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Children with cardiac diseases suffer from significant morbidity and mortality secondary to thromboembolic complications. Anticoagulant agents currently used for thromboprophylaxis have many limitations, including subcutaneous administration (low molecular weight heparins) and requirement for frequent monitoring via venipuncture (vitamin K antagonists). Edoxaban is an oral direct factor Xa inhibitor without need of monitoring. In the treatment of venous thromboembolism in adults, edoxaban has shown to be effective and safe.
This manuscript summarises the rationale and design of a phase 3, open-label, randomised controlled trial to evaluate and compare the safety and efficacy of edoxaban against standard of care (namely, vitamin K antagonist and low molecular weight heparin) in children with cardiac diseases.
A goal of 150 children with cardiac diseases at risk of thromboembolic complications who need primary or secondary anticoagulant prophylaxis will be recruited. Eligible children between 6 months and <18 years of age will be randomised in a ratio of 2 to 1 for edoxaban versus standard of care. Randomisation will be stratified based on underlying cardiac disease and concomitant use of aspirin for patients other than Kawasaki disease. The primary outcome will be safety, comprised of major and clinically relevant non-major bleeding in first 3 months of treatment. Bleeding beyond 3 months, symptomatic and asymptomatic thromboembolic events, and pharmacokinetic and pharmacodynamic parameters will be evaluated as secondary outcomes.
Randomised controlled anticoagulation trials are challenging in children. This study will evaluate a potentially valuable alternative of oral anticoagulant prophylactic use in children with cardiac diseases.
This retrospective case series study sought to describe the safety and clinical effectiveness of propafenone for the control of arrhythmias in children with and without CHD or cardiomyopathy.
Methods:
We reviewed baseline characteristics and subsequent outcomes in a group of 63 children treated with propafenone at 2 sites over a 15-year period Therapy was considered effective if no clinically apparent breakthrough episodes of arrhythmias were noted on the medication.
Results:
Sixty-three patients (29 males) were initiated on propafenone at a median age of 2.3 years. CHD or cardiomyopathy was noted in 21/63 (33%). There were no significant differences between demographics, clinical backgrounds, antiarrhythmic details, side effect profiles, and outcomes between children with normal hearts and children with CHD or cardiomyopathy. Cardiac depression at the initiation of propafenone was more common amongst children with CHD or cardiomyopathy compared to children with normal hearts. Systemic ventricular function was diminished in 15/63 patients (24%) prior to starting propafenone and improved in 8/15 (53%) of patients once better rhythm control was achieved. Other than one child in whom medication was stopped due to gastroesophageal reflux, no other child experienced significant systemic or cardiac side effects during treatment with propafenone. Propafenone achieved nearly equal success in controlling arrhythmias in both children with normal hearts and children with congenital heart disease or cardiomyopathy (90% versus 86%, p = 0.88).
Conclusion:
Propafenone is a safe and effective antiarrhythmic medication in children.
Turner syndrome is a clinical syndrome caused by autosomal abnormalities in women. It is often accompanied by congenital cardiovascular malformations commonly including a bicuspid aortic valve malformation and aortic coarctation, but the presence of multiple pulmonary venous abnormalities is extremely rare. We present a 27-year-old woman who was diagnosed with Turner syndrome. She was revealed an anomalous right upper pulmonary venous connection, left upper pulmonary vein obstruction, and varicose vein malformations of the left pulmonary veins by a series of examination. Cardiac catheterisation and selective pulmonary angiography can further confirm the diagnosis, morphological characteristics, haemodynamic significance and provide a reference for the next step of treatment.
A 10-day-old neonate with pulmonary consolidation was referred for echocardiography to rule out CHD. At first glance, the morphology appeared to be a bipartite right ventricle with normal tricuspid and pulmonary valves. In-depth analysis, however, of the images showed a double-chambered right ventricle, in which the inlet and outlet portions of the right ventricle were isolated from the apical component of the right ventricle, which itself communicated with the left ventricle through a ventricular septal defect. There was a normal pulmonary valve and tricuspid annulus.
Tetralogy of Fallot is a common CHD. Studies have shown a close link between heart failure and myocardial fibrosis. Interleukin-6 has been suggested to be a post-independent factor of heart failure. This study aimed to explore the relationship between IL-6 and myocardial fibrosis during cardiopulmonary bypass.
Material and Methods:
We downloaded the expression profile dataset GSE132176 from Gene Expression Omnibus. After normalising the raw data, Gene Set Enrichment Analysis and differential gene expression analysis were performed using R. Further, a weighted gene correlation network analysis and a protein–protein interaction network analysis were used to identify HUB genes. Finally, we downloaded single-cell expression data for HUB genes using PanglaoDB.
Results:
There were 119 differentially expressed genes in right atrium tissues comparing the post-CPB group with the pre-CPB group. IL-6 was found to be significantly up-regulated in the post-CPB group. Six genes (JUN, FOS, ATF3, EGR1, IL-6, and PTGS2) were identified as HUB genes by a weighted gene correlation network analysis and a protein–protein interaction network analysis. Gene Set Enrichment Analysis showed that IL-6 affects the myocardium during CPB mainly through the JAK/STAT signalling pathway. Finally, we used PanglaoDB data to analyse the single-cell expression of the HUB genes.
Conclusion:
Our findings suggest that high expression of IL-6 and the activation of the JAK/STAT signalling pathway during CPB maybe the potential mechanism of myocardial fibrosis. We speculate that the high expression of IL-6 might be an important factor leading to heart failure after ToF surgery. We expect that these findings will provide a basis for the development of targeted drugs.
The incidence of airway obstruction in patients with complex CHD other than vascular rings and absent pulmonary valve syndrome is unknown. We reviewed pre-operative CT and clinical data of children with conotruncal abnormalities to assess for airway obstruction. Airway obstruction was common (41% of patients), often moderate to severe, of diverse aetiology, and most commonly associated with a right aortic arch. Patients with airway obstruction showed a trend towards a higher mortality rate. Patients with complex conotruncal abnormalities should be assessed for airway obstruction as it may help predict the need for additional interventions and assist with prognostication.
Anorexia nervosa has a prevalence of 0.5–3% in adolescents, placing this population at increased risk of cardiac anomalies including arrhythmias, pericardial effusion, and myocardial dysfunction. Our objective is to describe cardiovascular anomalies observed by tissue Doppler imaging in patients with anorexia nervosa.
Methods:
We retrospectively reviewed electrocardiogram, Holter, and echocardiography findings in 28 patients diagnosed with anorexia nervosa.
Results:
Electrocardiogram was abnormal in 71% of patients with sinus bradycardia observed in 57%. Holter confirmed sinus bradycardia without significant pauses. Prolonged QTc, low voltage, and ectopic beats were each seen in 14% of patients. Wenckebach atrioventricular block was observed in one patient. Supraventricular or ventricular tachycardia was not observed. Echocardiography showed structurally normal heart in all patients. Pericardial effusion was seen in 7.1% of patients and left ventricular mass was decreased in 10.7%. Mean ejection fraction was 0.73 and mean fractional shortening was 38.4%. Tissue Doppler imaging revealed systolic or diastolic dysfunction in four patients with e’, a’, and s’ velocities in the lateral and septal basal segments more than two standard deviations below the mean. Two patients had decreased left ventricular mass but no significant difference in disease duration from the group. Basal segment velocities below one standard deviation were also observed in an additional seven patients.
Conclusion:
A trend for decreased tissue Doppler imaging velocities was seen in 25.0% of patients, while significant systolic and diastolic dysfunction was seen in 14.3% of patients, associated with a significant reduction in left ventricular mass and independent of disease duration.
Extensive screening in a newborn with prenatal suspicion of VACTERL syndrome identified an anomalous origin of the left pulmonary artery from the descending aorta with an arterial duct and left aortic arch, and normal intra-cardiac anatomy. Other anatomical anomalies suggested heterotaxy syndrome. At one-month-old, re-implantation of the 3.5 mm left pulmonary artery was performed by direct tension-low anastomosis. Post-operative course was complicated by severe left pulmonary atelectasis, and the patient died 20 days later.
Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.
Women with underlying cardiac conditions have an increased risk of adverse pregnancy outcomes. Counselling reproductive age women with heart disease is important to assist them in deciding whether to pursue pregnancy, to ensure their best cardiovascular status prior to pregnancy, and that they understand the risks of pregnancy for them and baby. This also provides an opportunity to explore management strategies to reduce risks. For this growing cohort of women, there is a great need for pre-conceptual counselling.
This retrospective comparative audit assessed new referrals and pre-conceptual counselling of women attending a joint obstetric–cardiology clinic at a tertiary maternity centre in a 12-month period of 2015–2016 compared with 2018–2019. This reflected the timing of the introduction of a multidisciplinary meeting prior to clinics and assessed the impact on referrals with the introduction of the European Society of Cardiology guidelines.
Data were reviewed from 56 and 67 patients in respective audit periods. Patient’s risk was stratified using modified World Health Organization classification.
Less than 50% of women with pre-existing cardiac conditions had received pre-conceptual counselling, although half of them had risks clearly documented. The majority of patients had a recent electrocardiograph and echocardiogram performed prior to counselling, and there was a modest improvement in the number of appropriate functional tests performed between time points. One-third of patients in both cohorts were taking cardiac medications during pregnancy.
There was a significant increase in the number of pregnant women with cardiac disease and in complexity according to modified World Health Organization risk classification. While there have been improvements, it is clear that further work to improve availability and documentation of pre-pregnancy counselling is needed.
We describe two cases of an unusual variant of double outlet right ventricle with intact ventricular septum diagnosed prenatally and confirmed by foetal autopsy in a case. The first case had mitral valve atresia, slit-like left ventricle, and normally related great arteries. The second case had mitral valve atresia, hypoplastic left ventricle, parallel outflows with an interrupted aortic arch.
High take-off coronary artery anomaly is a quite rare anomaly which is usually seen in isolated form and diagnosed incidentally. Association with tetralogy of Fallot is also rare and it is not one of the well-known coronary anomalies seen in this disease. Here, we describe high take-off right coronary artery in a 10-month-old female patient with tetralogy of Fallot which was diagnosed during catheter angiography. It is very important to show this anomaly sometimes with additional imaging techniques as it alters all the surgical approach including aortic cannulation.
Total anomalous pulmonary venous connection is a rare cyanotic CHD that requires surgical repair. An unligated vertical vein after total anomalous pulmonary venous connection surgery may help to decrease the episodes of post-operative pulmonary hypertensive crises, low cardiac output syndrome, and mortality. The aim was to assess long-term outcome and our post-operative transcatheter vertical vein closure experiences in five patients with repaired total anomalous pulmonary venous connection patients.
Methods:
A retrospective study was conducted in five cases with an unligated vertical vein following repair of supra-cardiac total anomalous pulmonary venous connection at our hospital from 2011 through 2018. Patients characteristics, cardiac catheterisation findings, surgical, and transcatheter procedural details were retrospectively analysed.
Results:
Transcatheter closure of the unligated vertical vein was technically successful in all the patients. Procedure-related complications were not observed in any of the patients. No long-term complication was found.
Conclusions:
We suggest that transcatheter closure of the patent vertical vein is an effective and well-tolerated alternative to the surgical approach.
Supracardiac total anomalous pulmonary venous drainage is commonly associated with a left-sided ascending vein draining into innominate vein. We present a case of a newborn with a right-sided ascending vein, draining into the right superior caval vein with stenosis at the SVC-ascending vein junction, posing a surgical dilemma in corrective surgery. Usage of three-dimensional computed tomographic scan was essential in delineating the anatomy and aiding surgery. The case demonstrates the rarity of this type of cardiac disease and the complications that develop.
A 6-year-old male with heterotaxia, abnormal systemic and pulmonary venous drainage, and a history of Fontan completion presented with desaturations and was found by cardiac catheterisation to have a hepatic vein to coronary sinus connection. This was successfully occluded using an Amplatzer Muscular Ventricular Septal Defect Occluder.
Diaphragmatic eventration is an anomaly of the diaphragm. In Scimitar syndrome, a curved-shaped anomalous pulmonary venous drainage is seen. Association between these conditions is rare. We present a newborn with diaphragmatic eventration, whose diagnosis of Scimitar syndrome was made after surgical repair. Scimitar syndrome is a congenital disorder often associated with other heart and lungs anomalies. Diagnosis can be fortuitous but with important prognostic features.
After congenital heart surgery, some patients may need long-term mechanical ventilation because of chronic respiratory failure. In this study, we analysed outcomes of the patients who need tracheostomy and home mechanical ventilation.
Methods:
Amongst 1343 patients who underwent congenital heart surgery between January, 2014 and June, 2018, 45 needed tracheostomy and HMV. The median age of these patients was 6.4 months (12 days–6.5 years). Nineteen patients underwent palliation while 26 patients underwent total repair. Post-operative diaphragm plication was performed in five patients (11%). Median duration of mechanical ventilation before tracheostomy was 32 days (8–154 days). The patients were followed up with their home ventilators in ward and at home. Mean follow-up time was 36.24 ± 11.61 months.
Results:
The median duration of ICU stay after tracheostomy was 27 days (range 2–93 days). Follow-up time in ward was median 30 days (2–156 days). A total of 12 patients (26.6%) were separated from the ventilator and underwent decannulation during hospital stay. Thirty-two patients (71.1%) were discharged home with home ventilator support. Of them, 15 patients (46.9%) were separated from the respiratory support in median of 6 weeks (1 week–11 months) and decannulations were performed. Total mortality was 31.1%. in which four patients are still HMV dependent. There was no significant difference for decannulation between total repair and palliation patients.
Conclusion:
HMV via tracheostomy is a useful option for the treatment of children who are dependent on long-term ventilation after congenital heart surgery although there are potential risks.
Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.
Pulmonary pressure is one of the most important parameters in the postoperative follow-up of patients who have undergone the Glenn procedure. Platelet activation markers, which are inexpensive and easily accessible blood count parameters, have been shown to be associated with the aetiology and pathogenesis of primary pulmonary artery hypertension. We examined the relationship between platelet activation markers and pulmonary pressures in the early postoperative period of patients who underwent the Glenn procedure.
Eighty-five patients who underwent the Glenn procedure in our clinic between January 2011 and March 2020 were included in the study retrospectively. Fifty-one patients were male and 34 were female, and age varied from 4 to 416 months, with a mean of 28.64 ± 51 months.
Patients with increased pulmonary blood flow on palliation before Glenn surgery had higher mean platelet volume values. However, no correlation was found between pulmonary pressures and platelet activation markers in the early postoperative period.
There was not similar study evaluating platelet activation markers in the paediatric age group before and after postoperative Glenn surgery in the literature. Therefore, even if platelet activation markers provide information about the pulmonary bed, they may be misleading due to other reasons that trigger bleeding and inflammatory processes in the early postoperative period.
We present a rare and challenging case of left ventricular aneurysm in an African child with no history of previous infection or trauma, admitted for surgical treatment, who presented non reversible cardiorespiratory arrest with cardiorespiratory resuscitation before surgery.