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Objective: To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients. Methods: This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the antegrade aortic flow across the aortic valve. At birth, a biventricular circulation (BVC) strategy was decided assuming the left ventricular (LV) systolic and diastolic function would ensure the systemic circulation. Results: Sixty‐three FAV procedures were performed in 58 fetuses, at a median (range) gestational age of 26.2 (20.3–32.2) weeks. The procedure was technically successful in 50/58 (86.2%) fetuses. There were 11/58 (19.0%) cases of in‐utero demise and 9/58 (15.5%) terminations of pregnancy. No patient was liveborn after an unsuccessful procedure. Thirty‐eight (65.5%) infants were liveborn, at a median (range) gestational age of 38.1 (29.0–40.6) weeks, of whom 21 (55.3%) required prostaglandin treatment. Twenty‐eight of the 38 (73.7%) liveborn children (48.3% of the study population) entered the BVC pathway at birth. Among them, 20 (71.4%) required an aortic valvuloplasty procedure at birth (11 (55.0%) percutaneous balloon, nine (45.0%) surgical) and eight (28.6%) did not require any treatment at birth, but, of these, five (62.5%) underwent surgical valvuloplasty between day 26 and day 1200 of age. Eleven (39.3%) of the infants with BVC at birth required a second intervention and four (14.3%) of them required a third intervention. Two (7.1%) infants who entered the BVC pathway at birth underwent conversion to univentricular circulation (UVC). None of the surviving children with BVC developed pulmonary hypertension. The overall survival rate in those with BVC at birth was 22/28 (78.6%) at a median (range) follow‐up of 23.3 (2.0–112.6) months. Ten of the 58 (17.2%) patients had UVC at birth. Among these, six (60.0%) received compassionate care from birth and four (40.0%) underwent surgery. Three of the 10 patients who had UVC at birth were still alive at the latest follow‐up assessment, at a median (range) gestational age of 24.3 (8.3–48.7) months. Conclusions: FAV for critical AS led to increase of antegrade aortic flow in 86.2% of fetuses, with BVC being achieved in 48.3% (73.7% of the liveborn cases). Among patients with BVC at birth, the rate of reintervention was high, but 78.6% of these children were alive at the latest evaluation. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Congenital heart defects in the context of heterotaxy are severe, with a complex anatomy. These defects are described using the binary concept of clinical loop (D-Loop or L-Loop), which implies that ventricle position is determined during heart looping. Recent work in the mouse has shown unexpected plasticity of ventricle position after heart looping. Based on a analysis of Nodal mutants with heterotaxy and right bronchus isomerism, 27% of D-Loop mutants at birth underwent leftward embryonic heart looping. These mice with a revertant loop, all display abnormal ventricle position. Analysis of 3D ventricle position in human patients with heterotaxy and right bronchus isomerism, using the strategy developed in the mouse based on the 3D orientation of the interventricular septum. Constitution of an heterotaxy cohort from the Necker database. Controls were selected as patients with transposition of the great arteries who had a systematic CT scan at 6 years. Three dimensions reconstructions of CT scans and quantitative analyses were performed using the Imaris software. Control patients had their right ventricle-left ventricle axis in the expected anteroposterior and left-right orientation. We collected 506 patients with heterotaxy syndrome, 186 of whom had CT scan. Forty patients had right bronchial isomerism. Among them, we found 29 (72%) patients who had an orientation of the interventricular septal axis similar to control, 7 (18%) patients with abnormal supero-inferior ventricles and 4 (10%) patients with abnormal left-right ventricles (Fig. 1). In total, 32% of patients in D-Loop have a malpositioned interventricular septum whereas 0 of patients in L-Loop. There is a high proportion of malpositioned ventricles in patients with D-Loop (32%), similar to what is observed in Nodal mouse mutants. This result supports conserved mechanisms in the mouse and human, including plasticity of ventricle position after heart looping. [ABSTRACT FROM AUTHOR]

Mediastinitis is a severe complication of heart surgery well described in the adult population. However very few studies focused on the description of mediastinitis and its outcomes in pediatric patients. To describe the clinical features and outcomes of postoperative mediastinitis in children and identify risk factors for mortality. We retrospectively reviewed all children with mediastinitis over a period of 10 years (2013–2023). Mediastinitis was defined by a purulent discharge of the mediastinum requiring surgery or organisms cultured from discharge of the mediastinal area. The incidence of mediastinitis was 0.74% (57 cases). Median age at surgery was 12 days with 67% patients younger than 3 months old. Sixty percent had delayed sternal closure. Median delay between surgery and diagnosis was 8 days (range: 2–39). The most frequent germs were Staphylococcus (46%), gram negative bacteria (35%) and fungi (9%). All patients had surgical debridement: 46 (81%) in the operating room and 11 (19%) in the ICU. Median duration of antibiotic therapy was 44 days. Median duration of stay was 21 days (range: 10–113) in the ICU and 35 days (range: 11–188) until hospital discharge. Outcomes in the ICU included a median of 8 days of assisted ventilation. Nine patients needed veno-arterial ECMO. Mortality rate was 27% with 12 out of 15 deaths occurring in the ICU. Mortality risk factors were surgical revision in the ICU compared to the operating room (OR 4.9; CI95% 1.3 to 19.9), delayed sternal closure superior to 3 days (OR: 5.0; 95% CI: 1.3 to 16.5) and fungal mediastinitis (OR: 14.9; 95% CI: 2.0 to 185.4). Mediastinitis leads to high mortality and morbidity in children. Mortality risk factors are: surgical revision in the ICU, prolonged delayed sternal closure and fungus infection. Patients with prolonged delayed sternal closure should be regularly screened for fungal colonization to prevent fungal mediastinitis. [ABSTRACT FROM AUTHOR]

Neurological disorders following heart surgery are mainly attributable to strokes. However there is a rare cause of abnormal movement following heart surgery: post-pump chorea. This complication is rare and poorly described particularly with regard to its therapeutic management and evolution. Descriptive monocentric retrospective study including all children with post-pump chorea over a 10-year period (2014–2023). Clinical presentation, radiological findings, and outcomes were analyzed based on clinical, biological, and cerebral imaging data. Over 10 years, 7059 pediatric cardiac surgeries with cardiopulmonary bypass (CBP) were performed at Necker hospital, and 11 patients experienced post-pump chorea (0.15%), including 5 boys (45%). Median age at the diagnosis of post-pump chorea was 5.7 years [0.45–9.9]. One patient was premature, and none had genetic syndromes. Median BMI was low (14.8 [11.9–17.4]). Six patients had cyanotic heart disease. Six patients (55%) had previously undergone surgery with CBP. Median CBP duration and length of stay in the intensive care unit were typical, at 132 minutes [64–362] and 6 days [1–186], respectively. The interval between surgery and symptom onset was 20 days [4–64], with a median duration of 44 days [3–181]. Abnormal movements primarily affected the face (100%) and were often bilateral (82%), involving the upper limbs (91%) and lower limbs (82%). Initial cerebral MRI did not show recent ischemic lesions. Six patients (55%) received medical treatment: tetrabenazine (n = 2), intravenous immunoglobulins (n = 1), corticosteroids (n = 1), L-Dopa (n = 1), cyamemazine (n = 1). Chorea persisted beyond 6 months in only 1 out of 11 patients. Post-pump chorea is extremely rare and can occur several months after cardiac surgery. It does not appear to be directly related to perioperative severity criteria. The prognosis is generally favorable within 6 months following diagnosis. [ABSTRACT FROM AUTHOR]

Congenitally corrected transposition of the great arteries (ccTGA) is a rare, complex and challenging structural heart disease. Therapeutic management remains controversial, particularly in patients with left ventricle outflow tract obstruction (LVOTO). As the natural history of ccTGA with LVOTO is overall very satisfactory and interventions that increase sub-pulmonary left ventricle (LV) pressure load are associated with a reduction of tricuspid regurgitation (TR) and systemic right ventricle (RV) dysfunction, we began to use restrictive left ventricle-to-pulmonary artery (LV-PA) conduit without ventricular septal defect (VSD) closure as part of the physiological repair of ccTGA/VSD/LVOTO. To report on the outcome of ccTGA/VSD/LVOTO patients treated with restrictive LV-PA conduit without VSD closure. Between 1979 and 2024, 9 consecutive patients with ccTGA/non-restrictive VSD/LVOTO underwent restrictive LV-PA conduit interposition without VSD closure at Necker Sick Children's Hospitals (Paris, France). Six (66.7%) patients had pulmonary stenosis and 3 (33.3%) patients had pulmonary atresia. Median age and weight at surgery were 5 (IQR: 16.6) years and 18 (IQR: 36.5) kgs. Five (55.6%) patients had previously undergone surgical palliation. No patient died after surgery with a median follow-up of 9.8 (IQR: 13.4) years. No patient required permanent pacemaker implantation for complete AV block. Freedom from reintervention was 62.5% (95% CI: 22.9–86.1) at 10 years. Late reintervention consisted in LV-PA conduit replacement (n = 3), PA stenting (n = 1) and one and half ventricle repair (hemi-Senning, Glenn, Rastelli, n = 1). At last follow-up, only one patient was significantly limited in physical activity (NYHA class III). No patient developed atrial or ventricular arrythmia. No patients developed moderate or severe TR and only 2 (22.2%) patients had decreased RV function and dilatation. We believe that restrictive LV-PA conduit placement without VSD closure represents a worthwhile biventricular alternative strategy to anatomic repair when it is deemed too risky and/or impractical. [ABSTRACT FROM AUTHOR]

Mitral valve replacement in infants is associated with high mortality and a significant rate of re-intervention. There is limited research examining the long-term consequences of valvuloplasty in this particular age group. Evaluate patient outcomes with mitral disease (stenosis, regurgitation or both) who had mitral valvuloplasty or replacement in the first year of life. Descriptive monocentric retrospective study including all children with mitral valve repair or replacement under 1 year of age over a period of 22 years (2001–2023). The outcomes assessed were: early mortality (at 30 days), late mortality, need for re-intervention. Fifty-six patients were identified. Median age at surgery was 147 days and median weight was 5.1 kg. Thirty-nine patients underwent mitral valve repair and 17 mitral valve replacement. Mean duration of ICU stay was 7 days. Overall median hospital stay was 11 days. Mortality rate was 17.9% (7.1% before discharge). Overall survival was 96% at 30 days, 86% at 1 year 82% at 3 years. Overall survival was significantly higher in patient with mitral valve repair compared to mitral valve replacement (P = 0.039). The only mortality risk factor identified was replacement of the mitral valve compare to repair, HR: 9 (1.3–94.8; P = 0.038). Twenty-three patients (41.1%) needed re-intervention. The re-intervention free survival rate after repair was 81% at 1 year; 65% at 5 years and 46% at 10 years. The re-intervention free survival rate after replacement was 74% at 1 year; 55% at 5 and 10 years. A log-rank test showed no difference in re-intervention free survival between mitral valve repair or replacement. Mitral valve surgery in infants carries particularly high risks and is associated with a high rate of re-interventions. While mitral valvuloplasty demonstrates superior outcomes in term of mortality, it frequently serves as a temporary measure, postponing the need for valve replacement. [ABSTRACT FROM AUTHOR]

Presents information on a study which investigated the In1-xGaxAs1-yPy/InP-based multiple quantum well (MQW) solar cell structures with built-in sequential resonant tunneling. Theoretical background; Samples used; Experimental method; Results and discussion; Conclusions.