Impact of cardiac surgical timing on the neurodevelopmental outcomes of newborns with Complex congenital heart disease (CHD).

Background: More than half of infants with complex congenital heart disease (CHD) will have a neurodevelopmental disorder of multifactorial causes. The preoperative period represents a time-window during which neonates with complex CHD are in a state of hypoxia and hemodynamic instability, which fosters the emergence of brain injuries and, thus, affects early brain networks and neurodevelopmental outcomes. Currently, there is no consensus regarding the optimal age for cardiac surgery in terms of neurodevelopmental outcomes, and its definition is a real challenge. Our aim is to determine the relationship between cardiac surgical timing and long-term neurodevelopmental outcomes for various types of complex CHD. Methods: We hypothesize that earlier surgical timing could represent a neuroprotective strategy that reduces perioperative white matter injuries (WMIs) and postoperative morbidity, leading to improved neurodevelopmental outcomes in infants with complex CHD. Firstly, our prospective study will allow us to determine the correlation between age at the time of surgery (days of life) and neurodevelopmental outcomes at 24 months. We will then analyze the correlation between age at surgery and (i) the incidence of WMIs (through pre- and postoperative MRIs), (ii) postoperative morbidity, and (iii) the duration of the hospital stay. Implications and Dissemination: This research protocol was registered in the Clinical Trial Registry (National Clinical Trial: NCT04733378). This project aims to help launch the first Neurocardiac Investigation Clinic in Marseille - AP-HM - to propose an overall personalized monitoring and treatment program for patients operated on for complex CHD.

Ventricular septal defect with anomalous origin of the left descending coronary artery from the right sinus of Valsava and an intraseptal course.

Patients with anomalous aortic origin of the left anterior descending coronary artery (AAOCA) from the right sinus of Valsava, and associated with a trans-septal course, are recommended for surgery only when symptoms of ischemia are present. The transconal unroofing method is straightforward and provides good anatomic result. In absence of significant coronary compression, surgical management of the trans-septal coronary course is proposed if the patient is a candidate to cardiac surgery for another reason, such as congenital heart disease. We describe a transconal approach in a patient with a trans-septal coronary artery and a ventricular septal defect.

Antibiotic Resistance Acquisition in the First Week of Life.

Objectives: The fetus is considered sterile but recent studies have suggested that gut colonization could start before birth. Scarce data are available for the acquisition of resistant Gram-negative bacteria (GNB) during the first days of life. Several studies have shown that integrons play a major role in antibiotic resistance acquisition. In this work, we studied the dynamics of human intestinal acquisition of GNB and integrons during the first days of life. Methods: Meconium was collected at birth and a stool sample before hospital discharge (days 2 or 3) on 185 term neonates. GNB were searched by culture on each sample and class 1, 2, and 3 integrons from each GNB or directly from samples. Eight risk factors for integron and GNB acquisition were studied. Results: We isolated 228 GNB, 46 from meconium and the remainder from stools. No link was found between GNB isolation and antibiotic exposure during delivery, but antibiotic exposure during labor significantly selected blaTEM-positive amoxicillin-resistant Enterobacteria. Two-thirds of GNB were antibiotic-susceptible and most of the resistant isolates were acquired after birth. Integrons were detected in 18 of the 228 GNB isolates from 3 meconium and 20 stools. Antibiotic administration during delivery and vaginal carriage of Streptococcus agalactiae appeared as risk factors for integron acquisition. Conclusion: Gram-negative bacteria and integrons are mostly acquired after birth during the first days of life even if for some term neonates, meconium was not sterile. Antibiotic administration during delivery is a major risk for integron acquisition and for selection of amoxicillin-resistant Enterobacteria.

Repeat mitral valve repair for haemolysis in children.

BACKGROUND: Severe haemolysis is a rare complication after mitral valve repair in congenital heart disease. AIM: We describe four children with severe mitral regurgitation who underwent valve repair and subsequently developed profound haemolytic anaemia. METHODS: Clinical, echocardiographic and surgical data were collected retrospectively from a surgical centre in France during a 5-year period. RESULTS: Two patients had atrioventricular septal defects, one patient had congenital mitral dysplasia and one had anomalous left coronary artery from the pulmonary artery with mitral regurgitation. Haemolysis was diagnosed 20 to 75 days after surgery, as a result of clinical and biological examination; it was severe, and blood transfusion support was necessary in all cases. Haemolysis was always associated with eccentric mitral regurgitation with a variable degree of severity (from low to severe). After exclusion of other haemolysis aetiology, redo mitral repair surgery was performed successfully in all cases. The haemolysis was considered to be mechanical in origin, caused by regurgitation of blood through the residual mitral regurgitation and stitches. CONCLUSION: Severe haemolysis is a rare complication that can occur after mitral valve repair in congenital heart disease. All patients underwent successful redo repair and had resolution of haemolysis after surgery. Even if redo surgery is required, iterative mitral repair is possible.

Atrio-ventricular valve regurgitation in univentricular hearts: outcomes after repair†.

OBJECTIVES: The aim was to describe the early and mid-term outcome after atrio-ventricular valve (AVV) repair in patients with univentricular hearts (UVHs) and to identify risk factors for AVV reoperation and death. METHODS: This study is a retrospective review of patients undergoing valve repair for AVV regurgitation at any stage of univentricular palliation from 1998 to 2014. Patient- and procedure-related variables were analysed. RESULTS: A total of 31 consecutive patients underwent 38 procedures for ≥ moderate AVV regurgitation at a median age of 3.6 years. Thirty-two percent of patients had a common AVV, 26% had two AVVs, 22% had a dominant tricuspid valve and 19% had a dominant mitral valve. All patients underwent valve repair as a first procedure without early mortality. At discharge, patients preserved their ventricular function (fractional shortening <30%: preoperative 16% vs postoperative 22.5%, NS). In 19% (n = 6) of patients, the procedure was considered as failed because of significant residual regurgitation. There were three late deaths [median delay: 1 year (range 0.7-13.6)] and three heart transplantations. Six patients underwent seven AVV reoperations [median delay: 2 years (range 0.2-7.6)]. Longer intensive care stay (P = 0.022), longer total postoperative hospital stay (P = 0.039), higher total number of surgeries (P = 0.039), lower body mass index (P = 0.042) and higher preoperative mean pulmonary pressure (P = 0.047) were univariate risk factors for death/transplantation. Failed first AVV repair (P = 0.01), higher total number of surgeries (P = 0.026), lower body mass index (P = 0.031), male gender (P = 0.031) and need for valve repair before bidirectional cavopulmonary connection (P = 0.036) were univariate risk factors for AVV reoperation. In multivariate analysis, no univariate risk factor reached statistical significance. Freedom from death/transplantation was 84% (CI 95%: 70%-98%) at 5 and 10 years. Survival free from AVV reoperation was 72% (CI 95%: 52%-92%) at 5 years and 62% at 10 years (CI 95%: 36%-88%). Mean follow-up of survivors was 4.7 years (SD ± 4.3; range 0.2-15.6). At last visit, 96% of survivors were in NYHA Class I-II. Ninety-two percent had a ≤ mild residual regurgitation. CONCLUSIONS: In patients with a UVH and ≥ moderate AVV regurgitation, AVV repair is feasible without postoperative deterioration of their ventricular function. Nevertheless, these patients remain at increased risk for death/transplantation and AVV reoperation.

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.

Real-time three-dimensional foetal echocardiography using a new transabdominal xMATRIX array transducer.

BACKGROUND: Foetal echocardiography has been used to diagnose congenital heart disease. However, conventional echocardiography can only display two-dimensional (2D) structural images of the intricate three-dimensional (3D) foetal heart. AIM: The purpose of this study was to report the first use of a new transabdominal xMATRIX array transducer and to describe its ability to perform all 3D modalities: intelligent spatiotemporal image correlation (iSTIC) acquisition, xPlane imaging and 3D surface imaging. METHODS: Eighty foetuses without congenital heart disease were included consecutively, with a gestational age between 20 and 37 weeks. 2D and 3D scans were performed with a transabdominal xMATRIX array transducer. Cardiac-STIC volume datasets were acquired and postprocessed with new automatic software (the 'Fetal Heart Navigator'). RESULTS: A total of 224 iSTIC acquisitions were performed (mean time for each, 2 seconds). Only 78 iSTIC acquisitions (35%) were able to detect the ductal arch automatically. 'Fetal Heart Navigator' feasibility varied according to foetal position, including the descending aorta. Live xPlane imaging had excellent feasibility regardless of foetal position; using rotation, lateral and vertical tilts, all cardiac structures were identified from a unique reference plane. Live 3D surface imaging had variable feasibility depending on the target structure. Only 10% of the volume dataset offered comprehensive imaging of intracardiac views. CONCLUSION: The new xMATRIX transabdominal transducer allows a multimodality approach to the foetal heart. Further studies that include foetuses with cardiac malformations are required.

Comparison of two- and three-dimensional transthoracic echocardiography for measurement of aortic annulus diameter in children.

BACKGROUND: Accurate evaluation of aortic root geometry is necessary in congenital aortic valve lesions in children, to guide surgical or angiographical intervention. AIM: To compare aortic annulus diameters measured by two- and three-dimensional transthoracic echocardiography (2D- and 3D-TTE), to determine the feasibility and reproducibility of 3D imaging and assess the dynamic changes during the cardiac cycle. METHODS: Thirty children without heart disease were prospectively included. Two orthogonal aortic annulus diameters were measured offline using multiplanar reconstruction in diastole and in systole and were compared with the measurement of the aortic annulus diameter by 2D-TTE. RESULTS: Mean age was 11±3.6 years. Feasibility of 3D imaging was 100%. The coefficients of intra- and interobserver variability were 3.5% and 6%, respectively. The 2D mean diameter was significantly smaller than the 3D maximum diameter in systole (1.94 vs. 2.01mm; p=0.005). 2D and 3D measurements were well correlated (p<0.0001). The maximum and minimum diameters in 3D were significantly different both in systole and in diastole (p<0.001) underlining an aortic annulus eccentricity. The mean aortic annulus diameters were not significantly different between systole and diastole, with important individual variability during the cardiac cycle. CONCLUSION: This study demonstrated the feasibility and reproducibility of 3D-TTE for the assessment of the aortic annulus diameter in a normal paediatric population. Because of an underestimation of the maximum diameter by 2D-TTE and the asymmetry of the aortic annulus, 3D measurements could be important before percutaneous aortic valvuloplasty or surgical replacement.