During the initial surge of the COVID-19 pandemic in the spring and summer of 2020, paediatric heart centres were forced to rapidly alter the way patient care was provided to minimise interruption to patient care as well as exposure to the virus. In this survey-based descriptive study, we characterise changes that occurred within paediatric cardiology practices across the United States and described provider experience and attitudes towards these changes during the pandemic. Common changes that were implemented included decreased numbers of procedures, limiting visitors and shifting towards telemedicine encounters. The information obtained from this survey may be useful in guiding and standardising responses to future public health crises.

Intrapericardial teratoma is a rare but recognised cause of respiratory distress in neonates. Patients often present with the compressive effects of the mass within the thorax. Prompt diagnosis should be followed swiftly by surgical resection. We report an unusual case of intrapericardial teratoma in a neonate presenting with collapse of the lung which was successfully treated by surgery.

In the spectrum of mitral valve anomalies, unguarded mitral orifice is an exceedingly rare malformation, with only four cases described in the current literature. All previously reported cases have been associated with discordant atrioventricular connections. We describe the first known case of unguarded mitral valve orifice, in the setting of atrioventricular concordance, in a newborn with hypoplastic left heart syndrome.

The present study assessed the usefulness of brain natriuretic peptide in evaluating the cardiovascular status in children and grown-ups with congenital or acquired disease affected by chronic cardiac failure. Brain natriuretic peptide should be included in the risk stratification of children with dilated cardiomyopathy. In patients with congenital heart defects, it is useful to tailor the optimal medical strategy to individual patients.

Neonatal cardiac hypertrophy associated with diabetic pregnancy is transient and regresses naturally, but is associated with increased morbidity and mortality.

This study was undertaken to analyse the changes in expression of 5 cardiac genes, including atrial natriuretic peptide, α- and β-myosin heavy chain, and cardiac and skeletal α-actin genes, using a rat neonatal model, in which cardiac hypertrophy was induced via maternal diabetes.

In the hypertrophied left ventricle of neonates from diabetic mothers, the levels of mRNA from all the above genes except skeletal α-actin were increased by between 1.8- and 12-fold compared with the controls at birth (p < 0.05). In the first 28 days, the level of mRNA for α-myosin heavy chain increased slightly, while that for atrial natriuretic peptide and β-myosin heavy chain decreased continuously similar to the controls, but at a significantly faster rate. No significant difference between the two groups of neonates was observed in all 5 genes after 1 month, indicating complete regression.

Expression of 5 cardiac genes in the neonatal cardiac hypertrophy was characterised in both hypertrophic and regressive phases. Hypertrophic regression provides a unique model for the testing of new drugs or genetic modifying factors in cardiac hypertrophy.

As patients with congenital cardiac malformations increasingly survive therapeutic interventions, and our understanding of primary electrical diseases increases, the landscape of paediatric and congenital electrophysiology is expanding. Electrophysiologic abnormalities, both tachycardic and bradycardic, are commonly seen in post-operative patients with congenital cardiac disease, as well as being part of the natural history of congenital malformations and cardiomyopathies. Disturbances of rhythm represent an increasing morbidity in this population, and therapies using devices in the form of pacemakers and implantable cardioverter-defibrillators have taken on a correspondingly important role. In this review, we discuss some of the key features and recent advances in pacing for bradycardia, resynchronization pacing, anti-tachycardia pacing, and use of implantable cardioverter-defibrillators.

All newborn infants have limited pulmonary reserve compared with older children. This puts them at increased risk of respiratory complications, such as those associated with infection by the respiratory syncytial virus. Young children with congenital cardiac disease are particularly likely to suffer severe disease related to infection by the virus. In these children, the extreme vulnerability of the lung to pulmonary oedema is compounded by the additional burden caused by the respiratory syncytial virus.

In addition to the well-documented acute pulmonary effects of infection with the respiratory syncytial virus, there may also be consequent long-term respiratory morbidity. Clinical studies have shown that infection by the virus in infancy is associated with a higher risk of developing subsequent bronchial obstructive disease. Much debate surrounds the mechanisms underlying this association. It is thought that a combined immunological and neurogenic response mechanism is likely. Prevention of severe respiratory disease in infants and young children with congenital heart disease due to infection by the virus may, therefore, offer both immediate and long-term benefits. Indeed, an increasing body of evidence supports this hypothesis, indicating a clinical rationale for prophylaxis against the virus in infancy, in order to reduce the chance of developing reactive airways disease and asthma in later life.

We present a case of a 2-year-old girl who presented with respiratory distress and umbilical abscess, and was found to have an inferior sinus venosus defect, malposition of the atrial septum primum, absent septum secundum, and anomalous drainage of the right upper and lower pulmonary veins to the right atrium.

Congenital atresia of the left coronary artery is a very rare condition. We report a case of a 13-year-old boy who was followed-up at our outpatient clinic from 6 months of age because of heart murmur. He had no symptoms for more than 10 years, and his left ventricular contraction was normal. Exercise electrocardiogram was performed at 7 and 10 years of age, and ST depression was not observed. He presented with chest pain and syncope on exertion at the ages of 11 and 12 years, respectively, and ST depression on exercise electrocardiogram was detected for the first time at 13 years of age. Cardiac echocardiography showed a high-choic antero-lateral papillary muscle of the mitral valve, prolapse of the mitral anterior leaflet, and mild mitral valve regurgitation. Coronary angiography did not demonstrate antegrade left coronary flow or dilation of the right coronary artery. We performed coronary artery bypass grafting using the left internal thoracic artery. Postoperatively, the ST depression resolved, and his exercise tolerability improved.

Objective: Medline classifies publications as clinical trials, randomised control trials, meta-analyses, practice guidelines, reviews, case reports, editorials, and letters. We tested the hypothesis that cardiology-related publications have increased with a shift in the type of publications over the past 10 years by age category. Methods: To retrieve from Medline the cardiology articles, we used the keyword “heart disease”, but limited the search to articles in English from 2000 to 2009. We repeated the search using one limit according to the publication type and using age tags. We used regression analysis to determine the effect of the year of publication on the number of publications of each type. Results: During the 10-year period, Medline registered 152,849 cardiology articles, doubling from 10,452 in 2000 to 20,841 in 2009, of which 8.5% were tagged as both paediatric and adult. There was a linear increase in the number over the study period in the total number of publications and in all categories, except for practice guidelines. There was almost a twofold increase in adult and neonatal articles, but ∼70% in paediatric articles. The rate of increase was 66% for randomised control trials, 73% for clinical trials, 124% for meta-analyses, 117% for editorials, 36% for reviews, and 103% for case reports. Practice guidelines remained very low, increasing significantly for paediatric and neonatal articles. Conclusions: There was a substantial increase in cardiology articles over the past 10 years, being greater for adult and neonatal articles compared with paediatric articles. The increase varied according to the type of article.

Takayasu arteritis is a chronic, idiopathic, granulomatous vasculitis involving the aorta, its major branches, and occasionally the pulmonary arteries. Although rare in children, it is the third most common vasculitis in the paediatric population. Although aneurysmal disease has been reported in adults with Takayasu arteritis, it is a rare entity in children. We present the case of a 10-year-old boy with a giant ascending and arch aneurysm that necessitated follow-up surgery for a new aneurysm and occlusive disease. This is also the first published case involving endovascular aortic graft placement for the management of vascular sequela of Takayasu arteritis in a child.

Two neonates are described presenting in severe cardiac failure within a few hours of birth. Echocardiography and the demonstration by color-coded Doppler of retrograde diastolic flow in the proximal descending aorta suggested the presence of large cerebral arteriovenous malformations. Such lesions were demonstrated by cranial ultrasound. The early diagnosis permitted early intervention, albeit with less than satisfactory outcome.

A ventricular septal defect is, almost always, an integral part of double outlet right ventricle and has been classified into the subaortic, subpulmonary, doubly committed and non-committed varieties. This study was performed to correlate the cross-sectional imaging characteristics of such ventricular septal defect in double outlet right ventricles using pathological specimens. The extent and the orientation of the outlet septum were the most important in the differentiation of the four varieties of ventricular septal defect. In the subaortic variety, the outlet septum fused with the left anterior margin of the defect, this being marked by the anterior limb of the septomarginal trabeculation. In the subpulmonary variety, the outlet septum fused with the right posterior margin of the defect, this being the posterior limb of the septomarginal trabeculation. The outlet septum was vestigial in case with doubly committed defects. In those with non-committed defects, the defect was not shown in those images or sections which demonstrated the outlet septum.

Mitral valve stenosis caused by a discrete supravalvular membrane is a rare congenital malformation haemodynamically leading to significant mitral valve stenosis. When the supravalvular mitral stenosis consists of a discrete supravalvular membrane adherent to the mitral valve, it is usually not clearly detectable by routine echocardiography. We report about the typical echocardiographic finding in three young patients with this rare form of a discrete membranous supravalvular stenosis caused by a membrane adherent to the mitral valve. These cases present a typical echocardiographic feature in colour Doppler generated by the pathognomonic supramitral flow acceleration. Whereas typical supravalvular mitral stenosis caused by cor triatriatum or a clearly visible supravalvular ring is easily detectable by echocardiography, a discrete supravalvular membrane adjacent to the mitral valve leaflets resembling valvular mitral stenosis is difficult to differentiate by routine echocardiography. In our opinion, this colour phenomenon does resemble the visual impression of polar lights in the northern hemisphere; owing to its typical appearance, it may therefore be named as “Polar Light Sign”. This phenomenon may help to detect this anatomical entity by echocardiography in time and therefore improve the prognosis for repair.

A 24-year-old woman with a history of idiopathic dilated cardiomyopathy status post heart transplant gave birth to a healthy term female infant. At 5 months of age, the infant was diagnosed with severe left ventricular dysfunction with an ejection fraction of 18% and moderate non-compaction of the left ventricle. She received a heart transplant at 7 months of age. Familial dilated cardiomyopathy was diagnosed. Genetic testing revealed a likely pathogenic variant in the TPM1 gene. Fetal cardiac screening is critical for offspring of heart transplant recipients, especially when the reason for transplant was cardiomyopathy. Early genetic consultation and counselling is necessary for all heart transplant recipients, preferably prenatally. Postnatal screening of offspring is essential at birth, at 3-month intervals until 1 year of age, and then annually until the risk for familial cardiomyopathy is assessed.