A 9-year-old boy who was born with bicuspid aortic stenosis underwent two unsuccessful aortic valvuloplasty interventions, and by 2 years of age he developed restrictive cardiomyopathy caused by left ventricular endocardial fibroelastosis and diastolic dysfunction. The attending cardiologist referred the patient to a high-volume, high-profile congenital cardiac surgical programme 1000 miles away that has a team with considerable experience with left ventricular endocardial fibroelastosis resection and a reputation of achieving good results. Owing to problems with insurance coverage, the parents sought other options for the care of their child in their home state. Dr George Miller is a well-respected local congenital and paediatric cardiac surgeon with considerable experience with the Ross operation as well as with right ventricular endocardial fibroelastosis resection. When talking with Dr Miller, he implied that there is little difference between right ventricular endocardial fibroelastosis and left ventricular endocardial fibroelastosis resection, and stated that he would perform the operation with low mortality based on his overall experience. Dr Miller stated that the local institution could provide an equivalent surgical procedure with comparable outcomes, without the patient and family having to travel out of state. A fundamental dilemma that often arises in clinical surgical practice concerns the conduct of assessing and performing new procedures, especially in rare cases, for which the collective global experience is scant. Although Dr Miller has performed right ventricular endocardial fibroelastosis resection, this procedure differs from left ventricular endocardial fibroelastosis resection, and he cannot be sure that he will indeed be able to perform the procedure better than the high-volume surgeon. This ethical situation is best understood in terms of the principles of respect for patient autonomy, beneficence, non-maleficence, and justice. The tension between the imperatives of beneficence and the obligation to respect the autonomy of the patient by acting only with the patient’s best interest in mind is discussed.

Most children born with even the most critical forms of CHD are now surviving well into adulthood. However, with increased survival has come increased recognition of the diverse neurobehavioural and psychosocial challenges these children experience. Among these challenges are deficits in executive function skills, including inhibitory control, working memory, and cognitive flexibility. Over the past several years, whereas inhibitory control and working memory deficits have garnered particular attention among clinicians and interventionists, relatively less attention has been paid to cognitive flexibility. This is unfortunate given both the high prevalence of cognitive flexibility deficits observed in children and adolescents with critical CHD, and also the far-reaching relevance of cognitive flexibility in helping individuals achieve optimal quality of life across the lifespan. This paper reviews the construct of cognitive flexibility, including its definition, development, measurement, and neuroanatomical basis, provides a summary of how cognitive flexibility is affected by CHD, and offers evidence-based recommendations to systematically support the development of cognitive flexibility within the context of CHD.

Coronavirus disease 2019 causes respiratory and systemic disease and has led to a sudden epidemic affecting people of all ages. Patients with congenital heart disease represent a high-risk population. In this article, we present a newborn who required extracorporeal membrane oxygenation support for acute respiratory failure in the early postoperative period due to exposure to severe acute respiratory syndrome coronavirus 2 after aortic arch repair and ventricular septal defect closure. To the best of our knowledge, this patient represents the first neonatal case of severe acute respiratory syndrome coronavirus 2 infection after congenital heart surgery and is the youngest patient to need extracorporeal membrane oxygenation support.

We describe a neonate with DiGeorge syndrome undergoing diagnosis and successful repair of interrupted right aortic arch and origin of the left pulmonary artery from the aorta. We discuss a link between this lesion and persistence of a left fifth arch.

One of the most challenging applications of cardiopulmonary bypass is corrective cardiac surgery in the neonate. The small size and high metabolic demand of these patients require miniaturized but efficient equipment. Even with the most advanced components, the volume required to prime the perfusion circuit is typically more than twice the blood volume of a neonate. Neonates have limited cardiac and pulmonary reserves and, therefore, great care is required to preserve those organs that have often already been subjected to hypoxemia, congestive heart failure, or low cardiac output prior to surgery. There is a tendency toward extravascular movement of fluids in newborns subjected to bypass that can adversely affect outcome. Careful monitoring and precise management of perfusion are essential to a successful procedure and optimal recovery of these patients.

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.

Single-nucleotide polymorphisms in miRNA-machinery genes may alter the biogenesis of miRNAs affecting disease susceptibility. In this case–control study, we aimed to evaluate the impact of three single-nucleotide polymorphisms (DICER rs1057035, DROSHA rs10719, and XPO5 rs11077) and their combined effect in a genetic risk score model on congenital heart disease (CHD) risk. A total of 639 participants was recruited, including 125 patients with CHD (65 males; age 9.2 ± 10 years) and 514 healthy controls (289 males; age 15.8 ± 18 years). Genotyping of polymorphisms in miRNA-machinery genes was performed using a TaqMan®SNP genotyping assay. A genetic risk score was calculated by summing the number of risk alleles of selected single-nucleotide polymorphisms. There was a significantly increased risk of CHD in patients with XPO5 rs11077 CC genotype as compared to AC heterozygote and AA homozygote patients (ORadjusted = 1.7; 95% CI: 1.1–2.8; p = 0.018). A clear tendency to significance was also found for DROSHA rs10719 AA genotype and CHD risk for both codominant and recessive models (ORadjusted = 1.8; 95% CI: 0.91–3.8; p = 0.09 and ORadjusted = 1.9; 95% CI: 0.92–4; p = 0.08, respectively). The resulting genetic risk score predicted a 1.73 risk for CHD per risk allele (95% CI: 1.2–2.5; p = 0.002). Subjects in the top tertile of genetic risk score were estimated to have more than three-fold increased risk of CHD compared with those in the bottom tertile (ORadjusted = 3.52; 95% CI: 1.4–9; p = 0.009). Our findings show that the genetic variants in miRNA-machinery genes might participate in the development of CHD.

The problems that exist in maintaining a partnership in paediatric cardiac surgery are considerable. They relate to fairness in allocation of time for leisure, the apportioning of cases between the partners, internal competition between them, financial considerations, and promotion of the ego. In this review, we discuss our own experiences in maintaining a partnership over a period of more than 18 years, relating such a “virtuous partnership” to the writings of Plato and Aristotle, and setting it against the tenets of the ancient Greek polis.

The polis, or city state, came to prominence in ancient Greece during the golden age of Pericles, this period seeing the initial evolution of Western philosophy, as well as numerous other scientific, artistic and architectural advances. The concept of the polis was to create a natural association with its citizens that nurtured all that is best in people, at the same time defining their character. In this respect, according to Plato, the person and the polis are mirror images. Aristotle then expanded this notion to incorporate the various forms of friendship, which he pointed out last only as long as the interrelated pleasure survives.

Using these principles as the point of departure, we argue that cardiac surgeons should respect moral virtue in each other. Extending this process means that we should also respect, and celebrate, our relationships with affiliated physicians, nurses, perfusionists, administrators, and all concerned in the care of children with congenitally malformed hearts. In this way, we create a virtuous partnership for congenital cardiac surgery that promotes all that was good, as engendered in the ancient Greek polis.

As we extend these observations to the modern world, we discuss some of the features that have permitted us to work so well together. One of the most important is a summoning and unwritten tenet that greets us as we enter the operating room, namely “check your ego at the door”. The operative choice should always be dictated by discussion, citations of literature, considered opinions, and relevance to the particular patient. Continuity of care should lead inexorably to the paediatric intensive care unit, where collegial relationships should be maintained with all those working therein. We need to recognize that there are various “captains of the ship”, who must work in harmony so as to bring the best possible care to our patients. We always endeavour to empower others to act on our behalf, based on their experience and training.

Whether we have achieved our desired moral excellence, and produced the completed or perfected friendship, is for others to judge. From our stance, we believe we have created a favourable environment by hard work, unselfish attitudes, and celebration of our mutual accomplishments. We were not the first to forge this kind of professional association, and happily we will not be the last.

We describe a case of an 11-year-old boy who underwent orthotopic heart transplant for dilated cardiomyopathy. He developed a normocytic, normochromic anaemia with a low reticulocyte count 1 month after transplant. A bone marrow biopsy was performed, which showed a mildly hypocellular bone marrow with few red blood cell precursors with giant pro-erythroblasts indicative of a pure red cell aplasia. Parvovirus B19 polymerase chain reaction in the blood was positive 2 months after transplant. Intravenous immunoglobulin administration resulted in a resolution of the anaemia over several months. Unexplained pure red cell aplasia in immunosuppressed patients should alert one to the possibility of parvovirus B19 infection.

This review offers a critical-care perspective on the pathophysiology, monitoring, and management of acute heart failure syndromes in children. An in-depth understanding of the cardiovascular physiological disturbances in this population of patients is essential to correctly interpret clinical signs, symptoms and monitoring data, and to implement appropriate therapies. In this regard, the myocardial force–velocity relationship, the Frank–Starling mechanism, and pressure–volume loops are discussed. A variety of monitoring modalities are used to provide insight into the haemodynamic state, clinical trajectory, and response to treatment. Critical-care treatment of acute heart failure is based on the fundamental principles of optimising the delivery of oxygen and minimising metabolic demands. The former may be achieved by optimising systemic arterial oxygen content and the variables that determine cardiac output: heart rate and rhythm, preload, afterload, and contractility. Metabolic demands may be decreased by a number of ways including positive pressure ventilation, temperature control, and sedation. Mechanical circulatory support should be considered for refractory cases. In the near future, monitoring modalities may be improved by the capture and analysis of complex clinical data such as pressure waveforms and heart rate variability. Using predictive modelling and streaming analytics, these data may then be used to develop automated, real-time clinical decision support tools. Given the barriers to conducting multi-centre trials in this population of patients, the thoughtful analysis of data from multi-centre clinical registries and administrative databases will also likely have an impact on clinical practice.

We report the first case of double-barrelled aorta associated with tetralogy of Fallot and pulmonary atresia, and we discuss the difficulties in the terminology of this fascinating anomaly.

We present a unique case of unilateral lung agenesis and anomalous pulmonary venous return in a newborn with Goldenhar syndrome.

A young child presented with hepatomegaly, ascites and bradycardia in the setting of COVID-19. Permanent complete atrioventricular block and severe right heart failure were diagnosed. He was treated with surgical epicardial pacemaker implantation. This report is the first description of COVID-19-induced permanent complete atrioventricular block in a child.

The incidence of novel coronavirus disease-19 (nCoV-19) and its associated complications is higher in high-risk groups. In this article, we explain the symptoms and course of the disease and the treatment for an adult patient with CHD who has been infected with novel nCoV-19.