Systematic skin and nasal decolonization lowers Staphylococcus infection in pediatric cardiac surgery.

BACKGROUND: Postoperative infections occur in approximately 10% of pediatric cardiac surgeries, involving Staphylococcus species in most cases. Nasal decontamination of Staphylococcus with mupirocin has been reported to reduce postoperative Staphylococcus infections after cardiac surgery in adults, but the effect of preoperative decontamination in children undergoing cardiac surgery has not been sufficiently studied to reach consensus. METHODS: We conducted a single-center retrospective study to evaluate the impact of systematic preoperative decolonization with intranasal mupirocin application and skin-washing with chlorhexidine soap on postoperative Staphylococcus infection in children undergoing cardiac surgery. Our population was divided into three groups according to decolonization protocol (group N: no decolonization; group T: targeted decolonization in Staphylococcus aureus [SA] carriers only; and group S: systematic decolonization). RESULTS: A total of 393 children were included between October 2011 and August 2015 (122 in group N, 148 in group T, and 123 in group S). The Staphylococcus infection rate significantly decreased in group S compared to group N (0.8% vs. 7.7%; p < 0.05) and tended to decrease in group S compared to group T (0.8% vs. 4.7%; p = 0.06). Systematic decontamination also significantly reduced the rate of infections starting from the skin (including surgical site infections and bloodstream infections) compared to targeted decolonization or lack of decolonization, but had no effect on the rate of pulmonary infections. CONCLUSION: The results of our study suggest that systematic preoperative skin and nasal decontamination, regardless of SA carriage status, could reduce the rate of postoperative Staphylococcus infections after cardiac surgery in children.

[Clinical variability and diagnosis steps in childhood mitochondrial disease]. / Variabilité clinique et conduite diagnostique des cytopathies mitochondriales : à propos d'une série de 18 cas pédiatriques.

OBJECTIVES: Mitochondrial respiratory chain deficiencies are known for their high clinical variability. Difficult to diagnose, the prevalence of these diseases is probably underestimated. METHODS: We report 18 children diagnosed with respiratory chain deficiency at the Tours University Hospital over the past 10 years. RESULTS: Three clinical profiles can be distinguished depending on the age at onset of the first symptoms: the neonatal period (4 cases), between 1 month and 2 years of age (10 cases), and after 10 years (4 cases). However, no clinical feature appears specific of any age group. In contrast, respiratory chain analysis on liver biopsy was very informative for all our patients at any age and with any clinical presentation, even with predominant neurological symptoms. CONCLUSIONS: These biochemical analyses support the diagnosis of mitochondrial disorders in view of molecular analysis, which nevertheless frequently remains inconclusive. These investigations should benefit from the new molecular screening technologies based on DNA chips that can identify the genomic mutations responsible for these severe and relatively frequent diseases.

[Enzyme replacement therapy in a boy with infantile Pompe disease: cardiac follow-up]. / Enzymothérapie substitutive chez un nourrisson atteint de maladie de Pompe : évolution cardiologique.

Pompe disease is an autosomal recessive glycogen storage disorder caused by acid-alpha-glucosidase deficiency. The infantile form is usually fatal by 1 year of age in the absence of specific therapy. We report the cardiac follow-up of a 4-month-old boy treated with enzyme replacement therapy (ERT) for 8 months. The patient had no cardiac failure at the age of 1 year. Before starting ERT, ECG showed a shortened PR interval, with huge QRS complexes and biventricular hypertrophy; echocardiography demonstrated major hypertrophic cardiomyopathy. The QRS voltage (SV1+RV6) decreased from 13 to 2.9 mV after 32 weeks of ERT, suggesting a progressive reduction of cardiac hypertrophy and intracellular glycogen excess. The PR interval increased from 60 to 90 ms. A block of the right bundle branch appeared after 13 weeks of treatment. The indexed left ventricular mass decreased from 240 to 90 g/m2 after 30 weeks of ERT. The left ventricular ejection fraction decreased transitorily between the 5th and the 15 th weeks of treatment. In summary, ERT is an efficient therapeutic approach for the cardiomyopathy of infantile Pompe disease. However, the possible occurrence of a right bundle branch block and a transitory alteration in the ejection fraction highlight the importance of cardiac follow-up.

[The role of aortic morphology in the occurrence of hypertension in patients undergoing surgery for coarctation of the aorta before the age of 1 year]. / Rôle de la morphologie aortique dans la survenue d'une hypertension artérielle chez des malades opérés d'une coarctation de l'aorte avant l'âge de 1 an.

OBJECTIVE: Hypertension on effort can be observed following surgical treatment of coarctation of the aorta even if the operation has been performed early and has been judged to be satisfactory. The pathophysiology of this hypertension has not been completely elucidated. The aim of our study was to look for a residual morphological anomaly of the aortic arch which might be responsible. METHODS: This was a retrospective study of patients aged over 10 years who had undergone surgery for coarctation of the aorta before the age of 1 year, consecutively between 1979 and 1993, and free from re-coarctation clinically or with Doppler. All of them had a blood pressure effort test on a cycling machine, echocardiography and an MRI of the thoracic aorta. RESULTS: 61 patients were included. Their median age was 15 years, 59% were male, 15% were hypertensive at rest and 56% on effort. With univariate analysis, only the average blood pressure (p=0.03) and the resting pulse pressure (p=0.001) were associated with hypertension on effort. With multivariate analysis the only factors that correlated independently with maximum arterial pressure on effort were the height of the patient (p=0.02) and the pulse pressure (p<0.0001). The surgical technique and the age at intervention were not associated with hypertension on effort (p=0.96 and 0.69 respectively). The diameter of the horizontal aorta and the aorta at the site of repair, measured on MRI, added to the diameter of the descending aorta at the diaphragm were not lower in patients with hypertension on effort (p=0.77 and 0.38). There were proportionally more Roman type aortic arches than Gothic types in patients with hypertension on effort, but this difference was not significant (p=0.18). CONCLUSION: In our study, the patients with hypertension on effort following correction of coarctation of the aorta did not display any residual obstruction at the level of the aortic cross on MRI. However, they had a resting pulse pressure that was significantly higher than the non hypertensive patients, reflecting an increase in the aortic wall rigidity.

[Quality of life of patients with total cavopulmonary anastomosis]. / Qualité de vie des patients avec une dérivation cavo-pulmonaire totale.

INTRODUCTION: Total cavo-pulmonary connection (TCPC) represents the current surgery standard for congenital univentricular heart disease (CUHD) patients. We studied the health related quality of life (HRQOL) expressed by TCPC operated patients. PATIENTS AND METHODS: This transversal study is based on 24 children and 10 adult TCPC patients aged from 5 to 41 years old. The HRQOL evaluation relies on generic self report instruments such as the PedsQL 4.0 for the children and their parents, and the Duke Health profile for the adult patients. Additional questions were specifically designed for this study as well. RESULTS AND DISCUSSION: The estimated HRQOL is 68.15 for the children and 68.71 for their parents. In these young cases, the global HRQOL or some specific score are altered when any comorbidity or scholar difficulties are present, or when TCPC was performed more than 10 years ago. The estimated HRQOL is 74.67 in adulthood. In these patients, the young age, the lifestyle, the onset of more than 2 early complications ans the necessity of anti-arrhythmia therapy decrease various HRQOL scores. The physical handicap is considered as the major limitation for children and adult patients. Thanks to adaptative and cognitive compensatory mechanisms, such as resilience, both groups express a satisfying HRQOL assessment. CONCLUSION: Our study indicates that TCPC provides a good HRQOL in CUHD patients despite the complex management and follow-up of these diseases, the risk of complication onset and the physical handicap usually observed.

[Heart murmurs in asymptomatic children: When should you refer?]. / Souffle cardiaque chez l'enfant asymptomatique : quand demander un avis cardiologique ?

Heart murmurs are common in children and adolescents. Although most are innocent, an isolated heart murmur in asymptomatic children may be the sole finding indicating serious heart disease. Historical elements of familial heart disease, cardiovascular symptoms and a well-conducted medical examination can identify children with an increased risk of heart disease. The distinction between an innocent heart murmur and a pathologic heart murmur is not always easy for primary care physicians because most of them have little experience with auscultation searching for congenital heart malformation. Echocardiography provides a definitive diagnosis of heart disease but is not required in case of innocent murmur. Inappropriate pediatric cardiologist and echocardiographic referral leads to useless and expensive examinations, resulting in a work overload for pediatric cardiologists. The objective of this review is to provide the keys to differentiate innocent and pathologic murmurs, and to help physicians decide on the optimal diagnostic strategy.

[Cardiomyopathy induced by frequent premature ventricular contractions]. / Myocardiopathie rythmique induite par des extrasystoles ventriculaires chez un enfant.

We report on the rare case of 7-year-old child presenting with frequent and apparently benign premature ventricular complexes (VPC) and left ventricular dysfunction. No structural disease of the heart was seen at cardiological evaluation. Eighteen months after the introduction of an antiarrhythmic treatment with low-dose bisoprolol, Holter monitoring showed 70,000 VPC in 24h with left ventricular function remaining decreased. Six months after increasing the dose of bisoprolol, the VPC were less frequent and left ventricular function was completely normalized. This case was characteristic of a cardiomyopathy induced by frequent premature ventricular contractions.

[Ventricular septal defects in the adult]. / Communications interventriculaires de l'adulte.

Ventricular septal defects (VSD) are very common in children but much rarer in adults because the majority either close spontaneously or are operated. The persistent VSD are usually membranous with a small or moderate shunt without pulmonary hypertension. They are usually well tolerated clinically but are at risk of complications, mainly bacterial endocarditis and aortic regurgitation. Doppler echocardiography plays a leading part in diagnosing the site and evaluating the left-to-right shunt and its complications. Surgery may be indicated in cases of large left-to-right shunt (QP/QS > 2) and when there are complications. Patients operated for VSD in childhood are usually considered to be cured. However, anatomical, haemodynamic, functional, electric and echocardiographic abnormalities may persist, justifying long-term cardiological follow-up.

[Hemifacial microsomia and cardiac malformations. Apropos of 2 cases]. / Microsomies hémifaciales et malformations cardiaques. A propos de 2 observations.

The authors report two cases of infants with complex heart disease comprising transposition of the great vessels, and a major unilateral facial anomaly involving the ear and the mandible, conforming to the description of hemifacial microsomia. The facial anomalies seen are defined and placed in the classification of known syndromes. The incidence of heart disease associated with facial microsomia is about 20% and in 2/3 of cases it is a ventricular septal defect or a tetralogy of Fallot. An analysis of aetiological factors shows that syndromes of the first two branchial arches are usually sporadic and that they may be the result of intra-uterine disease. The mechanism of the facial lesions is probably vascular.

[Right coronary artery arising from the pulmonary artery. Surgical treatment]. / Coronaire droite naissant de l'artère pulmonaire. Traitement chirurgical.

The authors report the case of an anomalous right coronary artery arising from the pulmonary artery. The patient was an active 18 years old boy who had a continuous murmur with diastolic accentuation, the cause of which was determined at angiography. There were electrical and echocardiographic signs of moderate left ventricular dilatation and thallium scintigraphy showed a myocardial perfusion defect. These were the only detectable consequences of this rare malformation. Normal coronary circulation was reestablished surgically, the indication being the risk of sudden death which has been previously reported rather than acute myocardial ischemia.