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Background: Children with advanced pulmonary disease due to cystic fibrosis (CF) are at risk of acute respiratory failure due to pulmonary exacerbations leading to their admission to pediatric intensive care units (PICU). The objectives of this study were to determine short and medium-term outcomes of children with CF admitted to PICU for acute respiratory failure due to pulmonary exacerbation and to identify prognosis factors., Methods: This retrospective monocentric study included patients less than 18 years old admitted to the PICU of a French university hospital between 2000 and 2020. Cox proportional hazard regression methods were used to determine prognosis factors of mortality or lung transplant., Results: Prior to PICU admission, the 29 patients included (median age 13.5 years) had a severe lung disease (median Forced Expiratory Volume in 1 s percentage predicted at 29%). Mortality rates were respectively 17%, 31%, 34%, 41% at discharge and at 3, 12 and 36 months post-discharge. Survival rates free of lung transplant were 34%, 32%, 24% and 17% respectively. Risk factors associated with mortality or lung transplant using the univariate analysis were female sex and higher pCO2 and chloride levels at PICU admission, and following pre admission characteristics: home respiratory and nutritional support, registration on lung transplant list and Stenotrophomonas Maltophilia bronchial colonization., Conclusion: Children with CF admitted to PICU for acute respiratory failure secondary to pulmonary exacerbations are at high risk of death, both in the short and medium terms. Lung transplant is their main chance of survival and should be considered early., (© 2024. The Author(s).)

Objectives: We compared the risk of mortality and reintervention after common arterial trunk (CAT) repair for different surgical techniques, in particular the reconstruction of the right ventricle outflow tract with left atrial appendage (LAA) without a monocusp., Methods: The study population comprised 125 patients with repaired CAT who were followed-up at our institution between 2000 and 2018. Statistical analysis included Cox proportional hazard models., Results: Median follow-up was 10.6 years. The 10-year survival rate was 88.2% (95% confidence interval [CI], 80.6-92.4) with the poorest outcome for CAT type IV (64.3%; 95% CI, 36.8-82.3; P < .01). In multivariable analysis, coronary anomalies (hazard ratio [HR], 11.63 [3.84-35.29], P < .001) and CAT with interrupted aortic arch (HR, 6.50 [2.10-20.16], P = .001) were substantial and independent risk factors for mortality. Initial repair with LAA was not associated with an increased risk of mortality (HR, 0.37 [0.11-1.24], P = .11). The median age at reintervention was 3.6 years [7.3 days-13.1 years]. At 10 years, freedom from reintervention was greater in the group with LAA repair compared with the valved conduit group, 73.3% (95% CI, 41.3-89.4) versus 17.2% (95% CI, 9.2-27.4) (P < .001), respectively. Using a valved conduit for repair (HR, 4.79 [2.45-9.39], P < .001), truncal valve insufficiency (HR, 2.92 [1.62-5.26], P < .001) and DiGeorge syndrome (HR, 2.01 [1.15-3.51], P = .01) were independent and clinically important risk factors for reintervention., Conclusions: For the repair of CAT, the LAA technique for right ventricle outflow tract reconstruction was associated with comparable survival and greater freedom from reintervention than the use of a valved conduit., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: The main challenge of aortic coarctation (CoA) repair in infants is to obtain durable results without morbidity. We aimed to describe predictors of aortic arch reintervention after aortic CoA repair., Methods: Between January 2000 and March 2014, we retrospectively included consecutive infants with isolated CoA or CoA with ventricular septal defect (CoA + VSD) who had surgical repair of the aortic arch before 3 months of age., Results: Five hundred and thirty patients were included: 308 (58%) patients had isolated CoA and 222 (42%) patients had CoA + VSD. Three hundred and eighty-five patients (72.6%) had CoA repair, 51 patients (9.6%) had CoA repair with closure of VSD and 94 patients (17.8%) had CoA repair with pulmonary artery banding. Mean age at operation was 13 ± 1.6 days, with 294 patients (55.5%) operated on before 2 weeks. Median follow-up was 7.57 years. Sixty-one patients (11.5%) needed reintervention on the aortic arch. Freedom from aortic arch reintervention was 90% at 1 year and 88.5% at 5 years. Proportions of aortic arch reintervention were similar in the different surgical strategy groups (P = 0.80). However, in patients receiving prostaglandin E1 (PGE1), the end-to-end repair was at higher risk of recoarctation compared to the extended end-to-side repair (P = 0.033). The risk factors of aortic arch reintervention were age at repair <15 days (P = 0.034) and the need for PGE1 infusion at surgery (P = 0.0043)., Conclusions: CoA repair in young infants has an overall good outcome. The use of PGE1 may modify the aortic arch anatomy and mask the boundaries of the resection to be performed. PGE1 treatment should be studied more specifically in another study to improve preoperative management., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)