery sheath. Data were collected from three centres in the United Kingdom and Ireland. A total of 13 covered stents were delivered via 12 NuDEL™ delivery systems in 12 patients. Among them, six patients had coarctation of the aorta, five patients had right ventricular outflow tract stenosis, and one patient had severe stenosis of a Mustard systemic venous baffle. There were no complications, and all the stents were deployed in the desired position with satisfactory haemodynamic results.
ConclusionsThe development of a bespoke system of a pre-mounted, pre-loaded covered stent may negate some of the technical challenges that complicate large-calibre stent deployment. Our preliminary results suggest that the NuDEL™ system is a safe and effective means of covered stent deployment in challenging anatomy.
Segregating bodily isomerism or heterotaxy: potential echocardiographic correlations of morphological findings
- Cornelia Tremblay, Rohit S. Loomba, Peter C. Frommelt, Donald Perrin, Diane E. Spicer, Carl Backer, Robert H. Anderson
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- Published online by Cambridge University Press:
- 03 April 2017, pp. 1470-1480
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Background
Bodily isomerism, also referred to as heterotaxy, involves predominantly the thoracic organs, although other organs are usually abnormally positioned. Previously assessed on the basis of splenic anatomy, it is now understood that isomerism is better segregated on the basis of atrial appendage morphology. This allows for anticipation of associated findings. We aimed to assess the accuracy of segregation based on the morphology of the atrial appendages and other structures more easily identified by echocardiography.
MethodsWe reviewed postmortem specimens of hearts from the archives at four institutions categorised as obtained from patients with “heterotaxy”. The cardiac structures were analysed using sequential segmental analysis. Non-cardiac structures were also examined if available. Statistical analyses were performed to compare differences in the settings of right as opposed to left isomerism.
ResultsSpecimens were available from 188 patients. Of these, 57 had left isomerism, and 131 had right isomerism. Atrial appendages were isomeric in all patients. A coronary sinus was found only in left isomerism, whereas a terminal crest, or a Eustachian valve, was found only in right isomerism. Interruption of the inferior caval vein was associated with left isomerism, whereas totally anomalous pulmonary venous connection was associated with right isomerism.
ConclusionIsomerism is uniformly segregated on the basis of the morphology of the atrial appendages, itself defined by the extent of the pectinate muscles. Other features such as the presence of a coronary sinus and systemic venous return can further help with such segregation of isomerism.
Fungal endocarditis in paediatrics: a review of 192 cases (1971–2016)
- Vithiya Ganesan, Shunmuga Sundaram Ponnusamy, Raja Sundaramurthy
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- Published online by Cambridge University Press:
- 27 March 2017, pp. 1481-1487
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Background
The aims of this article were to review the published literature on fungal endocarditis in children and to discuss the aetiology and diagnosis, with emphasis on non-invasive methods and various treatment regimes.
MethodsWe systematically reviewed published cases and case series of fungal endocarditis in children. We searched the literature, including PubMed and individual references for publications of original articles, single cases, or case series of paediatric fungal endocarditis, with the following keywords: “fungal endocarditis”, “neonates”, “infants”, “child”, and “cardiac vegetation”.
ResultsThere have been 192 documented cases of fungal endocarditis in paediatrics. The highest number of cases was reported in infants (93/192, 48%) including 60 in neonates. Of the neonatal cases, 57 were premature with a median gestational age of 27 weeks and median birth weight of 860 g. Overall, 120 yeast – fungus that grows as a single cell – infections and 43 mould – fungus that grows in multicellular filaments, hyphae – infections were reported. With increasing age, there was an increased infection rate with moulds. All the yeast infections were detected by blood culture. In cases with mould infection, diagnosis was mainly established by culture or histology of emboli or infected valves after invasive surgical procedures. There have been a few recent cases of successful early diagnosis by non-invasive methods such as blood polymerase chain reaction (PCR) for moulds. The overall mortality for paediatric fungal endocarditis was 56.25%. The most important cause of death was cardiac complications due to heart failure. Among the various treatment regimens used, none of them was significantly associated with better outcome.
ConclusionsNon-invasive methods such as PCR tests can be used to improve the chances of detecting and identifying the aetiological agent in a timely manner. Delays in the diagnosis of these infections may result in high mortality and morbidity. No significant difference was noted between combined surgical and medical therapy over exclusively combined medical therapy.
Using benchmarking to identify inter-centre differences in persistent ductus arteriosus treatment: can we improve outcome?
- Esther J. S. Jansen, Koen P. Dijkman, Richard A. van Lingen, Willem B. de Vries, Daniel C. Vijlbrief, Willem P. de Boode, Peter Andriessen
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- Published online by Cambridge University Press:
- 12 April 2017, pp. 1488-1496
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Objective
The aim of this study was to identify inter-centre differences in persistent ductus arteriosus treatment and their related outcomes.
Materials and methodsWe carried out a retrospective, multicentre study including infants between 24+0 and 27+6 weeks of gestation in the period between 2010 and 2011. In all centres, echocardiography was used as the standard procedure to diagnose a patent ductus arteriosus and to document ductal closure.
ResultsIn total, 367 preterm infants were included. All four participating neonatal ICU had a comparable number of preterm infants; however, differences were observed in the incidence of treatment (33–63%), choice and dosing of medication (ibuprofen or indomethacin), number of pharmacological courses (1–4), and the need for surgical ligation after failure of pharmacological treatment (8–52%). Despite the differences in treatment, we found no difference in short-term morbidity between the centres. Adjusted mortality showed independent risk contribution of gestational age, birth weight, ductal ligation, and perinatal centre.
ConclusionsUsing benchmarking as a tool identified inter-centre differences. In these four perinatal centres, the factors that explained the differences in patent ductus arteriosus treatment are quite complex. Timing, choice of medication, and dosing are probably important determinants for successful patent ductus arteriosus closure.
Prevalence and composition of CHD at different altitudes in Tibet: a cross-sectional study
- Jian-Yong Zheng, Yi-Gang Qiu, Dong-Tao Li, Jiang-Chun He, Yu Chen, Yi Cao, Ying-Ming Liu, Xian-Feng Li, Hai-Tao Chi, Tian-Chang Li
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- Published online by Cambridge University Press:
- 10 April 2017, pp. 1497-1503
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Background
The prevalence of CHD has been well described worldwide except in Tibet. This study aimed to illustrate the prevalence and composition of CHD in Tibetan children according to altitude.
Methods and resultsIn the first part, we prospectively recruited 7088 unselected Tibetan children (4–17 years) from south-west Tibet. The total prevalence of CHD increased from 4.6/1000 below 4200 m to 13.4/1000 above 4700 m, with a female-to-male ratio of 1.3:3.1. The total prevalence and female prevalence of patent ductus arteriosus increased more than 10-fold. Females living above 4700 m had exceptionally high prevalence of patent ductus arteriosus (14.9/1000). The prevalence of atrial septal defect was comparable among different altitudes (3.3–3.8/1000). The prevalence of ventricular septal defect was 1.3/1000 below 4700 m, and no cases were found above this altitude. In the second part, we retrospectively reviewed the clinical data of 383 CHD children in Tibet and 73 children at lower altitudes. The percentage of isolated ventricular septal defect decreased from 54.8 to 3.1%, and the percentage of isolated patent ductus arteriosus increased from 8.2 to 68.4% with elevation. Children living below 4200 m (10.4–13.7%) had a larger proportion of complex CHD than those above this altitude (2.0–3.1%). Of the 20 Tibetan children with complex CHD, 14 (70.0%) lived below 4200 m.
ConclusionsA wide variation in CHD prevalence and composition existed in Tibetan children among different altitudes.
CHD and respiratory syncytial virus: global expert exchange recommendations
- Robert M. R. Tulloh, Constancio Medrano-Lopez, Paul A. Checchia, Claudia Stapper, Naokata Sumitomo, Matthias Gorenflo, Eun Jung Bae, Antonio Juanico, Juan M. Gil-Jaurena, Mei-Hwan Wu, Talal Farha, Ali Dodge-Khatami, Rocky Tsang, Gerard Notario, Colleen Wegzyn
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- Published online by Cambridge University Press:
- 16 June 2017, pp. 1504-1521
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Background
Palivizumab is the standard immunoprophylaxis against serious disease due to respiratory syncytial virus infection. Current evidence-based prophylaxis guidelines may not address certain children with CHD within specific high-risk groups or clinical/management settings.
MethodsAn international steering committee of clinicians with expertise in paediatric heart disease identified key questions concerning palivizumab administration; in collaboration with an additional international expert faculty, evidence-based recommendations were formulated using a quasi-Delphi consensus methodology.
ResultsPalivizumab prophylaxis was recommended for children with the following conditions: <2 years with unoperated haemodynamically significant CHD, who are cyanotic, who have pulmonary hypertension, or symptomatic airway abnormalities; <1 year with cardiomyopathies requiring treatment; in the 1st year of life with surgically operated CHD with haemodynamically significant residual problems or aged 1–2 years up to 6 months postoperatively; and on heart transplant waiting lists or in their 1st year after heart transplant. Unanimous consensus was not reached for use of immunoprophylaxis in children with asymptomatic CHD and other co-morbid factors such as arrhythmias, Down syndrome, or immunodeficiency, or during a nosocomial outbreak. Challenges to effective immunoprophylaxis included the following: multidisciplinary variations in identifying candidates with CHD and prophylaxis compliance; limited awareness of severe disease risks/burden; and limited knowledge of respiratory syncytial virus seasonal patterns in subtropical/tropical regions.
ConclusionEvidence-based immunoprophylaxis recommendations were formulated for subgroups of children with CHD, but more data are needed to guide use in tropical/subtropical countries and in children with certain co-morbidities.
Right ventricular function after repair of tetralogy of Fallot: a comparison between bovine pericardium and porcine small intestinal extracellular matrix
- Ronak Naik, Jason Johnson, T. K. S. Kumar, Ranjit Philip, Umar Boston, Christopher J. Knott-Craig
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- Published online by Cambridge University Press:
- 29 May 2017, pp. 1522-1528
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Background
The porcine small intestinal extracellular matrix reportedly has the potential to differentiate into viable myocardial cells. When used in tetralogy of Fallot repair, it may improve right ventricular function. We evaluated right ventricular function after repair of tetralogy of Fallot with extracellular matrix versus bovine pericardium.
MethodSubjects with non-transannular repair of tetralogy of Fallot with at least 1 year of follow-up were selected. The extracellular matrix and bovine pericardium groups were compared. We used three-dimensional right ventricular ejection fraction, right ventricle global longitudinal strain, and tricuspid annular plane systolic excursion to assess right ventricular function.
ResultsThe extracellular matrix group had 11 patients, whereas the bovine pericardium group had 10 patients. No differences between the groups were found regarding sex ratio, age at surgery, and cardiopulmonary bypass time. The follow-up period was 28±12.6 months in the extracellular matrix group and 50.05±17.6 months in the bovine pericardium group (p=0.001). The mean three-dimensional right ventricular ejection fraction (55.7±5.0% versus 55.3±5.2%, p=0.73), right ventricular global longitudinal strain (−18.5±3.0% versus −18.0±2.2%, p=0.44), and tricuspid annular plane systolic excursions (1.59±0.16 versus 1.59±0.2, p=0.93) were similar in the extracellular matrix group and in the bovine pericardium group, respectively. Right ventricular global longitudinal strain in healthy children is reported at −29±3% in literature.
ConclusionIn a small cohort of the patients undergoing non-transannular repair of tetralogy of Fallot, there was no significant difference in right ventricular function between groups having extracellular matrix versus bovine pericardium patches followed-up for more than 1 year. Lower right ventricular longitudinal strain noted in both the groups compared to healthy children.
Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review
- Michelle Keir, Catriona Bhagra, Debra Vatenmakher, Francisca Arancibia-Galilea, Katrijn Jansen, Norihisa Toh, Candice K. Silversides, Jack Colman, Samuel C. Siu, Mathew Sermer, Andrew M. Crean, Rachel M. Wald
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- Published online by Cambridge University Press:
- 17 April 2017, pp. 1529-1537
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Objectives
Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population.
MethodsWe performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death.
ResultsA total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease.
ConclusionsWomen with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.
Exercise restriction is not associated with increasing body mass index over time in patients with anomalous aortic origin of the coronary arteries
- James M. Meza, Matthew D. Elias, Travis J. Wilder, James E. O’Brien, Richard W. Kim, Constantine Mavroudis, William G. Williams, Julie Brothers, Meryl S. Cohen, Brian W. McCrindle, for the Congenital Heart Surgeons’ Society
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- Published online by Cambridge University Press:
- 02 May 2017, pp. 1538-1544
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Anomalous aortic origin of the coronary arteries is associated with exercise-induced ischaemia, leading some physicians to restrict exercise in patients with this condition. We sought to determine whether exercise restriction was associated with increasing body mass index over time. From 1998 to 2015, 440 patients ⩽30 years old were enrolled into an inception cohort. Exercise-restriction status was documented in 143 patients. Using linear mixed model repeated-measures regression, factors associated with increasing body mass index z-score over time, including exercise restriction and surgical intervention as time-varying covariates, were investigated. The 143 patients attended 558 clinic visits for which exercise-restriction status was recorded. The mean number of clinic visits per patient was 4, and the median duration of follow-up was 1.7 years (interquartile range (IQR) 0.5–4.4). The median age at first clinic visit was 10.3 years (IQR 7.1–13.9), and 71% (101/143) were males. All patients were alive at their most recent follow-up. At the first clinic visit, 54% (78/143) were exercise restricted, and restriction status changed in 34% (48/143) during follow-up. The median baseline body mass index z-score was 0.2 (IQR 0.3–0.9). In repeated-measures analysis, neither time-related exercise restriction nor its interaction with time was associated with increasing body mass index z-score. Surgical intervention and its interaction with time were associated with decreasing body mass index z-score. Although exercise restriction was not associated with increasing body mass index over time, surgical intervention was associated with decreasing body mass index z-score over time in patients with anomalous aortic origin of the coronary arteries.
Transcatheter closure of the patent foramen ovale in children: intermediate-term follow-up results
- Kutay Sel, Hakan H. Aykan, Derya Duman, Ebru Aypar, Süheyla Özkutlu, Dursun Alehan, Tevfik Karagöz
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- Published online by Cambridge University Press:
- 02 May 2017, pp. 1545-1549
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The patent foramen ovale is almost a normal anatomical hole between the atria with ~30% incidence in the general population. It has been suggested that the patent foramen ovale is the cause of some neurological events, which is explained by paradoxical embolism. Transcatheter closure of the patent foramen ovale is a common procedure in adult patients with cerebral ischaemic events, but there are limited data investigating the results in children. Between January, 2005 and February, 2014, 17 patients’ patent foramen ovales were closed by the transcatheter approach in our department. The indications for closure were transient ischaemic attack in 10 patients, stroke in four patients, and migraine in three patients. The mean age and mean weight at the time of the procedure were 11.1±3.7 years and 42.1±15.4 kg, respectively. We asked our patients whether their previous ailments continued. All patients responded to the study survey. In 15 patients, ailments did not continue after patent foramen ovale closure and they significantly decreased in two of them. We suggest that under the right conditions device closure of the patent foramen ovale is a safe solution for these cryptogenic ischaemic events and migraine.
Long-term outcome after percutaneous closure of persistent left superior caval vein draining into the left atrium: a contrast-enhanced CT study
- Davide Marini, Matteo Castagno, Michele Millesimo, Francesca Ferroni, Gaetana Ferraro, Carlo Pace Napoleone, Gabriella Agnoletti
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- Published online by Cambridge University Press:
- 08 May 2017, pp. 1550-1556
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Background
Data regarding long-term outcome after percutaneous closure of left superior caval vein draining into the left atrium are lacking. The aim of the present study was to report the long-term follow-up by using contrast-enhanced CT.
MethodsIn all, three patients underwent percutaneous closure of left superior caval vein draining into the left atrium between 2005 and 2015. All of them were evaluated clinically and underwent contrast-enhanced CT.
ResultsIn one patient, the Amplatzer® Septal Occluder was used. In two patients, the Amplatzer® Vascular Plug type-1 was preferred: the device size/LSVC diameter ratio was 1.7 in the child and 1.2 in the adult. There were no early-onset or long-term onset complications. CT was performed 1, 2, and 10 years after the procedure, respectively. Complete occlusion of the vessel was documented in all. After 10 years since the procedure, CT revealed a persistent trivial residual shunt through the accessory hemiazygos vein in one patient, in whom the device was implanted above its drainage into the left superior caval vein. When an Amplatzer® Vascular Plug type-1 is oversized compared with the venous vessel diameter, it immediately assumes a dog-bone shape that disappears early to regain its shape memory and nominal size.
ConclusionsPercutaneous occlusion of left superior caval vein draining into the left atrium has excellent early and long-term outcomes. The optimal implantation of the device is below the drainage of the accessory hemiazygos vein, when present. The device might be oversized compared with the left superior caval vein diameter according to the age of the patient.
Comparison of two-dimensional and three-dimensional echocardiographic strain in children with CHD
- Bethany L. Wisotzkey, Brian D. Soriano, Sujatha Buddhe
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- Published online by Cambridge University Press:
- 15 May 2017, pp. 1557-1565
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Background
In CHD, three-dimensional strain analysis may overcome limitations of Doppler and two-dimensional strain of the left ventricle. The aims of this study were to evaluate feasibility and reproducibility of three-dimensional longitudinal, circumferential, and radial systolic strain by three-dimensional speckle-tracking echocardiography compared with two-dimensional echocardiography.
MethodsPatients with CHD, biventricular circulation with a systemic left ventricle, and who had two- and three-dimensional imaging performed on the same day from 2010 to 2014 were included. Quantitative two- and three-dimensional strain analyses were performed (two-dimensional cardiac performance analysis version 1.2 and four-dimensional left ventricular analysis version 3.1). Intra- and inter-observer variabilities were calculated on 25 studies.
ResultsA total of 30 patients, including 19 (61%) males, with a median age of 3.6 years (0.1–22 years) were included. The mean fractional shortening was 34.6±5.3%, and the mean ejection fraction was 62.0±6.4%. Measurement of two- and three-dimensional strain was feasible in >95% of segments. Good correlation was observed between longitudinal and circumferential strain (r=0.92, p⩽0.001 and r=0.87, p⩽0.001), but not radial strain (r=0.29, p=0.2). Intra- and inter-observer agreements were better for three-dimensional compared with two-dimensional strain, and better for both two- and three-dimensional longitudinal and circumferential strains compared with radial strain.
ConclusionLeft ventricular three-dimensional strain analysis is feasible in children with CHD. The reproducibility of longitudinal and circumferential strain by three-dimensional analyses is better. Further longitudinal studies are warranted for the potential clinical application of this new technology.
Results of pulmonary balloon valvuloplasty persist and improve at late follow-up in isolated pulmonary valve stenosis
- John J. Parent, Michael M. Ross, Edgard A. Bendaly, John P. Breinholt
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- Published online by Cambridge University Press:
- 27 June 2017, pp. 1566-1570
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Background
Pulmonary balloon valvuloplasty is a safe and effective treatment for children with pulmonary valve stenosis. A few studies evaluate the long-term outcomes of the procedure, particularly the degree of pulmonary regurgitation. We evaluated the outcomes of children >1 year following valvuloplasty for pulmonary valve stenosis.
MethodsA retrospective analysis of children with pulmonary valve stenosis following pulmonary balloon valvuloplasty at a single institution was performed. Clinic summaries, catheterisation data, and echocardiographic data were reviewed. Inclusion criteria were isolated pulmonary valve stenosis, age <19 years at the time of intervention, and at least one echocardiogram performed at least 1 year after valvuloplasty.
ResultsA total of 53 patients met inclusion criteria. The median age at valvuloplasty was 0.4 years (0.01–10.6 years). The last follow-up was 4.8±2.3 years following valvuloplasty. The pre-valvuloplasty peak instantaneous gradient by echocardiography was 60.6±14.6 mmHg. The peak gradient at the first postoperative echocardiography was reduced to 25.5±12 mmHg (p<0.001), and further decreased to 14.8±15.8 mmHg (p<0.001) at the most recent follow-up. The degree of regurgitation increased from before valvuloplasty to after valvuloplasty (p<0.001) but did not progress at the most recent follow-up (p=0.17). Only three patients (5.7%) required re-intervention for increasing pulmonary stenosis (two surgical; one repeat balloon). No significant procedural complications occurred.
ConclusionsPulmonary balloon valvuloplasty remains a safe and effective treatment for children with isolated pulmonary valve stenosis, with excellent long-term outcomes and no mortality. A few patients require further intervention. Long-term follow-up demonstrates decreased, residual stenosis. Patients have a small, acute increase in pulmonary regurgitation following valvuloplasty, but no long-term progression.
Determinants of quality of life in adults with CHD: an Australian cohort*
- Sarah L. Eaton, QiFeng Wang, Samuel Menahem
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- Published online by Cambridge University Press:
- 27 June 2017, pp. 1571-1576
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Background
Following improved survival rates in children with CHD, their quality of life and its determinants have become increasingly important. As part of a multicentre study entitled “Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease – International Study”, this article reviews the relationships among quality of life, anxiety and depression, sense of coherence, and severity of disease in an Australian cohort of adults with CHD.
Methods and resultsAdults with CHD were recruited from a single, community-based cardiology practice. All patients completed a self-reported questionnaire. A total of 135 patients, 71 males and 64 females, were recruited with a mean age of 26 years. The median quality of life in this cohort was 90; one-fifth of the patients experienced symptoms of anxiety. There was a significant negative correlation between quality of life and symptoms of anxiety and depression and a positive correlation between quality of life and sense of coherence.
ConclusionsThe quality of life of this cohort was generally excellent; however, one-fifth of them experienced symptoms of anxiety. Those with less anxiety and depression symptoms appeared to have a better quality of life, as did those who reported a higher sense of coherence. Interestingly, there was no significant relationship between complexity of CHD and quality of life.
The use of nesiritide in patients with critical cardiac disease
- Ronald A. Bronicki, Michele Domico, Paul A. Checchia, Curtis E. Kennedy, Ayse Akcan-Arikan
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- Published online by Cambridge University Press:
- 23 June 2017, pp. 1577-1584
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Objective
We evaluated the use of nesiritide in children with critical CHD, pulmonary congestion, and inadequate urine output despite undergoing conventional diuretic therapy.
DesignWe conducted a retrospective analysis of 11 patients with critical CHD, comprising 18 infusions, each of which occurred during separate hospitalisations. Haemodynamic parameters were assessed, and the stage of acute kidney injury was determined before and throughout the duration of therapy using a standardised definition of acute kidney injury – The Kidney Disease: Improving Global Outcomes criteria.
PatientsChildren with critical CHD, pulmonary congestion, and inadequate urinary output despite undergoing diuretic therapy were included.
Measurements and main resultsThe use of nesiritide was associated with a significant decrease in the maximum and minimum heart rate values and with a trend towards a significant decrease in maximum systolic blood pressure and maximum and minimum central venous pressures. Urine output increased but was not significant. Serum creatinine levels decreased significantly during the course of therapy (−0.26 mg/dl [−0.50, 0.0], p=0.02), and the number of patients who experienced a decrease in the stage of acute kidney injury of 2 or more – where a change in the stage of acute kidney disease of 2 or more was possible, that is, baseline stage >1 – was highly significant (five of 12 patients, 42%, p<0.001).
ConclusionsNesiritide had a favourable impact on haemodynamics, and its use was not associated with deterioration of renal function in patients with critical CHD.
Key drivers of patient experience in ambulatory paediatric cardiology
- Shalini D. Allam, Mary Mehta, Bertha Ben Khallouq, James F. Burrows, Paul Rosen
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- Published online by Cambridge University Press:
- 18 May 2017, pp. 1585-1590
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Patient experience is becoming a central focus of healthcare. A broad range of studies on how to increase patient satisfaction ratings exists; however, they lack the specificity to adequately guide physicians and hospitals on how to improve patient experience. The objective of this study was to define the aspects of patient experience within paediatric cardiologist practices that can serve as predictors of excellent patient satisfaction. From 1 January, 2013 to 28 February, 2015 (26 months), outpatients who visited paediatric cardiologists were asked to complete a 39-question patient satisfaction survey regarding their experience. Surveys were collected over a 26-month period by Press Ganey, an independent provider of patient satisfaction surveys. Participants were asked to rate their experience on a 1–5 Likert-scale: a score of 1 demonstrated a “poor” experience, whereas a score of 5 demonstrated a “very good” experience. This retrospective study of 2468 responses determined that cheerfulness of the practice (r=0.85, p<0.001), a cohesive staff (r=0.83, p<0.001), and a care provider explaining problems and conditions (r=0.81, p<0.001) were key aspects of a paediatric cardiologist’s practice that can be used as predictors of overall patient satisfaction. Awareness of how doctors can personalise a patient’s experience is vital to achieve greater patient satisfaction and, ultimately, better patient outcomes.
Impaired cardiac output during exercise in adults operated for ventricular septal defect in childhood: a hitherto unrecognised pathophysiological response
- Benjamin Asschenfeldt, Johan Heiberg, Steffen Ringgaard, Marie Maagaard, Andrew Redington, Vibeke E. Hjortdal
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- Published online by Cambridge University Press:
- 25 May 2017, pp. 1591-1598
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Background
Recent studies have demonstrated that surgical ventricular septal defect closure in childhood is associated with reduced functional capacity and disruption of the right ventricular force–frequency relationship during exercise. To further describe long-term cardiac function, we performed a non-invasive assessment of cardiac index during exercise in adults having undergone surgery for ventricular septal defect in early childhood.
MethodsA total of 20 patients (surgical age 2.1±1.4 years, age at examination 22.1±2.2 years) and 20 healthy, matched controls (23.4±2.1 years at examination) underwent continuous supine bicycle ergometry during MRI. Their blood flow was recorded in the ascending aorta and the pulmonary trunk at increasing exercise levels. Cardiac index, retrograde flow, and vessel diameters were determined by blinded, post hoc analyses.
ResultsThe patient group had normal cardiac index at rest (2.9±0.7 L/minute/m2), which was comparable with that of the controls (3.0±0.6 L/minute/m2); however, they had a lower increase in cardiac index during exercise (reaching 7.3±1.3 L/minute/m2 at submaximal exercise) compared with controls (8.2±1.2 L/minute/m2), p<0.05. Patients had a significantly higher ascending aorta retrograde flow than controls at rest and throughout exercise. In the pulmonary artery, the retrograde flow was minimal at rest in both groups, but increased significantly in patients during exercise compared with controls.
ConclusionsYoung adults with a surgically closed ventricular septal defect have a reduced cardiac index during exercise compared with healthy, young adults. The impaired cardiac index appears to be related to an increasing retrograde flow in the pulmonary artery with progressive exertion.
A rapid echocardiographic screening protocol for rheumatic heart disease in Samoa: a high prevalence of advanced disease
- Marvin Allen, John Allen, Take Naseri, Rebecca Gardner, Dennis Tolley, Lori Allen
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- Published online by Cambridge University Press:
- 15 June 2017, pp. 1599-1605
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Background
Echocardiography has been proposed as a method to screen children for rheumatic heart disease. The World Heart Federation has established guidelines for echocardiographic screening. In this study, we describe a rapid echocardiogram screening protocol according to the World Heart Federation guidelines in Samoa, endemic for rheumatic heart disease.
MethodsWe performed echocardiogram screening in schoolchildren in Samoa between 2013 and 2015. A brief screening echocardiogram was performed on all students. Children with predefined criteria suspicious for rheumatic hear diseases were referred for a more comprehensive echocardiogram. Complete echocardiograms were classified according to the World Heart Federation guidelines and severity of valve disease.
ResultsEchocardiographic screening was performed on 11,434 children, with a mean age of 10.2 years; 51% of them were females. A total of 558 (4.8%) children underwent comprehensive echocardiography, including 49 students who were randomly selected as controls. Definite rheumatic heart disease was observed in 115 students (10.0 per 1000): 92 students were classified as borderline (8.0 per 1000) and 23 with CHD. Advanced disease was identified in 50 students (4.4 per 1000): 15 with severe mitral regurgitation, five with severe aortic regurgitation, 11 with mitral stenoses, and 19 with mitral and aortic valve disease.
ConclusionsWe successfully applied a rapid echocardiographic screening protocol to a large number of students over a short time period – 28 days of screening over a 3-year time period – to identify a high prevalence of rheumatic heart disease. We also reported a significantly higher rate of advanced disease compared with previously published echocardiographic screening programmes.