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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).

Objective An important aspect of perioperative care in pediatric cardiac surgery is maintenance of optimal hemodynamic status using vasoactive/inotropic agents. Conventionally, this has focused on maintenance of cardiac output rather than perfusion pressure. However, this approach has been abandoned in our center in favor of one focusing primarily on perfusion pressure, which is presented here and compared to the conventional approach. Design A retrospective study. Setting Regional center for congenital heart disease. University Hospital of Lausanne, Switzerland. Patients All patients with Aristotle risk score ≥8 that underwent surgery from 1996 to 2012 were included. Patients operated between 1996 and 2005 (Group 1: 206 patients) were treated according to the conventional approach. Patients operated between 2006 and 2012 (Group 2: 217 patients) were treated according to our new approach. Interventions All patients had undergone surgery for correction or palliation of congenital cardiac defects. Outcome measurements Mortality, duration of ventilation and inotropic treatment, use of ECMO, and complications of poor peripheral perfusion (need for hemofiltration, laparotomy for enterocolitis, amputation). Results The two groups were similar in age and complexity. Mortality was lower in group 2 (7.3% in group 1 vs 1.4% in group 2, P

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.

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75 years (IQR 0.65-6.63). Forty-seven children had persistent LSVC (12.7 %), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83 %). In three patients (6.4 %) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17 %), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4 %) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0 days, p = 0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6 %, p = 0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery.

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 (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.

Introduction Gestational diabetes mellitus (GDM) is a state of glucose intolerance with onset during pregnancy. GDM carries prenatal and perinatal risks as well as long-term risks for the mother and her child. GDM may be involved in the foetal programming of long-term cardiovascular health. However, evidence is sparse and the effect of GDM on cardiovascular health is unknown. To address these issues, we will conduct MySweetHeart Cohort study. The objectives are to assess the effect of GDM on offspring’s cardiovascular health early in life by using surrogate markers of cardiovascular disease and atherosclerosis. Methods and analysis This is a cohort study of 100 offspring of women with GDM and 100 offspring of women without GDM. At inclusion, a baseline assessment of the mothers will be conducted through means of self-report questionnaires, a researcher-administrated interview, blood pressure and anthropometric measurements, and a maternal blood sampling. Between the 30th and 34th weeks of gestation, a foetal echography will be performed to assess the foetal cardiac structure and function, the fetomaternal circulation and the hepatic volume. At birth, maternal and neonatal characteristics will be assessed. An echocardiography will be performed to assess cardiac structure and function 2–7 days after birth; carotid intima-media thickness will be also measured to assess vascular structure. MySweetHeart Cohort is linked to MySweetHeart Trial (clinicaltrials.gov/ct2/show/NCT02890693), a randomised controlled trial assessing the effect of a multidimensional interdisciplinary lifestyle and psychosocial intervention to improve the cardiometabolic and mental health of women with GDM and their offspring. A long-term follow-up of children is planned. Ethics and dissemination Ethical approval has been obtained through the state Human Research Ethics Committee of the Canton de Vaud (study number 2016–00745). We aim to disseminate the findings through regional, national and international conferences and through peer-reviewed journals. Trial registration number ClinicalTrials.gov (clinicaltrials.gov/ct2/show/NCT02872974).

Pulmonary artery sling is a rare congenital condition in which the left pulmonary artery (LPA) arises from the right pulmonary artery, and then passes between the trachea and the esophagus to reach the left lung, thereby forming a sling around the airway. It is often associated with intrinsic tracheal stenosis due to complete cartilaginous rings. Therapeutic management nowadays consists of one-stage reimplantation of the LPA and tracheoplasty with cardiopulmonary bypass support. Here we present a 7-weeks-old boy with pulmonary artery sling and long-segment distal tracheal stenosis who underwent surgical intervention consisting of reimplantation of the LPA and slide tracheoplasty. Multiple respiratory and cardiovascular complications marked the postoperative course. They consisted of recurrent failed attempts in weaning off mechanical ventilation due to bronchomalacia, left vocal cord paralysis, development of granulation tissue at the anastomosis and restenosis of the trachea and the main stem bronchi requiring balloon dilatation. The patient also developed bilateral pulmonary artery thrombosis and stenosis of the LPA. After a prolonged hospitalization, the patient is doing well without any respiratory symptoms and has a good result on follow-up bronchoscopy 1 year after the initial surgery. The stenosis of the LPA responded well to percutaneous balloon dilatation 12 months after the primary surgery. The case illustrates that even though surgical techniques are improving and are in general associated with a low morbidity and mortality, management of pulmonary artery sling and tracheal stenosis can still be challenging. However, good long-term outcome can be achieved if the initial postoperative phase is overcome.

Cardiac arrhythmias are very frequent in fetuses and newborns. The prognosis depends on the nature of the arrhythmias but is most often either spontaneously benign or following short-term medication administration. A correct diagnosis is essential for both management and prognosis. It is based on echocardiography during the fetal period and mainly on history, physical exam, and electrocardiogram after birth, but other modalities are available to record transient arrhythmic events. Irregular rhythms are mostly benign and rarely require therapy. In most fetuses and infants, tachyarrhythmias resolve spontaneously or require short-term administration of antiarrhythmics. Approximately one third of these may recur later on, especially during adolescence. Persistent bradyarrhythmias might require pacemaker implantation when associated with failure to thrive or with risk of sudden death. CONCLUSION: Arrhythmias in fetuses and infants are very common and mostly benign. History, physical exam, and recording of the arrhythmia are essential to make a correct diagnosis and establish an appropriate management for the rare potentially harmful arrhythmias.

OBJECTIVES: Coarctation of the aorta is one of the most common congenital heart defects. Its diagnosis may be difficult in the presence of a patent ductus arteriosus, of other complex defects or of a poor echocardiographic window. We sought to demonstrate that the carotid-subclavian artery index (CSA index) and the isthmus-descending aorta ratio (I/D ratio), two recently described echocardiographic indexes, are effective in detection of isolated and complex aortic coarctations in children younger and older than 3 months of age. The CSA index is the ratio of the distal aortic arch diameter to the distance between the left carotid artery and the left subclavian artery. It is highly suggestive of a coarctation when it is

Perinatal adverse events such as limitation of nutrients or oxygen supply are associated with the occurrence of diseases in adulthood, like cardiovascular diseases and diabetes. We investigated the long-term effects of perinatal hypoxia on the lung circulation, with particular attention to the nitric oxide (NO)/cGMP pathway. Mice were placed under hypoxia in utero 5 days before delivery and for 5 days after birth. Pups were then bred in normoxia until adulthood. Adults born in hypoxia displayed an altered regulation of pulmonary vascular tone with higher right ventricular pressure in normoxia and increased sensitivity to acute hypoxia compared with controls. Perinatal hypoxia dramatically decreased endothelium-dependent relaxation induced by ACh in adult pulmonary arteries (PAs) but did not influence NO-mediated endothelium-independent relaxation. The M3muscarinic receptor was implicated in the relaxing action of ACh and M1muscarinic receptor (M1AChR) in its vasoconstrictive effects. Pirenzepine or telenzepine, two preferential inhibitors of M1AChR, abolished the adverse effects of perinatal hypoxia on ACh-induced relaxation. M1AChR mRNA expression was increased in lungs and PAs of mice born in hypoxia. The phosphodiesterase 1 (PDE1) inhibitor vinpocetine also reversed the decrease in ACh-induced relaxation following perinatal hypoxia, suggesting that M1AChR-mediated alteration of ACh-induced relaxation is due to the activation of calcium-dependent PDE1. Therefore, perinatal hypoxia leads to an altered pulmonary circulation in adulthood with vascular dysfunction characterized by impaired endothelium-dependent relaxation and M1AChR plays a predominant role. This raises the possibility that muscarinic receptors could be key determinants in pulmonary vascular diseases in relation to “perinatal imprinting.”

Background For free-breathing cardiovascular magnetic resonance (CMR), the self-navigation technique recently emerged, which is expected to deliver high-quality data with a high success rate. The purpose of this study was to test the hypothesis that self-navigated 3D-CMR enables the reliable assessment of cardiovascular anatomy in patients with congenital heart disease (CHD) and to define factors that affect image quality. Methods CHD patients ≥2 years-old and referred for CMR for initial assessment or for a follow-up study were included to undergo a free-breathing self-navigated 3D CMR at 1.5T. Performance criteria were: correct description of cardiac segmental anatomy, overall image quality, coronary artery visibility, and reproducibility of great vessels diameter measurements. Factors associated with insufficient image quality were identified using multivariate logistic regression. Results Self-navigated CMR was performed in 105 patients (55 % male, 23 ± 12y). Correct segmental description was achieved in 93 % and 96 % for observer 1 and 2, respectively. Diagnostic quality was obtained in 90 % of examinations, and it increased to 94 % if contrast-enhanced. Left anterior descending, circumflex, and right coronary arteries were visualized in 93 %, 87 % and 98 %, respectively. Younger age, higher heart rate, lower ejection fraction, and lack of contrast medium were independently associated with reduced image quality. However, a similar rate of diagnostic image quality was obtained in children and adults. Conclusion In patients with CHD, self-navigated free-breathing CMR provides high-resolution 3D visualization of the heart and great vessels with excellent robustness.

A 2-year-old boy was admitted for a 16-day history of intermittent fever (up to 40.5°C) with no other symptom. He had been followed since birth for a large midmuscular ventricular septal defect with a left-to-right shunt and signs of congestive heart failure. At 2 months of age, the patient had a pulmonary artery banding, and at 16 months of age he underwent hybrid periventricular closure of a muscular ventricular septal defect by Amplatzer occluder and removal of the pulmonary band. A 12-mm Amplatzer muscular ventricular septal defect (VSD) occluder was implanted through a direct puncture of the right ventricular free wall. Postoperative echocardiography initially showed a 7-mm residual muscular VSD at the inferior border of the device with a moderate left-to-right shunt that progressively decreased to a 3- to 4-mm residual VSD over the next few months. During postoperative follow-up, the patient’s condition improved with decreasing heart failure signs. Treatment with diuretics and angiotensin-converting enzyme inhibitor, and aspirin as well, was continued. Over the next 8 months he received several courses of antibiotics for various infections. On admission, the patient was subfebrile (37.7°C). His heart rate was 144 bpm, blood pressure was 121/75 mm Hg. Chest examination revealed a holosystolic …

Background In neonates and young infants (less than 3 months), coarctation may be missed or underestimated by echocardiography, especially with a patent ductus arteriosus or severe concurrent illness. A reliable noninvasive screening tool for coarctation would be useful for these patients. Methods From 1997 to 2003, echocardiographic evaluation was performed in 63 consecutive patients with coarctation (47 neonates and 16 infants) as well as in 23 controls (16 neonates and 7 infants). End-systolic measurements were obtained from 12 different sites of the aortic arch. Results In patients, the diameters of the ascending and descending aorta were comparable to controls, but the dimensions of the transverse arch were significantly smaller. The distances between the origins of the great vessels were longer in patients with coarctation than in controls. The ratio of the aortic arch diameter at the left subclavian artery, to the distance between the left carotid artery and the left subclavian artery, which we propose as the carotid-subclavian artery index, was significantly smaller in patients with coarctation. A cut-off point at 1.5 showed a sensitivity of 97.7% and 94.7%, and a specificity of 92.3% and 100%, for neonates and young infants, respectively. The positive predictive value to have coarctation was 97.7% and 100%, for neonates and infants, respectively. Conclusions The carotid-subclavian artery index is a simply obtainable noninvasive screening parameter, showing high sensitivity and specificity for coarctation, and may be useful in unstable patients or in those with a patent ductus arteriosus in which coarctation may be overlooked.

CLINICAL SUMMARY A 78-year-old man with symptomatic aortic stenosis was evaluated for TAVI. Concomitant comorbidities were coronary artery sclerosis, peripheral vascular disease, bilateral carotid stenosis, and pulmonary hypertension (50 mm Hg). He also had alcohol-related liver cirrhosis (Child score A) complicated by grade 2 esophageal varices (treated with propranolol) and a platelet count chronically less than 80 3 10/dL. The echocardiogram showed severe aortic stenosis (orifice area, 0.55 cm) with transvalvular peak and mean gradients of 68 mm Hg and 38 mm Hg, respectively, moderate left ventricular hypertrophy, and preserved function (left ventricular ejection fraction, 60%). The logistic EuroSCORE was 18%. However, the EuroSCORE does not take into consideration the liver cirrhosis, which is an important risk factor negatively influencing the outcome of highrisk patients undergoing standard cardiac surgery. Therefore, we offered the patient a less-invasive procedure, and a transapical TAVI was scheduled. Computed tomography scan measurements showed the following: aortic annulus 1⁄4 24 mm diameter; distances between the annulus and the coronary ostia 1⁄4 10.5 mm (left) and 11 mm (right); and C-arm fluoroscopy pre-orientation 1⁄4 18 degrees left (lateral position) and 11 degrees cranial. Before the procedure, a gastroscopy (performed with care under general anesthesia) confirmed the presence of esophageal varices contraindicating TEE use.

Background In late-diagnosed transposition of the great arteries (TGA), the left ventricle (LV) involutes as it pumps against low resistance and needs retraining by applying a pulmonary artery band (PAB) in preparation for an arterial switch operation. We report our experience with a telemetrically adaptable band compared with classic banding. Methods Ten patients underwent retraining of the LV, 4 patients with an adaptable band and progressive weekly tightening of the band (group 1) and 6 patients with a traditional band (group 2). Results Mean weight and age at pulmonary band placement was 5.8 ± 2.36 kg and 11.7 ± 11.1 months for group 1 and 5.0 ± 2.3 kg and 6.4 ± 7.6 months for group 2. Time between palliation and switch procedure was 4.2 months in both groups. Group 1 showed an initial mean pulmonary gradient of 25.5 ± 4.43 mm Hg with a 5% closure of the device. The mean gradient increased with progressive closure to 63.5 ± 9.8 mm Hg at the time of the arterial switch operation. There were no reinterventions or deaths in this group. In group 2, the mean pulmonary gradient increased with growth from 49 ± 21.4 mm Hg to 68.4 ± 7.86 mm Hg at the time of the switch procedure. However, 4 of these patients required reoperations during retraining: 2 needed 1 reoperation and 2 needed 2 reoperations. Two patients died—1 after banding and 1 after the switch operation. Conclusions Retraining of the LV by the adaptable device allows precise control of the tightening, avoids repetitive operations, and diminishes morbidity.

Kawasaki disease is an acute vasculitis of childhood. Its clinical presentation is well known, and coronary artery aneurysms are classical complications. Shock and pleural or pericardiac effusion are rare presentations of the disease. In intensive care units, the disease may be mistaken for septic shock or toxic shock syndrome. Owing to the fact that immunoglobulin therapy improves the course of the disease, especially if given early, and thus the diagnosis should not be delayed.

Background Mycoplasma hominis is a fastidious micro-organism causing systemic infections in the neonate and genital infections in the adult. It can also be the cause of serious extra-genital infections, mainly in immunosuppressed or predisposed subjects. Case Presentation We describe a case of severe pneumonia and pericarditis due to Mycoplasma hominis in a previously healthy adolescent who did not respond to initial therapy. Conclusions Mycoplasma hominis could be an underestimated cause of severe pneumonia in immunocompetent patients and should be particularly suspected in those not responding to standard therapy.

Studies in adults have shown that late gadolinium enhanced cardiac magnetic resonance is a safe and noninvasive diagnostic tool which allows one to differentiate myocardial infarction from myocarditis. We believe that it may also be highly useful in the paediatric population for the same purpose.

Aims Diabetes in pregnant women is increasing and with that the complications in their offspring. We studied our population of diabetic mothers (2003–2005) for pathologic ventricular hypertrophy (PVH). Methods and results In our retrospective study of all 87 diabetic pregnancies (92 neonates), 16 were type 1, 17 were type 2, and 54 were gestational diabetes (GD). Haemoglobin glycated (HbA1c) median was 5.8% (5.3–6.5): 17 with HbA1c above normal 2 with congenital heart disease (CHD) and six with PVH. A total of 75 neonates were normal, five had CHD, and 12 had PVH (1/12 died post-natally, 1/12 stillborn, 2/12 required premature delivery, 8/12 normal). The 16 type 1 pregnancies resulted in three neonates with CHD and in 50% PVH, including one death, one premature Cesarean section because of PVH. The 17 neonates of type 2 pregnancies showed in one CHD and in 25% PVH. Of the 54 GD pregnancies, one had CHD and one had PVH. Conclusion Pregnancies of both type 1 and 2 diabetes carry an increased risk for foetal development of PVH compared with those with GD. The insufficient effect of preventive glycaemia controls leads to conclude that although no definite predictive parameters for malignant outcome can be presented, close monitoring of these pregnancies may prevent perinatal catastrophes.

We report on a youngster followed by his paediatrician from birth until 14 years of age for premature beats, most likely of ventricular origin. The sudden death of his sister provoked a re-assessment of his electrocardiograms (ECG), resulting in the diagnosis of Brugada syndrome and the subsequent implantation of a cardioverter defibrillator. This syndrome is a well known entity in adult cardiology, first described by Brugada and Brugada in 1992. It is considered to be the second most common cause of death in young adults after road traffic accidents. In children, however, the Brugada syndrome is not well known and we believe to be certainly underdiagnosed.