Nationwide hospitalizations of patients with down syndrome and congenital heart disease over a 15-year period.

Down syndrome is one of the most common genetic diseases, generally associated with an increased probability of congenital heart diseases. This increased risk contributes to escalated levels of morbidity and mortality. In this study, we sought to analyze nationwide data of pediatric and adult patients with Down syndrome and congenital heart disease over a 15-year period. Data obtained from the hospital discharge form between 2001 and 2016 of patients diagnosed with Down syndrome in Italy and at least one congenital heart disease were included. Information on 12362 admissions of 6527 patients were included. Age at first admission was 6.2 ± 12.8 years and was a predictor of mortality (HR = 1.51, 95% CI 1.13-2.03, p = 0.006). 3923 (60.1%) patients underwent only one admission, while 2604 (39.9%) underwent multiple (> 1) admissions. There were 5846 (47.3%) admissions for cardiac related symptoms. Multiple admissions (SHR: 3.13; 95% CI: 2.99, 3.27; P < 0.01) and cardiac admissions (SHR: 2.00; 95% CI: 1.92, 2.09; P < 0.01) were associated with an increased risk of additional potential readmissions. There was an increased risk of mortality for patients who had cardiac admissions (HR = 1.45, 95% CI: 1.08-1.94, p = 0.012), and for those who underwent at least 1 cardiac surgical procedure (HR = 1.51, 95% CI 1.13-2.03, p = 0.006). CONCLUSIONS: A younger age at first admission is a predictor for mortality in patients with Down syndrome and congenital heart disease. If patients undergo more than one admission, the risk of further readmissions increases. There is a pivotal role for heart disease in influencing the hospitalization rate and subsequent mortality. WHAT IS KNOWN: • Down syndrome individuals often face an increased risk of congenital heart diseases. • Congenital heart diseases contribute significantly to morbidity and mortality in Down syndrome patients. WHAT IS NEW: • This study analyzes nationwide data covering a 15-year period of pediatric and adult patients in Italy with Down syndrome and congenital heart disease. • It identifies a younger age at first admission as a predictor for mortality in these patients, emphasizing the criticality of early intervention. • Demonstrates a correlation between multiple admissions, particularly those related to cardiac issues, and an increased risk of further readmissions, providing insights into the ongoing healthcare needs of these individuals.

Enhancing parental understanding of congenital heart disease through personalized prenatal counseling with 3D printed hearts.

OBJECTIVES: In addition to a correct prenatal diagnosis of congenital heart disease (CHD), comprehensive parental counseling is crucial to ensure that parents are well-informed about the condition of the fetus. This study aims to investigate whether there is a significant difference in the information acquired by parents through traditional counseling, utilizing 2-dimensional (2D) illustrations and images, compared to an advanced approach utilizing personalized three-dimensional (3D) printed models of the fetal heart developed from 3D ultrasound imaging. METHODS: This study, designed as a pilot randomized control trial, enrolled pregnant women with gestational ages greater than 18 weeks, whose fetuses were diagnosed with CHD and referred to our center between November, 2020 and June, 2021. Two groups of patients were included in the study. The first group received standard medical counseling with 2D images and illustrations, while the second group underwent advanced counseling with 3D-printed patient-specific heart models. Both groups were then required to complete the same survey in which the knowledge of the CHD was investigated. The 3D models were created from 3D ultrasound imaging and printed using resin materials in both 1:1 and 5:1 scale. RESULTS: A comparison of the scores obtained from the two groups revealed that 3D visualization of the fetus's heart has the potential to increase parental knowledge about CHD and the required surgical procedures. Furthermore, all couples expressed interest in receiving a 1:1 scale model of their baby's heart. CONCLUSION: Personalized prenatal counseling with 3D-ultrasound-based heart models positively impacts parents' understanding of CHD. The use of 3D models provides a more comprehensive and accessible representation of the condition, contributing to an increased knowledge gain, and potentially helping to support informed decisions regarding their child's care.

Maternal cardiovascular adaptation to pregnancy in obese pregnant women.

INTRODUCTION: Obesity is known to be associated with cardiovascular compromise and a major risk factor for the development of hypertensive disorders in pregnancy. However, little is known about the effect of obesity on maternal cardiac function. The aim of this study was to investigate the effect of obesity on the maternal cardiovascular system. MATERIAL AND METHODS: This was a prospective, observational, longitudinal study. Pregnant women with booking body mass index (BMI) ≥30 kg/m2 were compared with pregnant women with normal booking BMI 20-24.9 kg/m2. Participants were seen at three time points during pregnancy; 12-14, 20-24 and 30-32 weeks. At all visits, maternal blood pressure (BP) was measured, and cardiac geometry and function were assessed using two-dimensional trans-thoracic echocardiography. Multilevel linear mixed-effects models were used for all the comparisons. RESULTS: Fifty-nine pregnant women with obesity were compared with 14 pregnant women with normal BMI. In women with obesity, the maternal BP, heart rate and cardiac output were higher and peripheral vascular resistance was lower (p < 0.01 for all comparisons) compared with normal BMI women. Women with obesity had altered cardiac geometry with higher left ventricular end diastolic diameter, intraventricular septal thickness, posterior wall diameter, relative wall thickness and left ventricular mass (p < 0.001 for all comparisons). There was also evidence of impaired diastolic indices in the obese group with a lower E/A ratio, tissue Doppler imaging E' lateral and medial and higher left atrial volume (p < 0.01 for all comparisons). Finally, women with obesity had reduced longitudinal function, as assessed by mitral plane annular systolic excursion, between the second and third trimester of pregnancy, indicating possible early cardiac dysfunction in this group. CONCLUSIONS: Obesity is associated with maternal hyperdynamic circulation, altered cardiac geometry and suboptimal diastolic function, compared with normal BMI pregnant women, and these factors may contribute to the increased risk of complications in obese pregnant women.

Intracorporeal LVAD implantation in pediatric patients: A single-center 10 years' experience.

BACKGROUND: Mechanical cardiac support is currently an effective strategy to reduce morbidity and mortality in pediatric patients. However, solid evidence regarding the feasibility of intracorporeal devices in children still needs to be provided. We report our 10-year experience with intracorporeal left ventricular assist devices (LVAD) in children. MATERIALS AND METHODS: We included all patients undergoing intracorporeal, continuous-flow LVAD implantation between 2012 and 2022. Baseline and postoperative data were collected from the institutional database. RESULTS: Seven HeartWare and 4 HeartMate3 were implanted in 11 patients (median age 13.9 years, median body surface area - BSA - 1.42 m2, IQR 1.06-1.68). The most frequent indication to LVAD implant was dilated cardiomyopathy (72.7%). All candidates underwent a thorough preoperative advanced imaging. Three-dimensional reconstructions and implant fit simulation were performed when BSA was <1.2 m2, weight <30 kg, or internal transverse thoracic diameter <20 cm. There was no operative death. The most common postoperative complication was surgical re-exploration due to bleeding (27.3%). One patient died of severe neurological complications after about 3 months of hospitalization. No late deaths or unplanned re-hospitalizations occurred in the remaining 10, 6 of whom were discharged home. There were no major complications at the follow-up. All survivors underwent successful heart transplantation. CONCLUSIONS: Intracorporeal LVAD implantation proved to be a potentially feasible and safe option in young teenagers and children whose BSA was >1.0 m2. In borderline cases, the 3D reconstruction with implant fit simulation can effectively help to identify those patients who can safely undergo intrathoracic LVAD implantation.

Percutaneous approach to residual pulmonary bifurcation stenosis in conotruncal diseases.

Residual stenosis after right ventricle outflow tract surgery represents a major issue to manage in the children and adult patient with conotruncal defects. Despite a detailed multimodality imaging, the anatomy of distal pulmonary trunk and pulmonary artery bifurcation may be challenging in these patients.The aim of this study was to analyse retrospectively the outcome of the percutaneous transcatheter treatment in children with post-surgical stenosis of pulmonary artery bifurcation.We enrolled 39 patients with a median age of 6.0 years. Standard high-pressure balloon dilation was attempted in 33 patients, effective in 5 of them. Pulmonary branch stenting was performed in 10 patients, effective in 6. A kissing balloon approach was chosen in 17 patients (6 after angioplasty or stenting failure), and this technique was effective in 16 cases. Finally, a bifurcation stenting was performed in 10 patients (second step in 9 cases), effective in all the cases. None of the patients approached by kissing balloon needed a bifurcation stenting.In conclusion, standard balloon angioplasty and standard stenting might be ineffective in post-surgical stenosis involving pulmonary artery bifurcation. In this population, kissing balloon or bifurcation stenting, followed by side branch de-jailing, may be more effective in relieving the gradient.

Hypertension in children and adolescents.

Definition and management of arterial hypertension in children and adolescents are uncertain, due to different positions of current guidelines. The European Society of Cardiology task-force, constituted by Associations and Councils with interest in arterial hypertension, has reviewed current literature and evidence, to produce a Consensus Document focused on aspects of hypertension in the age range of 6-16 years, including definition, methods of measurement of blood pressure, clinical evaluation, assessment of hypertension-mediated target organ damage, evaluation of possible vascular, renal and hormonal causes, assessment and management of concomitant risk factors with specific attention for obesity, and anti-hypertensive strategies, especially focused on life-style modifications. The Consensus Panel also suggests aspects that should be studied with high priority, including generation of multi-ethnic sex, age and height specific European normative tables, implementation of randomized clinical trials on different diagnostic and therapeutic aspects, and long-term cohort studies to link with adult cardiovascular risk. Finally, suggestions for the successful implementation of the contents of the present Consensus document are also given.

Hybrid approach for end-stage heart failure treatment in a 6-month-old baby.

A 6-month-old baby girl, with a history of 2 months of intensive care management and two episodes of cardiac arrest, was transferred from another European country to initiate the "Giessen approach" for end-stage heart failure in children. At the admission, left ventricular ejection fraction was 20%. Severe mitral valve regurgitation and severe left atrial dilatation were present. Right ventricular function was preserved, and tricuspid valve regurgitation was mild. As a result, the patient underwent surgical pulmonary artery banding. Additionally, unloading of the left atrium was achieved by implanting an 8 mm atrial flow regulator device through a hybrid per-atrial approach. Two months after the procedure, the patient was progressively weaned from the inotropes and transferred to the ward.

Segmental and global longitudinal strain differences between Kawasaki disease and multi-system inflammatory syndrome in children.

BACKGROUND: Multi-system inflammatory syndrome in children and Kawasaki disease have overlapping clinical features but comparative echocardiographic studies are lacking. METHODS: We reviewed echocardiography findings of all multi-system inflammatory syndrome cases between 1st April and 31st July, 2020 and typical Kawasaki disease patients with coronary arteries abnormalities consecutively followed between 1st October, 2016 and June 30th, 2019. RESULTS: We included 40 multi-system inflammatory syndrome children (25 males, 62.5%) and 45 Kawasaki disease patients (31 males, 68.9%) at a mean age of 6.4 years old and 8 years old, respectively. Four out of 40 multi-system inflammatory syndrome children had coronary arteries abnormalities. Left ventricle ejection fraction was normal in both groups. Global longitudinal strain was normal although Kawasaki disease group had significantly lower values (-20.0 versus -21.7%; p = 0.02). Basal segments were the most affected in Kawasaki disease patients with significant differences in the basal anterior, anterolateral, and anteroseptal strain: -18.2 versus -23.0% (p = 0.002), -16.7 versus -22.0% (p < 0.001), -16.7 versus -19.5% (p = 0.034), respectively. The basal anterolateral and anteroseptal segments in Kawasaki disease patients were the only ones with an absolute reduction of longitudinal strain (-16.7% both) consistent with the greater left main coronary involvement in this cohort. CONCLUSIONS: Our findings are consistent with the transient cardiac involvement in multi-system inflammatory syndrome, as opposed to the subtle and chronic myocardial involvement in Kawasaki disease children with coronary arteries abnormalities. We speculate that the mechanism of cardiac impairment in the few multi-system inflammatory syndrome children with reduced global longitudinal strain is not related to coronary arteries abnormalities.

Unusual case of severe aortic regurgitation in a child with bicuspid aortic valve.

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

Unveiling the gothic aortic arch and cardiac mechanics: insights from young patients after arterial switch operation for d-transposition of the great arteries.

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

Maternal cardiovascular adaptation to pregnancy in women with previous bariatric surgery.

BACKGROUND: 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. OBJECTIVE: 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. STUDY DESIGN: 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. RESULTS: 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. CONCLUSION: 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.

Effectiveness of Repair of Aortic Coarctation in Neonates: A Long-Term experience.

To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in neonates. This is a retrospective clinical review of neonates with CoAo, who underwent surgery between 1995 and 2019. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics. Statistical analysis was performed by means of relative risk ratio and Cox and logistic multivariate analysis. 218 consecutive neonates (M/F: 129/89, median age 11 days, IQR 7-17 days) were included; 202 (92.7%) had a left thoracotomy; 178 underwent extended end-to-end anastomosis (EEEA, 81.6%). Hypoplastic aortic arch (HAA) was present in 102 patients (46.8%); complex cardiac anomalies in 85 (39%). Significant postoperative complications occurred in 20 (9.2%). Thirty-day mortality was 2.3% (most in complex types). At a median follow-up of 10.4 years (IQR 5.6-15.0 years; FU completeness 95.9%), there were 8 late deaths (3.7%), all associated to complex CoAo. Among 196 survivors, 177 (93.2%) were in NYHA class I; re-interventions on aortic arch occurred in 9.2% (2.0% were surgical). Freedom from mortality and re-intervention on aorta at 10 years were 94.3% and 96.7%, respectively. Surgical repair of CoAo in newborns without CPB in our series was safe and low-risk, with excellent early and late outcomes.

Myocardial involvement in children with post-COVID multisystem inflammatory syndrome: a cardiovascular magnetic resonance based multicenter international study-the CARDOVID registry.

BACKGROUND: Recent evidence shows an association between coronavirus disease 2019 (COVID-19) infection and a severe inflammatory syndrome in children. Cardiovascular magnetic resonance (CMR) data about myocardial injury in children are limited to small cohorts. The aim of this multicenter, international registry is to describe clinical and cardiac characteristics of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 using CMR so as to better understand the real extent of myocardial damage in this vulnerable cohort. METHODS AND RESULTS: Hundred-eleven patients meeting the World Health Organization criteria for MIS-C associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), having clinical cardiac involvement and having received CMR imaging scan were included from 17 centers. Median age at disease onset was 10.0 years (IQR 7.0-13.8). The majority of children had COVID-19 serology positive (98%) with 27% of children still having both, positive serology and polymerase chain reaction (PCR). CMR was performed at a median of 28 days (19-47) after onset of symptoms. Twenty out of 111 (18%) patients had CMR criteria for acute myocarditis (as defined by the Lake Louise Criteria) with 18/20 showing subepicardial late gadolinium enhancement (LGE). CMR myocarditis was significantly associated with New York Heart Association class IV (p = 0.005, OR 6.56 (95%-CI 1.87-23.00)) and the need for mechanical support (p = 0.039, OR 4.98 (95%-CI 1.18-21.02)). At discharge, 11/111 (10%) patients still had left ventricular systolic dysfunction. CONCLUSION: No CMR evidence of myocardial damage was found in most of our MIS-C cohort. Nevertheless, acute myocarditis is a possible manifestation of MIS-C associated with SARS-CoV-2 with CMR evidence of myocardial necrosis in 18% of our cohort. CMR may be an important diagnostic tool to identify a subset of patients at risk for cardiac sequelae and more prone to myocardial damage. CLINICAL TRIAL REGISTRATION: The study has been registered on ClinicalTrials.gov, Identifier NCT04455347, registered on 01/07/2020, retrospectively registered.

High take off left main coronary artery accompanied by multicryptic left ventricle myocardium detected by cardiac computerized tomography in a young male: case report.

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.

Surgical strategies for the management of end-stage heart failure in infants and children: A 15-year experience with a patient-tailored approach.

End-stage heart failure (ESHF) in pediatric age is an ongoing challenge. Heart transplantation is the final option, but its long-term outcomes are still suboptimal in children. An alternative patient-tailored surgical protocol to manage ESHF in children is described. Retrospective, single-center analysis of pediatric patients admitted to our institution between April 2004 and February 2021 for ESHF. Our current protocol is as follows: (a) Patients <1 year with isolated left ventricular dysfunction due to dilated cardiomyopathy underwent pulmonary artery banding (PAB). (b) Patients <10 years and <20 kg, who did not meet previous criteria were managed with Berlin Heart EXCOR. (c) Patients >10 years or >20 kg, underwent placement of intracorporeal Heartware. Primary outcomes were survival, transplant incidence, and postoperative adverse events. A total of 24 patients (mean age 5.3 ± 5.9 years) underwent 26 procedures: PAB in 6 patients, Berlin Heart in 11, and Heartware in 7. Two patients shifted from PAB to Berlin Heart. Overall survival at 1-year follow-up and 5-year follow-up was 78.7% (95%CI = 62%-95.4%) and 74.1% (95%CI = 56.1%-92.1%), respectively. Berlin Heart was adopted in higher-risk settings showing inferior outcomes, whereas a PAB enabled 67% of patients to avoid transplantation, with no mortality. An integrated, patient-tailored surgical strategy, comprehensive of PAB and different types of ventricular assist devices, can provide satisfactory medium-term results for bridging to transplant or recovery. The early postoperative period is critical and requires strict clinical vigilance. Selected infants can benefit from PAB that has demonstrated to be a safe bridge to recovery.

Bicuspid aortic valve and sports: From the echocardiographic evaluation to the eligibility for sports competition.

Bicuspid aortic valve (BAV) is the most common congenital heart defect in adults. Although a BAV may remain without clinical consequences for a lifetime, it can deteriorate in aortic valve stenosis and regurgitation and aortic dilatation. Unfortunately, the impact of regular training on patients with BAV and its natural course is not fully understood, although preliminary evidence suggests that the progression of valvular disease occurs primarily in an independent manner from sports practice. The current review aims to report how to perform a comprehensive echocardiographic examination in athletes with BAV and analyze the current literature on the influence of sports practice and how it impacts the aortic valve in athletes with BAV. The article also summarizes the current recommendations on sports eligibility and disqualification for competitive athletes with BAV.

Vector flow mapping: A review from theory to practice.

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.

A case of atrial septal defect associated with anomalous sinoatrial node artery in pulmonary atresia with intact ventricular septum.

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). Intra-procedural 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.

Measurement of Left Ventricular Dimensions and Ejection Fraction in Neonates by Three-Dimensional Echocardiography: A Comparative Study Between Philips QLAB and TOMTEC Software-Are the Values Interchangeable?

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.

Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database.

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.