B. Romefort

Procalcitonin as a marker of bacterial infection in children undergoing cardiac surgery with cardiopulmonary bypass.

BACKGROUND Owing to systemic inflammatory response syndrome, the diagnosis of post-operative infection after cardiopulmonary bypass is difficult to assess in children with the usual clinical and biological tools. Procalcitonin could be informative in this context. METHODS Retrospective study in a paediatric intensive care unit. Blood samples were collected as soon as infection was clinically suspected and a second assay was performed 24 hours later. Using referenced criteria, children were retrospectively classified into two groups: infected and non-infected. RESULTS Out of the 95 children included, 14 were infected. Before the third post-operative day, procalcitonin median concentration was significantly higher in the infected group than in the non-infected group - 20.24 nanograms per millilitre with a 25th and 75th interquartile of 15.52-35.71 versus 0.72 nanograms per millilitre with a 25th and 75th interquartile of 0.28 to 5.44 (p = 0.008). The area under the receiver operating characteristic curve was 0.89 with 95% confidence intervals from 0.80 to 0.97. The best cut-off value to differentiate infected children from healthy children was 13 nanograms per millilitre with 100% sensitivity - 95% confidence intervals from 51 to 100 - and 85% specificity - 95% confidence intervals from 72 to 91. After the third post-operative day, procalcitonin was not significantly higher in infected children - 2 nanograms per millilitre with a 25th and 75th interquartile of 0.18 to 12.42 versus 0.37 nanograms per millilitre with a 25th and 75th interquartile of 0.24 to 1.32 (p = 0.26). The area under the receiver operating characteristic curve was 0.62 with 95% confidence intervals from 0.47 to 0.77. A procalcitonin value of 0.38 nanograms per millilitre provided a sensitivity of 70% with 95% confidence intervals from 39 to 89 for a specificity of 52% with 95% confidence intervals from 34 to 68. After the third post-operative day, a second assay at a 24-hour interval can improve the sensitivity of the test. CONCLUSIONS Procalcitonin seems to be a discriminating marker of bacterial infection during the post-operative days following cardiopulmonary bypass in children.

Late repair of tetralogy of Fallot during childhood in patients from developing countries

OBJECTIVES Although the current surgical approach of tetralogy of Fallot (TOF) is primary complete repair in infancy, late diagnosis and lack of surgical facilities in developing countries may delay surgical treatment. Some of these patients exposed to prolonged chronic hypoxaemia are transferred to more privileged countries to undergo surgical repair with the support of non-governmental organizations. The objective of this single-centre retrospective study was to compare the postoperative outcomes of these foreign patients undergoing delayed repair with those of patients born in France undergoing timely repair during the same time period. METHODS The computer database of our institution was searched for all cases of TOF in foreign patients younger than 15 years, supported by two non-profit organizations, who underwent complete repair between January 2007 and December 2013. The control population consisted of the patients with TOF born in France, who underwent timely complete repair during the same period. RESULTS The 47 foreign children were older (57.6 ± 38.4 vs 8.3 ± 9.1 months, P < 0.0001), more hypoxaemic (SaO2 79 ± 11 vs 91 ± 8%, P < 0.0001), more growth-retarded (body mass index Z-score -1.35 ± 1.5 vs -0.46 ± 1.3, P = 0.0034), and had higher haematocrit level (52.5 ± 11.7 vs 37.5 ± 6.1%, <0.0001) and worse left ventricular ejection fraction (LVEF 62 ± 8 vs 69 ± 3.8%, P < 0.0001) than the 90 French patients. Postoperative mortality and morbidity (sepsis, arrhythmia, bleeding and need for surgical revision) were similar in the two groups, except for a higher rate of pericardial and/or pleural effusion in foreign children (36 vs 17%, P = 0.02). Length of stay was shorter in foreign than in French patients (11 ± 6 vs 15 ± 15 days, P = 0.0012). CONCLUSIONS In our experience, despite the presence of several risk factors (growth retardation, chronic hypoxaemia, polycythaemia and left ventricular dysfunction), late repair of TOF was undertaken during childhood in patients from developing countries with no difference in postoperative morbidity and mortality compared with that of timely repair during infancy. These older patients can be discharged sooner. However, patients undergoing late surgery may be at higher risk of complications of right ventricular failure, such as pleural and/or pericardial effusion.

Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus

BACKGROUND Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. METHODS We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. RESULTS Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM (P=0.0199; HR=3.13 [95% CI: 1.20-8.16]), non-European origin (P=0.0052; HR=4.10 [95% CI: 1.81-9.28]) and pacemaker implantation (P=0.0013; HR=5.48 [95% CI: 1.94-15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth-4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months-22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM (P=0.27; HR=1.65 [95% CI: 0.63-4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. CONCLUSION Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB.

0346: Neonatal arterial switch operation: the sooner the better!

Objective To evaluate the impact of preoperative management on in-hospital post-operative outcomes after an arterial switch operation (ASO) in newborns with transposition of the great arteries (TGA) Methods In this retrospective monocentric study, we included all newborns with TGA±ventricular septal defect who underwent an ASO between 2008 and 2014. Collected data included demographics, clinical and anatomic characteristics, and preoperative management (Rashkind, prostaglandin infusion, mechanical ventilation, age at surgery). Univariate and multivariate analyses were performed to study the impact of preoperative characteristics on time to extubation, a surrogate marker of postoperative morbidity. Results Among the 59 included newborns, 47 (79%) had an antenatal diagnosis, 31 (52%) received prostaglandins (median duration: 96 h; range 0-192h), and 38 (65%) underwent a Rashkind procedure. Mean age at surgery was 5±2 days. The mortality rate was 5% (n=3). Younger age at surgery significantly increased the probability to be extubated within 2 days after surgery, both in univariate analysis (Odd ratio 0.64; confidence interval 0.44-0.92) (p=0.01) and after adjustment for preoperative characteristics and management (OR 0.61; CI 0.39-0.95) (p=0.03). The presence of coronary anomalies tended to increase time to extubation, whereas Rashkind procedure and prostaglandin treatment had no impact. Conclusion Our study shows that younger age at surgery is associated with lower postoperative morbidity in newborns undergoing an ASO. These findings suggest that early surgery is preferable to delay with palliative management such as Rashkind and/or Prostaglandins.

Neonatal Marfan Syndrome: Report of a Case with an Inherited Splicing Mutation outside the Neonatal Domain.

We report a child and her mother affected by Marfan syndrome. The child presented with a phenotype of neonatal Marfan syndrome, revealed by acute and refractory heart failure, finally leading to death within the first 4 months of life. Her mother had a common clinical presentation. Genetic analysis revealed an inherited FBN1 mutation. This intronic mutation (c.6163+3_6163+6del), undescribed to date, leads to exon 49 skipping, corresponding to in-frame deletion of 42 amino acids (p.Ile2014_Asp2055del). FBN1 next-generation sequencing did not show any argument for mosaicism. Association in the same family of severe neonatal and classical Marfan syndrome illustrates the intrafamilial phenotype variability.