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Acute clinical manifestations of COVID-19 are generally less severe in childhood, however a proportion of them can develop a severe systemic hyperinflammatory syndrome after SARS-CoV-2 infection, known as the multisystem inflammatory syndrome (multisystem inflammatory syndrome in children, MIS-C). Cardiovascular manifestations in MIS-C are frequent (34-82%), including myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. The most affected cases can develop cardiogenic shock needing intensive care unit admission, inotropic support and sometimes even mechanical circulatory support. The elevation of myocardial necrosis markers, the frequently transient left ventricular systolic dysfunction and the presence of changes on magnetic resonance imaging, support the hypothesis of an immune-mediated post-viral pathogenesis similar to myocarditis. Although MIS-C shows excellent short-term survival, further studies are needed to demonstrate complete reversibility of residual subclinical heart damage.

Background: A multidisciplinary study group involving physicians and jurists was established to review and approve an informed consent about the most frequent interventional procedures for congenital heart diseases., Methods: The authors worked together with representatives of the Italian Society of Pediatric Cardiology and Congenital Heart Disease (SICP) Council and Jurist's expert in the field of health case-law. The final draft was shared with the major Italian centers involved in congenital interventional procedures and with AICCA, the Italian Patients Association of Congenital Heart Diseases - Adults and Children., Results: At the end of this review process, a final informed consent form was developed for the most frequent procedures performed in our catheterization laboratories. All of them consist of two parts: a general statement and a procedure-related one., Conclusions: The work performed by this multidisciplinary study group, under the supervision of the SICP, resulted in a new dedicated informed consent about interventional procedures in the field of congenital cardiology, taking into account the new legal requests. This informed consent is intended to be both a document that can be used as such and a document from which to derive a specific document for each center. We believe that using similar informed consents in all Congenital Heart Disease Centers or at least have informed consents all inspired by the same setting, could be a further improvement in taking care of the patients and their families.

Over recent years, thanks to remarkable advances in pediatric cardiology, cardiac surgery and catheter interventions, survival of children with congenital heart disease has significantly increased with the majority of patients surviving into adulthood. Therefore, the prevalence of adult patients with congenital heart disease has dramatically increased, as well as the need for specific and dedicated programs. Acute heart failure, infective endocarditis and arrhythmias represent the most common causes of visit in the emergency department in this population. Our task force aimed at guiding physicians taking care of this peculiar cohort of patients in the emergency department.

Patent foramen ovale (PFO) is implicated in the pathogenesis of different clinical syndromes in which it plays variable roles. In 2017 and 2018, four randomized clinical trials were published, allowing for the clarification of certain issues pertaining to cryptogenic stroke. Recently, eight European scientific societies collaborated to the writing of an interdisciplinary international position paper on PFO and cryptogenic stroke, based upon best available evidence, with the aim of defining the principles needed to guide decision making. Nonetheless, a tailored approach is not suitably addressed by standard position documents, considering that decisions about optimal management of PFO patients with left circulation thromboembolism are often challenging, mostly due to comorbidities and complex clinical scenarios.A panel of Italian cardiology experts gathered under the auspices of the Italian Society of Interventional Cardiology (SICI-GISE) for comprehensive discussion and consensus development, with the aim of providing practical recommendations, for both clinical and interventional cardiologists, regarding optimal management of PFO in patients with cerebral or systemic thromboembolism. In this position paper, various clinical scenarios in patients with and without high-risk PFO features are presented and discussed, including PFO patients with associated conditions (e.g. hypercoagulable states, deep vein thrombosis/pulmonary embolism, short runs of atrial fibrillation), and special subsets (e.g. patients with risk factors for atrial fibrillation, patients aged ≥65 years, patients who refused percutaneous PFO closure), with the Panel's recommendations being provided for each scenario.

Background: The multidisciplinary standardized interventions to educate and support patients with congenital heart disease (CHD) are described as "Transition Clinic" (TC). TC represents a key element to deliver care for patients during the transition from childhood to adulthood. So far, there is a lack of empirical evidence regarding the impact of TC models on the improvement of health perception in adolescent patients with CHD (CHD-specific TC model). For this reason, the aim of this study is assess the impact of the TC model on CHD adolescent patients' health perception outcomes., Methods: This study has a quasi-experimental design. Quality of life, satisfaction and health perception were assessed in T0 and after 1 year from enrollment (T1). During the follow-up period, the patients enrolled (aged 14 to 21 years) were involved in the CHD-specific TC model., Results: The results are referred to the first 100 patients enrolled (mean age 14.79 ± 1.85 years; 60% male), as they have already completed the follow-up. The overall study is currently ongoing. According to Warnes' classification, 29% of patients had simple heart defects, 46% showed moderate complexity, and 25% showed severe complexity. The comparison between T0 and T1 showed statistically significant improvement in T1 regarding pain/discomfort, anxiety/depression and perception of health status (EQ-5D), general satisfaction and quality of life (LAS QoL)., Conclusions: The preliminary results showed in this study are encouraging, and confirm the need to create a multidisciplinary standardized intervention of education and support to deliver care for adolescent patients with CHD.

Background: Transcranial Doppler echocardiography and transthoracic echocardiography may detect with high accuracy right-to-left shunt (RLS) due to patent foramen ovale (PFO). However, a standardized diagnostic protocol has never been established. It has been suggested that standing or sitting positions may improve sensitivity of transcranial Doppler due to increased LRS in such positions. We aimed to test efficacy of the sitting position in addition to the supine position in the grading of RLS in patients with PFO. We have also evaluated whether an increase in RLS is associated with a fall in oxygen saturation (SaO2)., Methods: From September 2015 to February 2016, 70 consecutive patients with suspected PFO underwent transcranial Doppler echocardiography and transthoracic echocardiography with microbubble count and evaluation of SaO2., Results: RLS due to PFO was diagnosed in 58 patients (82.9%). An increase in RLS grade in the sitting position was observed in 28 out of 58 patients (48.3%) (group 1), whereas no increase was detected in 30 patients (51.7%) (group 2). Among the clinical and echocardiographic variables considered, group 1 and group 2 differed only in the prevalence of atrial septal aneurysm (35.7 vs 3.3%, p=0.002). Postural changes were not associated with a fall in SaO2., Conclusions: RLS increases in about half of patients with PFO in the sitting position compared with the supine position, whereas no changes occur in SaO2.

The success of cardiac surgery over the past 50 years has increased numbers and median age of survivors with congenital heart disease (CHD). Adults now represent two-thirds of patients with CHD; in the United States alone the number is estimated to exceed 1 million.In this population many affected women reach reproductive age and wish to have children. While in many CHD patients pregnancy can be accomplished successfully, some special situations with complex anatomy, iatrogenic or residual pathology are associated with an increased risk of severe maternal and fetal complications. Pre-conception counseling allows women to come to truly informed choices. Risk stratification tools can also help high-risk women to eventually renounce to pregnancy and to adopt safe contraception options. Once pregnant, women identified as intermediate or high-risk should receive multidisciplinary care involving a cardiologist, an obstetrician and an anesthesiologist with specific expertise in managing this peculiar medical challenge.This document is intended to provide cardiologists working in hospitals where an Obstetrics and Gynecology Department is available with a streamlined and practical tool, useful for them to select the best management strategies to deal with a woman affected by CHD who desires to plan pregnancy or is already pregnant.

There is no generally accepted consensus on the management of patent foramen ovale (PFO) in the presence of cryptogenic cerebral ischemia, because of the lack of conclusive evidence. The aim of this position paper was to develop and promote a joint approach based on available data that may be shared by different specialists, while waiting for definite results from randomized controlled trials. A position statement was produced involving the major national scientific societies. The task force members were nominated by the presidents and/or executive boards of each society or working group, as appropriate, based on their previous work in relevant topic areas. Specific task force working groups prepared the drafts. In order to achieve maximum agreement, these drafts were merged and distributed to the scientific societies for local evaluation. The ensuing final draft, merging all the revisions, was reviewed by the task force and finally approved by all scientific societies. The following issues were addressed: definitions of transient ischemic attack (TIA) and both symptomatic and asymptomatic cryptogenic stroke; formulation of a diagnostic workup for patients with clinical event(s) and PFO; recommendations regarding medical and interventional treatment options considering individual risk factors based on the three available randomized trials and other observational studies; recommendations regarding requirements for operators and centers in Italy; definition of a follow-up evaluation protocol. In conclusion, available data provided the basis for the first multi-society position paper on the management of cryptogenic stroke/TIA and PFO.

The current work is the presentation of a new project at the IRCSS San Donato Milanese University hospital, in the sphere of Psychocardiology. Hospitalised children and adolescents often face psychosocial difficulties and the psychological condition of their parents frequently has an impact on their wellbeing. A strong need to take care, beyond the mere cure, is necessary in the hospital settings - that is a need to pay attention also to psychological aspects apart from the medical ones. Art therapy could be an answer for this need: the literature has outlined its efficacy in hospital, also due to the higher inclination of children and adolescents toward creativity. By providing and analysing the drawings of 10 young patients with congenital heart disease (CHD), this study outlines how the art therapy program gives these patients the opportunity to freely and directly express fears and anxieties about medical procedures and their disease. Moreover, through the creation of a tangible product, psychologists can better evaluate the psychological troubles of young patients and provide them and their parents with more focused and personalized support. This study also focuses on the perception of the utility that parents have of this new therapeutic intervention, offered at the Department of Paediatric Cardiac Surgery, confirming that art therapy is perceived as being effective and is definitely a good instrument in helping to "take care" of children and adolescents suffering from CHD.

Advances in medical and surgical management of congenital heart disease have changed the prognosis of infants and children with cardiac defects, so that an increasing number of patients reach adolescence and adult life, even those with complex defects. Recent data suggest that the number of adults with congenital heart disease, either repaired or not, approaches the number of children with the disorder. A cure is rarely achieved and ongoing surveillance and management in conjunction with specialists in this highly specialized field is mandatory to provide optimal care for patients. The profile of this patient population is going to change over the next few decades. Ideally specialist units should be established in appropriate geographic locations; patients need to be concentrated for expertise, experience, and optimal management. Less specialized regional centers and outpatient clinics in districts in connection with grown-up congenital heart disease units should be created. Specialist units should accept responsibility for educating the professionals, training the specialists, and sharing particular skills between each other. Guidelines and recommendations should help physicians to make decision in their daily practice. However, the final judgment regarding the care of an individual patient must be made by his/her physician. This article will briefly discuss some aspects of these dedicated guidelines and how they influence the clinical daily practice.

Adults with congenital heart disease, especially the ones who require constant medical care, encounter many psychosocial difficulties. These difficulties also occur in the interventional departments of cardiology and cardiac surgery, where they often have to make important decisions, undergo cardiology interventions and cardiac surgeries, and more rarely they also have to deal with unexpected complications that can lengthen their hospitalisation duration considerably. The literature has also outline predictive factors when it comes to the psychological difficulties encountered in this population. For these reasons, the inclusion of the psychologist in this context is essential.

Cardiopulmonary exercise testing (CPET) gives important information about functional capacity by direct measurement of exercise respiratory gas exchange. It provides assessment of the integrative exercise responses involving the cardiovascular, respiratory and muscle-skeletal systems which are not adequately investigated through the measurement of individual organ system function. CPET involves measurements of oxygen uptake (VO2), carbon dioxide production (VCO2) and several ventilatory measures. CPET is increasingly being used as a clinical tool to determine functional capacity, prognosis and potential need for medical or surgical interventions in patients affected by congenital heart disease (CHD). Also in this population, peak VO2 and slope VE/VCO2 are the most predictive parameters in terms of mortality and need of hospitalization. Cyanotic patients with Esisenmenger syndrome show the worst functional limitation and consequently the worst prognosis. This article provides basic and practical information about CPET and focuses on its interpretation in patients with CHD.

The incidence of infective endocarditis (IE) in patients with congenital heart disease (CHD) is higher than in general population; this is a major problem considering the continuous expansion of such group of patients. Generally the more complex is the congenital heart disease the higher is the risk of IE. The aetiology, clinical features, complications, basis for diagnosis and treatment of IE in CHD patients don't differ from those in acquired cardiac disease; however, right-sided IE is more frequent in CHD patients. Due to the complex anatomy or presence of artificial material in many CHD, the transesophageal echocardiogram is extremely useful although echocardiographic assessment remains difficult. Prognosis is better than in other forms of IE with a mortality rate <10%. Primary prevention is crucial: a good oral-dental hygiene and regular dental review are as important as antibiotic prophylaxis; however this awareness in the CHD population is still not satisfactorily spread due to an educational problem. New IE guidelines from International Cardiology Societies emphasize the role of primary prevention and limit antibiotic prophylaxis to the highest risk patients undergoing the highest risk procedures. This article reviews the main reasons justifying the revision of previous IE guidelines, focuses on criteria to select CHD patients requiring antibiotic prophylaxis and gives information about antibiotic therapy to use.

Many paediatric cardiac patients now survive to adulthood following early surgery. This population of adult patients with a congenital heart disease offer distinct challenges such as unusual anatomy and demands such as pregnancy and exercise tolerability not found in conventional paediatric or traditional adult interventional patients. Starting with neonatal balloon atrial septostomy for transposition of the great arteries, improving and changing percutaneous interventional techniques have increasingly replaced surgery as a treatment option in several congenital heart diseases. There has been a rapid growth in interventional cardiology techniques to treat adults with congenital heart disease, mirroring the rise of interventional cardiology as a cardiology subspecialty and the increasing population of adults with congenital abnormalities. We review current indications for adult congenital heart disease intervention and best practice, reviewing the patient spectrum commonly treated, devices used, and emerging treatments.

The improvement of surgical procedures over the past 40 years has changed the medical history of the congenital heart defect. It is estimated that 85% of children with congenital heart disease now have the opportunity to reach adulthood and that this percentage will increase over the next two decades. This special issue of the Journal aims to offer a basic information on key topics of this field of cardiology not newer, but still rapidly evolving.

Objective: Pulmonary regurgitation may cause progressive right ventricular dilatation and dysfunction in adult patients previously repaired for tetralogy of Fallot. To assess the optimal surgical timing, the impact of the right ventricular restoration with a new surgical ventriculoplasty technique is evaluated following TFO repaired adult patients with severe pulmonary regurgitation and right ventricular dilatation., Methods: Sixteen patients with severe pulmonary valve regurgitation (PVR) and right ventricular dilatation with RVOT aneurysm underwent right ventricular remodelling since January 2002. Each underwent preoperative evaluation by Doppler echocardiography, magnetic resonance imaging (MRI), and right ventricular myocardial acceleration during isovolumic contraction (IVC). The surgical procedure included pulmonary valve implantation and RVOT restoration achieved by removal of the aneurysm tissue, coupled with a ventriculoplasty to reduce volume, accomplished by creating a satisfactory RVOT dimension by placing with 2-0 Gortex suture to allow acceptance of a 26 Hegar dilator to avoid restriction. Nine patients had associated surgical procedures., Results: All patients survived the operative procedure and underwent a 16-month follow-up interval. A reduction of cardio thoracic index and a clinical improvement occurred in each patient. Significant reduction of RVEDV and RVESV and increased right ventricular ejection fraction was observed., Conclusions: This preliminary database implies that the right ventricular restoration is a simple and effective procedure, and introduces a structural component that should be added in repaired TFO patients with right ventricular dilatation and underlying aneurysm or akinesia of the right ventricular outflow tract.

This review is divided into two parts: the first of which traces the evolution of medical and palliative techniques; the following is a selective account of surgical concepts and procedures; the emphasis is on therapeutic developments during the last 50 years. The second part approaches the surgical corrections and tries to explain the reinterventional causes and the residual defects responsible of cardiac failure.

Despite Tetralogy of Fallot is a well-known cardiac congenital disease, still an important cardiovascular surgery and intensive care challenge. The following is a selective account of medical and surgical concepts and procedures; the emphasis is on therapeutic developments during the last 50 years. This review is divided into two parts: the first of which traces the evolution of medical and palliative techniques; the second part approaches the surgical corrections and try to explain the reinterventional causes and the residual defects responsible of cardiac failure.

Isolated patent ductus arteriosus accounts for 10 to 18% of all cardiovascular malformations. Transcatheter closure of the patent ductus arteriosus has now become established practice with a high success rate and a low incidence of complications. Many devices have been proposed during the last 20 years. The use of some of these devices has largely been abandoned for different reasons: high costs, the need of large transvenous sheaths and the high incidence of late residual shunts. The devices currently available (Amplatzer duct occluder, buttoned device, DuctOcclud coil, Gianturco-Grifka vascular occlusion device, detachable coils) render the embolization of a patent ductus arteriosus relatively easy, with a short hospital stay. We feel that this is the treatment of choice for the majority of patients with a patent ductus arteriosus. The aim of this article was to review our experience and report the state-of-the-art in transcatheter patent ductus arteriosus therapy.

A normal lung is supplied by a pulmonary artery branching from the pulmonary trunk. Major aorto-pulmonary collateral arteries (MAPCAs) are found in combination with various congenital heart malformations such as pulmonary atresia with ventricular septal defect (PA-VSD). Now that MAPCAs are used for unifocalization in patients with PA-VSD, the question arises as to whether the morphologic criteria of these collateral arteries could help to provide better results. We compared the morphology of the pulmonary vascular bed, the origin, course and connections of the MAPCAs in 40 consecutive infants with PA-VSD with or without 22q deletion (del22q11.2.). All underwent echocardiographic evaluation and catheterization. Identification of del22q11.2. was performed by FISH study. Del22q11.2. was identified in 16 pts (40%); the presence of MAPCAs was significantly higher in patients with del22q11.2. (9/16 vs 3/24, p = 0.01). While complex morphology of MAPCAs, anastomoses with the central pulmonary artery outside the lung and absent ductus arteriosus were associated with del22q11.2, confluence of the pulmonary arteries was not a relevant phenotypic difference. The size of the right and left pulmonary arteries expressed as a standard deviation difference of the normal range for body surface area was -4.2 (quartiles -3.1/-1.8) for PA-VSD with del22q11.2. and -2.6 (quartiles -5.3/-2.9) for PA-VSD without del22q11.2. (p = 0.02). The difference between measured and theoretical Nakata index was -373 +/- 94 for PA-VSD with del22q11.2. vs. -245 +/- 93 for PA-VSD without del22q11.2. (p = 0.0002). A specific pulmonary vascular bed phenotype could be defined in patients with PA-VSD with del22q11.2. deletion: MAPCAs with complex loop morphology and small but confluent central pulmonary arteries. These findings indicate a different timing of the faulty development pathway of the pulmonary vascular bed in patients with and without del22q11.2. This phenotype difference may help our understanding of maldevelopment and facilitate decisions concerning the suitability of these arteries for unifocalization procedures.