Patients with cyanotic congenital cardiac disease often develop major aortopulmonary collaterals. Vascular endothelial growth factor is a key promoter of angiogenesis. Its soluble receptor-1 acts as a potent antagonist. We studied 30 infants with cyanotic congenital cardiac disease and 27 infants with acyanotic congenital cardiac disease. Central venous plasma vascular endothelial growth factor and soluble vascular endothelial growth factor receptor-1 levels were measured before, and 24 and 96 hours after surgery. There was no difference between plasma vascular endothelial growth factor levels in infants with cyanotic and those with acyanotic congenital cardiac disease. In cyanotic infants, the soluble vascular endothelial growth factor receptor-1 levels tended to be higher than in the acyanotic infants. In conclusion, there is no significant difference in the plasma levels of vascular endothelial growth factor and its soluble receptor-1 between infants with cyanotic and those with acyanotic congenital cardiac disease.

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.

Right atrial appendage aneurysm is an extremely rare congenital malformation with unknown aetiology. The most common potential complication is atrial arrhythmias including atrial flutter, atrial fibrillation, and atrial tachycardia. These arrhythmias are usually refractory to medication therapy. Radiofrequency catheter ablation has poor efficacy with low success rate and high recurrence rate. Aneurysm resection is the recommended treatment with satisfactory efficacy. We report a child with chaotic atrial tachycardia due to giant right atrial appendage aneurysm who was successfully treated by aneurysm resection.

We describe the clinical history of a nine years old girl with Shone's syndrome. She underwent balloon angioplasty of the aortic coarctation in infancy, and later developed severe sub-aortic stenosis and moderate mitral valvar stenosis. The mitral valve was therefore replaced with a mechanical prosthesis, and the sub-aortic shelf was resected. Immediately after the operation, she developed signs of myocardial ischemia. Coronary angiography showed compression of the middle part of the circumflex artery by the mechanical prosthesis, the obstructed segment being successfully dilated using a coronary arterial stent.

Transcatheter closure of ventricular septal defects is considered first-line therapy when anatomically appropriate but is often challenged by proximity to the conduction system in perimembranous defects, or irregular defect shape, especially residual defects that may remain post-operatively. Advancements in device design, however, have allowed for significant improvements in deployment techniques and overall safety. Here we describe the first use of the Lifetech Konar-multifunction™ occluder device in North America, and our specific use of this device to close complex post-operative muscular and a perimembranous-ventricular septal defects in the same patient.

In the early 1980s cardiology was dominated by “triple threat individuals” – doctors who were the very best clinicians, very best researchers, and very best teachers. This type of individual usually became the director of the division. Now, however, the amount of knowledge and specialisation in each of these three areas of expertise has exploded. It is not certain that triple threat individuals can or do exist today. Instead, we need to be aiming higher, for a “quintuple threat profession”. Not only do we need expertise in clinical care, teaching, and research, but also we now require business leadership and alignment. No single person can be expert in all five of these areas. In paediatric cardiology we need to invest in the right people. Our new “quintuple threat profession” needs to match the expertise and passion of the right people with our current and future needs. Going forward, we should focus on leadership, teams, and alignment. We need to identify, develop, and empower leaders at all levels within our profession, including physicians, nurses, and hospital administrators. Ultimately, we need a profession that remains clinically led, but professionally managed. The second step of the formula is the development of successful teams, and teamwork. Successful “team behaviour” is not a part of traditional medical education. The third step is alignment. Alignment can be defined as representing the degree to which physicians and organisations, such as hospitals, acting out of enlightened self-interest, operate inside a common vision, mutual goals, and the acceptance of a shared destiny. Imagine the benefits to our profession and our kids if we can provide a new level of leadership, teamwork, and alignment going forward.

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.

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.

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.

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.

Double outlet left ventricle is an extremely rare anomaly. Until recently, the diagnosis was usually established by angiography or at postmortem. There are only a few reports describing the echocar-diographic findings in this lesion, and as far as we know, no report showing the anatomy as well as the velocity and pattern of flow by color Doppler echocardiography. The patient reported here underwent surgery at the age of four years, when an aortic homograft was placed between the right ventricle and the pulmonary trunk. This biventricular repair had to be changed into a Fontan-type procedure, 15 years later since the hypoplastic right ventricle did not grow adequately.

In 2004, practice guidelines for the management of heart failure in children by Rosenthal and colleagues were published in conjunction with the International Society for Heart and Lung Transplantation. These guidelines have not been updated or reviewed since that time. In general, there has been considerable controversy as to the utility and purpose of clinical practice guidelines, but there is general recognition that the relentless progress of medicine leads to the progressive irrelevance of clinical practice guidelines that do not undergo periodic review and updating. Paediatrics and paediatric cardiology, in particular, have had comparatively minimal participation in the clinical practice guidelines realm. As a result, most clinical practice guidelines either specifically exclude paediatrics from consideration, as has been the case for the guidelines related to cardiac failure in adults, or else involve clinical practice guidelines committees that include one or two paediatric cardiologists and produce guidelines that cannot reasonably be considered a consensus paediatric opinion. These circumstances raise a legitimate question as to whether the International Society for Heart and Lung Transplantation paediatric heart failure guidelines should be re-reviewed.

The time, effort, and expense involved in producing clinical practice guidelines should be considered before recommending an update to the International Society for Heart and Lung Transplantation Paediatric Heart Failure guidelines. There are specific areas of rapid change in the evaluation and management of heart failure in children that are undoubtedly worthy of updating. These domains include areas such as use of serum and imaging biomarkers, wearable and implantable monitoring devices, and acute heart failure management and mechanical circulatory support. At the time the International Society for Heart and Lung Transplantation guidelines were published, echocardiographic tissue Doppler, 3 dimensional imaging, and strain and strain rate were either novel or non-existent and have now moved into the main stream. Cardiac magnetic resonance imaging (MRI) had very limited availability, and since that time imaging and assessment of myocardial iron content, delayed gadolinium enhancement, and extracellular volume have moved into the mainstream. The only devices discussed in the International Society for Heart and Lung Transplantation guidelines were extracorporeal membrane oxygenators, pacemakers, and defibrillators. Since that time, ventricular assist devices have become mainstream. Despite the relative lack of randomised controlled trials in paediatric heart failure, advances continue to occur. These advances warrant implementation of an update and review process, something that is best done under the auspices of the national and international cardiology societies. A joint activity that includes the International Society for Heart and Lung Transplantation, American College of Cardiology/American Heart Association, the Association for European Paediatric and Congenital Cardiology (AEPC), European Society of Cardiology, Canadian Cardiovascular Society, and others will have more credibility than independent efforts by any of these organisations.