Infants with complex cyanotic CHD can become symptomatic from insufficient pulmonary blood supply following either ductal closure or due to outflow tract obstruction. Blalock–Taussig shunt mortality remains significant and recent studies have highlighted the advantages of using transcatheter alternatives. We present here our experience in changing our primary choice of palliation from the Blalock–Taussig shunt to transcatheter palliation with either a ductal stent or, if antegrade flow is present, a right ventricular outflow tract stent.

This is a retrospective, single-unit cohort study. Eighty-seven infants underwent palliation for insufficient pulmonary blood flow at under 3 months of age between 2012 and 2019. On an intention-to-treat basis, 29 underwent insertion of a Blalock–Taussig shunt, 36 duct stents, and 22 right ventricular outflow tract stents at median ages of 15, 9, and 32 days, respectively, and median weights of 3.3, 3.1, and 3.1 kg, respectively. No primary Blalock–Taussig shunts have been performed in our institution since 2017.

At 30-days there had been one death in each group (univariable p = 0.93) and deaths prior to repair totalled three in the shunt group, four in the ductal stent group, and two in the right ventricular outflow tract stent group (univariable p = 0.93). Reintervention on the pulmonary circuit prior to next stage of surgery was more frequent in those undergoing transcatheter intervention, reaching statistical significance by logrank (p = 0.012).

In conclusion, within this work we provide further evidence of the safety and efficacy of transition from a primary surgical to primary transcatheter palliation pathway in infants with insufficient pulmonary blood supply.

A 14-year-old female patient underwent surgical treatment of multiple atrial septal defects associated with unroofed coronary sinus and pulmonary valvar stenosis. One of the defects was that of the superior oval fossa and the other a large ellipsoidal defect positioned inferior to the inferior rim of the oval fossa. The patient underwent primary closure of the defects with a favorable result. To the best of our knowledge, this is the first surgical experience of an unusual atrial septal defect or the vestibular defect.

Radiofrequency ablation is the first-line treatment for arrhythmias with high success and low complication rates. Skin burns have been reported rarely after electrophysiological procedures, especially procedures in which higher-power energy is used and multiple ablations are performed. Here, we report a case of skin burn that developed after radiofrequency ablation for ventricular tachycardia originating from the right ventricular outflow tract.

Amongst all primary cardiac tumors, myxomas are the commonest, and their commonest site of origin is the left atrium. Myxomas originating in the left ventricle are rare. When seen, they usually present with a history of systemic embolisation and/or syncopal episodes, with constitutional symptoms being absent. We report here a child with left ventricular myxoma who presented with a prolonged febrile illness.

An indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m2 is a quantitative criterion for the diagnosis of Ebstein’s anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebstein’s anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebstein’s anomaly and 66 with pulmonary atresia with intact ventricular septum, were enrolled. Diagnosis of each anomaly was based on typical morphologic features. Echocardiographic variables, including the offset distance of the tricuspid septal leaflet, were measured via an offline analysis of images recorded before 1 month of age. The offset distance of the tricuspid septal leaflet was indexed by the body surface area, and the indexed offset distances in the Ebstein’s anomaly and pulmonary atresia with intact ventricular septum groups were 34.2 mm/m2 (7.1–119.1 mm/m2) and 7.2 mm/m2 (0.0–25.6 mm/m2), respectively. The indexed offset distance was ⩾8 mm/m2 in 29 (43.9%) of the patients with pulmonary atresia with intact ventricular septum; clinical and echocardiographic characteristics were comparable between these 29 patients and the remaining 37 patients with pulmonary atresia with intact ventricular septum. When an indexed offset distance ⩾8 mm/m2 was applied as a cut-off for the diagnosis of Ebstein’s anomaly, the sensitivity was 0.963 and the specificity was 0.561. In conclusion, indexed offset distance ⩾8 mm/m2 cannot be used as a cut-off for the diagnosis of complicated Ebstein’s anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

Paediatric cardiomyopathy is a progressive and often lethal disorder and the most common cause of heart failure in children. Despite their severe outcomes, their genetic etiology is still poorly characterised. The current study aimed at uncovering the genetic background of idiopathic primary hypertrophic cardiomyopathy in a cohort of Egyptian children using targeted next-generation sequencing. The study included 24 patients (15 males and 9 females) presented to the cardiomyopathy clinic of Cairo University Children’s Hospital with a median age of 2.75 (0.5–14) years. Consanguinity was positive in 62.5% of patients. A family history of hypertrophic cardiomyopathy was present in 20.8% of patients. Ten rare variants were detected in eight patients; two pathogenic variants (8.3%) in MBPC3 and MYH7, and eight variants of uncertain significance in MYBPC3, TTN, VCL, MYL2, CSRP3, and RBM20.

Here, we report on the first national study in Egypt that analysed sarcomeric and non-sarcomeric variants in a cohort of idiopathic paediatric hypertrophic cardiomyopathy patients using next-generation sequencing. The current pilot study suggests that paediatric hypertrophic cardiomyopathy in Egypt might have a particular genetic background, especially with the high burden of consanguinity. Including the genetic testing in the routine diagnostic service is important for a better understanding of the pathophysiology of the disease, proper patient management, and at-risk detection. Genome-wide tests (whole exome/genome sequencing) might be better than the targeted sequencing approach to test primary hypertrophic cardiomyopathy patients in addition to its ability for the identification of novel genetic causes.

A complex malformation of the heart was diagnosed by fetal echocardiography at 27 weeks of gestation. The study of the relationship of the abdominal great vessels, showing the inferior caval vein anterior and to the left of the aorta, made possible the diagnosis of right isomerism. This inferential guide to the arrangement of the thoraco abdominal organs proves to be as valid in the fetus as it is in the neonate and child. The examination of the abdominal great vessels as part of the fetal scan is of great value, enabling us to counsel mothers appropriately in the light of the poor prognosis of neonates with isomerism of atrial appendages and complex intracardiac lesions.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is an inherited cardiomyopathy characterised by ventricular arrhythmias and an increased risk of sudden cardiac death. Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnosis is based on criteria that take into account electrical and structural cardiac abnormalities, as well as mutation analysis. Appropriate pharmacological therapy and the prevention of sudden death with implantable defibrillators are important in the management of these patients. Exercise is considered an important environmental factor for the development and progression of the disease.

A 16-year-old girl with history of treated congenital mitral valve disease and signs of respiratory infection was admitted to our paediatric cardiology department. She was tested positive for severe acute respiratory syndrome coronavirus 2. Despite her severe pre-existing cardiac conditions with pulmonary hypertension, atrial arrhythmias and mitral valve stenosis, the infection did not lead to any cardiac or pulmonary deterioration. In adults, cardiac co-morbidities are known risk factors for a severe course of coronavirus disease 2019 infections. This case illustrates that in children even severe cardiac disease is not necessarily associated with a severe course of coronavirus disease 2019.

Coronary ostial atresia seen with pulmonary atresia and coronary-cameral fistulae or, more rarely, in isolation manifested as left main coronary artery atresia, is well described. We describe the clinical course and post-mortem findings in a neonate who suffered a fatal cardiac arrest and was found to have congenital absence of both coronary ostia in a single/common coronary system.

We present the case studies of two adult patients with tetralogy of Fallot who were scheduled for surgery. After addressing the right ventricular outflow tract obstruction, the aorta was opened and the ventricular septal defect was approached in a straightforward manner as it was located just under the overriding aortic valve. The second patient presented with was a situs inversus, dextroapex Fallot. In this setting, the aortic approach simplified the repair expeditiously. After 2 years, both patients are in New York Heart Association class I, with no residual ventricular septal defect, no aortic regurgitation, and complete relief of right ventricular outflow tract obstruction.

Right ventricular dysfunction is known to occur after the first stage of the Norwood sequence for treatment of patients with hypoplasia of the left heart. In a subset of patients, such ventricular dysfunction occurs without a specific anatomical cause. We describe two such cases with severe right ventricular dysfunction. In both cases, magnetic resonance imaging was used accurately to measure ventricular function and assess the arterial trunks. In both cases, cardiac transplantation was considered, but right ventricular function improved without invasive management. Transient right ventricular dysfunction in these cases may be due to the reduced ability of the right ventricle to adapt to the systemic vasculature. The improvement in ventricular function in these two cases may be due to delayed adaptation.

Between 1981 and 1990, 765 symptomatic neonates with major congenital heart malformations were admitted into the Grantham Hospital. This represented an incidence of 10 per 10,000 live births for Hong Kong. The figure was comparable to those reported for Caucasians. Among the 744 Oriental neonates, obstruction of the pulmonary outflow tract occurred most frequently (281, 37.8%), followed by left ventricular outflow tract obstruction (169, 22.7%), left-to-right shunting (115, 15.5%), complete transposition (92, 12.4%), common mixing situations (62,8.3%), and miscellaneous causes (25, 3.3%). When compared with the available reports from the West, Chinese neonates had a high preference for pulmonary outflow tract obstruction (p<0.005), especially the anomaly of pulmonary atresia and intact ventricular septum. This correlated well with cyanosis as the commonest neonatal presentation (64%). Contrary to previous reports that aortic coarctation was rare among Orientals, this abnormality was observed frequently in our study. The rare occurrence of critical aortic valvar stenosis among Chinese, however, was supported by our present analysis. Other lesions, such as left-to-right shunting and complete transposition, showed no significant racial difference in the frequency of occurrence. Such knowledge concerning the pattern of congenital heart disease amongst Oriental neonates can facilitate early diagnosis and timely referral of babies to the appropriate center for management.