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. This second document also examines the impact of devices and prostheses for CMR and CCT in CHD and additionally presents some indications for CMR and CCT exams when sedation or narcosis is needed.

Spectral Photon-Counting Computed Tomography: Technical Principles and Applications in the Assessment of Cardiovascular Diseases.

Spectral Photon-Counting Computed Tomography (SPCCT) represents a groundbreaking advancement in X-ray imaging technology. The core innovation of SPCCT lies in its photon-counting detectors, which can count the exact number of incoming x-ray photons and individually measure their energy. The first part of this review summarizes the key elements of SPCCT technology, such as energy binning, energy weighting, and material decomposition. Its energy-discriminating ability represents the key to the increase in the contrast between different tissues, the elimination of the electronic noise, and the correction of beam-hardening artifacts. Material decomposition provides valuable insights into specific elements' composition, concentration, and distribution. The capability of SPCCT to operate in three or more energy regimes allows for the differentiation of several contrast agents, facilitating quantitative assessments of elements with specific energy thresholds within the diagnostic energy range. The second part of this review provides a brief overview of the applications of SPCCT in the assessment of various cardiovascular disease processes. SPCCT can support the study of myocardial blood perfusion and enable enhanced tissue characterization and the identification of contrast agents, in a manner that was previously unattainable.

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).

Background: 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. Design: The CMR/CT WG of the Italian Pediatric Cardiology Society set up a multicenter observational clinical database of repaired TOF evaluations. This registry will enroll patients retrospectively and prospectively assessed by CMR for clinical indication in many congenital heart diseases (CHD) Italian centers. Data collection in a dedicated platform will include surgical history, clinical data, imaging data, and adverse cardiac events at 6 years of follow-up. Summary: The multicenter repaired TOF clinical database will collect data on patients evaluated by CMR in many CHD centers in Italy. The registry has been set up to allow future research studies in this population to improve clinical/surgical management and risk stratification of this population.

Biventricular Tissue Tracking with Cardiovascular Magnetic Resonance: Reference Values of Left- and Right-Ventricular Strain.

We derived reference values of left-ventricular (LV) and right-ventricular (RV) strain parameters in a cohort of 100 healthy subjects by feature tracking cardiac magnetic resonance (FT-CMR). Global and regional strain values were calculated for the LV; circumferential and radialSAX strain parameters were derived from the short-axis (SAX) stack, while longitudinal and radialLAX strain parameters were assessed in three long-axis (LAX) views. Only global longitudinal strain (GLS) was calculated for the RV. Peak global LV circumferential strain was -16.7% ± 2.1%, LV radialSAX strain was 26.4% ± 5.1%, LV radialLAX strain was 31.1% ± 5.2%, LV GLS was -17.7% ± 1.9%, and RV GLS was -23.9% ± 4.1%. Women presented higher global LV and RV strain values than men; all strain values presented a weak relationship with body surface area, while there was no association with age or heart rate. A significant association was detected between all LV global strain measures and LV ejection fraction, while RV GLS was correlated to RV end-diastolic volume. The intra- and inter-operator reproducibility was good for all global strain measures. In the regional analysis, circumferential and radial strain values resulted higher at the apical level, while longitudinal strain values were higher at the basal level. The assessment of cardiac deformation by FT-CMR is feasible and reproducible and gender-specific reference values should be used.

Cardiovascular Applications of Photon-Counting CT Technology: A Revolutionary New Diagnostic Step.

Photon-counting computed tomography (PCCT) is an emerging technology that can potentially transform clinical CT imaging. After a brief description of the PCCT technology, this review summarizes its main advantages over conventional CT: improved spatial resolution, improved signal and contrast behavior, reduced electronic noise and artifacts, decreased radiation dose, and multi-energy capability with improved material discrimination. Moreover, by providing an overview of the existing literature, this review highlights how the PCCT benefits have been harnessed to enhance and broaden the diagnostic capabilities of CT for cardiovascular applications, including the detection of coronary artery calcifications, evaluation of coronary plaque extent and composition, evaluation of coronary stents, and assessment of myocardial tissue characteristics and perfusion.

Role of Imaging in Cardiomyopathies.

Imaging has a central role in the diagnosis, classification, and clinical management of cardiomyopathies. While echocardiography is the first-line technique, given its wide availability and safety, advanced imaging, including cardiovascular magnetic resonance (CMR), nuclear medicine and CT, is increasingly needed to refine the diagnosis or guide therapeutic decision-making. In selected cases, such as in transthyretin-related cardiac amyloidosis or in arrhythmogenic cardiomyopathy, the demonstration of histological features of the disease can be avoided when typical findings are observed at bone-tracer scintigraphy or CMR, respectively. Findings from imaging techniques should always be integrated with data from the clinical, electrocardiographic, biomarker, genetic and functional evaluation to pursue an individualised approach to patients with cardiomyopathy.

3D Printed Model-Guided Neonatal Transcatheter Closure of Left Main Coronary Artery-to-Right Ventricle Fistula.

We report on a 2-week-old infant with huge left main coronary artery-to-right ventricular outflow tract fistula causing myocardial ischemia due to global coronary steal who was successfully submitted to percutaneous closure guided by a 3-dimensional-printed model using a duct-occluder vascular plug. (Level of Difficulty: Advanced.).

Dual Source Photon-Counting Computed Tomography-Part II: Clinical Overview of Neurovascular Applications.

Photon-counting detector (PCD) is a novel computed tomography detector technology (photon-counting computed tomography-PCCT) that presents many advantages in the neurovascular field, such as increased spatial resolution, reduced radiation exposure, and optimization of the use of contrast agents and material decomposition. In this overview of the existing literature on PCCT, we describe the physical principles, the advantages and the disadvantages of conventional energy integrating detectors and PCDs, and finally, we discuss the applications of the PCD, focusing specifically on its implementation in the neurovascular field.

Dual-Source Photon-Counting Computed Tomography-Part I: Clinical Overview of Cardiac CT and Coronary CT Angiography Applications.

The photon-counting detector (PCD) is a new computed tomography detector technology (photon-counting computed tomography, PCCT) that provides substantial benefits for cardiac and coronary artery imaging. Compared with conventional CT, PCCT has multi-energy capability, increased spatial resolution and soft tissue contrast with near-null electronic noise, reduced radiation exposure, and optimization of the use of contrast agents. This new technology promises to overcome several limitations of traditional cardiac and coronary CT angiography (CCT/CCTA) including reduction in blooming artifacts in heavy calcified coronary plaques or beam-hardening artifacts in patients with coronary stents, and a more precise assessment of the degree of stenosis and plaque characteristic thanks to its better spatial resolution. Another potential application of PCCT is the use of a double-contrast agent to characterize myocardial tissue. In this current overview of the existing PCCT literature, we describe the strengths, limitations, recent applications, and promising developments of employing PCCT technology in CCT.

Dual-Source Photon-Counting Computed Tomography-Part III: Clinical Overview of Vascular Applications beyond Cardiac and Neuro Imaging.

Photon-counting computed tomography (PCCT) is an emerging technology that is expected to radically change clinical CT imaging. PCCT offers several advantages over conventional CT, which can be combined to improve and expand the diagnostic possibilities of CT angiography. After a brief description of the PCCT technology and its main advantages we will discuss the new opportunities brought about by PCCT in the field of vascular imaging, while addressing promising future clinical scenarios.

Transcatheter Treatment of Native Idiopathic Multiloculated Aortic Aneurysm Guided by 3D Printing Technology.

Pediatric idiopathic aortic aneurysm is rare. Single saccular malformation can complicate native or recurrent aortic coarctation; however, multiloculated dilatations of the descending thoracic aorta, associated with aortic coarctation, have so far never been described in literature. In our case, printed 3D model technology was crucial in planning transcatheter treatment. (Level of Difficulty: Intermediate.).

Photon-Counting Computed Tomography (PCCT): Technical Background and Cardio-Vascular Applications.

Photon-counting computed tomography (PCCT) is a new advanced imaging technique that is going to transform the standard clinical use of computed tomography (CT) imaging. Photon-counting detectors resolve the number of photons and the incident X-ray energy spectrum into multiple energy bins. Compared with conventional CT technology, PCCT offers the advantages of improved spatial and contrast resolution, reduction of image noise and artifacts, reduced radiation exposure, and multi-energy/multi-parametric imaging based on the atomic properties of tissues, with the consequent possibility to use different contrast agents and improve quantitative imaging. This narrative review first briefly describes the technical principles and the benefits of photon-counting CT and then provides a synthetic outline of the current literature on its use for vascular imaging.

Association of Circulating Neutrophils with Relative Volume of Lipid-Rich Necrotic Core of Coronary Plaques in Stable Patients: A Substudy of SMARTool European Project.

BACKGROUND AND AIMS: Coronary atherosclerosis is a chronic non-resolving inflammatory process wherein the interaction of innate immune cells and platelets plays a major role. Circulating neutrophils, in particular, adhere to the activated endothelium and migrate into the vascular wall, promoting monocyte recruitment and influencing plaque phenotype and stability at all stages of its evolution. We aimed to evaluate, by flow cytometry, if blood neutrophil number and phenotype-including their phenotypic relationships with platelets, monocytes and lymphocytes-have an association with lipid-rich necrotic core volume (LRNCV), a generic index of coronary plaque vulnerability, in a group of stable patients with chronic coronary syndrome (CCS). METHODS: In 55 patients, (68.53 ± 1.07 years of age, mean ± SEM; 71% male), the total LRNCV in each subject was assessed by a quantitative analysis of all coronary plaques detected by computed tomography coronary angiography (CTCA) and was normalized to the total plaque volume. The expression of CD14, CD16, CD18, CD11b, HLA-DR, CD163, CCR2, CCR5, CX3CR1, CXCR4 and CD41a cell surface markers was quantified by flow cytometry. Adhesion molecules, cytokines and chemokines, as well as MMP9 plasma levels, were measured by ELISA. RESULTS: On a per-patient basis, LRNCV values were positively associated, by a multiple regression analysis, with the neutrophil count (n°/µL) (p = 0.02), neutrophil/lymphocyte ratio (p = 0.007), neutrophil/platelet ratio (p = 0.01), neutrophil RFI CD11b expression (p = 0.02) and neutrophil-platelet adhesion index (p = 0.01). Significantly positive multiple regression associations of LRNCV values with phenotypic ratios between neutrophil RFI CD11b expression and several lymphocyte and monocyte surface markers were also observed. In the bivariate correlation analysis, a significantly positive association was found between RFI values of neutrophil-CD41a+ complexes and neutrophil RFI CD11b expression (p < 0.0001). CONCLUSIONS: These preliminary findings suggest that a sustained increase in circulating neutrophils, together with the up-regulation of the integrin/activation membrane neutrophil marker CD11b may contribute, through the progressive intra-plaque accumulation of necrotic/apoptotic cells exceeding the efferocytosis/anti-inflammatory capacity of infiltrating macrophages and lymphocytes, to the relative enlargement of the lipid-rich necrotic core volume of coronary plaques in stable CAD patients, thus increasing their individual risk of acute complication.

Intravenous Contrast Material for Cardiac Computed Tomography: Results From the Open-label Multicenter, Multivendor Italian Registry of Contrast Material Use in Cardiac Computed Tomography.

PURPOSE: The Italian Registry of Contrast Material use in Cardiac Computed Tomography (iRCM-CCT) is a multicenter, multivendor, observational study on the use of contrast media (CM) in patients undergoing cardiac computed tomography (CCT). The aim of iRCM-CCT is to assess image quality and safety profile of intravenous CM compounds. MATERIALS AND METHODS: iRCM-CCT enrolled 1842 consecutive patients undergoing CCT (≥50 per site) at 20 cluster sites with the indication of suspected coronary artery disease. Demographic characteristics, CCT, and CM protocols, clinical indications, safety markers, radiation dose reports, qualitative (ie, poor vascular enhancement) and quantitative (ie, HU attenuation values) image parameters were recorded. A centralized coordinating center collected and assessed all image parameters. RESULTS: The cohort included 891 men and 951 women (age: 63±14 y, body mass index: 26±4 kg/m2) studied with ≥64 detector rows computed tomography scanners and different iodinated intravenous CM protocols and compounds (iodixanol, iopamidol, iohexol, iobitridol, iopromide, and iomeprol). The following vascular attenuation was reported: 504±147 HU in the aorta, 451±146 HU in the right coronary artery, 474±146 HU in the left main, 451±146 HU in the left anterior descending artery, and 441±149 HU in the circumflex artery. In 4% of cases the image quality was not satisfactory due to poor enhancement. The following adverse reactions to CM were recorded: 6 (0.3%) extravasations and 17 (0.9%) reactions (11 mild, 4 moderate, 2 severe). CONCLUSIONS: In a multicenter registry on CM use during CCT the prevalence of CM-related adverse reactions was very low. The appropriate use of CM is a major determinant of image quality.

Use of cardiac imaging in chronic coronary syndromes: the EURECA Imaging registry.

BACKGROUND: The prospective, multicentre EURECA registry assessed the use of imaging and adoption of the European Society of Cardiology (ESC) Guidelines (GL) in patients with chronic coronary syndromes (CCS). METHODS: Between May 2019 and March 2020, 5156 patients were recruited in 73 centres from 24 ESC member countries. The adoption of GL recommendations was evaluated according to clinical presentation and pre-test probability (PTP) of obstructive coronary artery disease (CAD). RESULTS: The mean age of the population was 64 ± 11 years, 60% of patients were males, 42% had PTP >15%, 27% had previous CAD, and ejection fraction was <50% in 5%. Exercise ECG was performed in 32% of patients, stress imaging as the first choice in 40%, and computed tomography coronary angiography (CTCA) in 22%. Invasive coronary angiography (ICA) was the first or downstream test in 17% and 11%, respectively. Obstructive CAD was documented in 24% of patients, inducible ischaemia in 19%, and 13% of patients underwent revascularization. In 44% of patients, the overall diagnostic process did not adopt the GL. In these patients, referral to stress imaging (21% vs. 58%; P < 0.001) or CTCA (17% vs. 30%; P < 0.001) was less frequent, while exercise ECG (43% vs. 22%; P < 0.001) and ICA (48% vs. 15%; P < 0.001) were more frequently performed. The adoption of GL was associated with fewer ICA, higher proportion of diagnosis of obstructive CAD (60% vs. 39%, P < 0.001) and revascularization (54% vs. 37%, P < 0.001), higher quality of life, fewer additional testing, and longer times to late revascularization. CONCLUSIONS: In patients with CCS, current clinical practice does not adopt GL recommendations on the use of diagnostic tests in a significant proportion of patients. When the diagnostic approach adopts GL recommendations, invasive procedures are less frequently used and the diagnostic yield and therapeutic utility are superior.

Advantages of Photon-Counting Detector CT in Aortic Imaging.

Photon-counting Computed Tomography (PCCT) is a promising imaging technique. Using detectors that count the number and energy of photons in multiple bins, PCCT offers several advantages over conventional CT, including a higher image quality, reduced contrast agent volume, radiation doses, and artifacts. Although PCCT is well established for cardiac imaging in assessing coronary artery disease, its application in aortic imaging remains limited. This review summarizes the available literature and provides an overview of the current use of PCCT for the diagnosis of aortic imaging, focusing mainly on endoleaks detection and characterization after endovascular aneurysm repair (EVAR), contrast dose volume, and radiation exposure reduction, particularly in patients with chronic kidney disease and in those requiring follow-up CT.

Prognostic Value of Coronary Calcium Score in Asymptomatic Individuals: A Systematic Review.

Despite updated guidelines and technological developments that allow for an accurate diagnosis, many asymptomatic individuals have a high risk of developing CAD or cardiac events. The CAC score can estimate a correct risk level for these subjects, which is clinically significant for adequate management of risk factors and obtaining personalized preventive therapy. This systematic review aims to assess the prognostic value of CAC score in asymptomatic individuals. According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, a systematic literature search was performed to identify original articles since 2010 that evaluated the prognostic value of CAC score in asymptomatic individuals. The quality of the included studies was assessed by the QUIPS tool. A total of 45 articles were selected. Many of these (25 studies) evaluated the prognostic value of CAC score in asymptomatic subjects. In comparison, others (20 studies) evaluated the association of CAC score with other clinical parameters and imaging modalities or the comparison with computed tomography coronary angiography (CTCA). Our findings showed that the CAC score provides valuable prognostic information for predicting CAD risk in asymptomatic individuals.

Coronary Artery Bypass Salvage With Branched Aortic Endograft in a Patient With Aortic Arch Ulcer.

Treatment of multiple penetrating aortic ulcers becomes troublesome if they involve the aortic arch. We report a percutaneous aortic repair using a unibody design endoprosthesis with a precannulated side component for the left subclavian artery to manage a large aortic arch ulcer and preserve the patency of previous coronary artery bypass graft. (Level of Difficulty: Advanced.).

Etiology, clinical presentation, and management of left main coronary artery aneurysms.

BACKGROUND AND AIM OF THE STUDY: The widespread use of noninvasive/invasive coronary imaging increased the probability of recognition of coronary aneurysms. Left main coronary aneurysms (LMCA), though rare, are potentially life-threatening but in the absence of controlled studies, guidelines do not provide any specific recommendation for their management. We, therefore, aimed to investigate the epidemiology, clinical presentation, therapeutic strategies, and prognostic implication of LMCA. METHODS: A systematic review of the literature was performed to retrieve all the reported cases of LMCA as of December 2021, which were summarized and classified according to their etiology, clinical presentation, and therapeutic management. RESULTS: Out of 1997 works retrieved, 180 studies were analyzed, describing 209 LMCA cases (aged 51 ± 19 years, 68% males). Atherosclerosis was the most common etiology (40%), followed by inflammatory (12%), congenital (9%), or degenerative (6%) conditions. Stable angina (43%) and acute coronary syndromes (32%) were more often the first clinical manifestations, while 29 (14%) LMCA were incidental findings. Most cases were treated surgically (53%), while percutaneous intervention was rarely adopted (7%). Data about antithrombotic therapies were scarce and heterogeneous. Finally, when longitudinal data were reported (n = 81), LMCA resulted associated with a severe prognosis, with a 15% mortality over an 8-month median follow-up. CONCLUSIONS: LMCA are most frequently, but not exclusively, caused by advanced atherosclerosis. Irrespective of their etiology and clinical presentation, LMCA may be associated with high short-term mortality. In absence of controlled studies, a careful evaluation of each case is warranted to optimize therapeutic strategies.

Multimodality Approach to a Complex Scimitar Syndrome: How Advanced Diagnostics Can Guide Therapeutic Strategies.

We report an unusual association of scimitar syndrome with right diaphragmatic hernia, left-sided aortic arch with "aberrant right subclavian artery" in a 2-year-old boy who underwent stepwise transcatheter occlusion of a significant aortopulmonary collateral followed by surgical treatment for the repair of the diaphragmatic hernia and esophageal compression. (Level of Difficulty: Advanced.).