Cangrelor use in a 6-year-old patient undergoing complex percutaneous coronary intervention after post-surgical myocardial infarction.

Cangrelor is an intravenously administered P2Y12 receptor antagonist, which has been approved for adult patients undergoing percutaneous coronary intervention and, due to its unique pharmacokinetics, it allows effective and controllable peri-procedural platelet inhibition. We report the case of a 6-year-old child with anomalous origin of right coronary artery from aortic left coronary sinus, who underwent elective surgical replacement of stenotic and calcified conduit between the right ventricle and the main pulmonary artery. The surgery was complicated by acute myocardial infarction secondary to coronary extrinsic compression. The patient was successfully treated with urgent percutaneous coronary intervention (simultaneous V-stenting) and cangrelor infusion, subsequently switched to clopidogrel therapy.

The burden of radiation exposure in congenital heart disease: the Italian cohort profile and bioresource collection in HARMONIC project.

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·cm2/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.

[Management of postoperative arrhythmias in the tetralogy of Fallot: a literature review]. / Gestione delle aritmie cardiache dopo correzione chirurgica della tetralogia di Fallot: revisione della letteratura.

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.

[Chest pain in children: a practical approach]. / Gestione del dolore toracico in età pediatrica.

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.

Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy.

BACKGROUND: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. METHODS: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). RESULTS: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. CONCLUSIONS: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.

Lombardy regional urgent reorganization for congenital cardiac patients following the Covid-19 pandemic.

OBJECTIVE: By the end of February 2020, the COVID-19 pandemic infection had spread in Northern Italy, with thousands of patients infected. In Lombardy, the most affected area, the majority of public and private hospitals were dedicated to caring for COVID-19 patients and were organized following the 'Hub-and-Spoke' model for other medical specialties, like cardiac surgery and interventional procedures for congenital cardiac disease (CHD). Here, we report how the congenital cardiac care system was modified in Lombardy and the first results of this organization. METHODS: We describe a modified 'Hub-and-Spoke' model - that involves 59 birthplaces and three specialized Congenital Cardiac Centers -- and how the hub center organized his activity. We also reported the data of the consecutive cases hospitalized during this period. RESULTS: From 9 March to 15 April, we performed: a total of 21 cardiac surgeries, 4 diagnostic catheterizations, 3 CT scans, and 2 CMR. In three cases with prenatal diagnosis, the birth was scheduled. The spoke centers referred to our center six congenital cardiac cases. The postop ExtraCorporeal Membrane Oxygenation support was required in two cases; one case died. None of these patients nor their parents or accompanying person was found to be COVID-19-positive; 2 pediatric intensivists were found to be COVID-19-positive, and needed hospitalization without mechanical ventilation; 13 nurses had positive COVID swabs (4 with symptoms), and were managed and isolated at home. CONCLUSION: Our preliminary data suggest that the model adopted met the immediate needs with a good outcome without increased mortality, nor COVID-19 exposure for the patients who underwent procedures.

The impact of treatment of the fetus by maternal therapy on the fetal and postnatal outcomes for fetuses diagnosed with isolated complete atrioventricular block.

OBJECTIVES: to analyse retrospectively the data of fetuses diagnosed with isolated complete atrioventricular block and efficacy of treatment of the fetus by maternal therapy. MATERIALS: Between 1992 and 2004, we diagnosed complete atrioventricular block in 26 singleton and 2 twins fetuses of 27 pregnant women known to have anti Ro/La antibodies, 11 with autoimmune disease, one patient analysed in 2 pregnancies. At presentation, 20 of the fetuses were compensated and non-hydropic, while 8 had hydrops. Twenty patients were treated with dexamethasone, 2 with associated salbutamol and one mother with isoproterenol. RESULTS: Age at presentation was not different between the hydropic and non-hydropic fetuses. The fetuses with hydrops, however, had a lower mean heart rate at presentation, 48.5 +/- 9.25 with a range from 32 to 60, compared to 59.95 +/- 7.9 beats per minute, with a range from 50 to 80, in the non-hydropic fetuses (p less than 0.002). Equally, after birth the mean heart rate in hydropic fetuses was 42.6 +/- 5.1, with a range from 38 to 50, as opposed to 56.05 +/- 11.8 beats per minute, with a range from 29 to 110, in the non-hydropic fetuses (p less than 0.015), The hydropic fetuses were delivered at 31.7 +/- 3.8 weeks' gestation, with a range from 29 to 38 weeks (p less than 0.003) compared to 35.5 weeks' gestation +/-2.04, with a range from 31 to 38, in the non-hydropic fetuses. Mortality was 37.5% in the hydropic fetuses, versus 5% of those without hydrops (p less than 0.02). Pacemakers were implanted in 22 of 26 infants born alive, at a median of 45 days, with a range from 1 day to 5 years, in those without hydrops during fetal life, and 3 days, with a range from 1 day to 8 months in those afflicted by hydrops, of whom 2 died despite the implant of the pacemaker. The presence and degree of hydrops had a significantly negative predictive value. No significant differences were observed between the treated and non treated cases, albeit that administration of steroids ameliorated rapidly the hydrops in 3 of 5 cases. CONCLUSIONS: The outcome in our cases was mainly dependent on the presence and degree of fetal cardiac failure. Treatment of the fetus by maternal administration of steroids did not result in any regression of the conduction disorder, but had a favourable effect on fetal hydrops.

Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids).

Importance: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk. Objective: To develop and validate an SCD risk prediction model that provides individualized risk estimates. Design, Setting, and Participants: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017. Exposures: The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping. Main Outcomes and Measures: A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise). Results: Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95% CI, 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years. Conclusions and Relevance: This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

Pregnancy in women with cardiovascular disease in the guidelines era: an Italian single-center experience.

AIMS: The aim of the present study is to determine pregnancy outcome with regard to the risk class and the modality way of referral to our joint Cardiac Obstetric Clinic. METHODS: This is a prospective observational study. Patients referred to our clinic between 2011 and 2014 were included. Reason and timing for referral were recorded. Two groups were identified: women who were known to have cardiac disease before referral (group I) and women who were not (group II). Pregnancies were managed as recommended by the ESC Guidelines. Cardiac events were defined as death, heart failure requiring treatment, documented tachyarrhythmias, thromboembolic events and need for urgent endovascular procedures or surgery. RESULTS: Of the 110 pregnancies, 51 (47%) were in group I and 57 (53%) in group II. Congenital (44%) and valvular (27%) diseases were the most frequent diagnosis. Thirty-two percent of patients were in WHO risk classes III-IV. Thirty percent were referred for symptoms, 70% for risk assessment. Women in group II were evaluated later in pregnancy than those in group I (25.6 ±â€Š9 vs. 21.4 ±â€Š9; P < 0.01). Cardiovascular events occurred in 15 (13.6%) pregnancies and were more common in WHO risk classes III-IV (11, P < 0.001), in group II (12, P= 0.02) and in patients referred for symptoms (11, P < 0.001). Stillbirths occurred only in classes III-IV (three pregnant, 2.7%). CONCLUSION: There was no maternal or neonatal mortality and an overall acceptable incidence of cardiovascular events but a relevant percentage of pregnant were first referred late and/or for the onset of symptoms. Events were more frequent in these patients. Further efforts are needed to optimize referral to specialized centers.

Mitral valve endocarditis due to Abiotrophia defectiva in a 14th week pregnant woman.

Infective endocarditis during pregnancy carries a high mortality risk, both for the mother and for the foetus and requires a multidisciplinary team in the management of complicated cases. We report our experience with a 39-year old patient, affected by an acute active mitral endocarditis due to Abiotrophia defectiva at the 14th gestational week, strongly motivated to continue the pregnancy. Our patient successfully underwent mitral valve replacement with a normothermic high-flow cardiopulmonary bypass under continuous intraoperative foetal monitoring. Caesarean section occurred at the 38th gestational week. The delivery was uneventful and both the mother and child are doing well at the 16-month follow-up.

Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia.

BACKGROUND: Mutations in the cardiac ryanodine receptor gene (RyR2) underlie catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited arrhythmogenic disease occurring in the structurally intact heart. The proportion of patients with CPVT carrying RyR2 mutations is unknown, and the clinical features of RyR2-CPVT as compared with nongenotyped CPVT are undefined. METHODS AND RESULTS: Patients with documented polymorphic ventricular arrhythmias occurring during physical or emotional stress with a normal heart entered the study. The clinical phenotype of the 30 probands and of 118 family members was evaluated, and mutation screening on the RyR2 gene was performed. Arrhythmias documented in probands were: 14 of 30 bidirectional ventricular tachycardia, 12 of 30 polymorphic ventricular tachycardia, and 4 of 30 catecholaminergic idiopathic ventricular fibrillation; RyR2 mutations were identified in 14 of 30 probands (36% bidirectional ventricular tachycardia, 58% polymorphic ventricular tachycardia, 50% catecholaminergic idiopathic ventricular fibrillation) and in 9 family members (4 silent gene carriers). Genotype-phenotype analysis showed that patients with RyR2 CPVT have events at a younger age than do patients with nongenotyped CPVT and that male sex is a risk factor for syncope in RyR2-CPVT (relative risk=4.2). CONCLUSIONS: CPVT is a clinically and genetically heterogeneous disease manifesting beyond pediatric age with a spectrum of polymorphic arrhythmias. beta-Blockers reduce arrhythmias, but in 30% of patients an implantable defibrillator may be required. Genetic analysis identifies two groups of patients: Patients with nongenotyped CPVT are predominantly women and become symptomatic later in life; patients with RyR2 CPVT become symptomatic earlier, and men are at higher risk of cardiac events. These data provide a rationale for prompt evaluation and treatment of young men with RyR2 mutations.

[A matter of perspective: multimodality imaging of a giant cardiac mass]. / Questione di prospettiva: imaging multimodale di una massa cardiaca gigante.

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.

Surgical treatment of arrhythmias related to congenital heart diseases.

BACKGROUND: Supraventricular arrhythmias complicate operated and unoperated congenital heart disease, especially when atrial dilatation coexists. METHODS: We evaluated the results of intraoperative ablation in a group of 23 patients with chronic supraventricular tachyarrhythmias (mostly intraatrial reentry) that were unresponsive to conventional medical therapy. All procedures were performed consecutively between September 1999 and November 2001. Ablation was done during redo operations (Fontan conversion to total cavopulmonary connection in 16 patients) in 18 patients and during primary surgical correction in 5 patients. The mean age at operation was 25 +/- 12 years (2 to 50 years). Cryoablation was done in 10 patients and radiofrequency ablation in 13 patients. Nineteen patients had ablation in one atrium and 4 had ablation in both atria. A generous atrial reduction was always performed at the end of the operation. RESULTS: The operative mortality rate was 13% (3 patients) from causes unrelated to ablation. In 20 survivors, the ablation was effective immediately. Eight patients required a permanent pacemaker. During a mean follow-up of 22 +/- 4 months, atrial arrhythmias recurred in 25% (5 patients) and were controlled with medical therapy, whereas 1 patient required pacemaker implantation. CONCLUSIONS: Intraoperative treatment of unresponsive atrial tachyarrhythmias associated with operated or unoperated congenital heart disease is feasible and the midterm results are encouraging.

Eisenmenger syndrome: a case of survival after ventricular tachycardia due to inferior myocardial infarction in a 48-year-old patient with congenital large ventricular septal defect.

Eisenmenger syndrome is the most common consequence of congenital cyanotic heart disease seen in adults; survival to the fifth decade of life is rare. Death is very difficult to predict: it is related to sudden cardiac ventricular arrhythmia, massive hemoptysis and right heart failure. In this paper, a patient with ventricular septal defect and Eisenmenger reaction is described. The patient was relatively well until 48 years of age, when she underwent surgery because of a cerebral abscess without cerebral complications but with some deterioration of her cardiac function. After discharge, the patient was readmitted to the hospital because the electrocardiogram showed persistent ST inferior elevation. Echocardiography demonstrated poor contractility and inferior akinesia. Sudden ventricular tachycardia occurred and the patient became unconscious. She was successfully resuscitated and, following a period of ventilation, the hemodynamics stabilized and she was discharged 17 days later. She remained well two years later.

[Treatment of arrhythmias in children without heart disease]. / Trattamento delle aritmie nel bambino con cuore apparentemente sano.

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.

An unusual case of suspected microvascular angina in a newborn.

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.

Partial anomalous connection of both superior pulmonary veins.

Several patterns of anomalous pulmonary venous drainage have been described in the literature, and bilateral partial pulmonary anomalous vein connection (PAPVC) has been described as a rare congenital cardiac anomaly. We report an unusual type of bilateral PAPVC, involving both the superior right and left pulmonary veins draining into the left brachiocephalic vein in a young adult who was symptomatic with dyspnea and a dry cough.