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Background/Objective: The impact of subpulmonary left ventricle (LV) dysfunction in patients with a systemic right ventricle (SRV) is insufficiently characterized, with only a few studies suggesting its prognostic significance. Additionally, its evaluation through imaging techniques is a challenge. To assess the correlation between quantitative cardiac magnetic resonance-feature tracking (CMR-FT) data and the risk of clinical events related to the natural history of SRV failure. Methods: In this cross-sectional study, 21 patients with a diagnosis of transposition of the great arteries (TGA) and atrial switch operation (AtSO) or congenitally corrected transposition (ccTGA) were recruited. All participants underwent CMR-FT analysis. Considered clinical events included NYHA class deterioration (from I-II to III-IV), increased diuretic therapy, arrhythmias, sudden cardiac death, and hospitalizations. Results: The cohort consisted of 52.4% males (mean age: 25.4 ± 11.9 years). Eleven patients were diagnosed with ccTGA. Of the 10 patients with TGA post-AtSO, 50% had undergone Mustard repair. Clinical events occurred in 11 patients, with 47.6% experiencing hospitalizations and 28.6% developing arrhythmias. Left ventricular global longitudinal strain (LV GLS) was significantly associated with event-risk in both univariate and multivariate analyses (p = 0.011; p = 0.025). A cut-off value of LV GLS > −19.24 was proposed to stratify high-risk patients (p = 0.001). Conclusions: Our study confirms the role of subpulmonary LV function in determining outcomes of SRV patients. The assessment of LV GLS by using CMR-FT could significantly enhance clinical management during follow-up. [ABSTRACT FROM AUTHOR]

Heart failure (HF) remains a critical global health challenge, necessitating advancements in diagnostic and therapeutic strategies. This review explores the evolution of imaging technologies and their impact on HF management, focusing on three-dimensional echocardiography (3DE), myocardial strain imaging, and vortex dynamics imaging. Three-dimensional echocardiography enhances traditional echocardiography by providing more accurate assessments of cardiac structures, while myocardial strain imaging offers the early detection of subclinical myocardial dysfunction, crucial in conditions such as chemotherapy-induced cardiotoxicity and ischemic heart disease. Vortex dynamics imaging, a novel technique, provides insights into intracardiac flow patterns, aiding in the evaluation of left ventricular function, valve diseases, and congenital heart anomalies. The integration of these advanced imaging modalities into clinical practice facilitates personalized treatment strategies, enabling the earlier diagnosis and more precise monitoring of disease progression. The ongoing refinement of these imaging techniques holds promise for improving patient outcomes and advancing the field of precision medicine in HF care. [ABSTRACT FROM AUTHOR]

Background: Aortic coarctation (CoA) is a congenital heart disease affecting 5–8% of patients, with long-term complications persisting despite successful correction. Stress echocardiography (SE) is increasingly used for evaluating cardiac function under stress, yet its role in repaired CoA remains under-explored. Objective: This study aimed to assess the predictive value of SE and myocardial strain in repaired CoA patients with a history of hypertension without significant gradients or with borderline gradients at rest. Methods: Between June 2020 and March 2024, we enrolled 35 consecutive CoA patients with successful repairs and either a history of hypertension or borderline Doppler gradients. Baseline and peak exercise echocardiographic measurements, including left ventricular mass index (LVMi) and global longitudinal strain (LVGLS), were recorded. Patients were followed for up to 4 years. Results: At baseline, the positive SE group had higher systolic blood pressure (SBP) and diastolic blood pressure (DBP) compared to the negative SE group. The positive SE group also exhibited significantly higher basal and peak trans-isthmic gradients. Positive SE was found in 45.7% of patients, with 68.7% of these requiring re-intervention during follow-up. A peak trans-isthmic gradient > 61 mmHg during exercise predicted recoarctation with 100% sensitivity and 71% specificity (AUC = 0.836, p < 0.004). Conclusions: SE identifies at-risk patients post-CoA repair, aiding in early intervention. A peak trans-isthmic gradient > 61 mmHg during exercise is a strong predictor of recoarctation. These findings support incorporating SE into routine follow-up protocols for CoA patients, particularly those with a history of hypertension and borderline gradients, to improve long-term outcomes and quality of life. [ABSTRACT FROM AUTHOR]

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 enddiastolic 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 (GLS) correlated with systemic atrial strain (sAS) (r=-0.6, p=0.01). CMR-derived EF correlated with CMR-derived 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 that correlated with peak oxygen consumption 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. [ABSTRACT FROM AUTHOR]

Pediatric hypertrophic cardiomyopathy (HCM) has a wide range of clinical manifestations. Left ventricular outflow tract obstruction (LVOTO) at rest is present in up to one-third of children with HCM, with a further 50-60% of symptomatic children developing a gradient under exertion. Treatment options are limited, and there is a relative lack of data on the pediatric population. Disopyramide is a sodium channel blocker with negative inotropic properties. This therapy effectively reduces LVOTO in adults with HCM and delays surgical interventions, but it is not licensed for use in children. We aimed to review and analyze the influence of disopyramide over the pathophysiological, clinical, electrocardiographic, and echocardiographic characteristics of patients with HCM in infancy, childhood, adolescence, and adult age. While disopyramide remains a cornerstone in the management of pediatric HCM, the advent of mavacamten and aficamten heralds a new era of potential advancements. These emerging therapies could significantly improve the quality of life and prognosis for young patients with HCM. [ABSTRACT FROM AUTHOR]

Congenital heart diseases (CHDs) represent a heterogeneous group of congenital defects, with high prevalence worldwide. Non-invasive imaging is essential to guide medical and surgical planning, to follow the patient over time in the evolution of the disease, and to reveal potential complications of the chosen treatment. The application of cardiac magnetic resonance imaging (CMRI) in this population allows for obtaining detailed information on the defects without the necessity of ionizing radiations. This review emphasizes the central role of CMR in the overall assessment of CHDs, considering also the limitations and challenges of this imaging technique. CMR, with the application of two-dimensional (2D) and tri-dimensional (3D) steady-state free precession (SSFP), permits the obtaining of very detailed and accurate images about the cardiac anatomy, global function, and volumes' chambers, giving essential information in the intervention planning and optimal awareness of the postoperative anatomy. Nevertheless, CMR supplies tissue characterization, identifying the presence of fat, fibrosis, or oedema in the myocardial tissue. Using a contrast agent for angiography sequences or 2D/four-dimensional (4D) flows offers information about the vascular, valvular blood flow, and, in general, the cardiovascular system hemodynamics. Furthermore, 3D SSFP CMR acquisitions allow the identification of coronary artery abnormalities as an alternative to invasive angiography and cardiovascular computed tomography (CCT). However, CMR requires expertise in CHDs, and it can be contraindicated in patients with non-conditional devices. Furthermore, its relatively longer acquisition time and the necessity of breath-holding may limit its use, particularly in children under eight years old, sometimes requiring anesthesia. The purpose of this review is to elucidate the application of CMR during the pediatric age. [ABSTRACT FROM AUTHOR]

Current guidelines for the care of heart transplantation recipients recommend routine endomyocardial biopsy and invasive coronary angiography as the cornerstones in the surveillance for acute rejection (AR) and coronary allograft vasculopathy (CAV). Non-invasive tools, including coronary computed tomography angiography and cardiac magnetic resonance, have been introduced into guidelines without roles of their own as gold standards. These techniques also carry the risk of contrast-related kidney injury. There is a need to explore non-invasive approaches providing valuable information while minimizing risks and allowing their application independently of patient comorbidities. Echocardiographic examination can be performed at bedside, serially repeated, and does not carry the burden of contrast-related kidney injury and procedure-related risk. It provides comprehensive assessment of cardiac morphology and function. Advanced echocardiography techniques, including Doppler tissue imaging and strain imaging, may be sensitive tools for the detection of minor myocardial dysfunction, thus providing insight into early detection of AR and CAV. Stress echocardiography may offer a valuable tool in the detection of CAV, while the assessment of coronary flow reserve can unravel coronary microvascular impairment and add prognostic value to conventional stress echocardiography. The review highlights the role of Doppler echocardiography in heart transplantation follow-up, weighting advantages and limitations of the different techniques. [ABSTRACT FROM AUTHOR]