Diagnosis and Therapeutic Cardiac Catheterization of Symptomatic Bicuspid Aortic Stenosis in the Pediatric Population.

Bicuspid aortic valve (BAV) is the most common congenital heart disease with a prevalence of 0.5%-1.3% of the population. Many children with BAV are asymptomatic. Clinically relevant abnormal valve function usually occurs in adulthood. However, in rare cases, children can fail to thrive which requires valvular intervention. In this review, we will explore in more detail the anatomy of the BAV, clinical presentation of BAV, diagnosis of BAV, and its function by echocardiography, and indications for transcatheter intervention in the pediatric population.

Clinical presentations, Laboratory analysis and Linear Growth in 50 Neonates and Young Infants with Acute Meningitis: One Year Experience of a Single Center in Qatar.

BACKGROUND: Meningitis frequently occurs in neonates and can lead to a number of acute, severe complications and long-term disabilities. Although, long term growth delay and abnormal weight gain appear to be risk factors following an acute attack of both bacterial and aseptic meningitis in children, especially during the fast phase of infantile growth, the long-term effects of acute meningitis occurring during the neonatal and early infantile periods on linear growth (length, weight and head growth) have not fully reported. AIM OF THE STUDY: The objective of this study is to describe the clinical presentation of neonates and young infants with acute meningitis with different etiologies and to determine the clinical impact of the effect of acute meningitis on growth parameters. MATERIAL AND METHODS: We analyzed the clinical data and the growth parameters of 50 newborns and young infants (age: 1.6 ± 0.9 months) admitted to our hospital (Al Wakhra Hospital, Department of Pediatrics, Doha, Qatar), between 1-1-2016 to 1-1-2017, with acute meningitis. Anthropometric measurements included weight, length, and head circumference. Length SDS (L-SDS) and body-mass-index (BMI) were calculated and recorded at every clinic visit, every 3 months for 8 ± 2 months. RESULTS: In this age group of neonates and young infants with acute meningitis fever (84%) and hypoactivity (64%) were the major presenting manifestations. Acute bacterial meningitis (n: 10) was associated with higher morbidity [shock (n: 1), subdural empyema (n: 1) and hydrocephalus (n: 1)]. Cerebrospinal fluid (CSF) examinations showed that infants with bacterial meningitis had significantly higher pleiocytosis of mainly polymorphic leukocytes and protein levels, compared to those with aseptic meningitis.All infants showed normal linear growth and weight gain during the follow-up period (8 ± 2 months). The annualized growth rate of infants was 25.3 ± 3.5 cm per year. All had normal length standard deviation scores (LSDS) (-0.2 ± 0.9) and none of them had LSDS < -2. All infants had a normal BMI (16.7 ± 1.8 kg/m2). Head circumference growth was normal in 49/50 infants (43.8 ± 1.8 cm) at 8 ± 2 months. One infant developed hydrocephalus after group B streptococcus (GBS) meningitis. There was no statistical difference in linear growth between infants with aseptic and bacterial meningitis. CONCLUSION: Acute bacterial meningitis in newborns and young infants is still associated with considerably high morbidity and complications. Infantile linear growth appears to be normal in all newborns and young infants with both bacterial and aseptic meningitis.

Noninvasive evaluation of single-ventricle patients before Fontan operation.

OBJECTIVE: To determine whether patients with single-ventricle physiology who are free from known risk factors could skip routine pre-Fontan hemodynamic evaluation, without affecting the postoperative outcome. METHODS: A diagnostic algorithm including echocardiographic, clinical, and angiographic risk factors was used retrospectively to evaluate all 44 patients who underwent Fontan completion at Hamad General Hospital between January 2000 and December 2012. We evaluated the efficacy of this algorithm as a screening tool to identify inoperable and high-risk patients in whom pre-Fontan cardiac catheterization should be performed. RESULTS: Mean age at Fontan completion was 5.9 years (range 1.08-24.5 years), and mean weight was 18 kg (range 7.4-60 kg). Three patients were found to be inoperable based on hemodynamic cardiac catheterization results. The algorithm was highly sensitive in detecting all 3 inoperable patients who were classified as high-risk subjects. Of the other 41 patients, 17 (41%) had no risk factors and 24 (59%) had one or more risk factors. This noninvasive algorithm showed a sensitivity of 83% and specificity of 76% in detecting adverse postoperative outcomes. The sensitivity did not change after adding hemodynamic data obtained by cardiac catheterization as additional risk factors. CONCLUSION: This noninvasive diagnostic algorithm could be used as an effective screening tool to detect patients in whom pre-Fontan cardiac catheterization could be avoided; magnetic resonance imaging and computed tomography can be a good substitute.

Linear growth in relation to the circulating concentration of insulin-like growth factor-I in young children with acyanotic congenital heart disease with left to right shunts before versus after surgical intervention.

OBJECTIVES: The aim was to determine the degree of linear growth retardation of patients with congenital acyanotic heart disease at presentation and the magnitude of catch-up growth, if any, in relation to their echocardiographic changes and insulin-like growth factor- I (IGF-I) concentration before versus after surgical intervention. MATERIALS AND METHODS: This prospective study recorded the anthropometric data and measured the circulating IGF-I, free thyroxin (FT4), and thyrotropin (TSH) of 27 infants and children with congenital acyanotic heart disease with left to right shunt (10 with VSD, 8 with ASD, 9 PDA) without heart failure, or severe pulmonary hypertension, before and 12 months or more after surgical or catheter intervention. Eighty normal age and sex- matched normal siblings of these patients were included as controls for the auxologic data. RESULTS: At presentation, patients' mean age = 35.6 ± 26 months, height SDS (HtSDS) = -1.6 ± 1.1, and body mass index (BMI) = 15.1 ± 2.5. They were significantly shorter and with lower BMI compared to normal controls (HtSDS = 0.25 ± 0.3, BMI = 16.4 ± 1.5). One year or more after catheter or surgical treatment, the HtSDS and BMI increased significantly in patients to -0.55 ± 0.9 and 15.9 ± 1.5, respectively). IGF-I levels increased from 46.8 ± 29 mcg/L before to 77.3 ± 47.6 mcg/L after intervention. No significant change has been detected in circulating FT4 or TSH concentrations. The HtSDS after treatment was correlated with the IGF-I concentration (r = 0.804, P < 0.001). The change in the HtSDS after intervention was correlated significantly with BMI (r = 0.594, P 0.001) and negatively with age (r = -0.52, P< 0.01). The shunt size was correlated negatively with BMI and HtSDS before intervention (r = -0.35, P < 0.01 and 0.01 and r = -0.461, P < 0.05, respectively). GVSDS after intervention surgery was correlated with BMI after intervention (r = 0.495, P < 0.001) and negatively with the age at operation (r = -0.683, P < 0.001). CONCLUSIONS: In congenital acyanotic heart diseases, early surgical interference and weight gain have beneficial effect on postoperative growth spurt. This catch-up growth appears to be mediated through activation of the GH/IGF-I system and suggests an important role of increasing BMI (an indicator of nutrition) as an imperative factor.