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.

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.

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.

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.

[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.

[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.

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.

Percutaneous device closure of iatrogenic left ventricular wall pseudoaneurysm.

A 67-year-old man with ischemic cardiomyopathy was transferred to our hospital in cardiogenic. During a video-assisted mini-thoracotomy for left ventricular epicardial lead implantation, a left ventricular free-wall rupture occurred and an emergency surgical repair was performed. Postoperatively patients experience left ventricular wall pseudoaneurysm. After stabilization of clinical conditions with aggressive medical treatment, we decided to attempt a minimally invasive procedure (ie, a transcatheter pseudoaneurysm closure). To date, few cases of device closure of left ventricle pseudoaneurysm are reported in the literature, usually secondary to myocardial infarction, and we believe this is the first case of left ventricle pseudoaneurysm after iatrogenic left ventricle laceration and surgical closure.

Pulmonary valve implantation with the new Shelhigh Injectable Stented Pulmonic Valve.

BACKGROUND: Pulmonary regurgitation (PR) occurs frequently after tetralogy of Fallot (TOF) repair, impairing long-term prognosis and necessitating reinterventions. Myocardial damage, invasiveness, and the risks of pulmonary valve replacement (PVR) therefore need to be minimized. The new Shelhigh Injectable Stented Pulmonic Valve (Shelhigh Inc, Union, NJ) allows implantation without cardiopulmonary bypass (CPB) under direct control. METHODS: Twelve symptomatic patients (age, 21.3 +/- 12.5; range, 5.8 to 53.5 years) with severe PR and progressive right ventricular (RV) dilatation with dysfunction received the Shelhigh valve in sizes 21 (n = 1), 25 (n = 4), 27 (n = 3), 29 (n = 2), and 31 mm (n = 2). RESULTS: Valve insertion was successful and hemodynamic performance excellent in all: peak systolic gradient, 14.5 +/- 4.6 (range, 10 to 20) mm Hg; mean gradient, 6.3 +/- 1.6 (range, 4 to 8) mm Hg. Four patients underwent concomitant procedures on CPB: one reduction plasty of a dilated main pulmonary artery, two tricuspid valve repairs, and one VSD closure. Early recovery was uneventful. There were no reoperations. During a mean follow-up of 5.4 +/- 4.3 months (range, 0.3 to 10.6 months) echocardiography showed good results, with low gradients and recovered RV function in all. All presented in New York Heart Association functional class 1 at the latest follow-up. CONCLUSIONS: The Shelhigh valve allows easy PVR without CPB up to large valve sizes, with less invasiveness compared with a conventional approach. Further follow-up is needed to assess its durability and long-term performance.

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.

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.