Fulminant Infective Endocarditis Due to and Several Complications in a 6-Year-Old Girl: A Case Report.

is a gram-negative coccobacillus belonging to the HACEK group ( species, , and species) and is a common oropharyngeal colonizer of healthy young children. Osteoarticular infection is the most commonly reported invasive infection in children, usually presenting a mild clinical picture. However, it can also cause severe invasive infections, especially infective endocarditis, with a high complication rate.

A 13-Year-Old Male With Diagnosed Idiopathic Pulmonary Hypertension: Is it Really Idiopathic?

A 13-year-old male was referred after incidental finding of cardiomegaly on chest radiograph and signs of pulmonary hypertension on subsequent cardiology consult. He was diagnosed with idiopathic pulmonary hypertension, and came to our center for a second opinion. He was born from consanguineous parents.

Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients.

Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension.

Taurine treatment of retinal degeneration and cardiomyopathy in a consanguineous family with SLC6A6 taurine transporter deficiency.

In a consanguineous Pakistani family with two affected individuals, a homozygous variant Gly399Val in the eighth transmembrane domain of the taurine transporter SLC6A6 was identified resulting in a hypomorph transporting capacity of ~15% compared with normal. Three-dimensional modeling of this variant has indicated that it likely causes displacement of the Tyr138 (TM3) side chain, important for transport of taurine. The affected individuals presented with rapidly progressive childhood retinal degeneration, cardiomyopathy and almost undetectable plasma taurine levels.

Effectiveness of individual and group programmes to treat obesity and reduce cardiovascular disease risk factors in pre-pubertal children.

Childhood obesity results in premature atherosclerosis and requires early intervention. Compare the effectiveness of 6-month lifestyle interventions (with choice of either individual or group therapy) with standard care on body mass index (BMI) z-score and cardiovascular disease (CVD) risks factors in children with obesity. This 6-month randomized controlled trial with a 6-month follow-up included 74 pre-pubertal children with obesity (7.

Vasoreactive Pulmonary Arterial Hypertension Manifesting With Misleading Epileptic Seizure: Diagnostic and Treatment Pitfalls.

A 5-year-old girl presented with acute nocturnal episodes of loss of consciousness following abdominal pain and crying. Epilepsy was primarily diagnosed but the course of the disease was suggestive of pulmonary hypertension. An adapted invasive assessment of pulmonary pressure and pharmacological challenge allowed for diagnosing vasoreactive pulmonary arterial hypertension.

Serum cardiovascular risk biomarkers in pre-pubertal obese children.

Background: Childhood obesity is associated with premature cardiovascular complications. However, little is known about the effect of a family-based behavioural intervention on the relationship between arterial function, blood pressure and biomarkers in pre-pubertal children with obesity.

Design: This was a single centre randomized controlled trial (RCT) including 74 children randomized to a 6-month behavioural intervention to treat obesity.

Two-stage arterial switch for late-presenting transposition of the great arteries.

Objectives: Ventricular retraining and arterial switch have been described in late-presenting transposition of the great arteries (TGA) in older infants who were unable to undergo neonatal arterial switch operation (ASO) and late survivors of atrial switch with systemic right ventricular dysfunction. There are little data available on patients presenting between these 2 groups. This study aims to review the early and mid-term outcomes of the management of late-presenting TGA with an unprepared left ventricle (LV) by a 2-stage arterial switch.

Successful Lung Transplant After Prolonged Extracorporeal Membrane Oxygenation (ECMO) in a Child With Pulmonary Hypertension: A Case Report.

Introduction: The use of extracorporeal membrane oxygenation (ECMO) is considered a risk factor for, or even a potential contraindication to, lung transplantation. However, only a few pediatric cases have been described thus far.

Case Presentation: A 9-year-old boy with idiopathic pulmonary arterial hypertension developed cardiac arrest after the insertion of a central catheter.

Airway compression management in late-presenting absent pulmonary valve syndrome.

Introduction: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression.

Methods: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution.

Elevated E-selectin and diastolic blood pressure in diabetic children.

Background: Cardiovascular risk markers are related to micro-angiopathy in children with type 1 diabetes (T1DM), but there is no information about their relationship with blood pressure (BP) and endothelial function.

Materials And Methods: This was a case-control study including 29 children with T1DM (mean age 10·5 ± 2·7 years, disease duration: 3·8 ± 2·2 years) and 39 healthy controls (mean age: 9·8 ± 2·7 years). We assessed 24-h ambulatory BP, vascular function and serum level of lipids, vascular cell adhesion molecule-1 (VCAM-1; ICAM) and selectins (E-selectin; P-selectin).

Long-term follow-up of cardiovascular risk factors after exercise training in obese children.

Objective: We previously demonstrated beneficial effects of physical activity on cardiovascular disease (CVD) risk factors, body mass index (BMI) and fat mass in pre-pubescent obese children. The aim of this study was to determine whether these changes were maintained 2 years later.

Methods: Two years after the Randomised Controlled Trial, we performed a follow-up study with 20 of 38 subjects (11.

Leaflet suspension to the contralateral annulus to address restriction or tethering-induced mitral and tricuspid regurgitation in children: results of a case-control study.

Objectives: Acceptable coaptation cannot always be obtained using standard repair techniques. We assessed the safety and mid-term results using a novel technique to address leaflet retraction or tethering in children with type III mitral or tricuspid regurgitation as an addition to standard valve repair techniques.

Methods: Forty children were included, 36 for the mitral valve and 4 for the tricuspid valve, with a mean age of 11.

Preclinical noninvasive markers of atherosclerosis in children and adolescents with type 1 diabetes are influenced by physical activity.

Objectives: To measure preclinical noninvasive markers of atherosclerosis in youth with type 1 diabetes (T1DM), and to determine their associations between physical activity level and cardiorespiratory fitness (maximal oxygen consumption [VO2max]).

Study Design: This was a cross-sectional study including 32 patients with T1DM and 42 healthy subjects aged 6 to 17 years. Main outcome measures included arterial flow-mediated dilation (FMD) and intima-media thickness with high-resolution ultrasonography; physical activity by accelerometer (valid 26 patients with T1DM, 35 healthy subjects) and VO2max.

Aortic valve repair by cusp extension for rheumatic aortic insufficiency in children: Long-term results and impact of extension material.

Objective: Aortic valve repair has encouraging midterm results in selected patients. However, neither the long-term results of cusp extension nor the durability of different pericardial fixation techniques has been reported. Our goal was to address these issues.

No-patch technique for complete atrioventricular canal repair.

Although no-patch repair was the first surgical treatment for complete atrioventricular canal, patch repairs are currently more widely used. We assessed the safety of forgoing a patch during the correction of complete atrioventricular canal in 8 consecutive patients. The complete atrioventricular canal was repaired using sutures placed on the right of the ventricular septal defect crest, passed through the bridging leaflet, and to the facing part of the ostium primum defect.

Arterial alterations in severely obese children with obstructive sleep apnoea.

Background: Obstructive sleep apnoea (OSA) in obese adults is associated with cardiovascular disease independently of obesity. Vascular alterations exist in children with obesity and may constitute the first stage in the development of adulthood cardiovascular disease.

Objective: To investigate the relationship between OSA and early arterial alterations in obese children.

Influence of polymorphisms in candidate genes on early vascular alterations in obese children.

Background: Many studies performed in adults have reported the involvement of genetic determinants in vascular alterations that predispose to cardiovascular diseases later in life. To date, no study has assessed the co-involvement of gene polymorphisms as cardiovascular risk factors in children.

Aims: To search for variants involved in early vascular alterations in obese children.

Physical activity reduces systemic blood pressure and improves early markers of atherosclerosis in pre-pubertal obese children.

Objectives: The aim of this study was to determine the effects of physical activity on systemic blood pressure (BP) and early markers of atherosclerosis in pre-pubertal obese children.

Background: Hypertension and endothelial dysfunction are premature complications of obesity.

Methods: We performed a 3-month randomized controlled trial with a modified crossover design: 44 pre-pubertal obese children (age 8.

Rescue circumflex artery stenting for major right ventricular dysfunction after Ross-Konno operation.

A single coronary artery is an anomaly in which only 1 coronary artery arises from the aortic root, supplying the entire heart. We report the case of a 5-year-old girl in whom a single coronary artery ostium was discovered during a Ross-Konno operation. This patient required extracorporeal membrane oxygenation for postoperative right ventricular infarction due to proximal stenosis of a dominant left circumflex artery in combination with an occluded minor right coronary artery.

Transpulmonary stenting of both pulmonary arteries with a surgical access through redo sternotomy.

We report the case of a four-year-old boy with pulmonary atresia (PA) and ventricular septal defect (VSD) associated with MAPCAs who had a complete repair two times. He developed 21 months later stenosis at the origin of both pulmonary arteries. The conventional interventional catheterization approaches were not available due to femoral vein thrombosis and severe transient complete atrioventricular block due to the manipulations to catheterize the left pulmonary artery.

Mitral valve repair for rheumatic valve disease in children: midterm results and impact of the use of a biodegradable mitral ring.

Background: Mitral valve repair for rheumatic mitral valve disease in children has become the preferred surgical modality. A mitral valve ring is frequently used in the repair. A recently introduced biodegradable ring has shown promising results and allows for growth of the native annulus.

Association of arterial stiffness and endothelial dysfunction with metabolic syndrome in obese children.

Objective: We investigated whether metabolic syndrome, defined in 3 different ways (2 commonly used and 1 novel) is associated with arterial alterations in obese children.

Study Design: The study group comprised 384 obese children age 2.5 to 18 years.