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Background The European-funded Health Effects of Cardiac Fluoroscopy and Modern Radiotherapy in Pediatrics (HARMONIC) project aims to improve knowledge on the effects of medical exposure to ionizing radiation (IR) received during childhood. One of its objectives is to build a consolidated European cohort of pediatric patients who have undergone cardiac catheterization (Cath) procedures, with the goal of enhancing the assessment of long-term radiation-associated cancer risk. The purpose of our study is to provide a detailed description of the Italian cohort contributing to the HARMONIC project, including an analysis of cumulative IR exposure, reduction trend over the years and an overview of the prospective collection of biological samples for research in this vulnerable population. Methods In a single-center retrospective cohort study, a total of 584 patients (323 males) with a median age of 6 (2–13) years, referred at the Pediatric Cardiology in Niguarda Hospital from January 2015 to October 2023, were included. Biological specimens from a subset of 60 patients were prospectively collected for biobanking at baseline, immediately post-procedure and after 12 months. Results Two hundred fifty-nine (44%) patients were under 1 year old at their first procedure. The median KAP/weight was 0.09 Gy·cm 2 /kg (IQR: 0.03–0.20), and the median fluoroscopy time was 8.10 min (IQR: 4.00–16.25). KAP/weight ratio showed a positive correlation with the fluoroscopy time (Spearman’s rho = 0.679, p  < 0.001). Significant dose reduction was observed either after implementation of an upgraded technology system and a radiation training among staff. The Italian cohort includes 1858 different types of specimens for Harmonic biobank, including blood, plasma, serum, clot, cell pellet/lymphocytes, saliva. Conclusions In the Italian Harmonic cohort, radiation dose in cardiac catheterization varies by age and procedure type. An institution’s radiological protection strategy has contributed to a reduction in radiation dose over time. Biological samples provide a valuable resource for future research, offering an opportunity to identify potential early biomarkers for health surveillance and personalized risk assessment.

In children, arrhythmias have an etiology, evolution and treatment strategy that often differs from that of adults. Ectopic beats are very common, but rarely need to be treated. Macroreentrant supraventricular tachycardia is the arrhythmia that usually requires a treatment intervention. Adenosine for tachycardia interruption and 1C anti-arrhythmic drugs for prevention of recurrences are the first-line treatment. Automatic supraventricular tachycardias are infrequent but are typical of the pediatric age; treatment should be targeted at rate control in addition to tachycardia interruption. Beta-blockers and calcium antagonists are the most useful drugs for tachycardia control. Ventricular tachycardia is a very uncommon arrhythmia in children usually related to channelopathies; in this setting beta-blockers are often the first-choice treatment, but in many selected patients implantation of a cardioverter-defibrillator is required. Other types of ventricular tachycardia include fascicular and infundibular tachycardias that are usually well controlled by medical and/or ablation therapy. Ablation procedures are very effective in curing many tachyarrhythmias also in the pediatric age; the main indication for ablation is the need for continuing medical therapy after the age of 10-12 years. Hypokinetic arrhythmias are very rare and usually require pacemaker implantation.

Tetralogy of Fallot (ToF) occurs in about 4 births/1000/year and represents about one tenth of all congenital heart diseases. Nowadays 86% of patients reach adulthood with corrective surgery. Before the 1980s, these patients were treated only with \"surgical palliation\", which consisted in the creation of a systemic to pulmonary artery shunt or a pulmonary valvulotomy, whereas after the introduction of extracorporeal circulation, corrective surgery is performed electively between 3 and 6 months of life. After repair patients during their life may develop hemodynamic lesions, including right ventricular outflow tract dysfunction, and arrhythmias which can occur in over 30% of cases. It is estimated that these patients present a risk of sudden death of 0.2%/year. Therefore, for the prevention and treatment of arrhythmic events, a periodic follow-up in specialized centres for adult congenital heart disease is mandatory, because most often arrhythmias are triggered by the presence of hemodynamic lesions, first of all pulmonary regurgitation.

Myocardial ischemia in pediatric population is uncommon and usually due to congenital heart disease or extracardiac conditions leading to poor coronary perfusion. A 6-day-old newborn presented with respiratory distress and signs of heart failure. ECG, echocardiography, and laboratory results were consistent with myocardial ischemia. Coronary angiography was performed to exclude anomalous origin of coronary arteries, showing normal coronary artery origin and course. Thrombophilia and extra-cardiac causes were ruled out. Clinical conditions improved with mechanical ventilation and diuretics, enzyme levels lowered, repolarisation and systolic function abnormalities regressed, but ischemic electrocardiographic and echocardiographic signs still presented during intense crying. Becaues of suspicion of microvascular angina, therapy with ASA and beta-blocker was started. At 5 month followup, the baby was in good clinical condition and no more episodes were recorded. We believe it is an interesting case, as no similar cases have been recorded till now.

Chest pain presenting during childhood is a common cause of admission to the emergency departments or to pediatric outpatient consultations. In most cases chest pain is idiopathic or secondary to musculoskeletal disorders, whereas a cardiac origin is confirmed in a minority (0.5-1%). The peak of incidence can be registered between 10-14 years, mainly involving males. Early identification of chest pain requiring further investigation allows a prompt diagnosis, limiting unnecessary exams and prolonged hospitalization.

Hypertrophic cardiomyopathy (HCM) is a familial, genetically determined, primary cardiomyopathy caused by mutations in genes coding for proteins of the sarcomere, or, less frequently, genes involved in storage diseases. In pediatric settings, pure HCM has an estimated incidence of 4.7 per million children. The disease is often sub-clinical and goes unrecognized mainly because most patients with HCM have only mild symptoms, if any. However, sudden cardiac death, the most dramatic clinical occurrence and the primary concern for patients and physicians alike, may be the first manifestation of the disease. We describe a case of compound heterozygosity in the MYBPC3 gene (p.Glu258Lys and IVS25-1G>A) associated with biventricular hypertrophy, atrial enlargement and subsequent neonatal death 33 days postpartum. Other studies have reported compound and/or double heterozygosis in the same or different sarcomeric genes during childhood and adulthood, and neonatal presentations have also been described. Our observations show that the combination of a missense (p.Glu258Lys) and a splice-site mutation (IVS25-1G>A) profoundly affects the clinical course. In families in which parental mutations are known, preimplantation (where ethically and legally feasible) or prenatal genetic screening should be adopted because: (1) neonatal HCM in genetic heterozygosity is potentially lethal and (2) heart disease is the most common developmental malformation and the leading cause of neonatal mortality and morbidity.

Supraventricular tachycardia (SVT) is the most common symptomatic arrhythmias in children. Re-entry tachycardias are the most common form, on the contrary automatic tachycardias are relatively rare. There are four types or re-entry: along anomalous pathway with bi-directional (Wolff-Parkinson-White) or unidirectional conduction, intranodal re-entry, intra-atrial re-entry that is common after surgical procedure, and finally the uncommon sinus node re-entry. Automatic tachycardias may be atrial or junctional. The different types of tachycardia have a different incidence according to the age: in the first year of age re-entry along anomalous pathway is the dominant form, while intranodal reentry becomes common during adolescence. The age at the beginning of tachycardia is important for long term prognosis. When SVT starts in the first months of life it disappears in 80% of cases within the first year of life; on the contrary, if tachycardia starts later spontaneous remission is detected in only 15%-20% of patients. In infancy heart failure is the more common presenting symptom, thereafter palpitations become the principal cause of recognition of SVT. Syncope is reported in about 8% of cases and in another 15% usually neonates and infants, the SVT has an occasional detection. Electrocardiogram (ecg) usually allows the precise diagnosis of various types of SVT, and every effort should be made to record ecg during tachycardia. The parameters that should be evaluated are: heart rate, P wave axis, PR and RP interval, and finally presence or absence of AV block. Short lasting episodes should be difficult to be recorded; in these cases cardio-call and trans-telephonic transmission represent useful techniques to obtain SVT demonstration. Patients with SVT require a complete evaluation with others diagnostic techniques: echocardiogram, Holter monitoring, stress test, that should be chosen according the type of tachycardia. Electrophysiologic evaluation is now rarely performed for diagnostic purpose; trans-esophageal atrial stimulation being less invasive than intracardiac evaluation is more extensively employed when diagnosis of SVT is uncertain. Transesophageal stimulation is useful in the following situations: 1) evaluation of patients with symptoms suggestive of paroxistic tachycardia but without ecg documentation, 2) to assess the mechanism responsible for re-entry tachycardia: macro re-entry versus intranodal re-entry 3) to evaluate characteristics of anomalous pathway with bi-directional conduction, and 4)to terminate re-entrant SVT.

Chest pain presenting during childhood is a common cause of admission to the emergency departments or to pediatric outpatient consultations. In most cases chest pain is idiopathic or secondary to musculoskeletal disorders, whereas a cardiac origin is confirmed in a minority (0.5-1%). The peak of incidence can be registered between 10-14 years, mainly involving males. Early identification of chest pain requiring further investigation allows a prompt diagnosis, limiting unnecessary exams and prolonged hospitalization.

Questa ha dimostrato la presenza di un forame ovale pervio con evidenza di shunt destro-sinistro (Figura 2). La paziente è stata quindi sottoposta a intervento cardiochirurgico combinato di sostituzione dell’aorta ascendente e chiusura del forame ovale pervio. Nelle prime lo shunt destro-sinistro si verifica solitamente a livello del forame ovale e può occorrere anche in assenza di ipertensione delle camere destre. Dal punto di vista strumentale è necessario osservare la presenza di uno shunt destro-sinistro con ecocardiografia o Doppler transcranico. Nel caso da noi riportato la dilatazione aortica, determinando una modificazione della geometria cardiaca ed uno schiacciamento dell’atrio destro, può aver favorito la dislocazione del setto interatriale che, in presenza di pervietà del forame ovale, ha facilitato lo shunt visto l’allineamento del flusso della cava inferiore con il setto interatriale (Figura 1C). La dislocazione atriale destra era probabilmente più marcata in ortostatismo, promuovendo così un flusso venoso preferenziale dalla vena cava inferiore direttamente verso la pervietà del forame ovale. Shah AH, Osten M, Leventhal A, et al. Percutaneous intervention to treat platypnea-orthodeoxia syndrome: the Toronto experience. Anatomic interaction between the aortic root and the atrial septum: a prospective echocardiographic study.

A newborn was suspected of having situs inversus with levocardia based on chest X-ray. Echocardiography ruled out this hypothesis but revealed a giant cardiac mass that was confirmed by magnetic resonance imaging. Coronary angiography showed that the right coronary artery ran on the surface of the mass, and only partial debulking surgery was performed to relieve right heart compression. Histological examination classified the mass as cardiac fibroma. Complex diagnostic work-up allowed correct anatomic definition of the mass as well as its relationship with adjacent structures, and helped guide surgical planning.