Early postoperative beta-blockers are associated with improved cardiac output after late complete repair of tetralogy of Fallot: a retrospective cohort study.

Tetralogy of Fallot is the most common cyanotic congenital heart disease. For decades, our institution has cared for humanitarian patients with late presentation of tetralogy of Fallot. They are characterized by severe right ventricular hypertrophy with consecutive diastolic dysfunction, increasing the risk of postoperative low cardiac output syndrome (LCOS). By right ventricular restrictive physiology, we hypothesized that patients receiving early postoperative beta-blockers (within 48 h after cardiopulmonary bypass) may have better diastolic function and cardiac output. This is a retrospective cohort study in a single-center tertiary pediatric intensive care unit. We included > 1-year-old humanitarian patients with a confirmed diagnosis of tetralogy of Fallot undergoing a complete surgical repair between 2005 and 2019. We measured demographic data, preoperative echocardiographic and cardiac catheterization measures, postoperative mean heart rate, vasoactive-inotropic scores, LCOS scores, length of stay, and mechanical ventilation duration. One hundred sixty-five patients met the inclusion criteria. Fifty-nine patients (36%) received early postoperative beta-blockers, associated with a lower mean heart rate, higher vasoactive-inotropic scores, and lower LCOS scores during the first 48 h following cardiopulmonary bypass. There was no significant difference in lengths of stay and ventilation. Conclusion: Early postoperative beta-blockers lower the prevalence of postoperative LCOS at the expense of a higher need for vasoactive drugs without any consequence on length of stay and ventilation duration. This approach may benefit the specific population of children undergoing a late complete repair of tetralogy of Fallot. What is Known: • Prevalence of low cardiac output syndrome is high following a late complete surgical repair of tetralogy of Fallot. What is New: • Early postoperative beta-blockade is associated with lower heart rate, prolonged relaxation time, and lower prevalence of low cardiac output syndrome. • Negative chronotropic agents like beta-blockers may benefit selected patients undergoing a late complete repair of tetralogy of Fallot, who are numerous in low-income countries.

"Take it or leave it": Analysis of pediatric heart offers for transplantation in Switzerland.

BACKGROUND: There is a shortage of donor hearts in Switzerland, especially for pediatric recipients. However, the rate and reason for refusals of pediatric donor hearts offered in Switzerland has not been systematically analyzed. METHODS: The national transplant database, Swiss Organ Allocation System, was searched for all hearts from Swiss and foreign donors younger than 16 years from 2015 to 2020. The numbers of accepted and refused hearts and early outcome were assessed, and the reasons for refusal were retrospectively analyzed. RESULTS: A total of 136 organs were offered to the three Swiss pediatric heart centers and foreign donor procurement organizations. Of these, 26/136 (19%) organs were accepted and transplanted: 18 hearts were transplanted in Switzerland, and 13 of these were foreign. Reasons for refusal were (1) no compatible recipient due to blood group or weight mismatch, 89.4%; (2) medical, meaning organ too marginal for transplantation, 7.4%; (3) logistic, 1.4%; and (4) other, 1.8%. Five organs were refused in Switzerland by one center but later accepted and successfully transplanted by another center. Hearts from outside Switzerland were transplanted significantly less than Swiss hearts (n = 16/120 vs. 10/16, p < .001). CONCLUSION: The most common reason for refusing a pediatric donor heart is lack of compatibility with the recipient. Few hearts are refused for medical reasons. A more generous acceptance seems to be justified in selected patients. Switzerland receives a high number of foreign offers, but their rate of acceptance is lower than that of Swiss donations.

COVID-19 infection in patients with history of pediatric heart transplant in Germany, Austria, and Switzerland.

COVID-19 is a heterogenous infection-asymptomatic to fatal. While the course of pediatric COVID-19 infections is usually mild or even asymptomatic, individuals after adult heart transplantation are at high risk of a severe infection. We conducted a retrospective, multicenter survey of 16 pediatric heart transplant centers in Germany, Austria and Switzerland to evaluate the risk of a severe COVID-19 infection after pediatric heart transplantation between 02/2020 and 06/2021. Twenty-six subjects (11 male) with a median age of 9.77 years at time of transplantation and a median of 4.65 years after transplantation suffered from COVID-19 infection. The median age at time of COVID-10 infection was 17.20 years. Fourteen subjects had an asymptomatic COVID-19 infection. The most frequent symptoms were myalgia/fatigue (n = 6), cough (n = 5), rhinitis (n = 5), and loss of taste (n = 5). Only one subject showed dyspnea. Eleven individuals needed therapy in an outpatient setting, four subjects were hospitalized. One person needed oxygen supply, none of the subjects needed non-invasive or invasive mechanical ventilation. No specific signs for graft dysfunction were found by non-invasive testing. In pediatric heart transplant subjects, COVID-19 infection was mostly asymptomatic or mild. There were no SARS-CoV-2 associated myocardial dysfunction in heart transplant individuals.

Case Report: Transcatheter interventional procedure to innominate vein turn-down procedure for failing fontan circulation.

Fontan physiology creates a chronic state of decreased cardiac output and systemic venous congestion, leading to liver cirrhosis/malignancy, protein-losing enteropathy, chylothorax, or plastic bronchitis. Creating a fenestration improves cardiac output and relieves some venous congestion. The anatomic connection of the thoracic duct to the subclavian-jugular vein junction exposes the lymphatic system to systemic venous hypertension and could induce plastic bronchitis. To address this complication, two techniques have been developed. A surgical method that decompresses the thoracic duct by diverting the innominate vein to the atrium, and a percutaneous endovascular procedure that uses a covered stent to create an extravascular connection between the innominate vein and the left atrium. We report a novel variant transcatheter intervention of the innominate vein turn-down procedure without creating an extravascular connection in a 39-month-old patient with failing Fontan circulation complicated by plastic bronchitis and a 2-year post-intervention follow-up.

Successful heart transplant in a child with congenital core myopathy and delayed-onset restrictive cardiomyopathy due to recessive mutations in the titin (TTN) gene.

BACKGROUND: Mutations in the TTN gene, encoding the muscle filament titin, are a major cause of inherited dilated cardiomyopathy. Early-onset skeletal muscle disorders due to recessive TTN mutations have recently been described, sometimes associated with cardiomyopathies. CASE DESCRIPTION: We report the case of a boy with congenital core myopathy due to compound heterozygosity for TTN variants. He presented in infancy with rapidly evolving restrictive cardiomyopathy, requiring heart transplantation at the age of 5 years with favorable long-term cardiac and neuromuscular outcome. CONCLUSION: Heart transplantation may have a role in selected patients with TTN-related congenital myopathy with disproportionally severe cardiac presentation compared to skeletal and respiratory muscle involvement.

Diuretics in pediatrics.

Diuretics are frequently prescribed drugs and help managing several pathological conditions, including acute and chronic kidney disease, nephrotic syndrome, congestive heart failure, ascites, systemic and pulmonary hypertension. Diuretic classes include among others osmotic diuretics and carboanhydrase inhibitors, loop diuretics, thiazides, and potassium-sparing diuretics. In this educational article, we aim at reviewing indications, mechanisms of action, and side effects, as well as basic pharmacokinetics considerations and data on diuretics in children, supporting practicing clinicians in choosing (and understanding the background of) the best-suited diuretic regimen for the individual patient. Newer diuretic classes like vaptans and sodium glucose type 2 cotransporter inhibitors, the recent controversies on hydrochlorothiazide, and the issue of diuretic resistance, will also be briefly addressed. CONCLUSION: This educational review offers a didactical overview of diuretics in Pediatrics. WHAT IS KNOWN: • Diuretics are frequently prescribed drugs in both adults and children. • They increase water and sodium excretion, reducing fluid overload. WHAT IS NEW: • This article reviews indications, mechanisms of action, side effects, and basic pharmacokinetics facts on diuretics in Paediatrics. • It also addresses current issues, like the management of diuretic resistance, the recent controversy on hydrochlorothiazide, and the novel classes vaptans and gliflozins.

Consequences of gestational diabetes mellitus on neonatal cardiovascular health: MySweetHeart Cohort study.

BACKGROUND: Hyperglycaemic disorders of pregnancy are associated with offspring cardiovascular alterations. METHODS: MySweetHeart cohort study aimed to assess the effect of maternal gestational diabetes (GDM) on offsprings' cardiovascular health. Newborns underwent clinical and echocardiographic examinations between 2016 and 2020. RESULTS: Compared to mothers without GDM (n = 141), mothers with GDM (n = 123) were more likely to have had GDM in previous pregnancies and had higher weight, BMI, blood glucose, and HbA1c. Newborns of both groups showed similar clinical characteristics. Echocardiography was performed on the 3rd (interquartile range, IQR, 2nd-4th) day of life in 101 offsprings of mothers without and 116 offsprings of mothers with GDM. Left ventricular (LV) mass was similar. Children born to mothers with GDM had a thicker posterior LV wall (z-score +0.15, IQR -0.38/0.62, versus +0.47, IQR -0.11/+1.1, p = 0.004), a smaller end-systolic (1.3 mL, IQR 1.0-1.5 mL, versus 1.4 mL, IQR 1.2-1.8 mL, p = 0.044) but a similar end-diastolic LV volume. They also had shorter tricuspid valve flow duration and aortic valve ejection time, lower tricuspid E-wave and pulmonary valve velocities. CONCLUSIONS: Newborns of mothers with or without GDM had similar clinical characteristics and LV mass. However, some echocardiographic differences were detected, suggesting an altered myocardial physiology among infants of mothers with GDM. REGISTRATION: ClinicalTrials.gov (NCT02872974). IMPACT: Hyperglycaemic disorders of pregnancy are known to be associated with offspring cardiovascular alterations. Clinical characteristics and estimated left ventricular (LV) mass were similar in children issued from mothers with and without gestational diabetes (GDM). Children born to mothers with GDM had a thicker posterior LV wall and a smaller end-systolic LV volume. Although LV mass is not different, myocardial physiology may be altered in these infants. Further studies should investigate the endothelial function of this population and the cardiovascular evolution of these children over time.

Gestational diabetes mellitus and offspring's carotid intima-media thickness at birth: MySweetHeart Cohort study.

OBJECTIVE: Hyperglycaemia during pregnancy is associated with cardiometabolic risks for the mother and the offspring. Mothers with gestational diabetes mellitus (GDM) have signs of subclinical atherosclerosis, including increased carotid intima-media thickness (CIMT). We assessed whether GDM is associated with increased CIMT in the offspring at birth. DESIGN AND SETTING: MySweetHeart Cohort is a prospective cohort study conducted in Switzerland. PARTICIPANTS, EXPOSURE AND OUTCOME MEASURES: This work included pregnant women with and without GDM at 24-32 weeks of gestation and their singleton live-born offspring with data on the primary outcome of CIMT. GDM was diagnosed based on the criteria of the International Association of Diabetes and Pregnancy Study Groups. Offspring's CIMT was measured by ultrasonography after birth (range 1-19 days). RESULTS: Data on CIMT were available for 99 offspring of women without GDM and 101 offspring of women with GDM. Maternal age ranged from 18 to 47 years. Some 16% of women with GDM and 6% of women without GDM were obese. Smoking during pregnancy was more frequent among women with GDM (18%) than among those without GDM (4%). Neonatal characteristics were comparable between the two groups. The difference in CIMT between offspring of women with and without GDM was of 0.00 mm (95% CI -0.01 to 0.01; p=0.96) and remained similar on adjustment for potential confounding factors, such as maternal prepregnancy body mass index, maternal education, smoking during pregnancy, family history of diabetes, as well as offspring's sex, age, and body surface area (0.00 mm (95% CI -0.02 to 0.01; p=0.45)). CONCLUSIONS: We found no evidence of increased CIMT in neonates exposed to GDM. A longer-term follow-up that includes additional vascular measures, such as endothelial function or arterial stiffness, may shed further light on the cardiovascular health trajectories in children born to mothers with GDM. TRIAL REGISTRATION NUMBER: NCT02872974; Pre-results.

Epidemiology of Kawasaki Disease in children in Switzerland: a national prospective cohort study.

AIM OF THE STUDY: Kawasaki disease is a febrile illness which can lead to significant coronary artery lesions. Its incidence varies among countries and is highest in Japan (330.2 children under 5 years old/100,000 per year). Since the epidemiology of Kawasaki disease in Switzerland is unknown, we conducted a national prospective data collection between 2013 and 2017 to describe its incidence, diagnosis, and treatment. METHODS: We collected demographic and clinical data of the children under 17 years old hospitalised with Kawasaki disease in Switzerland between March 2013 and February 2017 using anonymous data collection forms with the help of the Swiss Paediatric Surveillance Unit (SPSU). We defined Kawasaki disease per the 2004 American Heart Association criteria: patients with ≥5 days of fever and ≥4 of the 5 main clinical features were included as complete Kawasaki disease and patients with ≥5 days of fever and <4 of the 5 main clinical features were included as incomplete Kawasaki disease. The incidence was calculated with the data of the Federal Statistical Office of Switzerland, considering permanent residents of the country. The different groups were compared by the unpaired student t-test for continuous variables and Pearson's chi squared test for categorical variables, respectively. RESULTS: We included 175 patients: 60% were boys, with a mean age of 38.2 months. The incidence of Kawasaki disease was 3.1/100,000 [95% CI 2.6-3.7] per year in children under 17 years of age and 8.4/100,000 [95% CI 6.7-10.2] per year in children under 5 years of age. The most frequent clinical signs were a rash (85.4%) and changes of the lips and oral/pharyngeal mucosa (83.4%). The diagnosis of Kawasaki disease was made at a mean of 7.3 days after the first symptom. Echocardiography was abnormal in 52.3%. The treatment with intravenous immunoglobulins (IVIG) and acetylsalicylic acid was administered in accordance with international guidelines. Subgroup analysis showed that children older than 5 years old had significantly more complete Kawasaki disease than the younger ones (78.8% vs 57.4%, p = 0.021). Children with "extreme ages" (<1 year old and >8 years old) were diagnosed later (8.6 (±0.9) vs 7.0 (±0.3) days, p = 0.0129), had longer duration of fever (9.8 (±0.9) vs 8.1 (±0.3) days, p = 0.013) and had more echocardiographic abnormalities (n = 26 (70.3%) vs n = 65 (47.5%), p = 0.014) at diagnosis. One child died during the acute phase of the illness. CONCLUSIONS: The incidence of Kawasaki disease in Switzerland is in the lower range of other European countries.

Interventions to Decrease Carotid-Intima Media Thickness in Children and Adolescents With Type 1 Diabetes: A Systematic Review and Meta-Analysis.

Introduction: Hyperglycemia is associated with a higher cardiovascular risk, as evidenced by increased carotid-intima media thickness (CIMT) in youth with diabetes. We conducted a systematic review and meta-analysis to assess the effect of pharmacological or non-pharmacological interventions on CIMT in children and adolescents with prediabetes or diabetes. Methods: We conducted systematic searches of MEDLINE, EMBASE, and CENTRAL, together with supplementary searches in trial registers and other sources for studies completed up to September 2019. Interventional studies assessing ultrasound CIMT in children and adolescents with prediabetes or diabetes were considered for inclusion. Where appropriate, data were pooled across studies using random-effect meta-analysis. Quality was assessed using The Cochrane Collaboration's risk-of-bias tool and a CIMT reliability tool. Results: Six studies involving 644 children with type 1 diabetes mellitus were included. No study involved children with prediabetes or type 2 diabetes. Three randomized controlled trials (RCTs) evaluated the effects of metformin, quinapril, and atorvastatin. Three non-randomized studies, with a before-and-after design, evaluated the effects of physical exercise and continuous subcutaneous insulin infusion (CSII). The mean CIMT at baseline ranged from 0.40 to 0.51 mm. The pooled difference in CIMT was -0.01 mm (95% CI: -0.04 to 0.01) for metformin compared to placebo (2 studies; 135 participants; I2: 0%). The difference in CIMT was -0.01 mm (95% CI: -0.03 to 0.01) for quinapril compared to placebo (1 study; 406 participants). The mean change from baseline in CIMT was -0.03 mm (95% CI: -0.14 to 0.08) after physical exercise (1 study; 7 participants). Inconsistent results were reported for CSII or for atorvastatin. CIMT measurement was rated at a higher quality on all reliability domains in 3 (50%) studies. The confidence in results is limited by the low number of RCTs and their small sample sizes, as well as the high risk of bias in before-and-after studies. Conclusions: Some pharmacological interventions may decrease CIMT in children with type 1 diabetes. However, there is great uncertainty with respect to their effects and no strong conclusions can be drawn. Further evidence from larger RCTs is required. Systematic Review Registration: PROSPERO, CRD42017075169.

Multisystem inflammatory syndrome with refractory cardiogenic shock due to acute myocarditis and mononeuritis multiplex after SARS-CoV-2 infection in an adult.

A 22-year-old male with a typical history of pauci-symptomatic COVID-19 3 weeks earlier, confirmed by positive serology for SARS-CoV-2 (IgG), was admitted to the intensive care unit because of severe myocarditis with refractory cardiogenic shock that required extracorporeal life support. Due to a clinical presentation suggestive of Kawasaki-like disease with coronary aneurysm and severe systemic inflammation, intravenous immunoglobulins were administered in combination with tocilizumab. The initial clinical course was favourable with these treatments. However, the patient subsequently developed a severe mononeuritis multiplex leading to bilateral foot drop, which required intensive immunosuppressive therapy (corticosteroids, cyclophosphamide and rituximab). The clinical presentation meets the criteria for multisystem inflammatory syndrome associated with SARS-CoV-2, but includes very severe organ damages. Early recognition, a multidisciplinary approach and aggressive therapeutic intervention can lead to a favourable outcome.

Risk factors during first 1,000 days of life for carotid intima-media thickness in infants, children, and adolescents: A systematic review with meta-analyses.

BACKGROUND: The first 1,000 days of life, i.e., from conception to age 2 years, could be a critical period for cardiovascular health. Increased carotid intima-media thickness (CIMT) is a surrogate marker of atherosclerosis. We performed a systematic review with meta-analyses to assess (1) the relationship between exposures or interventions in the first 1,000 days of life and CIMT in infants, children, and adolescents; and (2) the CIMT measurement methods. METHODS AND FINDINGS: Systematic searches of Medical Literature Analysis and Retrieval System Online (MEDLINE), Excerpta Medica database (EMBASE), and Cochrane Central Register of Controlled Trials (CENTRAL) were performed from inception to March 2019. Observational and interventional studies evaluating factors at the individual, familial, or environmental levels, for instance, size at birth, gestational age, breastfeeding, mode of conception, gestational diabetes, or smoking, were included. Quality was evaluated based on study methodological validity (adjusted Newcastle-Ottawa Scale if observational; Cochrane collaboration risk of bias tool if interventional) and CIMT measurement reliability. Estimates from bivariate or partial associations that were least adjusted for sex were used for pooling data across studies, when appropriate, using random-effects meta-analyses. The research protocol was published and registered on the International Prospective Register of Systematic Reviews (PROSPERO; CRD42017075169). Of 6,221 reports screened, 50 full-text articles from 36 studies (34 observational, 2 interventional) totaling 7,977 participants (0 to 18 years at CIMT assessment) were retained. Children born small for gestational age had increased CIMT (16 studies, 2,570 participants, pooled standardized mean difference (SMD): 0.40 (95% confidence interval (CI): 0.15 to 0.64, p: 0.001), I2: 83%). When restricted to studies of higher quality of CIMT measurement, this relationship was stronger (3 studies, 461 participants, pooled SMD: 0.64 (95% CI: 0.09 to 1.19, p: 0.024), I2: 86%). Only 1 study evaluating small size for gestational age was rated as high quality for all methodological domains. Children conceived through assisted reproductive technologies (ART) (3 studies, 323 participants, pooled SMD: 0.78 (95% CI: -0.20 to 1.75, p: 0.120), I2: 94%) or exposed to maternal smoking during pregnancy (3 studies, 909 participants, pooled SMD: 0.12 (95% CI: -0.06 to 0.30, p: 0.205), I2: 0%) had increased CIMT, but the imprecision around the estimates was high. None of the studies evaluating these 2 factors was rated as high quality for all methodological domains. Two studies evaluating the effect of nutritional interventions starting at birth did not show an effect on CIMT. Only 12 (33%) studies were at higher quality across all domains of CIMT reliability. The degree of confidence in results is limited by the low number of high-quality studies, the relatively small sample sizes, and the high between-study heterogeneity. CONCLUSIONS: In our meta-analyses, we found several risk factors in the first 1,000 days of life that may be associated with increased CIMT during childhood. Small size for gestational age had the most consistent relationship with increased CIMT. The associations with conception through ART or with smoking during pregnancy were not statistically significant, with a high imprecision around the estimates. Due to the large uncertainty in effect sizes and the limited quality of CIMT measurements, further high-quality studies are needed to justify intervention for primordial prevention of cardiovascular disease (CVD).

Coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply in pulmonary atresia and ventricular septal defect with absent left pulmonary artery.

Pulmonary atresia and ventricular septal defect is associated with variable sources of pulmonary blood supply. We present a case of a coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply.

Implantation of a HeartMate 3 in a 13-Year-Old Child with Dilated Cardiomyopathy.

Left ventricular assist device is a well-established therapy in heart failure adults, but less in children. A 13-year-old-boy with severe left ventricular dysfunction did not improve under medical treatment. A HeartMate 3 (HM3) was implanted as a bridge to transplantation. Despite the size limitation, the HM3 shows promising results and our case supports its feasibility in children.

Point-of-care hemostasis in children with congenital heart disease, the POCHEMO study: baseline reference values of thromboelastometry and impedance aggregometry.

: Viscoelastic tests and impedance aggregometry allow coagulation evaluation at the bedside, but reference values are scarce in pediatrics. The aim of this study was to establish reference values of thromboelastometry and impedance aggregometry for this population and compare it between age groups. This prospective, single-center, observational study evaluates viscoelastic tests and impedance aggregometry in children with congenital heart disease. A total of 204 children were included with a median age of 3.6 years old. We provide references values for this population with median, percentile 2.5 and percentile 97.5. Infants demonstrate for extrinsic activity a shorter coagulation time (52 [49-55] vs. 56 [51-62] s, P = 0.007) and clot formation time (90 [71-118] vs. 113 [93-146] s, P < 0.0001) so as for intrinsic activity a shorter clot formation time (53 [44-69] vs. 75 [59-92] s, P < 0.0001). The maximal clot firmness was significantly stronger in infants for extrinsic (65 [61-69] vs. 59 [54-63] mm, P < 0.0001), intrinsic (68 [64-70] vs. 61 [57-65] mm, P < 0.0001), and fibrinogen (12 [9-16] vs. 10 [8-13] mm, P = 0.02) activities. Platelet aggregation was significantly higher in infants with an amplitude at 6 min of 28 [23-34] vs. 22 [15-27] Ω, P less than 0.0001, a maximum speed of 11 [9-13] vs. 7 [5-10] Ω/min, P less than 0.0001, and an area under the curve of 120 [92-135] vs. 86 [59-112] Ω min, P less than 0.0001. We provided the first reference values for impedance aggregometry and thromboelastometry in children with congenital heart disease. We showed that these infants tend to have accelerated coagulation and stronger clot firmness compared with older children, but this finding may have only minimal relevance when treating a bleeding child. Trial registration number: ClinicalTrials.gov (clinicaltrials.gov/ct2/show/NCT02387944).

CytoSorb® hemoadsorption and mechanical circulatory support in a newborn with refractory shock after congenital heart surgery.

Refractory cardiogenic and vasoplegic shock after congenital heart surgery is a threatening condition leading to high morbidity and mortality. Control of hemodynamic and inflammatory response is fundamental in medical strategy. We report the case of a newborn with cardiogenic and vasoplegic shock secondary to cardiopulmonary bypass for atrioseptostomy and prostaglandin treatment in the context of hypoplastic left heart syndrome, successfully treated with a combination of mechanical circulatory support and cytokine hemoadsorption column (CytoSorb®). Vasopressor support was weaned during the time of treatment without multiple organ failure occurrence. The use of CytoSorb® cartridge inserted in an extra-corporeal circuit even in a newborn is easy and feasible, as long as some precautions are considered. Routine monitoring of drugs levels is mandatory during the use of the cartridge and immediately after.

Thirty Years of Kawasaki Disease: A Single-Center Study at the University Hospital of Lausanne.

Kawasaki disease is an acute vasculitis with a particular involvement of the coronary arteries. Coronary artery aneurysms develop in 20% of untreated children. It has been shown that early treatment with intravenous immunoglobulins and aspirin decreases this risk to 5%, but the medium to long term prognosis of children with Kawasaki disease is still unclear. To determine the outcome of the disease and risk factors for poor evolution, we reviewed retrospectively the medical records of all patients with a diagnosis of Kawasaki disease at our Institution between 1981 and 2014. Among the 207 patients included in the study, 96 patients had coronary diameter anomalies (46.4%) at diagnosis and children with atypical ages for Kawasaki disease (<1 year or >10 year of age) were more often affected with aneurysms or dilatations. Eighty-four of them had complete regression of coronary aneurysms during the follow-up (87.5%) Absence of immunoglobulins in the acute phase was associated with less regression rate (57.1 vs. 92.2%), and boys had greater z-scores at last echocardiography, statistically significant for the left anterior descending artery. We found rare complications after the acute phase documented in our patient charts (only 3.8%). Recurrence of the disease occurred in 5 children (2.4%) and myocardial ischemia in 3 patients (1.4%), all with initial coronary aneurysm. Conclusion: Medium to long term prognosis after Kawasaki disease is excellent. Boys, patients not treated with immunoglobulins or outside the usual age range are more at risk for an unfavorable outcome.

Leukemoid Reaction in Infant Pertussis: Is There a Place for Hydroxyurea? A Case Report.

A 73-days old infant of 34 weeks' gestation was hospitalized with a co-infection of respiratory syncytial virus (RSV) and Bordetella pertussis (BP). She required invasive ventilation for 9 days in the context of malignant pertussis with persistent hypoxemia and hypercapnia secondary to a leukemoid reaction. Despite an increase of white blood cell (WBC) count up to 70 G/L and ensuing pulmonary hypertension, no hemodynamic compromise occurred. Without clear indication for leukapheresis nor exchange transfusion, an off-label treatment with hydroxyurea was given for 5 days with gradual decrease of WBC count, without any complication and hospital discharge on day 29. To our knowledge, no effective therapy for malignant pertussis has been described in the literature and complications are frequent with leukoreduction procedures. We discuss an alternative to invasive procedures in young infants to fulfill the need to decrease rapidly leukocyte counts in a leukemoid reaction associated with Bordetella pertussis infection. To our knowledge, hydroxyurea has never been used in malignant pertussis but is a well-known medication for oncologic and hematologic diseases such as acute myeloid leukemia or sickle cell anemia. Its effects in this setting are not well understood but the positive outcome in our patient supports the need for further studies.

Risk factors and determinants of carotid intima-media thickness in children: protocol for a systematic review and meta-analysis.

INTRODUCTION: Carotid intima-media thickness (CIMT) is a surrogate marker of atherosclerosis that is measured in adults and children to better understand the natural history of cardiovascular disease (CVD). In adults, CIMT is predictive of myocardial infarction and stroke. In children and adolescents, CIMT is used to assess vascular changes in the presence of CVD risk factors (obesity, hypertension, smoking, etc) or clinical conditions associated with a high risk for premature CVD. However, there is no comprehensive overview, in a life-course epidemiology perspective, of the risk factors and determinants of CIMT in children. It is also important to evaluate between-study differences in CIMT measurement methods and take them into consideration when drawing conclusions. Our objective is to systematically review the evidence on the relationship between CIMT and prenatal and postnatal exposures or interventions in children, as well as documenting and discussing the CIMT measurement methods. METHODS AND ANALYSIS: Systematic searches of the Medical Literature Analysis and Retrieval System Online (MEDLINE), Excerpta Medica (EMBASE)and Central Register of Controlled Trials (CENTRAL) databases will be conducted. The reference lists and other literatures sources will be browsed. Observational and experimental studies in children from birth up to 18 years will be included. Prenatal and postnatal exposures or interventions assessed in relationship with CIMT will be considered for inclusion. Examples might include gestational age, obesity, hypertension, tobacco exposure, specific at-risk conditions (chronic kidney disease, diabetes, etc) or statin treatment. The outcome will be CIMT assessed by ultrasonography. The setting, scanning and measurement methods for each included study will be described in detail. Results will be synthesised descriptively and, if appropriate, will be pooled across studies to perform meta-analyses. Separate meta-analyses for each exposure or intervention type will be conducted. ETHICS AND DISSEMINATION: This systematic review will be published in a peer-reviewed journal. A report will be prepared for clinicians and other healthcare decision-makers. PROSPERO REGISTRATION NUMBER: CRD42017075169.

Anesthetic Management for Concomitant Correction of Congenital Cardiac Defects and Long "O" Ring Tracheal Stenosis: A Role for Heliox?: Report of 2 Cases.

We present 2 infants with the rare association of long congenital tracheal stenosis, ventricular septal defect, and pulmonary hypertension. We describe a step-by-step assessment of the patients and the necessary procedures for a successful concomitant repair of both cardiac and tracheal malformations. The use of a helium-oxygen mixture (heliox) for the induction of anesthesia and pre-cardiopulmonary bypass is discussed.