Efficacy of a Transition Program in Adolescents and Young Adults With Congenital Heart Disease: The TRANSITION-CHD Randomized Controlled Trial.

PURPOSE: Currently, nearly 90% of patients with congenital heart disease (CHD) reach adulthood in relatively good health. Structured transition programs have emerged to support adolescents and young adults in transitioning to adult care structures, improve their autonomy, and limit healthcare ruptures. The TRANSITION-CHD randomized controlled trial aimed to assess the impact of a transition program on health-related quality of life (HRQoL) in adolescents and young adults with CHD. METHODS: From January 2017 to February 2020, 200 subjects with a CHD, aged 13-25 years, were enrolled in a prospective, controlled, multicenter study and randomized in two balanced groups (transition program vs. standard of care). The primary outcome was the change in PedsQL self-reported HRQoL score between baseline and 12-month follow-up, using an intention-to-treat analysis. The secondary outcomes were the change in disease knowledge, physical health (cardiopulmonary fitness, physical activity), and mental health (anxiety, depression). RESULTS: The change in HRQoL differed significantly between the transition group and the control group (mean difference = 3.03, 95% confidence interval (CI) = [0.08; 5.98]; p = .044; effect size = 0.30), in favor of the intervention group. A significant increase was also observed in the self-reported psychosocial HRQoL (mean difference = 3.33, 95% CI = [0.01; 6.64]; p = .049; effect size = 0.29), in the proxy-reported physical HRQoL (mean difference = 9.18, 95% CI = [1.86; 16.51]; p = .015; effect size = 0.53), and in disease knowledge (mean difference = 3.13, 95% CI = [1.54; 4.72]; p < .001; effect size = 0.64). DISCUSSION: The TRANSITION-CHD program improved HRQoL and disease knowledge in adolescents and young adults with CHD, supporting the generalization and systematization of similar preventive interventions in pediatric and congenital cardiology.

Correlation between three-dimensional echocardiography and cardiopulmonary fitness in patients with univentricular heart: A cross-sectional multicentre prospective study.

BACKGROUND: The prognosis of patients with a functional single ventricle has improved, with better cardiopulmonary fitness, health-related quality of life and survival. Conventional echocardiography remains the first-line technique in single ventricle follow-up. Three-dimensional (3D) echocardiography has shown recent value in congenital cardiology, but its ability to predict functional status in patients with a single ventricle remains unknown. AIM: To evaluate, in patients with a single ventricle, the association between 3D echocardiography variables and functional status determined by cardiopulmonary fitness. METHODS: Children and adults with a functional single ventricle were prospectively enrolled in this multicentre study. Cardiopulmonary fitness was assessed by cardiopulmonary exercise test, with measures of maximum oxygen uptake (VO2max) and ventilatory efficiency (VE/VCO2 slope). 3D echocardiography was performed with off-line reproducibility analyses, using TomTec Arena™ software. Health-related quality of life was assessed using the SF-36 questionnaire. RESULTS: A total of 33 patients were screened, and 3D echocardiography analyses were feasible in 22 subjects (mean age 28±9years). 3D echocardiography ejection fraction correlated with percent-predicted VO2max (r=0.64, P<0.01), VE/VCO2 slope (r=-0.41, P=0.05), two-dimensional echocardiography ejection fraction (r=0.55, P<0.01) and health-related quality of life physical functioning dimension (r=0.56, P=0.04). 3D echocardiography indexed end-systolic volume correlated with percent-predicted VO2max (r=-0.45, P=0.03) and VE/VCO2 slope (r=0.65, P<0.01). 3D echocardiography reproducibility was good. CONCLUSIONS: Single ventricle ejection fraction and volumes measured by 3D echocardiography correlated with cardiopulmonary fitness, as determined by two main prognostic cardiopulmonary exercise test variables: VO2max and VE/VCO2 slope. Despite good reproducibility, 3D echocardiography feasibility remained limited. 3D echocardiography may be of value in single ventricle follow-up, provided that the technique and analysis software are improved.

Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure.

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14-64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018­2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review.

BACKGROUND: Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient. CASE PRESENTATION: We report the case of a 3-year-old patient, initially managed for metastatic WT. During his clinical course, the diagnosis of a PPB was made after detecting the DICER1 mutation and subsequent management was therefore modified. CONCLUSION: This case highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, the DICER 1 mutations should be looked for as these could help adapt management and schedule the surgical procedures.

Cardiac strangulation: An atypical complication from epicardial pacemaker leads in a newborn.

We report a rare and serious complication of cardiac strangulation arising from the implantation of epicardial pacing leads in a newborn. Patient's follow-up 9-month postsurgery revealed compression under the pulmonary valve annulus by a pacemaker lead, causing progressive stenosis of the right ventricular outflow tract. The epicardial leads were replaced to relieve compression, and stenosis of the right ventricular outflow tract was rectified. Pacemaker implantation in newborns is not without challenges; epicardial leads should be carefully positioned to avoid any compression of cardiac structures.

Tetralogy of Fallot with critical biventricular dysfunction: is surgical correction achievable?

We describe the case of a 32-month-old patient from a developing country with tetralogy of Fallot associated with a severe biventricular dysfunction. This association is rare but makes the surgical strategy complex and potentially contraindicated. An acute severe hypoxic episode led us to perform palliative rescue intervention involving the placement of an undersized systemic-to-pulmonary shunt. This surgery was well tolerated and allowed a fast and impressive recovery of the ventricular function, making complete repair possible.

Thymic-Specific Serine Protease Limits Central Tolerance and Exacerbates Experimental Autoimmune Encephalomyelitis.

The genetic predisposition to multiple sclerosis (MS) is most strongly conveyed by MHC class II haplotypes, possibly by shaping the autoimmune CD4 T cell repertoire. Whether Ag-processing enzymes contribute to MS susceptibility by editing the peptide repertoire presented by these MHC haplotypes is unclear. Thymus-specific serine protease (TSSP) is expressed by thymic epithelial cells and thymic dendritic cells (DCs) and, in these two stromal compartments, TSSP edits the peptide repertoire presented by class II molecules. We show in this article that TSSP increases experimental autoimmune encephalomyelitis severity by limiting central tolerance to myelin oligodendrocyte glycoprotein. The effect on experimental autoimmune encephalomyelitis severity was MHC class II allele dependent, because the lack of TSSP expression conferred protection in NOD mice but not in C57BL/6 mice. Importantly, although human thymic DCs express TSSP, individuals segregate into two groups having a high or 10-fold lower level of expression. Therefore, the level of TSSP expression by thymic DCs may modify the risk factors for MS conferred by some MHC class II haplotypes.

Image of a left atrial mass after a cardiopulmonary bypass in a child.

Invagination of an appendage into the left atrium is a rare complication. It occurs spontaneously or after open-heart surgery. In our case, a postoperative transesophageal echocardiogram, after closure of a ventricular septal defect in a 5-month-old infant, revealed a large mass in the left atrium. A diagnosis of a left appendage inversion was confirmed after external examination of the heart. Herein, we provide echocardiographic images before, during, and after manual reversion of the left appendage. Misdiagnosis of this complication could have led to an additional unnecessary surgical procedure that could have impacted on the patient's morbidity.

Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair.

OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage. METHODS: Three patients presenting with situs solitus and extreme leftward displacement of the septum primum were included. All of the pulmonary veins drained anomalously into the anatomical right atrium, and the distance between the mitral valve and the abnormal septum primum was greatly reduced, compromising the size of the left atrial chamber, but with normal left ventricle diameters. Preoperative 3D echocardiographic findings are reported. We achieved a biventricular surgical repair in all cases. The atrial septation was accomplished using an autologous pericardial patch after removing the abnormal septal membrane. RESULTS: The postoperative course was free from any cardiovascular complications. Echocardiographic scans showed a harmonious reconstruction without pulmonary venous obstructions or stenosis. CONCLUSIONS: This article reports the severe leftward displacement of the septum primum presented as an isolated cardiac malformation; 3D transthoracic echocardiography allowed an accurate diagnosis of this malformation and helped in choosing the best surgical strategy.

Two-dimensional right ventricular strain by speckle tracking for assessment of longitudinal right ventricular function after paediatric congenital heart disease surgery.

BACKGROUND: Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD). AIMS: To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers. METHODS: In this prospective single-centre study, RV peak systolic strain (RV-PSS) was measured using 2D speckle tracking in 37 consecutive children undergoing CHD surgery. Examinations were performed the day before surgery, a few hours after surgery and before discharge. Relationships with the z score of tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (TA Sa) were assessed. RESULTS: Median (interquartile range) age was 19 months (5-63); median weight was 9.2 kg (5.3-18.0). RV-PSS analysis was feasible in 92.9% (95% confidence interval [CI]: 86.0-97.1) of examinations. The coefficient of variation was 9.7% (95% CI: 7.4-11.9) for intraobserver variability and 15.1% (95% CI: 12.7-17.6) for interobserver variability. Correlations between RV-PSS and z score of TAPSE and TA Sa were strong (r=0.71, P<0.0001 and r=0.70, P<0.0001, respectively). RV-PSS was significantly reduced after surgery compared with baseline (-10.5±2.9% vs. -19.5±4.8%; P<0.0001) and at discharge (-13.5±4.0% vs. -19.5±4.8%; P<0.0001). Similar evolutions were observed with TAPSE and TA Sa (both P<0.0001). CONCLUSION: RV longitudinal strain by speckle tracking is a feasible and reproducible method of assessing perioperative evolution of RV function in children with CHD.

Assessment of Ventricular Septal Defect Size and Morphology by Three-Dimensional Transthoracic Echocardiography.

BACKGROUND: Morphologic description of ventricular septal defect (VSD) is mandatory before performing the newly developed transcatheter closure procedure. Inaccurate estimation of defect size has been reported using conventional two-dimensional (2D) transthoracic echocardiography (TTE). The aim of this study was to assess VSD morphology and size using three-dimensional (3D) TTE compared with 2D TTE and surgery. METHODS: Forty-eight children aged 21.4 ± 29.3 months with isolated muscular (n = 11 [22.9%]) and membranous (n = 37 [77.1%]) VSDs were prospectively included. Three-dimensional images were acquired using full-volume single-beat mode. Minimal diameter, maximal diameter, and systolic and diastolic VSD areas were measured from 3D data sets using multiplanar reconstruction mode (QLAB 9). Maximal-to-minimal VSD diameter ratio was used to assess VSD geometry. Linear regression analysis and the Bland-Altman method were used to compare 3D measurements with 2D and surgical measurements in a subgroup of 15 patients who underwent surgical VSD closure. RESULTS: VSD 3D diameters and areas were measured in all patients (100%; 95% CI, 92.6%-100%). Maximal diameter was lower on 2D TTE compared with 3D TTE (7.3 vs 11.3 mm, P < .0001). Mean bias was 4 mm, with 95% of values ranging from -1.76 to 9.75 mm. Correlation between 3D maximal diameter and surgical diameter was strong (r(2) = 0.97, P < .0001), while correlation between maximal 2D diameter and surgical diameter was moderate (r(2) = 0.63, P < .0001). VSDs had an oval shape when assessed by 3D TTE. Maximal-to-minimal diameter ratio assessed by 3D TTE was significantly higher in muscular VSDs compared with membranous VSDs (3.20 ± 1.51 vs 2.13 ± 1.28, respectively, P = .01). VSD area variation throughout the cardiac cycle was 32% and was higher in muscular compared with membranous VSDs (49% vs 26%, P = .0001). CONCLUSIONS: Three-dimensional TTE allows better VSD morphologic and maximal diameter assessment compared with 2D TTE. VSD shape and its changes during the cardiac cycle can be visually and quantitatively displayed. Three-dimensional echocardiography may thus be particularly useful before and during percutaneous VSD closure.

Mouse and human CD8(+) CD28(low) regulatory T lymphocytes differentiate in the thymus.

Regulatory T (Treg) lymphocytes play a central role in the control of immune responses and so maintain immune tolerance and homeostasis. In mice, expression of the CD8 co-receptor and low levels of the co-stimulatory molecule CD28 characterizes a Treg cell population that exerts potent suppressive function in vitro and efficiently controls experimental immunopathology in vivo. It has remained unclear if CD8(+) CD28(low) Treg cells develop in the thymus or represent a population of chronically activated conventional T cells differentiating into Treg cells in the periphery, as suggested by their CD28(low) phenotype. We demonstrate that functional CD8(+) CD28(low) Treg cells are present in the thymus and that these cells develop locally and are not recirculating from the periphery. Differentiation of CD8(+) CD28(low) Treg cells requires MHC class I expression on radioresistant but not on haematopoietic thymic stromal cells. In contrast to other Treg cells, CD8(+) CD28(low) Treg cells develop simultaneously with CD8(+) CD28(high) conventional T cells. We also identified a novel homologous naive CD8(+) CD28(low) T-cell population with immunosuppressive properties in human blood and thymus. Combined, our data demonstrate that CD8(+) CD28(low) cells can develop in the thymus of mice and suggest that the same is true in humans.

3D transthoracic echocardiography to assess pulmonary valve morphology and annulus size in patients with Tetralogy of Fallot.

BACKGROUND: Accurate evaluation of the pulmonary valve (PV) is crucial before surgical repair of Tetralogy of Fallot (TOF). AIMS: To assess PV and pulmonary annulus (PA) morphology using three-dimensional (3D) transthoracic echocardiography (TTE) in infants referred for surgical repair of TOF. Also, to compare PA measurements obtained by 3D TTE with those from other imaging modalities, including two-dimensional (2D) TTE and computed tomography (CT), with reference to surgical measurements. METHODS: 3D zoom mode was used to assess PV morphology. 2D TTE and CT PA diameters were compared to both vertical and horizontal diameters obtained from 3D datasets. Surgical PA diameters were measured using Hegar's dilators. RESULTS: A total of 29 patients with TOF (median [range] age 6 [3-24] months) were included and all successfully underwent 2D and 3D TTE; 22 also underwent CT. The number of pulmonary leaflets could be visualized in 24 patients (82.8%), with complete concordance with surgical findings. Vertical diameter was significantly longer than horizontal diameter (P<0.001)-underlying PA eccentricity-and was more important in bicuspid than tricuspid valves. Correlations between 2D and 3D TTE diameters were good. Surgical diameter was better correlated with 2D and 3D diameters than with CT diameter. 3D minimum, 2D and CT diameters were significantly lower than surgical diameters, but 3D mean and maximum diameters were not. CONCLUSION: 3D TTE is accurate to assess PV morphology and PA size in patients with TOF. 2D TTE and CT underestimate PA diameter with reference to surgical diameter, however 3D mean and maximum diameters did not differ significantly.

Aortic arch aneurysm: short- and mid-term results comparing open arch surgery and the hybrid procedure†.

OBJECTIVES: Open arch surgery for aortic arch aneurysm was historically associated with a high risk of postoperative morbi-mortality. Improved operative techniques have now lowered the incidence of these complications but in parallel, hybrid arch procedures have emerged. Nowadays, very little data are available about their mid-term results compared with open surgery. METHODS: From January 2002 to January 2014, 46 patients had treatment for an exclusive aortic arch aneurysm including 25 open arch surgeries and 21 type I hybrid arch procedures in our institution. All cases involved arch aneurysms involving at least one carotid artery (Zone 0 and Zone 1). Aneurysms of the distal arch and descending aorta were excluded (Zone 2 and beyond). Results from a retrospective database are reported. There were no patients lost to the follow-up. RESULTS: There was no significant difference in preoperative comorbidities between the two groups. The incidence of in-hospital mortality was similar at 20% (5/25) for open surgery and 19% (4/21) for hybrid procedure (P = 0.830). The incidence of permanent cerebral neurological deficit was comparable at 17.4% (4/23) for open surgery and 21.1% (4/19) for hybrid procedure (P = 1). Median survival was 109.5 months for open surgery and 56.3 months for hybrid procedure. Freedom from all-cause mortality was 78, 63, 63 and 57% at 1, 3, 5 and 7 years, respectively in the open surgical group. Freedom from all-cause mortality was 74, 55, 46 and 28% at 1, 3, 5 and 7 years, respectively in the hybrid group. Survival rates and incidence of major adverse cardiac and cerebro-vascular event between open surgery and hybrid procedure were not statistically different (P = 0.530 and P = 0.325, respectively). However, incidence of reintervention was in favour of open surgery [14.5 vs 44.8% at 7 years, P = 0.045; 95% confidence interval: (0.06-0.97)]. CONCLUSIONS: The type I hybrid arch procedure fails to demonstrate better results compared with open surgery, regarding morbi-mortality at the short- and mid-term follow-up. Moreover, it increases the risk of reintervention. Patients treated by this technique must undergo a closer follow-up because of this risk. Larger randomized studies are needed to better define the exact indications of this therapy.

The medical history of adults with complex congenital heart disease affects their social development and professional activity.

BACKGROUND: In recent decades, advances in surgery and therapeutic catheterization have steadily increased the life expectancy and prevalence of adults with congenital heart disease (CHD). AIMS: We assessed medical and psychosocial variables of adults with CHD, according to the disease complexity. METHODS: We included, from a single-centre observational cohort study, 135 consecutive adults with CHD (median age of 40 years, interquartile range: 28.0-51.0) followed in our cardiology unit, who answered a questionnaire assessing daily activity and psychosocial functioning. Disease complexity was classified according to the Bethesda conference. RESULTS: Cardiac malformation complexity was simple in 61 (45.2%), moderate in 50 (37.0%) and complex in 24 (17.8%) patients. Cardiac surgery had been performed in 86.5% of moderate and complex patients. Complications (such as heart failure, arrhythmia and pulmonary hypertension) were mainly observed in the complex group (P=0.003). Physical activity was lower in the complex group (no activity in 58.8%, but sport previously contraindicated in 50% of these; P=0.03). Education level tended to be lower in the complex and moderate groups than in the simple group (respectively, 31.2% and 33.3% vs. 45.7% had passed the Baccalaureate; P=0.47). The pass rate was lower in patients with complications (P=0.037) or more than one cardiac surgery (P=0.03). In the complex group, 56.3% of patients were unemployed (P=0.048). CONCLUSIONS: Complexity of heart disease and medical history affect education level and employment of adults with CHD. Academic education of children with a complex defect and career counselling are important to prevent unemployment among adults with CHD.

Peripheral regulatory T lymphocytes recirculating to the thymus suppress the development of their precursors.

Most T lymphocytes, including regulatory T cells (Treg cells), differentiate in the thymus. The age-dependent involution of this organ leads to decreasing production of T cells. Here we found that the output of new Treg cells from the thymus decreased substantially more than that of conventional T cells. Peripheral mouse and human Treg cells recirculated back to the thymus, where they constituted a large proportion of the pool of Treg cells and displayed an activated and differentiated phenotype. In the thymus, the recirculating cells exerted their regulatory function by inhibiting interleukin 2 (IL-2)-dependent de novo differentiation of Treg cells. Thus, Treg cell development is controlled by a negative feedback loop in which mature progeny cells return to the thymus and restrain development of precursors of Treg cells.

Safety and efficiency of the new micro-multiplane transoesophageal probe in paediatric cardiology.

BACKGROUND: Transoesophageal echocardiography (TOE) is feasible in neonates using a miniaturized probe, but is not widely used because of low imaging quality. AIMS: To assess handling and imaging quality of a new release of a micro-TOE probe in children. METHODS: Thirty-eight consecutive children, enrolled during February and May 2013, underwent TOE with the Philips S8-3t probe. Insertion, handling and image quality were assessed. RESULTS: The 38 children (aged 7days to 12years; weight 3.1-27kg) underwent 75 TOE (30 [40.0%] before cardiac surgery, 31 [41.3%] after cardiac surgery, 4 [5.3%] during a percutaneous procedure, 10 [13.3%] in the intensive care unit). Insertion of the micro-TOE probe was 'very easy' in 37/38 patients (97.4%). Handling was better in the lightest children (P=0.001). Image quality was mainly 'good' or 'very good', with no significant changes between preoperative and postoperative examinations or over time. Total scores (insertion, handling, image quality) were significantly better in the lightest children (P=0.02). Preoperative TOE did not provide additional information over transthoracic echocardiography. Postoperative TOE was useful to assess surgical results, but no residual lesions required extracorporeal circulation return. Micro-TOE was useful during the postoperative care of neonatal surgery with open breastbone to assess the surgical result and ventricular function. It was also useful to guide extracorporeal membrane oxygenation (ECMO) indication and withdrawal; and was a useful guide for percutaneous procedures. CONCLUSION: Micro-multiplane TOE is safe and efficient for use in neonates and children. This minimally invasive tool increases the impact of TOE in paediatric cardiology.