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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]

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]

The arterial switch operation (ASO) has become the standard surgical treatment for patients with d-transposition of the great arteries. While ASO has significantly improved survival rates, a subset of patients develop 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 for cardiac function and exercise capacity. The GAA has been associated with changes in ventricular function, hemodynamics, 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, and cardiac computed tomography, 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. [ABSTRACT FROM AUTHOR]

Background: Although most children may experience mild to moderate symptoms and do not require hospitalization, there are little data on cardiac involvement in COVID-19. However, cardiac involvement is accurately demonstrated in children with MISC. The objective of this study was to evaluate cardiac mechanics in previously healthy children who recovered from severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in a long-term follow-up by means of two-dimensional speckle-tracking echocardiography (STE). Methods: We analyzed a cohort of 157 paediatric patients, mean age 7.7 ± 4.5 years (age range 0.3–18 years), who had a laboratory-confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or mildly symptomatic for COVID-19. Patients underwent a standard transthoracic echocardiogram and STE at an average time of 148 ± 68 days after diagnosis and were divided in three follow-up groups (<180 days, 180–240 days, >240 days). Patients were compared with 107 (41 females—38%) age- and BSA-comparable healthy controls (CTRL). Results: Left ventricular (LV) global longitudinal strain (post-COVID-19: −20.5 ± 2.9%; CTRL: −21.8 ± 1.7%; p < 0.001) was significantly reduced in cases compared with CTRLs. No significant differences were seen among the three follow-up groups (p = NS). Moreover, regional longitudinal strain was significantly reduced in LV apical-wall segments of children with disease onset during the second wave of the COVID-19 pandemic compared to the first wave (second wave: −20.2 ± 2.6%; first wave: −21.2 ± 3.4%; p = 0.048). Finally, peak left atrial systolic strain was within the normal range in the post-COVID-19 group with no significant differences compared to CTRLs. Conclusions: Our study demonstrated for the first time the persistence of LV myocardial deformation abnormalities in previously healthy children with an asymptomatic or mildly symptomatic (WHO stages 0 or 1) COVID-19 course after an average follow-up of 148 ± 68 days. A more significant involvement was found in children affected during the second wave. These findings imply that subclinical LV dysfunction may also be a typical characteristic of COVID-19 infection in children and are concerning given the predictive value of LV longitudinal strain in the general population. [ABSTRACT FROM AUTHOR]

Aims Compared with adult patients, clinical manifestations of children's coronavirus disease-2019 (COVID-19) are generally perceived as less severe. The objective of this study was to evaluate cardiac involvement in previously healthy children with asymptomatic or mildly symptomatic severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. Methods and results We analysed a cohort of 53 paediatric patients (29 males, 55%), mean age 7.5 ± 4.7 years, who had a confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or only mildly symptomatic for COVID-19. Patients underwent standard transthoracic echocardiogram and speckle tracking echocardiographic study at least 3 months after diagnosis. Thirty-two age, sex, and body surface area comparable healthy subjects were used as control group. Left ventricular ejection fraction was within normal limits but significantly lower in the cases group compared to controls (62.4 ± 4.1% vs. 65.2 ± 5.5%; P  = 0.012). Tricuspid annular plane systolic excursion (20.1 ± 3 mm vs. 19.8 ± 3.4 mm; P  = 0.822) and left ventricular (LV) global longitudinal strain (−21.9 ± 2.4% vs. −22.6 ± 2.5%; P  = 0.208) were comparable between the two groups. Regional LV strain analysis showed a significant reduction of the LV mid-wall segments strain among cases compared to controls. Furthermore, in the cases group, there were 14 subjects (26%) with a regional peak systolic strain below −16% (−2.5 Z score in our healthy cohort) in at least two segments. These subjects did not show any difference regarding symptoms or serological findings. Conclusion SARS-CoV-2 infection may affect left ventricular deformation in 26% of children despite an asymptomatic or only mildly symptomatic acute illness. A follow-up is needed to verify the reversibility of these alterations and their impact on long-term outcomes. [ABSTRACT FROM AUTHOR]

Single coronary artery (SCA) from the right sinus is the rarest coronary anomaly. We describe 2 cases: 1 with SCA type-1RI; 2 with SCA type-2RII-A. Appropriate and successful treatment (CABG in case-1; PTCA in case-2) was chosen relying on accurate morphological description provided by MDCT, in order to recognize all the possible mechanisms of myocardial ischemia. athletes. Patients are usually asymptomatic and, in most of the cases, CAA are incidentally discovered [1]. There are several types of CAA according to their origination and course (e.g., separate ostia of left anterior descending (LAD) and left circumflex (LCX), intrinsic anatomy (e.g., ostial stenosis, atresia, ectasia, hypoplasia), coronary termination (e.g., fistulas) and anomalous collateral vessels. Among them, single coronary artery (SCA) is the rarest, with a prevalence of 0.02-0.06% in the general population [1,2]. As historical note, in one of the two vascular anatomical models in "Sansevero Chapel" in Naples (Italy), an origin of single coronary artery from the right aortic coronary sinus is showed in detail [3]. In the current, ECG-gated multi-detector CT (MDCT) is becoming a widespread technique for the detection of CAA, with an increasingly solid evidence-based role [4]. Its multi-planar capability allows a precise visualization of the origin, course, and termination of vessels. In patients with coronary artery disease, the detection of coronary anomalies, and above all SCA, may affect treatment (e.g., medical therapy over percutaneous intervention) even if no recent treatment guidelines or follow-up recommendations exist at the moment [5,6]. We report two cases of right-SCA confirmed by MDCT after development of ischemic cardiopathy. Treatment was different, with good result in both of them: percutaneous revascularization (PTCA) vs coronary artery bypass graft (CABG). [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes. METHODS We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A 'biventricular' Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation. RESULTS A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P  = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4–20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P  = 0.018). Statistical analysis showed an equal distribution of BVF across time (P  = 0.620), and there were no significant differences in terms of early and late survival (P  = 0.53 and P  = 0.72, respectively) or morbidity (P  = 0.45 and P  = 0.80, respectively). CONCLUSIONS A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle. [ABSTRACT FROM AUTHOR]

Objectives: The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes., Methods: Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed., Results: Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16-26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4-42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m 2 ) in patients with a right dominant ventricle ( p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome ( p = 0.043). Left ventricular dominance was associated with a better ejection fraction ( p = 0.04), less extent of late gadolinium enhancement ( p = 0.022), higher metabolic equivalents ( p = 0.01), and higher peak oxygen consumption ( p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support ( p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test., Conclusions: In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

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 (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.

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.

Aims: Compared with adult patients, clinical manifestations of children's coronavirus disease-2019 (COVID-19) are generally perceived as less severe. The objective of this study was to evaluate cardiac involvement in previously healthy children with asymptomatic or mildly symptomatic severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection., Methods and Results: We analysed a cohort of 53 paediatric patients (29 males, 55%), mean age 7.5 ± 4.7 years, who had a confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or only mildly symptomatic for COVID-19. Patients underwent standard transthoracic echocardiogram and speckle tracking echocardiographic study at least 3 months after diagnosis. Thirty-two age, sex, and body surface area comparable healthy subjects were used as control group. Left ventricular ejection fraction was within normal limits but significantly lower in the cases group compared to controls (62.4 ± 4.1% vs. 65.2 ± 5.5%; P = 0.012). Tricuspid annular plane systolic excursion (20.1 ± 3 mm vs. 19.8 ± 3.4 mm; P = 0.822) and left ventricular (LV) global longitudinal strain (-21.9 ± 2.4% vs. -22.6 ± 2.5%; P = 0.208) were comparable between the two groups. Regional LV strain analysis showed a significant reduction of the LV mid-wall segments strain among cases compared to controls. Furthermore, in the cases group, there were 14 subjects (26%) with a regional peak systolic strain below -16% (-2.5 Z score in our healthy cohort) in at least two segments. These subjects did not show any difference regarding symptoms or serological findings., Conclusion: SARS-CoV-2 infection may affect left ventricular deformation in 26% of children despite an asymptomatic or only mildly symptomatic acute illness. A follow-up is needed to verify the reversibility of these alterations and their impact on long-term outcomes., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.)

Objectives: The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes., Methods: We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A 'biventricular' Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation., Results: A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4-20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P = 0.018). Statistical analysis showed an equal distribution of BVF across time (P = 0.620), and there were no significant differences in terms of early and late survival (P = 0.53 and P = 0.72, respectively) or morbidity (P = 0.45 and P = 0.80, respectively)., Conclusions: A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)