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BackgroundCardiac dysfunction and endothelial damage are known complications of type 1 diabetes mellitus (T1D) mainly affecting adults. However, some studies have shown that subclinical myocardial impairment already present during adolescence. Myocardial work (MW) has emerged as an afterload-independent tool that allows early identification of subclinical damage. This study aims to provide a comprehensive non-invasive cardiovascular evaluation of T1D adolescents using both conventional and advanced echocardiography.MethodsWe enrolled 31 patients, aged between 13 and 19 years, who were diagnosed with T1D for at least 10 years and were followed up by the Paediatric Diabetology Unit of our institution. We collected data relating to anthropometry, lifestyle, blood tests, glycemic control parameters, and conventional and advanced echocardiographic measurements. A comparison of MW parameters with the data from 31 age- and sex-matched healthy volunteers from a previous study in our lab was carried out.ResultsIn our population, the glycemic control parameters showed suboptimal control. While diastolic parameters were in the normal range for all the patients, E′ velocities and left atrial diameter were significantly worse in patients with poorer glycemic controls. Global longitudinal strain (GLS), global work index (GWI), and global work efficiency (GWE) were significantly lower in the T1D population compared to those in the healthy population (p 99th percentile).ConclusionsTo the best of our knowledge, this was the first study to investigate MW in T1D adolescents. The descriptive parameters of GLS and MW showed subclinical cardiac damage already during this timeframe. Therefore, these tools should be integrated into the cardiovascular assessment of diabetic adolescents, and preventive strategies should be implemented to maximize glycemic and pressure control effectiveness.

Systemic right ventricle (sRV) dysfunction is frequent in patients with congenitally corrected transposition of great arteries (cc-TGA) and those with dextro-transposition of great arteries (D-TGA) after Mustard/Senning operations. This condition should be identified promptly. We aimed to compare echocardiographic parameters with cardiac magnetic resonance (CMR)-derived parameters in patients with sRV and to evaluate their correlation with clinical variables and exercise capacity. Patients with cc-TGA and D-TGA after Mustard/Senning who underwent standard and advanced (speckle tracking and 3D) echocardiography and CMR (including feature-speckle tracking) were included. Clinical and imaging parameters were collected. Echocardiographic-derived right ventricle end-diastolic area and end-systolic area correlated with 3D echocardiographic-derived right ventricle end-diastolic and end-systolic volume (r=0.6, p=0.006 and r=0.8, p=0.002). 3D ejection fraction (EF) correlated with fractional area change and tricuspid annular plane systolic excursion (TAPSE) (r=0.8, p=0.001 and r=0.7, p=0.03). sRV global longitudinal strain correlated with systemic atrial strain (sAS) (r=-0.6, p=0.01). CMR-derived EF correlated with CMR-derived global longitudinal strain (GLS) both endocardial and myocardial (r=-0.7, p=0.007 and r=-0.6, p=0.005). sRV areas as assessed by echo correlated with CMR-derived volumes (r=0.9, p=0.0001 for diastole and r=0.8, p=0.0001 for systole). Similarly, a correlation was found between sRV echo-derived GLS and CMR-derived GLS, both endocardial and myocardial (r=0.8, p=0.001 and r=0.7, p=0.01). The only imaging parameter which correlated with peak V02 was sAS (r=0.55, p=0.04). When comparing cc-TGA and D-TGA, the former showed better GLS-derived values as assessed by CMR (CMR-derived right ventricle endocardial longitudinal strain -23.2% versus -17.2%, p=0.002; CMR-derived right ventricle myocardial longitudinal strain -21.2% versus -16.7%; p=0.05), bigger systemic atrial area (20.2 cm2/m2 versus 8.4 cm2/m2, p=0.005) and higher TAPSE values (16.2 mm versus 12.2 mm, p=0.04). Echocardiography is valid to screen for sRV dilatation and function and to guide the timing for CMR. The investigation of atrial deformation imaging may help to better understand diastolic function. Patients with cc-TGA show better cardiac function compared to patients after atrial switch. Further investigations are needed to identify imaging parameters linked to exercise capacity.

Advances in pediatric cardiac surgery have resulted in a recent growing epidemic of children and young adults with congenital heart diseases (CHDs). In these patients, congenital defects themselves, surgical operations and remaining lesions may alter cardiac anatomy and impact the mechanical performance of both ventricles. Cardiac function significantly influences outcomes in CHDs, necessitating regular patient follow-up to detect clinical changes and relevant risk factors. Echocardiography remains the primary imaging method for CHDs, but clinicians must understand patients' unique anatomies as different CHDs exhibit distinct anatomical characteristics affecting cardiac mechanics. Additionally, the use of myocardial deformation imaging and 3D echocardiography has gained popularity for enhanced assessment of cardiac function and anatomy. This paper discusses the role of echocardiography in evaluating cardiac mechanics in most significant CHDs, particularly its ability to accommodate and interpret the inherent anatomical substrate in these conditions.

The complete transposition of the great arteries (C-TGA) is a congenital cardiac anomaly characterized by the reversal of the main arteries. Early detection and precise management are crucial for optimal outcomes. This review emphasizes the integral role of multimodal imaging, including fetal echocardiography, transthoracic echocardiography (TTE), cardiovascular magnetic resonance (CMR), and cardiac computed tomography (CCT) in the diagnosis, treatment planning, and long-term follow-up of C-TGA. Fetal echocardiography plays a pivotal role in prenatal detection, enabling early intervention strategies. Despite technological advances, the detection rate varies, highlighting the need for improved screening protocols. TTE remains the cornerstone for initial diagnosis, surgical preparation, and postoperative evaluation, providing essential information on cardiac anatomy, ventricular function, and the presence of associated defects. CMR and CCT offer additional value in C-TGA assessment. CMR, free from ionizing radiation, provides detailed anatomical and functional insights from fetal life into adulthood, becoming increasingly important in evaluating complex cardiac structures and post-surgical outcomes. CCT, with its high-resolution imaging, is indispensable in delineating coronary anatomy and vascular structures, particularly when CMR is contraindicated or inconclusive. This review advocates for a comprehensive imaging approach, integrating TTE, CMR, and CCT to enhance diagnostic accuracy, guide therapeutic interventions, and monitor postoperative conditions in C-TGA patients. Such a multimodal strategy is vital for advancing patient care and improving long-term prognoses in this complex congenital heart disease.

Systemic hypertension has been considered mainly as an adult health issue for a long time, but it is now being increasingly acknowledged as a significant problem also among pediatric patients. The frequency of pediatric hypertension has grown mostly because of increases in childhood obesity and sedentary lifestyles, but secondary forms of hypertension play a role as well. Considering that unaddressed hypertension during childhood can result in enduring cardiovascular complications, timely identification and intervention are essential. Strategies for addressing this disease encompass not only lifestyle adjustments, but also the use of medications when needed. Lifestyle modifications entail encouraging a nutritious diet, consistent physical activity, and the maintenance of a healthy weight. Moreover, educating both children and their caregivers about monitoring blood pressure at home can aid in long-term management. Thus, the aim of this review is to discuss the etiologies, classification, and principles of the treatment of hypertension in pediatric patients.

Aortic regurgitation (AR) is common after aortic balloon valvuloplasty in children and it has been associated with large balloon/annulus ratio, abnormal valve morphology and aortic valve prolapse. We present a rare case of severe AR after aortic balloon valvuloplasty due to aortic strands rupture, causing prolapse of the co-joined cusp. These findings were identified by 3D echocardiography, highlighting the importance of this imaging technique even in pediatric patients.

Long-term effects of systemic hypertension (HTN) and HTN-mediated damages have been largely studied in non-congenital adult populations. By contrast, robust data about the predisposing factors, prevalence, consequences, and treatment of HTN in adults with congenital heart diseases (ACHD) is still scarce. Different mechanisms including the underlying cardiac disease, cardiac surgery and its consequences, the development of metabolic syndrome and secondary forms seem to play a role in HTN in ACHDs. To mitigate the potential long-term effects of HTN in this complex population, a meticulous follow-up is mandatory to identify patients who should receive treatment, and tailored strategies should be applied to obtain the best as possible result.Thus, this review will investigate risk factors, effects, and treatments of HTN in ACHD patients.

The arterial switch operation (ASO) has become the standard surgical treatment for patients with d-transposition of the great arteries (d-TGA). While ASO has significantly improved survival rates, a subset of patients develops a unique anatomical anomaly known as the gothic aortic arch (GAA). Understanding cardiac mechanics in this population is crucial as altered mechanics can have profound consequences on cardiac function and exercise capacity. The GAA has been associated with changes in ventricular function, hemodynamic, and exercise capacity. Studies have shown a correlation between the GAA and decreased ascending aorta distensibility, loss of systolic wave amplitude across the aortic arch, and adverse cardiovascular outcomes. Various imaging techniques, including echocardiography, cardiac magnetic resonance imaging (CMR), and cardiac computed tomography (CCT), play a crucial role in assessing cardiac mechanics and evaluating the GAA anomaly. Despite significant advancements, gaps in knowledge regarding the prognostic implications and underlying mechanisms of the GAA anomaly remain. This review aims to explore the implications of the GAA anomaly on cardiac mechanics and its impact on clinical outcomes in young patients after ASO. Advancements in imaging techniques, such as computational modeling, offer promising avenues to enhance our understanding of cardiac mechanics and improve clinical management.

BackgroundIn children with congenital long QT syndrome (LQTS), the risk of arrhythmic events during exercise commonly makes it difficult to balance exercise restrictions versus promotion of physical activity. Nevertheless, in children with LQTS, cardiorespiratory fitness, muscle fitness, and physical activity, have been scarcely explored.Materials and methodsIn this prospective, controlled, cross-sectional study, 20 children with LQTS (12.7 ± 3.7 years old) and 20 healthy controls (11.9 ± 2.4 years old) were enrolled. All participants underwent a cardiopulmonary exercise test, a muscular architecture ultrasound assessment, (cross-sectional area on right rectus femoris and pennation angle), a handgrip muscular strength evaluation, and a standing long broad jump test. The level of physical activity was determined using with a waist-worn tri-axial accelerometer (Actigraph GT3X).ResultsPeak oxygen uptake (VO2peak) and ventilatory anaerobic threshold (VAT) were lower in children with LQTS than in healthy controls (33.9 ± 6.2 mL/Kg/min vs. 40.1 ± 6.6 mL/Kg/min, P = 0.010; 23.8 ± 5.1 mL/Kg/min vs. 28.8 ± 5.5 mL/Kg/min, P = 0.007, respectively). Children with LQTS had lower standing long broad jump distance (119.5 ± 33.2 cm vs. 147.3 ± 36.1 cm, P = 0.02) and pennation angle (12.2 ± 2.4° vs. 14.3 ± 2.8°, P = 0.02). No differences in terms of moderate-to-vigorous physical activity were observed (36.9 ± 12.9 min/day vs. 41.5 ± 18.7 min/day, P = 0.66), but nearly all children were below the WHO guidelines.ConclusionDespite similar physical activity level, cardiorespiratory fitness and muscle fitness in children with LQTS were lower than in healthy controls. The origin of this limitation seemed to be multifactorial, involving beta-blocker induced chronotropic limitation, physical and muscle deconditioning. Cardiovascular rehabilitation could be of interest in children with LQTS with significant physical limitation.

The field of prenatal cardiac imaging has revolutionized the way we understand and manage congenital heart diseases (CHD) in the developing fetus. In the prenatal period, cardiac imaging plays a pivotal role in the diagnostic pathway, from screening to classification and follow-up of CHD. The ability to visualize the fetal heart in utero allows healthcare professionals to detect abnormalities early, thus enabling timely interventions and informed decision-making processes for both the mother and the medical team. Early CHD detection improves preparation for delivery, postnatal care, and postnatal outcomes. Advancements in medical technology and imaging techniques have provided clinicians with insights into the fascinating workings of the fetal heart. Several imaging modalities have proven to be helpful in this field, with echocardiography undoubtedly representing the primary modality for evaluating the fetus. By providing detailed anatomical and functional information, fetal cardiac magnetic resonance (CMR) imaging contributes to better prenatal counseling and enhances the coordination of care between obstetricians, maternal–fetal medicine specialists, and pediatric cardiologists. Shortcomings of fetal CMR are due to technical concerns related to the intrauterine position of the fetus and subsequent challenges to following a standard scan protocol. The aim of this paper was to revise the current state-of-the-art in the field of fetal CMR and its clinical applications and to delve into methods, challenges, and future directions of fetal CMR in prenatal imaging.

Tetralogy of Fallot (TOF) is the most common complex congenital heart disease with long-term survivors, demanding serial monitoring of the possible complications that can be encountered from the diagnosis to long-term follow-up. Cardiovascular imaging is key in the diagnosis and serial assessment of TOF patients, guiding patients’ management and providing prognostic information. Thorough knowledge of the pathophysiology and expected sequalae in TOF, as well as the advantages and limitations of different non-invasive imaging modalities that can be used for diagnosis and follow-up, is the key to ensuring optimal management of patients with TOF. The aim of this manuscript is to provide a comprehensive overview of the role of each modality and common protocols used in clinical practice in the assessment of TOF patients.

The Fontan operation represents the last of multiple steps that are offered a wide range of congenital cardiac lesions with a single ventricle (SV) physiology. Nowadays this surgical program consists of a total cavopulmonary connection (TCPC), by anastomosing systemic veins to the pulmonary arteries (PAs), excluding the right-sided circulation from the heart. As a result of imaging, surgical, percutaneous, and critical care improvements, survival in this population has steadily increased. However, the Fontan physiology chronically increases systemic venous pressure causing systemic venous congestion and decreased cardiac output, exposing patients to the failure of the Fontan circulation (FC), which is associated with a wide variety of clinical complications such as liver disease, cyanosis, thromboembolism, protein-losing enteropathy (PLE), plastic bronchitis (PB), and renal dysfunction, ultimately resulting in an increased risk of exercise intolerance, arrhythmias, and premature death. The pathophysiology of the failing Fontan is complex and multifactorial; i.e., caused by the single ventricle dysfunction (diastolic/systolic failure, arrhythmias, AV valve regurgitation, etc.) or caused by the specific circulation (conduits, pulmonary vessels, etc.). The treatment is still challenging and may include multiple options and tools. Among the possible options, today, interventional catheterization is a reliable option, through which different procedures can target various failing elements of the FC. In this review, we aim to provide an overview of indications, techniques, and results of transcatheter options to treat cavopulmonary stenosis, collaterals, impaired lymphatic drainage, and the management of the fenestration, as well as to explore the recent advancements and clinical applications of transcatheter cavopulmonary connections, percutaneous valvular treatments, and to discuss the future perspectives of percutaneous therapies in the Fontan population.

Percutaneous interventions have completely refashioned the management of children with congenital heart diseases (CHD) and the use of non-invasive imaging has become the gold standard to plan and guide these procedures in the modern era. We are now facing a dual challenge to improve the standard of care in low-risk patients, and to shift our strategies from the classic open chest surgery to imaging-guided percutaneous interventions in high-risk patients. Such rapid evolution of ultrasound technologies over the last 20 years have permitted the integration of transthoracic, transesophageal and intracardiac echocardiography into the interventional workflow to improve image guidance and reduce radiation burden from fluoroscopy and angiography. Specifically, miniaturization of transesophageal probe and advances in three-dimensional (3D) imaging techniques have enabled real-time 3D image guidance during complex interventional procedure, In addition, multimodality and fusion imaging techniques harness the strengths of different modalities to enhance understanding of anatomical and spatial relationship between different structures, improving communication and coordination between interventionalists and imaging specialists. In this review, we aim to provide an overview of 3D imaging modalities and multimodal fusion in procedural planning and live guidance of percutaneous interventions. At the present times, 3D imaging can no longer be considered a luxury but a routine clinical tool to improve procedural success and patient outcomes.

AimsThis international multi-center study aimed to demonstrate the feasibility and reliability of non-invasive myocardial work (MW) parameters in the pediatric population, and to provide normal reference ranges for this useful echocardiographic tool in this specific subset of patients.Methods and ResultsIn this retrospective multi-center study involving three pediatric laboratories, 150 healthy children and adolescents (mean age of 10.6 ± 4.5, 91 males) were enrolled. A complete echocardiographic examination has been performed, including global longitudinal strain (GLS) assessment. The following parameters of non-invasive MW have been obtained through a dedicated software: global work index (GWI), global constructive work (GCW), global work waste (GWW), and global work efficiency (GWE), using left ventricular (LV) strain loops and non-invasive brachial artery cuff pressure values. The lowest expected values were for GWI 1,723 mmHg% in males and 1,682 mmHg% in females, for GCW 2,089 and 2,106 mmHg%, for GWE 95.9 and 95.5% whereas the highest expected value for GWW was 78 mmHg% in men and 90 mmHg% in women. The univariable and multivariable analysis showed significant associations between either GWI or GCW with SBP (β coefficient = 0.446, p < 0.001; β coefficient = 0.456, p < 0.001, respectively) and LV GLS (β coefficient = −0.268, p = 0.001; β coefficient = −0.233, p = 0.003, respectively). Inter- and intra-observer variability showed good reproducibility of non-invasive MW parameters.ConclusionNon-invasive MW parameters were feasible and reliable in the pediatric population. This study provided normal reference ranges of these useful echocardiographic indices.

Since its first description in 1971, the Fontan procedure and its modifications have led to a substantial improvement in the survival rates of patients with a variety of types of complex Congenital Heart Disease (CHD) characterised by the presence of a single, dominant ventricle. However, despite the significant improvement of the prognosis over the years, Fontan patients are still exposed to several cardiovascular and systemic complications. It is, therefore, important to fully understand the pitfalls hidden behind a Fontan anatomy and the potential predictors of ventricular failure. Cardiovascular imaging plays a key role in this context, allowing for the early identification of complications with important prognostic implications. Echocardiography remains the first-line imaging modality for serial evaluation of Fontan patients. However, there is a growing role of cardiovascular magnetic resonance and cardiac computed tomography from pre-operative assessment to longitudinal follow-up. The aim of this paper will be to provide a comprehensive overview of the role, strengths, and weaknesses of each imaging modality in the assessment of congenital cardiac conditions palliated with the Fontan procedure.