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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.

Introduction We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. Materials and methods From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). Results The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). Conclusions ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

The role played by the right ventricular (RV) dysfunction has long been underestimated in clinical practice. Recent findings are progressively confirming that when the RV efficiency deteriorates both the right and the left circulation is (significantly) affected, but studies dedicated to a detailed description of RV hemodynamic role still lack. In response to such a gap in knowledge, this work proposes a numerical model that for the first time evaluates the effect of isolated RV dysfunction on the whole circulation. Lumped parameter modelling was applied to represent the physio-pathological hemodynamics. Different grades of impairment were simulated for three dysfunctions i.e., systolic, diastolic, and combined systolic and diastolic. Hemodynamic alterations (i.e., of blood pressure, flow, global hemodynamic parameters), arising from the dysfunctions, are calculated and analysed. Results well accord with clinical observations, showing that RV dysfunction significantly affects both the pulmonary and systemic hemodynamics. Successful verification against in vivo data proved the clinical potentiality of the model i.e., the capability of identifying the degree of RV impairment for given hemodynamic conditions. This study aims at contributing to the improvement of RV dysfunction recognition and treatment, and to the development of tools for the clinical management of pathologies involving the right heart.

In 2018, the position paper ‘Imaging the adult with congenital heart disease: a multimodality imaging approach’ was published. The paper highlights, in the first part, the different imaging modalities applied in adult congenital heart disease patients. In the second part, these modalities are discussed more detailed for moderate to complex anatomical defects. Because of the length of the paper, simple lesions were not touched on. However, imaging modalities to use for simple shunt lesions are still poorly known. One is looking for structured recommendations on which they can rely when dealing with an (undiscovered) shunt lesion. This information is lacking for the initial diagnostic process, during repair and at follow-up. Therefore, this paper will focus on atrial septal defect, ventricular septal defect, and persistent arterial duct. Pre-, intra-, and post-procedural imaging techniques will be systematically discussed. This position paper will offer algorithms that might help at a glance. The document is prepared for general cardiologists, trainees, medical students, imagers/technicians to select the most appropriate imaging modality and to detect the requested information for each specific lesion. It might serve as reference to which researchers could refer when setting up a (imaging) study.

Aims To investigate pulmonary valve (PV) and right ventricular function by echocardiography in paediatric patients with repaired Tetralogy of Fallot (ToF), comparing PV preservation surgical strategies to standard transannular patch (TAP) repair. Methods and results All patients undergoing transatrial-transpulmonary repair for ToF at our institution between January 2007 and May 2020 were reviewed retrospectively. Patients were divided into 2 groups, according to the different techniques used (PV preservation strategy vs TAP repair). All patients underwent standard echo-Doppler study including RV areas, fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE); Pulmonary regurgitation (PR) was assessed by Color Doppler, continuous-wave (CW) Doppler, pressure half time (PHT) and PR index. By speckle tracking we measured also, in a subgroup of patients, right atrial strain (RAS), RV and left ventricle (LV) global longitudinal strain (RVGLS, LVGLS) and their time to peak (TTP) values. Eighty-two patients underwent a PV preservation strategy while 34 underwent a standard TAP repair. Five-year actuarial freedom from moderate/severe PV regurgitation was significantly higher in the PV preservation group compared to the TAP (61.3% [95% CI: 48-73%] vs 25.9% [95% CI: 12-43%], respectively; p = 0.02). After adjusting for age, gender, BSA, and type of PV, the use of a TAP was still significantly associated with an increased risk for PV regurgitation at Follow-up (HR: 1.85, 95% CI: 1.09, 3.15; p = 0.02). At a mean follow-up of 6.9 ± 0.3 years, patients undergoing PV preservation showed an increased right ventricular fractional area change (46.9 ± 0.8% vs 42.5 ± 1.7%, P Conclusion Surgical repair of ToF with PV preservation provides excellent outcomes in terms of PV competence and right ventricular function and should be advocated whenever possible.

Aortic regurgitation (AR) continues to be an important cause of morbidity and mortality in pediatric patients. Although echocardiographic parameters are well established for the adults, there are no clear cut-off values for AR severity in children. Cardiac magnetic resonance (CMR) imaging is considered a "gold standard" for a quantitative evaluation of the AR, but it is not widely available. This study assesses which echo parameter can accurately define AR severity as assessed by CMR in pediatric patients. A total of 27 pediatric patients (12 ± 3 years, range 6 to 18 years) with different degree of AR underwent echo assessment within an average of 35 days from CMR. CMR included phase-contrast velocity-encoded imaging for the measurement of regurgitant fraction (RF). Severe AR was defined as RF33%. Echo evaluation included vena contracta, pressure half time, the ratio between the AR jet and the left ventricular outflow tract diameter (jet/left ventricular outflow tract), presence of holodiastolic reversal flow in abdominal aorta, the ratio between the velocity-time integral of the reversal flow over the forward flow in descending aorta (echoRF). Among the studied parameters, the strongest predictor of severe AR, as assessed by CMR, was echoRF. Receiver-operating characteristic curve showed, for a cutoff0.38, an area under the curve of 0.886 (p0.0001), a sensitivity of 71%, and a specificity of 100%. Correlation coefficient between echoRF and RF was R = 0.929 (p0.0001). In conclusion, echoRF is a strong echo-Doppler marker of severe AR in the pediatric population. This parameter should be routinely added in the standard echo evaluation of pediatric patients with AR.

Background Myocardial crypts are discrete, narrow, blood filled invaginations within the left ventricular myocardium and high-take-off coronary artery are rare manifestations where coronary arteries originate above the sinotubuler junction. Case presentation A 41-year-old man with multiple coronary artery disease risk factors admitted to our outpatient department with progressive dyspnea and atypical chest pain. Physical examination revealed no pathological findings. His blood examination revealed only mild to moderately high IgE and LDL levels. Transthoracic echocardiography (TTE) was normal. His treadmill test was normal, yet in the 3rd stage of the test he had an atypically located chest pain which was relieved in the resting period. As he had multiple cardiovascular risk factors, we performed a coronary CT angiography to exclude coronary artery disease. Coronary CT angiography(CCTA) demonstrated multiple myocardial crypts, a muscular VSD like defect which were not detectable with TTE and a high take off left main coronary artery (LMCA). After CCTA, we repeated the TTE to investigate the crypts and VSD-like defect which were clear on CCTA, yet a precise TTE hardly showed crypts and didn’t confirm a shunt between the left and right ventricle. We defined the defect as ‘spontaneously closed muscular VSD’. None of these pathologies were clinically relevant with the patient’s symptoms, thus pneumonology started a montelukast therapy for 1 year and we decided to follow up the patient, as multiple crypts may indicate an early phase hypertrophic cardiomyopathy. Conclusions Considering that a high take-off LMCA is a congenital anomaly, encountering multiple crypts which are also congenital pathologies, is plausible, as congenital anomalies may accompany eachother. Echocardiography is a very useful, practical imaging tool but regrettably may be suboptimal due to various patient and method related reasons. Target combination of different cardiovascular imaging tools like echocardiography, cardiac CT(CCT), may be utilized in order to ensure a comprehensive diagnosis particularly.

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.

The goal of Level 1 training in echocardiography is to enable the trainee to select echocardiography appropriately for the evaluation of a specific clinical question, and then to interpret the report. It is not the goal of Level 1 training to teach how to perform the examination itself—that is the goal of higher levels of training. However, understanding the principles, indications, and findings of this crucial technique is valuable to many medical professionals including outside cardiology. This should be seen as part of a general understanding of cardiac imaging modalities. The purpose of this position statement is to define the scope and outline the general requirements for Level 1 training and competence in echocardiography. Moreover, the document aims to make a clear distinction between Level 1 competence in echocardiography and focus cardiac ultrasound (FoCUS).

Background Short-term sequelae of Multisystem Inflammatory Syndrome in Children (MIS-C), recently published by our institution, showed rapid improvement of the cardiac abnormalities within a few weeks after the onset of the disease. However, subtle residual abnormalities, affecting mainly the myocardial interstitium, were shown in some of the patients. The current study aimed to assess myocardial deformation with CMR shortly after MIS-C. Methods Sixty children were included into the study; 30 following MIS-C (onset-to-scan mean 27 days, SD 11) and 30 controls. Strain values were compared between patients and controls and additionally to published paediatric normal CMR values. U-Mann Whitney test was used for comparison of the myocardial deformation between patients and controls. Results Median age of the patients was 9.0 years (range 0.99–14.4) and controls 9.8 years (range 4.7–14.9). All conventional CMR parameters in patients were in normal range. Strain values were significantly lower in patients than in controls. When compared to published centile graphs, radial and circumferential global strain was within 2.5th and 97.5th centile in all patients. Eleven patients had global longitudinal strain between 2.5th centile and 50th centile, 1 patient was below 2.5th centile and all the others above 50th centile. Only 3 controls had global longitudinal strain between 2.5th centile and 50th centile, all other had higher strain. Conclusions This study demonstrates that myocardial deformation indices measured by CMR are within normal range in the vast majority of the patients within a few weeks after the onset of MIS-C. However, when compared to healthy controls, all strain parameters were lower in patients.

Background The most important determinant of long-term survival in patients with idiopathic pulmonary fibrosis is the right ventricular (RV) adaptation to the increased pulmonary vascular resistance. Our aim was to explore RV contractile reserve during stress echocardiography in early-stage IPF. Methods Fifty early-stage patients with IPF and 50 healthy control patients underwent rest and stress echocardiography, including RV two-dimensional speckle tracking echocardiography. At peak exertion, blood gas analysis and spirometry were also assessed. Results At rest, RV diameters were mildly increased in IPF; however, although RV conventional systolic function indexes were similar between the IPF and control groups, RV global longitudinal strain and RV lateral wall longitudinal strain (LWLS) were significantly reduced in the IPF cohort. During physical exercise, patients with IPF showed a reduced exercise tolerance with lower maximal workload (P o 2; P Conclusions RV myocardial dysfunction is already present at rest in early-stage IPF and worsens during exertion as detected by two-dimensional speckle-tracking echocardiography. The RV altered contractile reserve appears to be related to reduced exercise tolerability and impaired pulmonary hemodynamic.

Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy.A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037].Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified.

Background Right (R) or left (L) ventricular outflow tract (VOT) obstruction can be either a dynamic phenomenon or a congenital anatomic lesion, which requires a prompt and optimal timing of treatment to avoid a pathological ventricular remodelling. Objective To develop a simple and reliable numerical tool able to relate the R/L obstruction size with the pressure gradient and the cardiac output. To provide indication of the obstruction severity and be of help in the clinical management of patients and designing the surgical treatment for obstruction mitigation. Methods Blood flow across the obstruction is described according to the classical theory of one-dimensional flow, with the obstruction uniquely characterized by its size. Hemodynamics of complete circulation is simulated according to the lumped parameter approach. The case of a 2 years-old baby is reproduced, with the occlusion placed in either the R/ or the L/VOT. Conditions from wide open to almost complete obstruction are reproduced. Results Both R/LVOT obstruction in the in-silico model resulted in an increased pressure gradient and a decreased cardiac output, proportional to the severity of the VOT obstruction and dependent on the R/L location of the obstruction itself, as it is clinically observed. Conclusion The in-silico model of ventricular obstruction which simulates pressure gradient and/or cardiac output agrees with clinical data, and is a first step towards the creation of a tool that can support the clinical management of patients from diagnosis to surgical treatments.

The tricuspid valve (TV) has been neglected for many years. Only recently, new studies demonstrated the prognostic role of the tricuspid valve lesions. In addition to that, new interventional approaches offer the possibility to non-invasively treat tricuspid valve disease. In this review, our aim is to summarize the role of different imaging techniques in the assessment of tricuspid valve with particular regards to congenital heart diseases. Finally, we analyze the importance of the TV and its adjacent regions from an electrophysiological point of view, both in structurally normal hearts and in congenital heart diseases. The most relevant transthoracic echocardiography (TTE) views to visualize the TV are the "modified" parasternal long axis, the apical views and subcostal projections, such as right oblique or left oblique views. However, simultaneous visualization of the three leaflets is possible only with three-dimensional TTE, or, sometimes, in parasternal short axis and subcostal short axis views in children. 3D echocardiography (3DE) is promising in this field. Indeed, its unique projections, such as en face views from the right ventricular and atrial perspectives, are able to define the spatial relationship of the tricuspid leaflets with the surrounding structures. Moreover, multimodality imaging assessment has been recently proposed for the diagnostic assessment of the TV, especially before percutaneous intervention. Cardiac Computed Tomography (CCT) provides valuable anatomic spatial information of the TV apparatus. Cardiac Magnetic Resonance (CMR) is able to provide, non-invasively, detailed morphological and functional information of the valve.

Kawasaki disease (KD) can be associated with high morbidity and mortality due to coronary artery aneurysms formation and myocardial dysfunction. Aim of this study was to evaluate the diagnostic performance of non-invasive myocardial work in predicting subtle myocardial abnormalities in Kawasaki disease (KD) children with coronary dilatation (CADL). A total of 100 patients (age 8.7 ± 5 years) were included: 45 children with KD and CADL (KD/CADL) (Z-score > 2.5), 45 age-matched controls (CTRL) and, finally, an additional group of 10 children with KD in absence of coronary dilatation (KD group). Left ventricular (LV) systolic function and global longitudinal strain (GLS) were assessed. Global myocardial work index (MWI) was calculated as the area of the LV pressure-strain loops. From MWI, global Constructive Work (MCW), Wasted Work (MWW) and Work Efficiency (MWE) were estimated. Despite normal LV systolic function by routine echocardiography, KD/CADL patients had lower MWI (1433.2 ± 375.8 mmHg% vs 1752.2 ± 265.7 mmHg%, p . MWI, MCW and MWE were significantly reduced in KD children despite normal LVEF and normal GLS. These abnormalities seems independent from CADL. Thus, in KD with normal LVEF and normal GLS, estimation of MWI may be a more sensitive indicator of myocardial dysfunction.

An 11-year-old boy affected by pulmonary atresia with intact ventricular septum (AP-IVS) was listed for percutaneous pulmonary valvuloplasty and closure of multi-fenestrated atrial septal defect (ASD). Intraprocedural transesophageal echocardiography arose the suspect of abnormal coronary artery pattern while selective angiography documented a single sinoatrial node artery (SANa) with an unusual retro-aortic course. As consequence, we proceeded to effectively close the defects with a not self-centering device placed in the most central side hole. This case supports the hypothesis that sometimes arrhythmic complication during ASD closure procedures might be due to unrecognized injury of the SANa.

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.

Background: Non-invasive prediction of critical coronary artery stenosis (CAST) in patients with coronary artery disease (CAD) is challenging. Strain parameters can often capture an impairment of regional longitudinal function; however, they are load dependent. A novel non-invasive method to estimate Myocardial Work (MW) has been recently proposed, showing a strong correlation with invasive work measurements. Our aim was to investigate the ability of non-invasive MW to predict the ischaemic risk area underlying a CAST.Methods and Results: The study population comprises 80 individuals: 50 patients with CAST and 30 controls (CTRL). Echocardiography recordings were obtained before coronary angiography to measure global longitudinal strain (GLS), Myocardial Work Index (MWI), Myocardial Constructive Work (MCW), Myocardial Wasted work (MWW), Myocardial Work Efficiency (MWE). Global MWI (p = 0.048), MWE (p < 0.001), and MCW (p = 0.048) at baseline were significantly reduced in patients with CAST compared to controls (p < 0.05). Regional MWE within the myocardial segments underlying the CAST, but not LS, was significantly reduced compared to non-target segments (p < 0.001). At ROC analysis, the diagnostic performance to predict CAST for regional MWE (AUC = 0.920, p < 0.001) was higher compared to both regional post-systolic shortening index (PSI) (AUC = 0.600, p = 0.129) and regional LS (AUC = 0.546, p = 0.469).Conclusions: Non-invasive estimation of MW work indices is able to predict a CAST before invasive angiography.

Multisystem Inflammatory Syndrome in Children (MIS-C) is a known severe condition affecting children previously exposed to SARS-CoV-2. The aim of our study was to describe the early cardiac abnormalities in patients with MIS-C, evaluated by speckle tracking echocardiography (STE) and cardiac MRI (CMR). Clinical, laboratory and microbiological data were measured for all patients. All children underwent standard transthoracic echocardiography, STE with analysis of left ventricle global longitudinal strain (GLS). Seventeen (75%) of the children were evaluated with CMR. Twenty-three patients (13M, 10F) were recruited, mean age was 8.1 ± 4 years. Cardiovascular symptoms were present in 10 (43.5%). Nine children (39.1%) shared Kawasaki Disease-like symptoms. Four patients (17.4%) needed ICU admission. In-hospital survival was 100%. TnI was elevated in 15 (65.2%) and BNP in 20 (86.9%) patients. The median time to STE evaluation was 8 days and to CMR was 18 days after fever onset. Mean LVEF was 59 ± 10%. Coronary dilation was observed in six (26.1%) patients. STE showed a reduced mean LVGLS (−17 ± 4.3%). LGE with a non-ischemic pattern was evident in six out of seventeen patients (35.2%). The elevation of myocardial necrosis markers, the reduction of LVGLS and the presence of LGE on CMR in about a quarter of MIS-C patients supports the hypothesis of a post-viral immune-mediated myocarditis-like pathogenesis.

Three-dimensional echocardiography is being used to evaluate left ventricular dimensions and ejection fraction in clinical practice. The validity and normal values have been established in a large group of normal adults, children and neonates. The aim of this study was to compare left ventricular dimensions and ejection fraction obtained from the same 3-dimensional echocardiography datasets using 2 commercially available applications: Philips QLAB and Tomtec 4D LEFT VENTRICLE-ANALYSIS. Fifty consecutive newborns or neonates coming to their first newborn visit within the first 3 weeks of live, were recruited. 38 babies underwent full Echocardiographic evaluation, including the acquisition of several 3-dimensional datasets, while naturally sleeping. Left ventricular dimensions, volumes and ejection fraction were measured using Philips QLAB version 9.0 and results were compared to results of the same datasets, analysed using TomTec 4D LV software. Mean left ventricular diastolic, and systolic volumes indexed to body surface area and ejection fraction were: 24.7 ± 3.6 ml/m2, 9.2 ± 1.3 ml/m2 and 62 ± 3.8% using TomTec and 26.6 ± 3.8 ml/m2, 10.4 ± 2 ml/m2 and 63 ± 3.1% using QLAB, respectively. Mean indexed left ventricular diastolic, and systolic volumes measured with QLAB were significantly higher as compared to TomTec with insignificant difference in the ejection fraction. Normal left ventricular indices obtained from 3-dimensional echocardiography datasets were established, using Philips QLAB and TomTec 4D LV ANALYSIS software. Measurements obtained were significantly different between those venders, and hence, may not be used interchangeably.

The primary mission of the European Association of Cardiovascular Imaging (EACVI) is ‘to promote excellence in clinical diagnosis, research, technical development, and education in cardiovascular imaging’. Echocardiography is a key component in the evaluation of patients with known or suspected cardiovascular disease and is essential for the high quality and effective practice of clinical cardiology. The EACVI aims to update the previously published recommendations for training, competence, and quality improvement in echocardiography since these activities are increasingly recognized by patients, physicians, and payers. The purpose of this document is to provide the general requirements for training and competence in echocardiography, to outline the principles of quality evaluation, and to recommend a set of measures for improvement, with the ultimate goal of raising the standards of echocardiographic practice. Moreover, the document aims to provide specific guidance for advanced echo techniques, which have dramatically evolved since the previous publication in 2009.

Children with congenital and acquired heart disease may be exposed to relatively high lifetime cumulative doses of ionizing radiation from necessary medical invasive and non-invasive imaging procedures. Although these imaging procedures are all essential to the care of these complex paediatric population and have contributed to meaningfully improved outcomes in these patients, exposure to ionizing radiation is associated with potential risks, including an increased lifetime attributable risk of cancer. The goal of this manuscript is to provide a comprehensive review of radiation dose management and cardiac computed tomography performance in the paediatric population with congenital and acquired heart disease, to encourage informed imaging to achieve indication-appropriate study quality at the lowest achievable dose.

BackgroundThe ongoing pandemic of Novel Coronavirus Disease 2019 (COVID-19) infection has created a global emergency. Despite the infection causes a mild illness to most people, some patients are severely affected, demanding an urgent need to better understand how to risk-stratify infected subjects.DesignThis is a meta-analysis of observational studies evaluating cardiovascular (CV) complications in hospitalized COVID-19 patients and the impact of cardiovascular risk factors (RF) or comorbidities on mortality.MethodsData sources: PubMed, Scopus, and ISI from 1 December 2019 through 11 June 2020; references of eligible studies; scientific session abstracts; cardiology web sites. We selected studies reporting clinical outcomes of hospitalized patients with COVID-19. The main outcome was death. Secondary outcomes were cardiovascular symptoms and cardiovascular events developed during the COVID-19-related hospitalization. Extracted data were recorded in excel worksheets and analysed using statistical software (MedCalc, OpenMetanalyst, R). We used the proportion with 95% CI as the summary measure. A Freeman-Tukey transformation was used to calculate the weighted summary proportion under the random-effects model. Heterogeneity was assessed by using the Cochran Q test and I2 values.ResultsAmong 77317 hospitalized patients from 21 studies, 12.86% had cardiovascular comorbidities or RF. Cardiovascular complications were registered in 14.09% of cases during hospitalization. At meta-regression analysis, pre-existing cardiovascular comorbidities or RF were significantly associated to cardiovascular complications in COVID-19 patients (p = 0.019). Pre-existing cardiovascular comorbidities or RF (pConclusionsCardiovascular complications are frequent among COVID-19 patients, and might contribute to adverse clinical events and mortality, together with pre-existing cardiovascular comorbidities and RF. Clinicians worldwide should be aware of this association, to identifying patients at higher risk.

Background Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF). Aims To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO2). Methods and results All patients underwent standard echo-Doppler study, speckle tracking analysis, and CMR to assess PR and RV size and function. Thirty-six patients underwent also cardiopulmonary exercise test. Fourty-six patients (aged 13.7 ± 3.0) were included. Echo derived RV areas correlated with CMR RV volumes (p 21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume ≥ 150 ml/m2. RVEF was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. Flow-reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify CMR pulmonary regurgitant fraction (RF) ≥ 35%. None of the CMR parameters correlated with peak VO2. Among the Echo data only right atrial strain (RAS) correlated with peak VO2. Conclusion In children, flow-reversal in pulmonary branches identifies hemodynamically significant RF with a good sensitivity but poor specificity. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. RV longitudinal systolic dysfunction coexists with a still preserved EF. RAS correlates strongly with peak VO2 and should be added in their follow up.

Aims To describe the cardiac abnormalities in patients with COVID-19 and identify the characteristics of patients who would benefit most from echocardiography. Methods and results In a prospective international survey, we captured echocardiography findings in patients with presumed or confirmed COVID-19 between 3 and 20 April 2020. Patient characteristics, indications, findings, and impact of echocardiography on management were recorded. Multivariable logistic regression identified predictors of echocardiographic abnormalities. A total of 1216 patients [62 (52–71) years, 70% male] from 69 countries across six continents were included. Overall, 667 (55%) patients had an abnormal echocardiogram. Left and right ventricular abnormalities were reported in 479 (39%) and 397 (33%) patients, respectively, with evidence of new myocardial infarction in 36 (3%), myocarditis in 35 (3%), and takotsubo cardiomyopathy in 19 (2%). Severe cardiac disease (severe ventricular dysfunction or tamponade) was observed in 182 (15%) patients. In those without pre-existing cardiac disease (n = 901), the echocardiogram was abnormal in 46%, and 13% had severe disease. Independent predictors of left and right ventricular abnormalities were distinct, including elevated natriuretic peptides [adjusted odds ratio (OR) 2.96, 95% confidence interval (CI) 1.75–5.05) and cardiac troponin (OR 1.69, 95% CI 1.13–2.53) for the former, and severity of COVID-19 symptoms (OR 3.19, 95% CI 1.73–6.10) for the latter. Echocardiography changed management in 33% of patients. Conclusion In this global survey, cardiac abnormalities were observed in half of all COVID-19 patients undergoing echocardiography. Abnormalities were often unheralded or severe, and imaging changed management in one-third of patients.

Introduction Hypoplastic Left Heart Syndrome (HLHS) has high mortality and morbidity and systemic right ventricle (RV) dysfunction may play a key-role. Study aim is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting outcome in HLHS patients. Methods We studied 27 HLHS patients (17 male) who successfully completed Norwood palliation. All the patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed: baseline, one-month after Norwood, three-months after Norwood, one-week before bidirectional cavopulmonary anastomosis (BCPA) and two-months after BCPA. From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). Results After a mean follow-up of 1.18 (± 1.16) years, 8 out of 27 of the included patients met the composite endpoint of death/heart transplant (HT). At pre-Norwood assessment, there was no difference in echo measurements between survivors and patients with events. In death/HT group TAPSE and LS declined already one-month after Norwood procedure: TAPSE ≤5 mm had good sensitivity (85.71%) and moderate specificity (63.16%) for death/HT (AUC = 0.767); a decrease of LS > 8.7% vs baseline showed 100% sensitivity and 84.21% specificity for death/HT (AUC = 0.910). At multivariate analysis, one-month-after-Norwood LS drop >8.7% was the best predictor of outcome (P = 0.01). Conclusions RV dysfunction in HLHS carries prognostic value. Our findings encourage serial measurements of RV function to identify the subgroup of HLHS patients at higher risk. In our experience, ∆ LS showed the best predictive value.

Background Stroke volume response during stress is a major determinant of functional status in heart failure and can be measured by two-dimensional (2-D) volumetric stress echocardiography (SE). The present study hypothesis is that SE may identify mechanisms underlying the change in stroke volume by measuring preload reserve through end-diastolic volume (EDV) and left ventricular contractile reserve (LVCR) with systolic blood pressure and end-systolic volume (ESV). Methods We enrolled 4,735 patients (age 63.6 ± 11.3 yrs, 2800 male) referred to SE for known or suspected coronary artery disease (CAD) and/or heart failure (HF) in 21 SE laboratories in 8 countries. In addition to regional wall motion abnormalities (RWMA), force was measured at rest and peak stress as the ratio of systolic blood pressure by cuff sphygmomanometer/ESV by 2D with Simpson's or linear method. Abnormal values of LVCR (peak/rest) based on force were ≤1.10 for dipyridamole (n=1,992 patients) and adenosine (n=18); ≤2.0 for exercise (n=2,087) or dobutamine (n=638). Results Force-based LVCR was obtained in all 4,735 pts. Lack of stroke volume increase during stress was due to either abnormal LVCR and/or blunted preload reserve, and 57 % of patients with abnormal LVCR nevertheless showed increase in stroke volume. Conclusions Volumetric SE is highly feasible with all stresses, and more frequently impaired in presence of ischemic RWMA, absence of viability and reduced coronary flow velocity reserve. It identifies an altered stroke volume response due to reduced preload and/or contractile reserve.

Introduction: Many centers have recently adopted pulmonary valve (PV) preservation procedures to prevent the detrimental long-term effects of chronic pulmonary regurgitation after tetralogy of Fallot (ToF) repair. Hypothesis: Here, we investigated PV and right ventricular function after PV preservation surgical strategies compared to standard transannular patch (TAP) repair. Methods: All patients undergoing transatrial-transpulmonary repair for ToF with PV stenosis at our institution between January 2007 and May 2020 were reviewed retrospectively. Patients were divided into 2 main groups, according to the different techniques used: patients undergoing a PV preservation strategy and patients undergoing TAP repair. Results: Overall, 82 patients underwent a successful PV preservation strategy while 34 underwent a standard TAP repair. At index surgery, BSA (0.31±0.1 m 2 , P=0.3), age (4.8±0.3 months, P=0.5) and preoperative PV Z-score (-3.20±0.1, P=0.1) did not differ between groups. Five-year actuarial freedom from moderate/severe PV regurgitation was significantly higher in the PV preservation group compared to the TAP (61.3% [95% CI: 48-73%] vs 25.9% [95% CI: 12-43%], respectively; p=0.02). After adjusting for age, gender, BSA, and type of PV, the use of a TAP was still significantly associated with an increased risk for PV regurgitation at follow up (HR: 1.85, 95% CI: 1.09, 3.15; p=0.02). At a mean follow-up of 6.9±0.3 years, patients undergoing PV preservation showed an increased right ventricular fractional area change (46.9±0.8% vs 42.5±1.7%, P Conclusions: Surgical repair of ToF with PV preservation provides excellent outcomes in terms of PV competence and right ventricular function and should be advocated whenever possible.

Background During ischemia a close relationship exists between sub-endocardial blood flow and myocardial function. Strain parameters can capture an impairment of regional longitudinal function but are load dependent. Recently, a novel non-invasive method to calculate Myocardial Work (MW) showed a strong correlation with invasive work measurements. Our aim was to investigate the ability of non-invasive MW indices to identify the ischaemic risk area during transient acute coronary occlusion (TACO). Methods and results The study population comprises 50 individuals with critical coronary stenosis (CCS). Echocardiography recordings were obtained before coronary angiography, during TACO and after revascularization to measure global longitudinal strain (GLS), Myocardial Work Index (MWI), Myocardial Constructive Work (MCW), Myocardial Wasted work (MWW), Myocardial work efficiency (MWE). Compared to baseline, we found a significant reduction of GLS (p = 0.005), MWI, MCW and MWE (p Conclusions The non-invasive measurement of MW parameters is a sensitive and early marker of myocardial ischemia during TACO.

Objectives The purpose of this study was to assess the functional and prognostic correlates of B-lines during stress echocardiography (SE). Background B-profile detected by lung ultrasound (LUS) is a sign of pulmonary congestion during SE. Methods The authors prospectively performed transthoracic echocardiography (TTE) and LUS in 2,145 patients referred for exercise (n = 1,012), vasodilator (n = 1,054), or dobutamine (n = 79) SE in 11 certified centers. B-lines were evaluated in a 4-site simplified scan (each site scored from 0: A-lines to 10: white lung for coalescing B-lines). During stress the following were also analyzed: stress-induced new regional wall motion abnormalities in 2 contiguous segments; reduced left ventricular contractile reserve (peak/rest based on force, ≤2.0 for exercise and dobutamine, ≤1.1 for vasodilators); and abnormal coronary flow velocity reserve ≤2.0, assessed by pulsed-wave Doppler sampling in left anterior descending coronary artery and abnormal heart rate reserve (peak/rest heart rate) ≤1.80 for exercise and dobutamine (≤1.22 for vasodilators). All patients completed follow-up. Results According to B-lines at peak stress patients were divided into 4 different groups: group I, absence of stress B-lines (score: 0 to 1; n = 1,389; 64.7%); group II, mild B-lines (score: 2 to 4; n = 428; 20%); group III, moderate B-lines (score: 5 to 9; n = 209; 9.7%) and group IV, severe B-lines (score: ≥10; n = 119; 5.4%). During median follow-up of 15.2 months (interquartile range: 12 to 20 months) there were 38 deaths and 28 nonfatal myocardial infarctions in 64 patients. At multivariable analysis, severe stress B-lines (hazard ratio [HR]: 3.544; 95% confidence interval [CI]: 1.466 to 8.687; p = 0.006), abnormal heart rate reserve (HR: 2.276; 95% CI: 1.215 to 4.262; p = 0.010), abnormal coronary flow velocity reserve (HR: 2.178; 95% CI: 1.059 to 4.479; p = 0.034), and age (HR: 1.031; 95% CI: 1.002 to 1.062; p = 0.037) were independent predictors of death and nonfatal myocardial infarction. Conclusions Severe stress B-lines predict death and nonfatal myocardial infarction. (Stress Echo 2020 - The International Stress Echo Study [SE2020]; NCT03049995)

The recent outbreak of 2019 severe acute respiratory syndrome coronavirus-2 is having major repercussions on healthcare services provision in Italy and worldwide. Data suggest the virus has a strong impact on the cardiovascular system, and cardiac imaging will play an important role in patients affected by coronavirus disease-2019. Although paediatric patients are mildly affected, they represent a clear accelerator in spreading the virus, and healthcare workers are at higher risk of infection. The aim of this position paper is to provide clinical recommendation regarding the execution of imaging investigations for the cardiac diagnostic work-up of paediatric patients with suspected or confirmed infection.

An enlarged left atrial volume index (LAVI) at rest mirrors increased LA pressure and/or impairment of LA function. A cardiovascular stress may acutely modify left atrial volume (LAV) within minutes. Aim of this study was to assess the feasibility and functional correlates of LAV-stress echocardiography (SE) Out of 514 subjects referred to 10 quality-controlled labs, LAV-SE was completed in 490 (359 male, age 67 ± 12years) with suspected or known chronic coronary syndromes (n = 462) or asymptomatic controls (n = 28). The utilized stress was exercise in 177, vasodilator in 167, dobutamine in 146. LAV was measured with the biplane disk summation method. SE was performed with the ABCDE protocol. The intra-observer and inter-observer LAV variability were 5% and 8%, respectively. ∆-LAVI changes (stress-rest) were negatively correlated with resting LAVI (r = −0.271, p < 0.001) and heart rate reserve (r = -.239, p < 0.001). LAV-dilators were defined as those with stress-rest increase ≥ 6.8ml/m2, a cutoff derived from a calculated reference change value above the biological, analytical and observer variability of LAVI. LAV dilation occurred in 56 patients (11%), more frequently with exercise (16%) and dipyridamole (13%) compared to dobutamine (4%, p < 0.01). At multivariable logistic regression analysis, B-lines ≥ 2 (OR: 2.586, 95% CI = 1.1293–5.169, p = 0.007) and abnormal contractile reserve (OR: 2.207, 95% CI = 1.111–4.386, p = 0.024) were associated with LAV dilation. In conclusion, LAV-SE is feasible with high success rate and low variability in patients with chronic coronary syndromes. LAV dilation is more likely with reduced left ventricular contractile reserve and pulmonary congestion.

Background: Left ventricular noncompaction cardiomyopathy (LVNC) is associated with poor clinical outcome in childhood. Standard diagnostic criteria are still controversial, especially in young patients. Recent studies in adults demonstrated that left ventricular (LV) twist is abnormal in LVNC, but it has not been investigated in pediatric patients to date. Our aim was to assess LV cardiac mechanics, LV twist, and the prevalence of rigid body rotation, using 2-dimensional speckle tracking echocardiography, in young patients with LVNC and LV hypertrabeculation. Methods: Forty-seven children (age range: 0–18 years) were assessed for suspected LVNC. All patients underwent 2-dimensional speckle tracking echocardiography and cardiovascular magnetic resonance imaging at 1.5 Tesla (T). Twenty-three patients fulfilled the cardiovascular magnetic resonance imaging diagnostic criteria for LVNC (LVNC group), while the remaining 24 did not and were included in the LV hypertrabeculation group. Forty-seven age- and sex-matched healthy volunteers were used as controls. Results: The average LV twist was significantly reduced in LVNC compared with control and LV hypertrabeculation. Rigid body rotation was recognized in 13 (56%) children with LVNC and in 1 (4%) child with LV hypertrabeculation and a strong family history for LVNC. Multivariable analysis demonstrated that LV twist is an independent predictor of LVNC ( P =0.006; coefficient=0.462). The receiver operating characteristics curve showed that LV twist had optimal predictive value to discriminate patients with LVNC (cutoff value Conclusions: LV twist has good predictive value in diagnosing LVNC in young patients. Our findings strongly support the routine use of 2-dimensional speckle tracking echocardiography in the evaluation of young patients with suspected LVNC.

Introduction Blood speckle imaging (BSI) is a new speckle-tracking-based technique for blood flow study. Flows assessment is particularly challenging in Congenital heart diseases (CHDs), where a profound distortion in cardiac anatomy gives reason of alteration in fluid mechanics. Up until now doppler methods were the only ones used for the analysis of fluid. Overcoming standard Doppler limits, BSI is becoming a promising new approach for the study of fluid dynamics. The aim of the study was to assess fluid-dynamics of different CHDs by using BSI. Methods and results Ten children (7 with suspected CHDs and 3 controls) were enrolled for the purpose between January and June 2019. All of them underwent a complete 2D and Doppler echocardiographic assessment plus a further BSI study. We studied: one complex transposition of great arteries, one coarctation of aorta, one tetralogy of Fallot, one complete atrio-ventricular septal defect, one suspicious flow in pulmonary artery, one aortic stenosis, one hypertrophic cardiomyopathy and three normal controls. Conclusion Compared to conventional Doppler methods, BSI was able to better describe fluid dynamics and hemodynamics of these CHDs by showing laminar and turbulent flows and vortices. In our experience, it helped to define the diagnosis and, in some cases, drove the management.

The right ventricle plays a major role in congenital heart disease. This article describes the right ventricular mechanics in some selected congenital heart diseases affecting the right ventricle in different ways: tetralogy of Fallot, Ebstein anomaly, and the systemic right ventricle.

Background Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. Methods Fifty consecutive EA patients (1 day–18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ± 41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. Results Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5–83) vs 28.4 mm/m2 (IQR 17.5–47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ± 7.7% vs 36.7 ± 9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7–0.98) vs 0.55 (IQR 0.4–0.7); p = 0.000], a lower functional RV-longitudinal strain (−10.2 ± 6.2% vs −16.2 ± 7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ± 13.5% vs 36.3 ± 12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. Conclusion Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.

Doppler interrogation of blood flow in the hepatic veins (HVs) provides valuable information regarding a wide spectrum of pathological processes that affect the right heart. Systematic analysis of the direction, velocity, and phasicity of the HV waveforms allows one to distinguish normal from abnormal patterns and provides important diagnostic information. Abnormalities in heart rate, rhythm, and intracardiac conduction are commonly encountered during echocardiographic studies. Sinus bradycardia and tachycardia, bradyarrhythmias and tachyarrhythmias as well as atrioventricular conduction disturbances influence the flow pattern in the HVs and may pose a challenge to the correct interpretation of the HV Doppler. Alterations in HV flow that are induced by the electrical abnormalities may mimic right heart pathology. Awareness of these alterations allows one to avoid misinterpretation of the HV signal, helps diagnose the underlying rhythm or conduction abnormality, and permits assessment of the impact on right heart hemodynamics.

Spectral Doppler interrogation of the descending thoracic and abdominal aorta provides valuable information regarding cardiac and vascular hemodynamics. An abnormal aortic Doppler profile is encountered in pathological conditions that affect the aorta and its branches, the aortic valve, the left ventricle, and the pericardium. Characteristic findings on Doppler interrogation of the aorta are often noted in individuals with obstructive atherosclerotic disease of the aorto-iliac system including severe stenosis or occlusion of the distal aorta and/or iliac arteries. In this manuscript, we highlight the findings on spectral Doppler that led to the identification of occlusive disease in the distal aorta.

Aims: Three-dimensional speckle-tracking echocardiography (3D-STE) is a promising new technique to evaluate left ventricular (LV) mechanics. The feasibility and normal values of LV strain using 3D-STE have recently been established in adults and children. Unfortunately, no data are available in neonates. The aims of this study were to evaluate the feasibility and establish normal values of 3D LV volumes, ejection fraction (EF), and the 4 normal strains in healthy neonates. Materials and Methods: Of 50 consecutive newborns who were delivered at our hospital or returned to their first newborn follow-up within the first 3 weeks of life, 38 babies underwent full echocardiographic evaluation, including the acquisition of at least 3 full volume data sets from the apical window, while naturally sleeping. Data sets were analyzed offline. Global LV longitudinal, circumferential, and radial strain, as well as 3D LV volumes and EF, were measured using 3D-STE. Results: Of the 50 newborns, 2 patients were excluded because of significant intra-cardiac shunts, and in another 10 subjects, parents did not give consent. At least one data set was adequate for analysis in all the remaining subjects. Mean indexed LV diastolic, systolic volumes, and EF were 24.7 ± 3.6 ml/m 2 , 9.2 ± 1.3 ml/m 2 , and 63% ± 3.7%, respectively. Normal global longitudinal, circumferential, radial, and tangential 4D strain were −20.9% ± 2.8%, −32.4% ± 3.1%, 44.3% ± 3.4%, and −39.7% ± 3.4%, respectively. Conclusions: 3D-STE is feasible in newborns without the needed for sedation. Reference values of normal, regional, and global LV 4D strain and volumes were obtained.

Aim: To determine whether right atrial (RA) function has prognostic value in patients with idiopathic pulmonary arterial hypertension (PAH).Methods and results: Overall, 104 patients (70 female, mean age 58 +/- 13 years) with idiopathic PAH underwent standard Doppler echocardiography and strain and strain rate (SR) analysis before right heart catheterization. At a mean follow-up of 22 +/- 7 months, 30 patients (29%) had clinical worsening. On Cox multivariable proportional-hazards regression analysis, RA reservoir function measured as peak longitudinal SR (hazard ratio [HR] 0.5; P < 0.0001), RA area (HR 1.2; P < 0.01), right ventricular (RV) SR (HR 0.6; P < 0.0001), cardiac index (HR 0.79; P < 0.01), and mixed venous oxygen saturation (HR 0.82; P < 0.01) were found to be independent correlates of cardiac events. A RA SR reservoir cut-off value of

Background: In patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associatedwith abnormal aortic bioelastic properties.Hypothesis: GA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1-the occurrence of GA in a large sample of patients after ASO; 2-the association between GAand aortic bio-elastic properties; and 3-the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE). Methods: We studied one hundred and five asymptomatic patients, who have undergone first stage ASO for d-TGA, with normal left ventricular ejection fraction (LVEF >= 53%).Results: Forty-six (44%) patients showed a GA (mean age 11.5 +/- 7.2 years, 26 males) while fifty-nine (56%) patients (mean age 9.6 +/- 6.7 years, 37 males) did not present GA. The two groupswere comparable for age, sex, BSA, and office blood pressure values. In group GA aortic root was significantly dilated(27.4 +/- 7.5mm vs. 21.2 +/- 6.9 mm, p < 0.0001), aortic stiffness index (Group GA= 1.8 +/- 1.2 vs. 1.4 +/- 0.7, p = 0.025) was significantly increased, left atrial volume was larger (p = 0.0145), global longitudinal strain (Group GA= -18.4 +/- 2.5% vs. -20.1 +/- 3.3%, p= 0.012) and basal LV longitudinal strains (Group GA= -16.9 +/- 4.8% vs. -20.4 +/- 7.0%, p= 0.013) were significantly reduced.Conclusions: After ASO the presence of a GA is associated with a significantly dilated aortic root, stiffer aortic wall, larger left atrial volume, and worse LV longitudinal systolic deformations, well known predictors of cardiovascularmorbidity and mortality. (C) 2017 Elsevier B.V. All rights reserved.