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The alarming onset of some cases of myocarditis and pericarditis following the administration of Pfizer–BioNTech and Moderna COVID-19 mRNA-based vaccines in adolescent males has recently been highlighted. All occurred after the second dose of the vaccine. Fortunately, none of patients were critically ill and each was discharged home. Owing to the possible link between these cases and vaccine administration, the US and European health regulators decided to continue to investigate the potential causal relationship between COVID-19 mRNA vaccines and myocarditis. In any case, none of the patients fulfilled the criteria for multi-system inflammatory syndrome or Kawasaki-like disease and there was no evidence of acute SARS-CoV-2 infection.

Currently, the world is coping with the COVID-19 pandemic with a few vaccines. So far, the European Medicine Agency has approved four of them. However, following widespread vaccination with the recombinant adenoviral vector-based Oxford-AstraZeneca vaccine, available only in the United Kingdom and Europe, many concerns have emerged, especially the report of several cases of the otherwise rare cerebral sinus vein thrombosis and splanchnic vein thrombosis. The onset of thrombosis particularly at these unusual sites, about 5--14 days after vaccination, along with thrombocytopenia and other specific blood test abnormalities, are the main features of the vaccine side effects. The acronym vaccine-induced prothrombotic immune thrombocytopenia (VIPIT) has been coined to name this new condition, with the aim of highlighting the difference from the classic heparin-induced thrombocytopenia (HIT). VIPIT seems to primarily affect young to middle-aged women. For this reason, the vaccine administration has been stopped or limited in a few European countries. Coagulopathy induced by the Oxford-AstraZeneca vaccine (and probably by Janssen/Johnson & Johnson vaccine as well in the USA) is likely related to the use of recombinant vector DNA adenovirus, as experimentally proven in animal models. Conversely, Pfizer and Moderna vaccines use mRNA vectors. All vaccine-induced thrombotic events should be treated with a nonheparin anticoagulant. As the condition has some similarities with HIT, patients should not receive any heparin or platelet transfusion, as these treatments may potentially worsen the clinical course. Aspirin has limited rational use in this setting and is not currently recommended. Intravenous immunoglobulins may represent another potential treatment, but, most importantly, clinicians need to be aware of this new unusual postvaccination syndrome.

Since its outbreak in China at the end of 2019, the new coronavirus disease (COVID-19) was characterized by both easy spreading and high mortality. The latter proved to be way more elevated in the North of Italy -with a peak of 18.4% in region Lombardia and even 31% in the city of Bergamo and surrounding county- than in the rest of the world. In an attempt to conceptualize the reasons for such a dramatic situation, four key elements have been identified: COVID-19 itself, old age, lung disease, and heart failure. Their harmful combination has been named “The deadly quartet”. The underlying risk factors, among which a lot of them are distinctive features of the population in northern Italy, have been summarized as “unmodifiable”, “partially modifiable”, and “modifiable”, for the sake of clarity. Up-to-date scientific evidence in this field has been described in the form of a narrative and easy-to-read review.

Abstract: Familial hypercholesterolemia (FH) is a genetic disease, the underlying cause of which is represented by mutations capable of influencing the metabolism of low-density lipoproteins (LDL). The distinguishing characteristic of FH is increased LDL cholesterol blood levels since birth which trigger early development of atherosclerosis-related diseases. Diagnosis of FH is frequently either missed or made with a considerable delay. Prompt identification of the disease is pivotal in implementing early prevention measures. Safe and effective drugs have been approved for use in children and adolescents, with statins, with or without ezetimibe, representing first-line therapy. At times, however, these medications may not be sufficient to achieve the therapeutic target, particularly in homozygous FH patients. In such cases, lipoprotein apheresis, which has proved to be safe and efficient, is strongly suggested. New drugs still at the investigational stage may represent a promising and personalised therapy. The lowering of cholesterol levels in childhood hampers the formation of arterial atherosclerotic plaques, thus reducing cardiovascular events later in life. Accordingly, early detection, diagnosis, and therapy in FH subjects are priority aims.

COronavirus Infectious Disease which started in 2019 (COVID‐19) usually presents with the signs and symptoms of pneumonia. However, a growing number of recent reports highlight the fact that the infection may be by far more than only a respiratory disease. There is evidence of an increased thromboembolic risk in COVID‐19 patients, with a variety of manifestations in terms of ischemic stroke, deep vein thrombosis, acute pulmonary embolism, acute myocardial infarction, systemic arterial embolism, and placental thrombosis. The German physician Rudolph Virchow, about two centuries ago, described three pivotal factors contributing together to thromboembolic risk: endothelial injury, hypercoagulability, and blood stasis. COVID‐19‐associated hypercoagulability is unique and distinctive, and has its own features involving the immune system. Many of the drugs proposed and currently undergoing evaluation for the treatment of COVID‐19 have one or more of the Virchow's triad elements as a target. The three factors outlined by Virchow are still able to explain the venous and arterial hypercoagulable state in the dramatic COVID‐19 setting. Nowadays, we have decidedly more sophisticated diagnostic tools than Virchow had, but many of the challenges that we are facing are the same as Virchow faced in the 19th century.

Subjects formerly born preterm subsequently develop arterial - particularly isolated systolic- hypertension more frequently than their peers born at term. : Numerous factors may influence this predisposition, including an incomplete nephrogenesis, implying the presence of kidneys with a reduced number of nephrons and consequent reduction in haematic filtration, increased sodium absorption and activation of renin-angiotensin-aldosterone system, increased arterial rigidity produced by an elastin deficiency previously observed in anatomic specimens of human immature aorta, and reduced endothelial nitric oxide excretion, due to high blood levels of ADMA, a strong direct inhibitor of nitric oxide that exerts a vasoconstrictor effect. : Other possible factors (i.e. excretion of neuroendocrine compounds) may also be implicated. The aim of this paper was to review all possible mechanisms involved in the observed increase in blood pressure in individuals who had been born preterm and/or with intrauterine growth restriction. The outlook for new and promising laboratory techniques capable of identifying alterations in the metabolic pathways regulating blood pressure levels, such as metabolomics, is also provided.

A decline in sports activities among children and adolescents was noted during the stay-at-home restrictions imposed by COVID-19. With the easing of restrictions, physical activities are being resumed.A data search was conducted to identify the role of parents in resuming sporting activities, the risks and benefits of doing so, the physical examination to be conducted prior to physical activity, the existence of guidelines/protocols for return to sports and physical activity, the role of comorbidities in influencing the restart of the same.Parents should consult the child's physician prior to allowing he/she to resume physical exercise. In preparation for this, a careful physical examination should be performed. Children with COVID-19, even if asymptomatic, should not resume any physical activity until at least 10 days after testing positive. Those with moderate or severe COVID-19 should be referred for cardiological evaluation. The level of activity should be resumed gradually, as per the GRTP protocol. Athletes with a positive COVID-19 test in the presence of concomitant medical conditions (diabetes, cardiovascular or respiratory or renal disease) should undergo medical evaluation prior to adhering to a GRTP. Those with complicated COVID19 infection or long Covid may need additional investigations.While there is no doubt that it will bring multiple benefits in terms of general health, returning to play sport should be gradual and preceded by an accurate physical examination in those young subjects previously affected by the coronavirus disease, especially when their heart and/or lungs and/or kidneys were affected.

Background: right ventricular (RV) systolic dysfunction is associated with poor outcomes in Tetralogy of Fallot (ToF) patients. Conversely, the influence of diastolic dysfunction in this setting is poorly known. In addition, evaluation of RV diastolic function by cardiac MRI is rarely performed.Materials and methods: twenty-four surgically treated ToF patients were enrolled in the study (54% males; median age at study: 28 (18–50) years; median age at surgery: 1.9 (0.4–8.2) years. They were studied by echocardiography to assess RV diastolic function in terms of traditional and TDI-derived parameters. At cardiac MRI, RV diastolic function was assessed by using phase-contrast analysis of flow through the tricuspid valve in short axis view. Diastolic dysfunction was graded as impaired relaxation, pseudo-normal, or restrictive physiology.Results: fifteen (62.5%) ToF subjects had echocardiographic evidence of diastolic dysfunction and eleven (45.8%) at cardiac MRI. Statistically significant correlation between echocardiographic and cardiac MRI parameters of diastolic dysfunction was weak (p

‘An aspirin a day keeps the heart attack away’, is this also true in the prevention of thrombotic events associated with COVID-19? The rising COVID-19 pandemic has led to much work in the understanding of the pathophysiology of the disease. COVID-19 infection is thought to be an endothelial disease. Supporting this concept, it has been suggested that infection with SARS-CoV-2, the aetiological agent for COVID-19 infection, leads to a state mimicking Virchow’s triad, that is, vascular endothelial injury, blood stasis and clotting in concert with systemic inflammation resulting in systemic thrombosis. In keeping with this concept, moderately and critically ill patients with COVID-19 have been found to have thrombotic and thromboembolic events during the acute and convalescent state. Accordingly, there has been much interest in treating patients with COVID-19 with anticoagulants. In an analysis of several large series of critically ill patients in multiple studies (534 therapeutic anticoagulation, 564 usual care thromboprophylaxis), therapeutic anticoagulation did not improve survival or the number of days free of cardiovascular or respiratory organ support. On the other hand, among 2244 non-critically ill patients (1190 therapeutic anticoagulation, 1054 thromboembolic prophylaxis), therapeutic anticoagulation improved rates of hospital survival and reduced the use of cardiorespiratory organ support.1 Since there is intense platelet activation secondary to endothelial injury and inflammation, there is …

High blood pressure in pediatric age is meeting a growing interest owing to the possible aftermaths in adult age in terms of public health. In fact, children and adolescents with high blood pressure values are likely to become hypertensive adults, thus developing cardiovascular diseases. Over the last decade, numerous studies have been conducted in this field. That is the reason why the American Academy of Pediatrics in 2017 published un update on the previous recommendations of the United States Fourth Working Group on pediatric blood pressure. This update includes a new classification of hypertension, the endorsement of the 24-hour ambulatory blood pressure monitoring and the reduction in the blood pressure target for both chronic kidney disease and non-chronic kidney disease hypertensive children. This review discusses strengths and weaknesses of the recent practical guidelines of the American Academy of Pediatrics.

Individuals with the highest risk for adverse outcomes of COVID-19 should be prioritized by the vaccine allocation policies. We have conducted a literature review of published studies, which comprehend congenital heart disease (CHD) and COVID-19, in order to present the overall evidences of both exposure and clinical risk of patients with adult congenital heart disease (ACHD) and to propose a risk profile schema for those patients to be incorporated into vaccine distribution decisions.

At the beginning of coronavirus disease 2019 (COVID-19) children seemed to be less affected and with milder symptoms than adults. Afterward, however, a warning was released regarding the possible association between COVID-19 and Kawasaki disease (KD) or Kawasaki-like disease. Thereafter, labels of Paediatric Inflammatory Multisystem Syndrome Temporally associated with SARS-CoV-2 (PIMS-TS) in Europe and Multisystem Inflammatory Syndrome in Children (MIS-C) in the USA were coined to refer to this new disease entity. The reality is that PIMS-TS/MIS-C resembles certain KD complications such as toxic shock syndrome and macrophage activation syndrome than to classic KD. PIMS-TS/MIS-C and KD share the viral origin (however just supposed for KD) and consequent dysregulated innate immune system inflammatory reaction. PIMS-TS/MISC symptoms occur about 2-4 weeks after the onset of COVID-19 or having been exposed to somebody positive for COVID-19, rather than in the acute phase of the infection. Clinically PIMS-TS/MIS-C affects older children than KD and presents more often with gastrointestinal symptoms, shock, and multi-organ dysfunction. myocarditis is more common in PIMS-TS/MIS-C than coronary artery aneurysms formation seen in KD. There are also differences in laboratory tests and immunology responses in KD and PIMS-TS/MIS-C. Thus PIMS-TS/MIS-C seems to be a new and multifaceted entity, distinct from KD, notwithstanding some common features in both. The dysregulated innate immune system reaction is responsible for PIMS-TS/MIS-C onset and outcome. A multidisciplinary approach, involving paediatric intensivists, paediatric cardiologists, infectious disease specialists, immunologists, and rheumatologists, is needed for the treatment of these children.

ACE2 receptor has a broad expression pattern in the cellular membrane and provides a protective action against the development of cardiovascular diseases. Recently, this enzyme has become of extreme interest during the pandemic infection of COVID-19 (coronavirus disease 2019). This virus invades alveolar epithelium and cardiomyocytes using ACE2 as a transmembrane receptor. ACE2 is a counter-regulatory peptide that degrades Ang II into Ang 1-7, thereby attenuating the biological effects of the AT1 receptor. The binding between the spike protein of COVID-19 and the enzyme is crucial for the virus to enter the target cells, but whether an increase in ACE2 activity could facilitate the infection is not yet demonstrated. However, this aspect has raised many concerns about the use of ACE inhibitors or ARBs in infected patients or patients at risk of infection. It appears that cellular infection leads to a reduction in ACE2 expression and an increase in the activity of the Ang II--AT1 axis, which leads to the release of pro-inflammatory cytokines, ARDS, myocarditis, and hypercoagulability with the possibility of exacerbation of acute coronary syndrome, induction of pulmonary embolism, or appearance of disseminated intravascular coagulation. Therefore, ACE inhibitors or angiotensin receptor blocker drugs should be continued in infected patients, as their discontinuation can increase Ang II activity and induce injury to the lungs or cardiovascular system.

After the dramatic coronavirus outbreak at the end of 2019 in Wuhan, Hubei province, China, on 11 March 2020, a pandemic was declared by the WHO. Most countries worldwide imposed a quarantine or lockdown to their citizens, in an attempt to prevent uncontrolled infection from spreading. Historically, quarantine is the 40-day period of forced isolation to prevent the spread of an infectious disease. In this educational paper, a historical overview from the sacred temples of ancient Greece—the cradle of medicine—to modern hospitals, along with the conceive of healthcare systems, is provided. A few foods for thought as to the conflict between ethics in medicine and shortage of personnel and financial resources in the coronavirus disease 2019 era are offered as well.

Background. The pandemic of Novel Coronavirus Disease 2019 (COVID-19) is challenging, given the large number of hospitalized patients. Cardiovascular co-morbidities are linked to a higher mortality risk. Thus, patients with Congenital Heart Disease (CHD) might represent a high-risk population. Nevertheless, no data about them are available, yet. Hence, we conducted a nationwide survey to assess clinical characteristics and outcomes in patients with congenital heart disease affected by COVID-19. Methods and Results. This is a multi-centre, observational, nationwide survey, involving high-volume Italian CHD centres. COVID-19 diagnosis was defined as either &ldquo, clinically suspected&rdquo, or &ldquo, confirmed&rdquo, where a severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) test had been performed and was positive. Cardiovascular comorbidities were observed among adult patients&mdash, atrial fibrillation (seven, 9%), hypertension (five, 7%), obesity (seven, 9%) and diabetes (one, 1%)&mdash, but were absent among children. Cardiovascular complications were mainly observed in the &ldquo, COVID-19+ group, consisting of heart failure (9%), palpitations/arrhythmias (3%), stroke/TIA (3%) and pulmonary hypertension (3%). Cardiovascular symptoms such as chest pain (1%), myocardial injury (1%) and pericardial effusion (1%) were also recorded. On the contrary, CHD patients from the clinically suspected COVID-19 group presented no severe symptoms or complications. Conclusions. Despite previous reports pointing to a higher case-fatality rate among patients with cardiovascular co-morbidities, we observed a mild COVID-19 clinical course in our cohort of CHD patients. Although these results should be confirmed in larger cohorts to investigate the underlying mechanisms, the findings of low cardiovascular complications rates and no deaths are reassuring for CHD patients.

The recent outbreak of 2019 severe acute respiratory syndrome coronavirus-2 is having major repercussions on healthcare services provision in Italy and worldwide. Data suggest the virus has a strong impact on the cardiovascular system, and cardiac imaging will play an important role in patients affected by coronavirus disease-2019. Although paediatric patients are mildly affected, they represent a clear accelerator in spreading the virus, and healthcare workers are at higher risk of infection. The aim of this position paper is to provide clinical recommendation regarding the execution of imaging investigations for the cardiac diagnostic work-up of paediatric patients with suspected or confirmed infection.

The Fontan procedure is often the only definitive palliative surgical option for patients with a variety of complex CHD sharing in common, a single, dominant ventricle. In recent decades, imaging and therapeutic improvement have played a crucial role in those patients in whom many complications can hamper their life. After 50 years from the first procedure, heart transplantation remains the only definitive treatment for those with a failing Fontan circulation.

(1) Background: the impact of a series of laboratory parameters (haemoglobin, haematocrit, foetal haemoglobin, peripheral oxygen saturation, iron, transferrin, ferritin, and albumin) on perimembranous ventricular septal defects spontaneous healing was tested. (2) Methods: one hundred and seven patients were enrolled in the study (57% males; mean age 2.1 ± 0.4 years) and were subsequently subdivided into two groups: self-healing (n = 36) and in need of intervention (n = 71). Self-healing subjects were defined on the basis of an absence of residual shunts at colorDoppler across the previous defect. (3) Results: no statistically significant differences were reported in the size of perimembranous ventricular septal defects between the two groups (p = ns). Conversely, prevalence of anaemia was significantly higher in those requiring intervention than in the self-healing group (p < 0.03), while haemoglobin, iron, ferritin, and albumin levels were lower (p < 0.001, p < 0.05, p < 0.02, p < 0.007, respectively). In multivariable linear regression analysis, only haemoglobin and albumin were found to be associated with spontaneous closure (p < 0.005 and p < 0.02, respectively). In multiple logistic regression analysis, haemoglobin independently increased the probability of self-healing of perimembranous ventricular septal defects (p = 0.03). All patients needing an interventional closure of perimembranous ventricular septal defects presented with haemoglobin

Pulmonary hypertension (PH) is quite infrequent in pediatric age and its most common etiologies include idiopathic pulmonary arterial hypertension, PH related to congenital heart diseases, bronchopulmonary dysplasia (chronic lung disease), persistence of pulmonary hypertension of the newborn, and congenital diaphragmatic hernia. The developed for adult patients PH classification shows limitations when applied to pediatric subjects since the underlying causes are markedly different between the two ages. In 2011, the Pulmonary Vascular Research Institute Panama Task Force outlined the first specific pediatric pulmonary hypertensive vascular disease diagnostic classification, including 10 main categories and 109 subcategories, thus testifying PH complex pathophysiology during newborns/children growth and development. The unique, distinctive features of pediatric PH were recognized also during the fifth World Symposium on pulmonary hypertension in 2013 and then confirmed in the recent 2018 sixth World Symposium. For the sake of uniformity, an attempt to adapt the adult classification to pediatric patients was made. However, all these commendable classifications are very complex and maybe not of quick comprehension for clinicians. A clinical simpler and simplified method is now suggested, comprising only five groups: neonatal, cardiac, developmental, idiopathic, and syndromic PH. This approach is not aimed at replacing the already existing classifications but is mainly based on the kind of specialized physician (

A more in-depth, refined definition of pulmonary arterial hypertension in newborns is neededhttp://ow.ly/DQH530nDaLz

Aim To evaluate the effectiveness of glycoprotein (GP) IIb/IIIa receptor antagonists in patients with acute myocardial infarction (AMI) who have undergone unsuccessful thrombolysis.

The sequential chamber analysis and angiographic, hemodynamic and clinical features, including those seen on electrocardiography, are presented in 18 patients with a univentricular heart of right ventricular type. In all cases a main chamber with a right ventricular trabecular pattern received both (or the sole) atrioventricular (A-V) connections, and a rudimentary chamber with a left ventricular trabecular pattern receiving no A-V connection was also present. The 18 patients were seen between 1971 and 1979; 6 died and the diagnosis was confirmed at autopsy. Twelve patients had double inlet, four absent right and two absent left A-V connections. Electrocardiography revealed right ventricular hypertrophy in all, and 11 had a superior mean frontal QRS axis. Chest roentgenography disclosed dextrocardia in 7 and levocardia in 11. Of 12 infants presenting with a marked decrease in pulmonary flow, 10 presented as ill neonates with hypoxia and acidosis. Four infants with increased pulmonary flow presented before 8 weeks with heart failure and mild cyanosis. Two with moderate pulmonary stenosis had mild cyanosis, but were asymptomatic. All had associated cardiac anomalies, pulmonary stenosis and persistent ductus arteriosus being the most common. For accurate diagnosis, angiographic demonstration of a posterior rudimentary chamber in addition to the main chamber of right ventricular trabecular pattern is mandatory.

Three cases of anomalous aortic origin of the right pulmonary artery are reported. All patients presented with severe cardiac failure, two of them in the neonatal period. Clear visualization of the anomalous origin of the right pulmonary artery was obtained by cross-sectional echocardiography and the diagnosis was confirmed by cardiac catheterization. All patients underwent correction without the aid of cardiopulmonary bypass. The continuity between main and right pulmonary arteries was established by a polytetrafluoroethylene graft. One early death was due to bleeding and, probably, a pulmonary vasoconstriction crisis. In all patients the right ventricular pressure was systemic or suprasystemic before surgery and dropped to near normal in the operating room after correction. Two to five years after surgery the survivors are well. This rare, potentially lethal anomaly is amenable to correction; however, diagnosis should be followed by immediate surgical treatment.

In a series of 1036 routine electrocardiograms, 14 children, without clinical and echocardiographic evidence of heart disease, showed a left axis deviation in the standard electrocardiogram. Six children also had an incomplete right bundle branch block. Left axis deviation is defined as a mean frontal QRS complex axis being -30 degrees or less. The age varied from three months to 10 years, with a clear predominance of males (13/14). The unusual finding of left QRS axis deviation in normal children is emphasized.

Determination of the arrangement of the atrial chambers ("atrial situs") is the first step in sequential chamber analysis of complex congenital heart disease. Although bronchial pattern, as detected by chest X-ray, is an excellent guide for prediction of atrial arrangement, exceptions to this rule based on post-mortem examination have been described. We report a case in which discordance between the atrial arrangement (mirror-image) and bronchial morphology (usual) was diagnosed during life.

We performed retrograde pulmonary arteriography by means of a pulmonary venous wedge injection in 10 patients with no demonstrable intrapericardial pulmonary arteries by "conventional" angiographic techniques. In all cases but one, the procedure demonstrated the feasibility of a further operation. No complications were observed. Retrograde pulmonary arteriography is an important additional method for determining the existence of surgically accessible pulmonary arteries when other techniques have failed.

Dextrocardia can be defined as a heart in the right chest with the major axis to the right. This definition, however, conveys no information regarding the chamber arrangements and internal anatomy of the heart. Of 40 patients satisfying this definition in the files of the Brompton Hospital, 33 had angiocardiographic data adequate for complete analysis in terms of connections, relations, and morphology of cardiac segments. They form the subject of this report. There were 16 (48%) patients with situs solitus, 11 (33%) with situs inversus, and six (18%) with situs ambiguus. Of the cases of situs ambiguus, four exhibited laevoisomerism and two dextroisomerism. Of the 16 patients with situs solitus, six had two ventricles and 10 had univentricular hearts; two patients had concordant and three discordant ventriculoarterial connections, seven had double outlet ventricle, and four a single outlet heart. Of the 11 patients with situs inversus, nine had two ventricles and two a univentricular heart of right ventricular type; the arterial connection was concordant in two, discordant in two, double outlet in six, and single outlet in one. Of the six patients with situs ambiguus and laevo or dextroisomerism, four had two ventricles, and two univentricular hearts; the arterial connection was concordant in one, double outlet in three, and single outlet in two. Segmental analysis and the use of basic descriptive terms are essential to define the complex anatomy of such hearts.