Recommendations for cardiovascular magnetic resonance and computed tomography in congenital heart disease: a consensus paper from the CMR/CCT Working Group of the Italian Society of Pediatric Cardiology and the Italian College of Cardiac Radiology endorsed by the Italian Society of Medical and Interventional Radiology (Part II).

Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. This is the second of two complementary documents, endorsed by experts from the Working Group of the Italian Society of Pediatric Cardiology and the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, aimed at giving updated indications on the appropriate use of CMR and CCT in different clinical CHD settings, in both pediatrics and adults. In this article, support is also given to radiologists, pediatricians, cardiologists, and cardiac surgeons for indications and appropriateness criteria for CMR and CCT in the most referred CHD, following the proposed new criteria presented and discussed in the first document.

Impact of 4D-Flow CMR Parameters on Functional Evaluation of Fontan Circulation.

We sought to evaluate the potential clinical role of 4D-flow cardiac magnetic resonance (CMR)-derived energetics and flow parameters in a cohort of patients' post-Fontan palliation. In patients with Fontan circulation who underwent 4D-Flow CMR, streamlines distribution was evaluated, as well a 4D-flow CMR-derived energetics parameters as kinetic energy (KE) and energy loss (EL) normalized by volume. EL/KE index as a marker of flow efficiency was also calculated.

Overcoming Underpowering in the Outcome Analysis of Repaired-Tetralogy of Fallot: A Multicenter Database from the CMR/CT Working Group of the Italian Pediatric Cardiology Society (SICPed).

Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized.

Long-Term Cardiovascular Outcome in Children with MIS-C Linked to SARS-CoV-2 Infection-An Italian Multicenter Experience.

MIS-C is a multisystem inflammatory syndrome that is characterized by multi-organ failure and cardiac involvement. The aim of this study was to describe the long-term cardiovascular outcome in a cohort of MIS-C pediatric patients, who were admitted to two Italian Pediatric Referral Centers. Sixty-seven patients (mean age 8.

Long term follow-up in two siblings with Sengers syndrome: Case report.

Background: Sengers syndrome is characterized by congenital cataract, hypertrophic cardiomyopathy, mitochondrial myopathy, and lactic acidosis associated with mutations in AGK gene. Clinical course ranges from a severe fatal neonatal form, to a more benign form allowing survival into adulthood, to an isolated form of congenital cataract. Thus far few reported cases have survived the second decade at their latest examination, and no natural history data are available for the disease.

Dobutamine Stress Cardiac MRI in Pediatric Patients with Suspected Coronary Artery Disease.

Following current practice, pediatric patients with treated congenital coronary malformations or acquired coronary disease undergo Cardio-Pulmonary Exercise Test (CPET), stress Echocardiography and Electrocardiography (sEcho, sEKG), and Coronary Angiography (CA). Stress cMRI can assess cardiac function, myocardial viability, and stress/rest perfusion deficit-without radiation exposure, general anesthesia, and hospitalization-in a single non-invasive exam. The aim of our pilot study is to assess the feasibility and diagnostic accuracy of Dobutamine stress cMRI compared to the current procedures (sEcho, CPET, CA).

Cardiovascular Manifestations in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19 According to Age.

Cardiac involvement in multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus-19 disease is often observed with a high risk of heart failure. The aim is to describe cardiovascular involvement, management and early outcome in MIS-C by comparing cardiovascular manifestations in children younger and older than 6 years old. This retrospective observational study included 25 children with MIS-C, admitted to a single pediatric center between March 2020 and September 2021.

[An up-to-date telemedicine integrated clinical pathway for adult patients with congenital heart disease].

Clinical management of adult patients with congenital heart disease (GUCH) is a difficult task for multiple reasons, which include their own pathology and clinical history complexity, diagnostic complexity and organization of care. GUCH specialists are present in very small numbers and are concentrated in few centers, thus generating considerable transfer problems for patients. During the COVID-19 pandemic, telemedicine has become the standard of care, ensuring health assistance continuity, and implementing communication channels between patients and health professionals.

[Physical activity in patients with repaired and unrepaired congenital heart diseases. Task Force for exercise prescription in patients with congenital heart disease of the Italian Society of Pediatric Cardiology and Congenital Heart Disease].

Regular physical activity is essential for physical health and mental wellbeing in children and teenagers. However, patients with congenital heart disease are often restricted from being physically active due to parental overprotection and lack of physical activity promotion or exercise prescription from their physicians. A comprehensive medical evaluation is crucial for the development of personalized exercise programs for these patients.

Cardiac Manifestations in Children with SARS-COV-2 Infection: 1-Year Pediatric Multicenter Experience.

Since the spread of COVID-19, pediatric patients were initially considered less affected by SARS-COV-2, but current literature reported subsets of children with multisystem inflammatory syndrome (MIS-C). This study aims to describe the cardiac manifestation of SARS-COV-2 infection in a large cohort of children admitted to two Italian pediatric referral centers. Between March 2020 and March 2021, we performed a cardiac evaluation in 294 children (mean age 9 ± 5.

Cardiovascular MRI assessment of pectus excavatum in pediatric patients and postoperative simulation using vacuum bell.

Background: The sternal lift by Vacuum Bell (VB) is effective, as largely demonstrated by its intraoperative use during surgical procedure to elevate the sternum during the Nuss procedure routinely. Indeed, the thoracic remodelling during VB application is comparable to post-surgical scenario, and suitable to compare cardiovascular parameters of the two different thoracic configurations immediately.

Objective: We would quantify and correlate preoperative parameters which determine the severity of the pectus excavatum (PE), and the cardiovascular effects at the baseline.

Left ventricular dysfunction in repaired tetralogy of Fallot: incidence and impact on atrial arrhythmias at long term-follow up.

Left ventricle (LV) systolic dysfunction in repaired tetralogy of Fallot (TOF) has been identified as a risk factor for functional status and adverse outcome. The aims of this cross-sectional followed by a prospective study were: (1) to evaluate the prevalence of LV systolic dysfunction in a large cohort of adults with repaired tetralogy of Fallot, (2) to test the relationship between LV systolic dysfunction and other known risk factors and (3) to evaluate the impact of LV systolic dysfunction on adverse cardiac events. In a multicenter study, 237 adults repaired TOF (58 % males, age 30 ± 10 years) were evaluated by cardiac magnetic resonance (CMR).

Identification of TBX5 mutations in a series of 94 patients with Tetralogy of Fallot.

Tetralogy of Fallot (TOF) (OMIM #187500) is the most frequent conotruncal congenital heart defect (CHD) with a range of intra- and extracardiac phenotypes. TBX5 is a transcription factor with well-defined roles in heart and forelimb development, and mutations in TBX5 are associated with Holt-Oram syndrome (HOS) (OMIM#142900). Here we report on the screening of 94 TOF patients for mutations in TBX5, NKX2.

Tricuspid annular plane systolic excursion for the assessment of ventricular function in adults operated on with mustard procedure for complete transposition of the great arteries.

Background: In adult patients with d-transposition of the great arteries after atrial switch operation, dysfunction of the systemic right ventricle (RV) is a well-known complication. Echocardiographic variables may provide adequate estimation of subpulmonary RV function, but their applicability to the subaortic RV is not straightforward. We evaluate the concordance between tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging-derived ejection fraction of the RV (MRI-RVEF) in these patients.

Subclinical cardiac dysfunction and exercise performance in childhood cancer survivors.

Background: Although anthracycline cardiotoxicity is clearly related to the cumulative dose administered, subclinical cardiac dysfunction has been reported across a wide range of treatment regimens, and its clinical significance is still unclear. Purpose of this study is to investigate by exercise echocardiography for subclinical cardiac dysfunction in survivors of pediatric cancer treated with low-moderate anthracycline doses, and to evaluate whether it may alter the response of the cardiovascular system to dynamic exercise.

Procedure: Post-exercise left ventricular end-systolic wall stress (ESS), left ventricular posterior wall dimension and percent thickening at end systole, and cardiopulmonary exercise test-derived indexes of cardiac function were examined in 55 apparently healthy patients (mean age 13.

Stenting of aortic coarctation and exercise-induced hypertension in the young.

Objectives: To evaluate whether the stenting of aortic coarctation enhance the risk of exercise-induced hypertension (EIH).

Background: There is the theoretical concern that aortic stents may cause increased aortic wall impedance and therefore systolic hypertension during exercise.

Methods: Blood pressure and the Doppler derived peak and mean systolic pressure gradient (PSG and MSG) across the distal aorta at the peak of exercise were evaluated in young patients (mean age 14 +/- 3 years) with aortic coarctation successfully treated with surgery or with stent implantation at least 1 year before the test.

Aortic arch geometry and exercise-induced hypertension in aortic coarctation.

Hypertension at rest or during effort is not uncommon in patients with aortic coarctation (CoA), even those with a successful repair or mild degree of obstruction. Anatomic factors and functional abnormalities have been proposed as causes of this finding. Recently, aortic arch geometry was reported in association with hypertension at rest in patients with successful CoA repair.

Attenuation of induced bronchoconstriction in healthy subjects: effects of breathing depth.

The effects of breathing depth in attenuating induced bronchoconstriction were studied in 12 healthy subjects. On four separate, randomized occasions, the depth of a series of five breaths taken soon (approximately 1 min) after methacholine (MCh) inhalation was varied from spontaneous tidal volume to lung volumes terminating at approximately 80, approximately 90, and 100% of total lung capacity (TLC). Partial forced expiratory flow at 40% of control forced vital capacity (V(part)) and residual volume (RV) were measured at control and again at 2, 7, and 11 min after MCh.