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Bronchogenic cysts are considered rare airway anomalies that can have a significant impact on the well-being of infants. In this case report, we present a rare presentation of bronchogenic cyst presenting with early neonatal respiratory distress due to airway and vascular compression. Surgical excision was curative with an excellent prognosis.
Tetralogy of Fallot is a congenital heart defect diagnosed in infancy. Assessment of right ventricular size and function is important for evaluation of patients with tetralogy of Fallot, but these quantitative measures are challenging by echocardiography. This study evaluates a semi-automated software (EchoInsight®, Epsilon Imaging) by comparing its measures to manual measures in children with tetralogy of Fallot.
Methods:
Echocardiographic measurements were performed using manual techniques and semi-automated software. Right ventricular measurements included end-diastolic and end-systolic area, fractional area change, chamber dimensions, and tricuspid annular plane systolic excursion. Reliability, correlation, and agreement between manual and semi-automated measures were assessed.
Results:
Echocardiograms for 46 patients were analysed. Intra- and inter-observer reliabilities for semi-automated measures were good with intraclass correlation coefficients all over 0.95 and 0.85, respectively. There was high correlation between manual and semi-automated methods for areas and dimensions (r = 0.91–0.98). Tricuspid annular plane systolic excursion measures and fractional area change also correlated, albeit less strongly. The semi-automated measurements of end-systolic and end-diastolic area were a 20 and 47% higher than manual methods, respectively.
The semi-automated method yielded a relative 52% lower fractional area change compared to the manual method.
Conclusions:
The semi-automated software generates quantitative right ventricular measures in children with tetralogy of Fallot with good reliability and good correlation with manual methods for all measures, but with significant difference between manual and semi-automated techniques for area and functional measures. The specific right ventricular geometry in tetralogy of Fallot children may be why, compared to normal anatomy, greater differences were observed between the two techniques.
Webinars have recently replaced in-person medical conferences, including paediatric cardiology conferences, given the COVID-19 pandemic.
Methods:
With increasing environmental concerns, we analysed the differences between the environmental footprint of a paediatric cardiology webinar with a hypothetical conference. Travel data was collected, with assumptions made on the amount of computer use, internet use and accordingly the overall use of electricity for both forms of conference. Life Cycle Assessment methodology was used (OpenLCA and Ecovinvent v 3.7).
Results:
We showed that the theoretical environmental impact of a virtual conference is significantly less (4 tons CO2 equivalent) than the traditional international face-to-face conference (192 tons CO2 equivalent). The life cycle assessment methodology showed that resource use for a face-to-face conference lasting 2.5 days for 1374 attendees is equivalent to 400 times what an average person would use in one year, the climate change and photochemical ozone formation approximately 250 times and the eutrophication terrestrial equivalent to 225 times. However, using carbon equivalent emissions to measure environmental harm from flying is an under estimate of the potential damage, when one considers the additional production of airplane contrails. Notwithstanding this, there is a 98% reduction in climate change impact when meetings are held virtually.
Conclusions:
While the virtual conference may never completely replace the traditional in-person paediatric cardiology conference, due to networking benefits, the significant theoretical benefits to the environment highlighted in this study, warrants consideration for the virtual conference taking a more common place in sustainable academia.
In resource limited settings, children with cardiac disease present late, have poor outcomes and access to paediatric cardiology programmes is limited. Cardiac point of care ultrasound was introduced at several Médecins Sans Frontières sites to facilitate cardiopulmonary assessment. We describe the spectrum of disease, case management and outcomes of cases reviewed over the Telemedicine platform.
Methods:
Previously ultrasound naïve, remotely placed clinical teams received ultrasound training on focussed image acquisition. The Médecins Sans Frontières Telemedicine platform was utilised for remote case and imaging review to diagnose congenital and acquired heart disease and guide management supported by a remotely situated paediatric cardiologist.
Results:
Two-hundred thirty-three cases were reviewed between 2016 and 2018. Of 191 who underwent focussed cardiac ultrasound, diagnoses included atrial and ventricular septal defects 11%, atrioventricular septal defects 7%, Tetralogy of Fallot 9%, cardiomyopathy/myocarditis 8%, rheumatic heart disease 8%, isolated pericardiac effusion 6%, complex congenital heart disease 4% and multiple other diagnoses in 15%. In 17%, there was no identifiable abnormality while 15% had inadequate imaging to make a diagnosis. Cardiologist involvement led to management changes in 75% of cases with a diagnosis. Mortality in the entire group was disproportionately higher among neonates (38%, 11/29) and infants (20%, 16/81). There was good agreement on independent review of selected cases between two independent paediatric cardiologists.
Conclusion:
Cardiac point of care ultrasound performed by remote clinical teams facilitated diagnosis and influenced management in cases reviewed over a Telemedicine platform. This is a feasible method to support clinical care in resource limited settings.
Surgical repair of Tetralogy of Fallot has excellent outcomes, with over 90% of patients alive at 30 years. The ideal time for surgical repair is between 3 and 11 months of age. However, the symptomatic neonate with Tetralogy of Fallot may require earlier intervention: either a palliative intervention (right ventricular outflow tract stent, ductal stent, balloon pulmonary valvuloplasty, or Blalock-Taussig shunt) followed by a surgical repair later on, or a complete surgical repair in the neonatal period. Indications for palliation include prematurity, complex anatomy, small pulmonary artery size, and comorbidities. Given that outcomes after right ventricular outflow tract stent palliation are particularly promising – there is low mortality and morbidity, and consistently increased oxygen saturations and increased pulmonary artery z-scores – it is now considered the first-line palliative option. Disadvantages of right ventricular outflow tract stenting include increased cardiopulmonary bypass time at later repair and the stent preventing pulmonary valve preservation. However, neonatal surgical repair is associated with increased short-term complications and hospital length of stay compared to staged repair. Both staged repair and primary repair appear to have similar long-term mortality and morbidity, but more evidence is needed assessing long-term outcomes for right ventricular outflow tract stent palliation patients.
Previous echocardiographic studies failed to show residual alterations of heart function in paediatric patients that have received treatment for Chagas disease. While the echocardiogram is the fundamental front-line tool for evaluating heart function, the appearance of new techniques allows a more detailed analysis. We aimed to evaluate systolic and diastolic function with new techniques in a paediatric population with Chagas disease several years after treatment completion.
Material and methods:
Echocardiograms were obtained from 84 Chagas disease patients (48 female) and 27 healthy controls. All patients had received treatment concluding on average 10 years prior to the study. The prospective analysis considered cardiac dimensions and cardiac function using two-dimensional, M-mode, Doppler and tissue Doppler imaging with emphasis on measuring longitudinal strain in the left ventricle by speckle tracking. Ejection fraction was measured with three-dimensional echocardiography.
Results:
Patients had an age of 14.2 ± 5.7 years (6–33) at the time of evaluation. Global and segmental motility of the left ventricle was normal in all patients. Ejection fraction was 59.2 ± 6.5 and 57.4 ± 6.5% (p = 0.31) in patients and controls respectively. Left ventricular global longitudinal systolic strain was −19 ± 2.4% in patients and −19 ± 3.6% (p = 0.91) in controls. No significant differences were found in remaining systolic and diastolic function measurements.
Conclusions:
Paediatric patients that have received treatment for Chagas disease, evaluated with either conventional techniques or new tools, do not show significant long-term alterations of ventricular function.
Although cardiac catheterisation (cath) is the diagnostic test for pulmonary hypertension, it is an invasive procedure. Echocardiography (echo) is commonly used for the non-invasive diagnosis of pulmonary hypertension but maybe limited by lack of adequate signals. Therefore, emphasis has been placed on biomarkers as a potential diagnostic tool. No prior paediatric studies have simultaneously compared N-terminal pro-B-type-natriuretic peptide (NTproBNP) with cath/echo as a potential diagnostic tool. The aim of this study was to determine if NTproBNP was a reliable diagnostic tool for pulmonary hypertension in this population.
Methods:
Patients were divided into Study (echo evidence/established diagnosis of pulmonary hypertension undergoing cath) and Control (cath for small atrial septal defect/patent ductus arteriosus and endomyocardial biopsy post cardiac transplant) groups. NTproBNP, cath/echo data were obtained.
Results:
Thirty-one patients met inclusion criteria (10 Study, 21 Control). Median NTproBNP was significantly higher in the Study group. Echo parameters including transannular plane systolic excursion z scores, pulmonary artery acceleration time and right ventricular fractional area change were lower in the Study group and correlated negatively with NTproBNP. Receiver operation characteristic curve analysis demonstrated NTproBNP > 389 pg/ml was 87% specific for the diagnosis of pulmonary hypertension with the addition of pulmonary artery acceleration time improving the specificity.
Conclusions:
NTproBNP may be a valuable adjunctive diagnostic tool for pulmonary hypertension in the paediatric population. Echo measures of transannular plane systolic excursion z score, pulmonary artery acceleration time and right ventricular fractional area change had negative correlations with NTproBNP. The utility of NTproBNP as a screening tool for pulmonary hypertension requires validation in a population with unknown pulmonary hypertension status.
Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients.
Methods and results:
From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]).
Conclusions:
Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.
We aimed to assess the immediate haemodynamic response and the timing of cardiac remodelling in paediatric secundum atrial septal defect patients who underwent percutaneous transcatheter closure.
Methods:
In this longitudinal cohort study with 41 paediatric secundum ASD patients who underwent PTC with Amplatzer Occluder device were assessed for immediate post-interventional haemodynamic response measured by catheterisation and was evaluated for right and left cardiac remodelling during a follow-up period of 12 months by transthoracic echocardiography. SPSS 20.0 was used for statistical analyses of pre- and post-interventional invasive haemodynamic parameters of the patients, and pre- and post-interventional TTE data compared with the values of the control group consisted of 39 healthy children.
Results:
The mean diameter of ASD was 13.9 ± 4.7 mm. PTC intervention in all patients completed with 100% success and 0% complication rates. All invasive haemodynamic data, except the ratio of pulmonary resistance to systemic resistance, significantly reduced after PTC (p < 0.05). TTE and PW Doppler revealed that right and left cardiac remodelling started as soon as the post-interventional 24th hour and completed in the 12th month.
Conclusions:
This study with a very high interventional success rate can be counted as the first example of research on the haemodynamic response and timing of cardiac remodelling after PTC of secundum ASD in children. We suggest that future multicentric studies with larger cohorts and a comprehensive methodology like ours with longer follow-up periods would better serve to further assess the cardiac remodelling in children after PTC of secundum ASD.
Kawasaki disease is an acute systemic vascular disease, generally self-limited and typically affecting children <5 years old, which leads to coronary artery aneurysms in about 25% of untreated cases. Cardiovascular involvement is characterised by transient pancarditis, in acute phase, while coronary illness, ranging from mild dilation to giant CAAs occurs late, rarely before the 10th day since fever onset. Here, we describe a peculiar case of KD, which occurred in a 4-month-old infant and presented with exudate cardiac tamponade and early giant aneurism of both the proximal right coronary artery) and the left circumflex coronary artery, in acute phase of the disease.
To analyse the changes of different ventilation on regional cerebral oxygen saturation and cerebral blood flow in infants during ventricular septal defect repair.
Methods:
Ninety-two infants younger than 1 year were enrolled in the study. End-expiratory tidal pressure of carbon dioxide was maintained at 40–45 and 35–39 mmHg in relative low and high ventilation groups. Regional cerebral oxygen saturation and flow velocity of the middle cerebral artery were recorded after anaesthesia (T0), cut pericardium (T1), separation from cardiopulmonary bypass (T2), the end of modified ultrafiltration, (T3) and at the end of operation (T4).
Results:
The relative low ventilation group exhibited a significantly high regional cerebral oxygen saturation at each time point except for T2 (T0:77 ± 4, T1:76 ± 5, T3:76 ± 8, T4:76 ± 8, respectively, p < 0.001). Flow velocity of the middle cerebral artery in the relative low ventilation group was higher compared to the relative high ventilation group at each time point except for T2 (T0:53 ± 14, T1:54 ± 15, T3:53 ± 17, T4:52 ± 16, respectively, p < 0.001). Between the two groups, T2 showed the lowest middle cerebral artery flow velocity (relative low ventilation: 39 ± 15, relative high ventilation: 39 ± 11, p < 0.001).
Conclusion:
The infants’ regional cerebral oxygen saturation and middle cerebral artery flow velocity performed better in the range of 40–45 mmHg end-expiratory tidal pressure of carbon dioxide during CHD surgery. Modified ultrafiltration increased cerebral oxygen saturation. It was important to regulate ventilation in order to balance cerebral oxygen in infants.
Pulmonary hypertension is not the only cause of arterial desaturation in patients with atrial septal defect. Arterial desaturation can also occur with normal pulmonary artery pressure making it mandatory to understand the mechanism to avoid erroneous diagnosis. In this report, for the first time, we demonstrate atrial flutter as the cause of arterial desaturation in a patient with large atrial septal defect despite normal pulmonary artery pressure, which was normalised following successful radiofrequency ablation.
16p12.2 microdeletion has been associated with congenital heart defects and developmental delay. In this case, we describe the rare association between tetralogy of Fallot with an absent pulmonary valve a right-sided aortic arch and a retro-aortic innominate vein associated with a 16p12.2 microdeletion and epilepsy.
An otherwise healthy 17-year-old adolescent boy presented with upper respiratory tract symptoms and was diagnosed with coronavirus disease 2019 infection. A haemorrhagic pericardial effusion was found in the transthoracic echocardiography. He was treated with pericardiocentesis, ibuprofen, and hydroxychloroquine. A large pericardial effusion may complicate COVID-19 in children and should be considered in acute decompensation.
The authors briefly present two clinical cases and discuss the approach to the management of an incidental finding of patent foramen ovale (PFO) in a young child. We discuss the current controversies in the management of PFO in this age group.
Mammalian target of rapamycin inhibitors was found recently to be an effective treatment for manifestations of Tuberous sclerosis complex, including cardiac rhabdomyomas. Most cases with Cardiac rhabdomyoma treated with mammalian target of rapamycin inhibitors to date were diagnosed with Tuberous sclerosis. We report a case of cardiac rhabdomyoma and severe right ventricular outflow obstruction in a baby with negative genetics for Tuberous sclerosis that responded rapidly to Sirolimus.
Optimal pre-participative screening in young athletes is still controversial. We sought to evaluate the strategy of including point-of-care ultrasound to electrocardiogram. In total, 1188 young competitive athletes were screened in different sports institutions. This proved to be a useful strategy by improving diagnostic performance primarily with respect to detect structural abnormalities and also by minimising positive false cases of electrocardiogram alone.
We present a 53-year-old male with the rare constellation of stress cardiomyopathy, dextrocardia with situs inversus and anomalous coronary anatomy. This case highlights the difficulties faced when managing patients with uncommon disorders and demonstrates a rare overlap of acquired and CHD.
Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs. The purpose of this study is to demonstrate, with this rare anomaly, the accurate place of the echocardiography to establish diagnosis especially in the systemic or supra-systemic pulmonary hypertension.
Isolated subclavian artery is a rare anomaly. A second steal due to a patent arterial duct further reduces arm perfusion. Surgical anastomosis of the isolated vessel to aorta normalises arm perfusion. Simple echocardiographic clues aid in the diagnosis. An associated moderate sized ventricular septal defect was non-surgically closed along with catheter closure of the duct resulting in improved arm perfusion.