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Introduction: Pulmonary valve stenosis determines multiple effects on the right ventricular dimension and function. Percutaneous balloon valvuloplasty is the treatment of choice in severe pulmonary valve stenosis in patients of all ages. However, little is known regarding right ventricular function immediate changes after percutaneous balloon dilation. Pediatric patients with isolated pulmonary valve stenosis represent a pure clinical model of chronic RV pressure overload not affected by other confounders or comorbidities. Aim of the study: This study seeks to explore right ventricle (RV) mechanics in pediatric patients early after percutaneous balloon pulmonary valvuloplasty (BPV) for valvar pulmonary stenosis (PS). Materials and Methods: Forty-three pediatric patients (19 males), mean age 3.2 ± 4.9 years old, with severe pulmonary valve stenosis and indication for percutaneous balloon valvuloplasty were recruited. All patients underwent standard transthoracic echocardiography (TTE), and speckle-tracking echocardiography (STE) with an analysis of right ventricle free-wall longitudinal strain (RVFWLS) one day before and one day after the procedure. For each patient, we collected invasive parameters during the interventional procedure before and after BPV. Results: After the procedure, there was an immediate significant reduction in both peak-to-peak transpulmonary gradient (peak-to-peak PG) and ratio between the right ventricle and aortic systolic pressure (RV/AoP) with a drop of ∆29.3 ± 14.67 mmHg and ∆0.43 ± 0.03, respectively. Post-procedural echocardiography showed peak and mean transvalvar pressure gradient drop (∆50 ± 32.23 and ∆31 ± 17.97, respectively). The degree of pulmonary valve regurgitation was mild in 8% of patients before the procedure, affecting 29% of our patients post-BPV (p = 0.007). The analysis of right ventricular mechanics showed a significant improvement of fractional area change (FAC) immediately after BPV (40.11% vs. 44.42%, p = 0.01). On the other hand, right ventricular longitudinal systolic function parameters, TAPSE and global RVFWLS, did not improve significantly after intervention. The segmental analysis of the RVFWLS showed a significant regional increase in the myocardial deformation of the apical segments. Conclusions: Percutaneous BPV represents an efficient and safe procedure to relieve severe pulmonary valve stenosis. The analysis of the right ventricular function on echocardiography demonstrated an immediate global systolic function improvement, while longitudinal systolic function was persistently impaired 24 h after intervention, possibly due to the necessity of a longer recovery time.

Objective Wolff-Parkinson-White Syndrome (WPW) has been associated with reduced local myocardial deformation, and when left ventricular dysfunction is present, catheter ablation of the accessory pathway may be required, even in asymptomatic patients. We aimed to evaluate the diagnostic value of non-invasive myocardial work in predicting subtle abnormalities in myocardial performance in children with WPW. Methods and results Seventy-five paediatric patients (age 8.7 ± 3.5 years) were retrospectively recruited for the study: 25 cases with manifest WPW and 50 age- and sex- matched controls (CTR). Global myocardial work index (MWI) was measured as the area of the left ventricle (LV) pressure-strain loops. From MWI, global Myocardial Constructive Work (MCW), Wasted Work (MWW), and Work Efficiency (MWE) were estimated. In addition, standard echocardiographic parameters of LV function were evaluated. Despite normal LV ejection fraction (EF) and global longitudinal strain (GLS), children with WPW had worse MWI, MCW, MWW and MWE. At multivariate analysis, MWI and MCW were associated with GLS and systolic blood pressure, and QRS was the best independent predictor of low MWE and MWW. In particular, a QRS>110 msec showed good sensitivity and specificity for worse MWE and MWW values. Conclusion In children with WPW, myocardial work indices were found significantly reduced, even in presence of normal LV EF and GLS. This study supports the systematic use of myocardial work during the follow-up of paediatric patients with WPW, and myocardial work analysis may represent a sensitive measure of LV performance and aid in decision-making.

OBJECTIVES To investigate the long-term outcomes of concomitant suture bicuspidization to treat mild or moderate tricuspid regurgitation at the time of mitral valve surgery. METHODS Data from patients who underwent mitral valve surgery for degenerative mitral valve regurgitation with mild or moderate tricuspid regurgitation and annular dilatation between January 2009 and December 2017 were analyzed. The cohort was divided into 2 groups: mitral valve surgery alone and mitral valve surgery with concomitant tricuspid valve repair. RESULTS A total of 196 patients were included in the study. Mitral valve surgery alone and mitral valve surgery with concomitant tricuspid valve repair were performed in 91 (46.4%) and 105 (53.6%) patients, respectively. Propensity score matching analysis identified 54 pairs. In the matched cohort, 30-day mortality (0.0% vs 1.9%, P = 1.0) and new permanent pacemaker implantation (11.1% vs 7.4%, P = 0.740) did not differ significantly between groups. After a mean follow-up of 6.0 (2.8) years, mitral valve surgery with concomitant tricuspid valve repair was not associated with increased mortality risk compared to mitral valve surgery alone (hazard ratio 1.04, 95% confidence interval 0.47–2.28, P = 0.927) with 10-year overall survival rates of 69.9% and 77.2%, respectively. Furthermore, mitral valve surgery with concomitant tricuspid valve repair was associated with a significantly reduced progression of tricuspid valve regurgitation (P CONCLUSION Patients undergoing mitral valve surgery with concomitant tricuspid valve repair had similar 30-day and long-term survival, similar permanent pacemaker implantation rate, and reduced progression of tricuspid valve regurgitation compared to those undergoing mitral valve surgery alone.

(1) Background: Transthoracic echocardiography is the first-line non-invasive investigation for assessing pediatric patients’ cardiac anatomy, physiology, and hemodynamics, based on its accessibility and portability, but complete anatomic and hemodynamic assessment is time-consuming. (2) Aim: This study aimed to determine whether an automated software developed for adults could be effectively used for the analysis of pediatric echocardiography studies without prior training. (3) Materials and Methods: The study was conducted at the University Hospital of Bordeaux between August and September 2022 and included 45 patients with normal or near normal heart architecture who underwent a 2D TTE. We performed Spearman correlation and Bland-Altman analysis. (4) Results: The mean age of our patients at the time of evaluation was 8.2 years ± 5.7, and the main reason for referral to our service was the presence of a heart murmur. Bland-Altman analysis showed good agreement between AI and the senior physician for two parameters (aortic annulus and E wave) regardless of the age of the children included in the study. A good agreement between AI and physicians was also achieved for two other features (STJ and EF) but only for patients older than 9 years. For other features, either a good agreement was found between physicians but not with the AI, or a poor agreement was established. In the first case, maybe proper training of the AI could improve the measurement, but in the latter case, for now, it seems unrealistic to expect to reach a satisfactory accuracy. (5) Conclusion: Based on this preliminary study on a small cohort group of pediatric patients, the AI soft originally developed for the adult population, had provided promising results in the evaluation of aortic annulus, STJ, and E wave.

Objective Obesity is known to be associated with cardiovascular compromise and is a major risk factor for the development of hypertensive disorders in pregnancy. The aim of this study was to investigate the effect of obesity on the maternal cardiovascular system. Design This was a prospective, observational, longitudinal study. Setting A tertiary centre in London Population Pregnant women with booking body mass index (BMI) ≥ 30kg/m2 (n=64) were compared to pregnant women with normal booking BMI (20-24.9kg/m2) (n=14). Methods Two-dimensional trans-thoracic echocardiography. Main outcomes Longitudinal difference in blood pressure, cardiac geometry and cardiac function between the groups. Results In women with obesity, the blood pressure, heart rate and cardiac output were higher and peripheral vascular resistance was lower (p

Advanced echocardiographic and CMR features of patients with repaired Tetralogy of Fallot in the long-term follow-up. Anna Gozzi1, Jolanda Sabatino1, Elena Cuppini1, Irene Cattapan1, Alessia Basso1, Jennifer Fumanelli1, Angela Di Candia1, Martina Avesani1, Domenico Sirico1, Biagio Castaldi1, Elena Reffo1, Alessia Cerutti1, Roberta Biffanti1, Giovanni Di Salvo1 1Department for Women's and Children's Health, University Hospital of Padova, Pediatric and Congenital Cardiology Unit, Via Nicolo` Giustiniani, 2, 35128 Padova, Italy Background Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Children and, more frequently young adults, with repaired ToF develop late complications. Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in patients with repaired tetralogy of Fallot. The aim of this study was to describe and analyse advanced echocardiographic and CMR features of patients with repaired ToF in the long-term follow-up and to find non-invasive predictors of adverse prognosis. Methods This is a retrospective cohort study. Consecutive 200 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 19 ± 8. The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively. Right myocardial work index (RWI) was calculated as the area of the right ventricular pressure strain loops. From RWI, Right Constructive Work (RCW), Wasted Work (RWW) and Work Efficiency (RWE) were estimated. Patients were screened according the age at surgery (12 months) and the time since the repair (< 25 years and ≥ 25 years). A composite end-point of major adverse cardiovascular events (sudden cardiac death, sustained and non-sustained ventricular tachycardia, resuscitated sudden cardiac death, or syncope) was used. Results CPET values were not significantly different in patients with more than 25 years since the corrective surgery. Left ventricular ejection fraction measured either by CMR or echocardiography were slightly but significantly reduced in patients with more than 25 years since the corrective surgery. Patients with older age at surgery (>12 months) have significantly reduced V02max at CPET, larger RA and LA areas and larger RV basal diameter. During a median follow-up of 2.9 years, 1 patient died suddenly, 5 had documented ventricular tachycardia, and other 4 had syncope. On univariate analysis, echocardiographic left atrial area/BSA (P=0.044) and right ventricular longitudinal strain (P=0.010) were significantly related to the combined end point. Patients with reported VT at Holter ECG presented significantly reduced right ventricular function in terms of CMR-EF, echocardiographic FAC and RWI. Finally, TOF patients with borderline/impaired predicted VO2 max at CPET presented with significantly reduced RV freewall longitudinal strain and right ventricular myocardial work index. Conclusion Long-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF - operated patients could be excellent up to 25 years after the repair. However, patients with older age at surgery have relative reduced functional status compared with those undergoing early repair. The echocardiographic and CMR evaluation of RV function are usually related to worse prognosis, recurrency of VT and reduced functional status in patients with repaired TOF. Right ventricular myocardial work index is feasible in patients with repaired TOF; disadvantageous right ventricular work may be a more sensitive indicator of right ventricular impairment compared with standard echocardiographic parameters, and is able to predict exercise capacity.

OBJECTIVES The aim of this retrospective multicentre study was to investigate and compare clinical outcomes of unilateral and bilateral antegrade cerebral perfusion (ACP) strategies on cerebral protection during surgery for type A aortic dissection. METHODS Data from 646 patients who underwent surgical repair of thoracic type A aortic dissection using unilateral and bilateral ACP with moderate hypothermic circulatory arrest in 3 cardiac surgical institutions between 2008 and 2018 were analysed. Propensity matching was performed to assess which technique ensured better outcomes. RESULTS Unilateral and bilateral ACP techniques were performed in 250 (39%) and in 396 (61%) patients, respectively. Propensity score analysis identified 189 matched pairs. In the matched cohort, the lowest core temperature was 27.5°C and 28°C in the bilateral and unilateral groups, respectively (P CONCLUSIONS Unilateral and bilateral ACP are both valid brain protection strategies in the landscape of aortic arch surgery. While admitting all the study limitations, unilateral technique could offer some clinical advantages. Clinical registration number 76049

Background: The major limitation to the Ross operation is a progressive autograft dilation, possibly leading to reoperations. A murine model was created to evaluate pulmonary artery graft (PAG) adaptation to pressure overload. Methods: Lewis rats (n = 17) underwent heterotopic surgical implantation of a PAG, harvested from syngeneic animals (n = 17). A group of sham animals (n = 7) was used as a control. Seriated ultrasound studies of the PAG were performed. Animals were sacrificed at 1 week (n = 5) or 2 months (n = 15) and the PAG underwent mechanical and histopathological analyses. Results: Echography showed an initial increase in diameter (p < 0.001) and a decrease in peak systolic velocity (PSV). Subsequently, despite no change in diameter, an increase in PSV was observed (p < 0.01). After 1 week, the stiffness of the PAG and the aorta were similar, while at 2 months, the PAG appeared more rigid (p < 0.05). PAG’s histological analysis at 2 months revealed intimal hyperplasia development. The tunica media showed focal thinning of the elastic lamellae and normally distributed smooth muscle cells. Conclusions: We demonstrated a stiffening of the PAG wall after its implantation in systemic position; the development of intimal hyperplasia and the thinning of the elastic lamellae could be the possible underlying mechanism.

Background Management of pregnancy and risk stratification in women with congenital heart diseases (CHD) are challenging, especially due to physiological haemodynamic modifications that inevitably occur during pregnancy. Aims To compare the accuracy of the existing pregnancy cardiovascular risk scores in prediction of maternal complications during pregnancy in CHD patients. Method and results From 2007 to 2018, all pregnant women with a CHD who delivered birth after 20 weeks of gestation were identified. The discriminating power and the accuracy of the five existing pregnancy cardiovascular risk scores [CARPREG, CARPREG II, HARRIS, ZAHARA risk scores, and modified WHO (mWHO)] were evaluated. Out of 104 pregnancies in 65 CHD patients, 29% experienced cardiovascular complications during pregnancy or post-partum. For the five scores, the observed rate of cardiovascular events was higher than the expected risk. The values of area under the ROC curve were 0.75 (0.62–0.88) for mWHO, 0.65 (0.53–0.77) for CARPREG II, 0.60 (0.40–0.80) for HARRIS, 0.59 (0.47–0.72) for ZAHARA, and 0.58 (0.43–0.73) for CARPREG. Conclusion The modified WHO classification appeared to better predict cardiovascular outcome in pregnant women with CHD than the four other existing risk scores. Clinical Trial Registration: Clinicaltrials.gov: NCT04221048.

Systemic right ventricle (SRV) is commonly encountered in patients with congenital heart disease. This nomenclature includes diseases with different anatomic features, adaptation and clinical phenotypes, and has a variable - but overall guarded - prognosis. Right ventricular fibromuscular architecture, shape, adaptation to overload conditions, rhythmic disorders and - most of all - tricuspid regurgitation (TR) contribute to the pathophysiology of SRV failure. The pivotal role of TR is complex as it is due to both the intrinsic abnormalities of the valve (specific to each phenotype) and the consequence of SRV dilation and failure. Medical therapy has not been equivocally proven to be effective for TR. Surgery (valve repair or replacement) has shown conflicting long-term results, mainly dependent on preoperative SRV function. Thus, other management options have been proposed to improve SRV function and valve competency, such as early anatomical correction, pulmonary banding, resynchronization therapy and valvular edge-to-edge percutaneous repair. The aim of this review is to discuss the mechanisms of TR and SRV failure, as well as the available therapeutic options.

Congenital left ventricular (LV) diverticulum is a rare condition characterized by the presence of a contractile appendix originating usually from the cardiac apex, but with high variability in location, dimension, and clinical presentation. We describe the diagnostic process and clinical management of an isolated apical diverticulum discovered during fetal life. (Level of Difficulty: Advanced.), Central Illustration

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.

Duchenne muscular dystrophy (DMD) is the most frequent and severe form of MD. It firstly affects the skeletal muscles, causing severe disability, and subsequently the myocardium. The only two options to treat end‐stage heart failure in these patients are either a left ventricular assist device (LVAD) implantation as destination therapy or a heart transplant. These hypotheses are still controversial, and data are very limited. We describe the case of an 18‐year‐old male patient, affected by DMD and in a wheelchair from the age of 11. He progressively developed dilated cardiomyopathy, and in 2016, at the age of 14 years, he underwent HeartWare LVAD implantation, as destination therapy, without post‐operative complications. He has been followed up for 47 consecutive months; and 30 months after LVAD implantation, he developed an infection of the exit site, treated by antibiotics and surgical toilette. Following this event, on the basis of patient's good general conditions and willingness, we started to consider heart transplant as an option. Before the patient was listed, he underwent accurate workup, and we found higher values of forced vital capacity, forced expiratory volume in 1 s, and peak expiratory flow, compared with the predicted values of same‐age DMD patients. The patient have neither scoliosis nor need for non‐invasive mechanical ventilation, and finally, he was always treated with steroids with stable thoraco‐abdominal function over the years. According to these considerations, the patient was listed for heart transplant. In 12 February 2020, at the age of 18 years, the patient underwent heart transplant with no post‐operative complications. Cardiac transplantation is not considered a valid option for DMD patients, because of the shortage of donor availability and the systemic nature of DMD disease. Considering that this patient had already experienced an LVAD‐related complication and he had better general condition than his DMD peers, we listed him for a heart transplant. We described the case of a DMD patient who underwent successful heart transplantation after 47 months of HeartWare LVAD assistance. Three months' follow‐up is uneventful.

OBJECTIVES Many centres have recently adopted pulmonary valve (PV) preservation (PVP) during tetralogy of Fallot (ToF) repair. We sought to identify the midterm functional outcomes and risk factors for pulmonary regurgitation after this procedure. METHODS All patients undergoing PVP during transatrial–transpulmonary repair for ToF with PV stenosis at our institution between January 2007 and December 2020 were reviewed. RESULTS Overall, 73 patients were included. At the index surgery, the body surface area was 0.31 ± 0.04 m2, the age was 4.9 ± 2.9 months and the preoperative PV z-score was -3.02 ± 1.11. At a mean follow-up of 5.3 ± 2.7 years, the fractional area change of the right ventricle (RV) was 47.1 ± 5.2%, and the tricuspid annular plane systolic excursion z-score was -3.31 ± 1.89%. The 5-year freedom from moderate/severe PV regurgitation was 61.3% [95% confidence interval (CI): 48, 73%]. There was a significant correlation between RV function and moderate/severe PR at follow-up (R2: 0.08; P = 0.03). A comparison with a group of patients undergoing a transannular patch procedure (N = 33) showed superior outcomes for patients with PVP. The preoperative PV z-score and the degree of PR at discharge were risk factors for the early development of moderate/severe PR at follow-up [hazard ratio (HR): 0.64; 95% CI: 0.48, 0.86, P = 0.01 and HR: 2.31; 95% CI: 1.00, 5.36, P = 0.04, respectively]. A preoperative PV annulus z-score ≤ -2.85 was found to be predictive for moderate/severe PR at 5 years after PVP (HR: 2.56; 95% CI: 1.31, 5.01, P = 0.002). CONCLUSIONS A pulmonary valve preservation strategy during tetralogy of Fallot repair should always be attempted. However, a preoperative PV annulus z-score < -2.85 and moderate/severe regurgitation upon discharge are risk factors for midterm pulmonary regurgitation.

The Ross operation for aortic valve disease has regained new interest due to its outstanding long-term results. Nonetheless, when employed as freestanding root replacement, the possible dilation of the pulmonary autograft and subsequent aortic regurgitation is described. Several animal models have been proposed. However, these are usually limited to ex-vivo models or in-vivo experiments with relatively expensive large animal models. In this study, we sought to establish a rodent model of pulmonary artery graft (PAG) implantation in a systemic position. A total of 39 adult Lewis rats were included. Immediately after euthanasia, the pulmonary root was harvested from a donor animal (n=17). Syngeneic recipient (n=17) and sham-operated (n=5) rats were sedated and ventilated. In the recipient group, the PAG was implanted with an end-to-end anastomosis in infra-renal abdominal aortic position. Sham-operated rats underwent only transection and re-anastomosis of the aorta. Animals were followed with serial ultrasound studies for two months and post-mortem histological analysis. The median PAG diameter in the native position was 3.20 mm (IQR=3.18-3.23). At follow-up, the median diameter of the PAG was 4.03 mm (IQR=3.74-4.13) at 1 week, 4.07 mm (IQR=3.80-4.28) at 1 month, and 4.27 mm (IQR=3.90-4.35) at 2 months (p0.01). Peak systolic velocity was 220.07 mm/s (IQR=210.43-246.41) at 1 week, 430.88 mm/s (IQR=375.28-495.56) at 1 month, and 373.68 mm/s (IQR=305.78-429.81) at 2 months (p=0.02) and did not differ from the sham-operated group at the end of the experiment (p=0.5). Histological analysis did not show any sign of endothelial thrombosis. This study showed that rodent models may allow for the evaluation of the long-term adaptation of the pulmonary root to a high-pressure system. A systemically placed syngeneic PAG implantation represents a simple and feasible platform for the development and evaluation of novel surgical techniques and drug therapies to further improve the outcomes of the Ross operation.

The hypothermic circulatory arrest is a major limitation of the frozen elephant trunk (FET) technique, associated with a high incidence of postoperative mobility and mortality. The aim of this study was to evaluate the surgical outcomes of patients suffering from different aortic arch diseases treated with the release and perfuse technique (RPT).We retrospectively reviewed the records of patients who had undergone aortic arch repair with the RPT at our Institution between October 2019 and September 2021. Preoperative, intra-operative, and postoperative data were collected.A total of 18 patients undergoing aortic arch surgery with the use of RPT were analyzed. Twelve of them (67%) were males, and the median age of the entire cohort was 67 (56-73) years. The primary indications for surgery included acute type A aortic dissection (50%), chronic type B aortic dissection (33%), and chronic aneurysms (17%). The median cardiopulmonary bypass-, aortic cross-clamp- and circulatory arrest times were 163 (147-213) min, 69 (51-120) min, and 10 (8-13) min, respectively. The median intensive care unit and hospital stay were 4 (2-7) and 12 (11-16) days, respectively. One patient (6%) died during the first 30 days after surgery.Considering the anatomical limitations related to the use of this technique, the RPT can be safely performed and could represent a promising strategy to reduce the circulatory arrest time during aortic arch surgery. Nevertheless, further studies are required to demonstrate its efficacy.

Aims Paediatric patients with a diagnosis of Wolff–Parkinson–White (WPW) Syndrome may develop a reduction of local myocardial deformation because of accessory pathway-related electrical dyssynchrony, which may lead to an impairment of left ventricular systolic function. The presence of ventricular dysfunction may be an indication for these patients to undergo radiofrequency catheter ablation (RFCA), even if asymptomatic. However, myocardial abnormalities are sometimes subtle and cannot be detected by standard echocardiographic evaluation. The purpose of this study was to assess the diagnostic value of non-invasive myocardial work in predicting subtle myocardial abnormalities in paediatric patients with WPW Syndrome. Methods and results Forty-four paediatric patients (age 8.2 ± 4.3 years) were included in this study: 12 cases with manifest WPW Syndrome and 32 age-, sex-, and arterial pressure-matched controls (CTR). Left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) were evaluated. Global myocardial work index (MWI) was measured 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 standard echocardiographic parameters, paediatric patients with WPW Syndrome had lower MWI (1233.5 ± 281.6 mmHg% in WPW vs. 1624.0 ± 305.8 mmHg% in CTR, P = 0.0004), MCW (1833.4 ± 257.9 mmHg% in WPW vs. 2069.0 ± 319.9 mmHg% in CTR, P = 0.03), MWW (187.6 ± 117.7 mmHg% in WPW vs. 90.9 ± 58.9 mmHg% in CTR, P = 0.0008), and GWE (90.5 ± 4.8% in WPW vs. 95.2 ± 2.2% in CTR, P = 0.00006). There were no significant differences in GLS and LVEF between patients with WPW Syndrome and controls. Conclusions WPW Syndrome was found to be associated with a significant reduction of myocardial work indices in the paediatric population. The assessment of MWI may represent a sensitive measure to identify a subtle myocardial dysfunction in an early stage, even when LVEF and GLS are normal. It might be considered a further diagnostic parameter for referring little patients to RFCA.

Aims To describe the current application of exercise stress echocardiography (ESE) in our Paediatric Cardiology Departments. Methods and results Baseline and under stress symptoms, vital parameters, ECG, and echocardiograms from patients who underwent ESE in our centres, were retrospectively analysed and compared, as well as clinical management plans formulated based on ESEs results. Forty-five patients from Centre 1 (median age 16 years), including 87% of patients with congenital heart diseases (CHDs), and 20 patients from Centre 2 (median age 11 years), mainly tested to rule out myocardial ischemia, were included. Among patients from Centre 1, 28 had previously been treated surgically, 6 percutaneously, and 11 were under follow-up. Indications for ESE/patients’ native diagnosis are illustrated in the picture. Centre 1: Exercise was maximal in 17 patients, with 2 of them having symptoms at the peak of exercise. It was stopped beforehand in 28 patients because of dyspnoea (3) and muscle fatigue (25). No arrhythmia was detected. ESE was considered as positive in 14 patients; after that, 3 patients underwent percutaneous interventions, 2 underwent cardiac surgery, 3 received indication for cardiac catheterization, 4 for advanced cardiac imaging, and 2 for exercise restriction and medical therapy. Centre 2: 15 patients (75%) completed the exercise, and none of them developed symptoms. In the remaining 25%, exercise was stopped because of muscle exhaustion. No arrhythmia was detected, and all the ESEs were negative. Comparing the cohorts, no differences in terms of ejection fraction were noticed at rest and under stress. Patients in cohort 1 were older (P = 0.002), they achieved lower average maximal heart rate (P = 0.0001), performed less lasting exercise (P = 0.05), and ESE was maximal less frequently (P = 0.005). Lastly, they had significant changes in clinical decisions (P = 0.004). Conclusions In paediatric cardiology, particularly in children with CHDs, ESE is a promising technique and could influence significantly clinical management plans.

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

International audience; Background: This study aimed to detect late sub-clinical patterns of cardiac dysfunction using speckle tracking echocardiography (STE) in children with cancer remission more than 12 months after the end of anthracycline treatment.Methods: This prospective controlled study enrolled 196 children, 98 of which had been treated with anthracyclines (mean age 10.8 ± 3.6 years; 51% female) and 98 were age- and gender-matched healthy subjects in a 1:1 case-control design. Conventional echocardiographic variables were collected for left ventricle (LV) and right ventricle (RV). STE analyses were performed in the LV longitudinal, radial, and circumferential displacements and in the RV free wall longitudinal displacement. The association between LV global longitudinal strain (GLS) and the main clinical and biological parameters was evaluated.Results: After a mean time interval of 5.1 ± 3.2 years since the end of chemotherapy (mean cumulative anthracycline dose of 192 ± 96 mg/m2), conventional echocardiographic measures were normal. GLS was significantly decreased in the anthracycline group (-19.1% vs. -21.5%, P < 0.0001), with a higher proportion of children with abnormal values (Z-score < -2 in 18.6% vs. 1.0%, P < 0.0001). No association was found between GLS and clinical or biological parameters. Circumferential strain was significantly worse in the anthracycline group (-16.8% vs. -19.4%, P < 0.0001), and radial strain significantly better (+51.4% vs. +35.9%, P < 0.0001). RV conventional echocardiography and STE parameters were normal and not different between anthracycline and control groups.Conclusions: The existence of a modified LV strain despite normal LV function in children treated with anthracyclines represents an important perspective for cardiomyopathy surveillance in childhood cancer survivors. Clinical Trial Registration -ClinicalTrials.gov Identifier: NCT02893787.

Although some anecdotal experience concerning the use of modern sutureless aortic valve bioprosthesis for the treatment of endocarditis has been reported, no data is available on the mid-and long-term results. We describe a case of a patient successfully treated with Perceval sutureless bioprosthesis for aortic valve endocarditis and his seven years follow-up.

The heart transplantation (HT) is undoubtedly the best treatment for end-stage heart failure patients (2). However, the organ shortage remains a major challenge in cardiac surgery. Facing this problem, the medical community starts to extend the donor criteria to select more suitable organs for HT. The use of ECDs is still controversial, since it is associated with a high incidence of primary graft failure (3), and although it guarantees longer survival than without transplantation, there is still some hesitation in accepting this practice.

Heart transplantation represents the gold standard for end-stage heart failure. However, due to the increasing demand and the shortage of available organs, donor supply remains the main limitation. Marginal donor hearts in high-risk candidates who do not meet standard listing criteria are the only alternative when life expectancy is limited, but their use is still debated. Surgical correction of detected coronary lesions or valvular heart defects allows further enlargement of the number of available organs. In this article, we offer a literature review on this topic and report two marginal donor hearts with angiography evidence of coronary stenosis and preserved ventricular function, which underwent concomitant myocardial revascularization during heart implantation.

We describe the case of a 59-year-old female affected by aortic extensive endocarditis with communication between aortic annulus and right cavities, treated with double pericardial patch reconstruction and rapid-deployment aortic valve prosthesis implantation.

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 The interest in intra-cardiac blood flow analysis is rapidly growing, and it has encouraged the development of different non-invasive imaging techniques. Among these, Vector Flow Mapping (VFM), combing Color-Doppler imaging and speckle tracking data, seems to be a promising approach, feasible in adult and children population. Aim of the review The aim of this review is to give a historical perspective on the development of VFM method and a summary of the current algorithms and parameters potentially evaluable. Then, we will present the current state-of-the-art of VFM with an overview of clinical studies and applications of this technique.

Obesity in pregnancy is associated with substantial risks, notably hypertensive disorders. Bariatric surgery achieves sustained weight loss and has several cardiovascular benefits, including positive effects on blood pressure, cardiac geometry, and both systolic and diastolic function. Pregnancy following bariatric surgery is also associated with improved outcomes, including a reduced risk of hypertensive disorders. The underlying mechanisms, however, remain uncertain. Maternal cardiovascular adaptation plays a vital role in maintaining a healthy pregnancy, and maladaptation has been associated with adverse pregnancy outcomes. However, to date, the maternal cardiovascular adaptation to pregnancy after bariatric surgery has not been investigated.To investigate the maternal cardiovascular adaptation to pregnancy in women with previous bariatric surgery compared with women with a similar early-pregnancy body mass index, age, and race but no history of weight loss surgery.This was a prospective, observational, longitudinal study including pregnant women with (n=41) and without (n=41) a history of bariatric surgery. The participants were seen at 3 time points; at 12 to 14, 20 to 24, and 30 to 32 weeks of pregnancy. At each visit, the blood pressure was measured and the maternal cardiovascular system was assessed using transthoracic echocardiography. Two-dimensional speckle tracking was performed to assess the global longitudinal and circumferential strain on a subset of patients (15 in each group). Offline analysis was performed according to the European and American echocardiography guidelines. Multilevel linear mixed-effect models were used for all the comparisons.Compared with the no-surgery group, women with previous bariatric surgery, had lower systolic and diastolic blood pressure, heart rate, and cardiac output across all the trimesters (P.01 for all comparisons), with an evidence of more favorable diastolic indices, including a higher E-wave/A-wave ratio across the mitral valve (P.001), higher mitral velocity at the lateral and medial annulus (E') (P=.01 and P=.03, respectively), and a lower left atrial volume (P.05). Furthermore, women with previous bariatric surgery demonstrated lower global longitudinal (P.01) and circumferential strain (P=.02), which is suggestive of better systolic function.Our findings indicate better cardiovascular adaptation to pregnancy in women with previous bariatric surgery than in pregnant women of a similar early-pregnancy body mass index but no history of surgery.