Williams-Beuren syndrome (WBS) affects young infants and children. The underlying etiopathogenesis of this rare disease is due to the mutation of the elastin gene that is responsible for the elasticity of the arterial wall. As a result of inadequate elastin production, the major systemic arteries become abnormally rigid and can be manifested by an impediment to the blood flow. The most common cardiovascular abnormalities encountered in WBS are supravalvular aortic stenosis, pulmonary arterial stenosis, and mitral valve prolapse. Less frequently observed cardiovascular abnormalities include coarctation of the aorta, ventricular septal defect, patent ductus, subaortic stenosis, and hypertrophic cardiomyopathy. Coronary artery stenosis and severe impediment to the bi-ventricular outflow as a result of supravalvular aortic and pulmonary artery stenosis predispose patients to sudden death. Patients with progressed arterial stenosis and severe stenosis are likely to require intervention to prevent serious complications. Rarely, imaging findings may precede clinical presentation, which allows the radiologist to participate in the patient care. However, to be more prudent, the radiologist must be accustomed to the imaging characteristics of WBS as well as the patient's clinical information, which could raise the suspicion of WBS. We performed a retrospective analysis of all the available images from patients diagnosed with WBS in last 4 years at our institution, and present key imaging findings along with a review of the literature to summarize the clinically relevant features as demonstrated by multidetector computed tomography in WBS. Cross-sectional imaging plays a vital role in the diagnosis of WBS cases with equivocal clinical features. MDCT evaluation of complex cardiovascular abnormalities of WBS including coronary artery disease is feasible with modern MDCT scanners and in the future, this approach could provide accurate information for planning, navigation, and noninvasive assessment of the secondary arterial changes in WBS and thus reducing the dependence upon invasive contrast catherization techniques.
BackgroundCentral line insertion is a routine procedure in medical practice. Dislodgement of lines into the vascular system is a rare complication. We noticed that paediatric health care providers (PHCP) contact the cardiac or general paediatric surgeon for extraction of dislodged lines more frequently than using the less invasive percutaneous approach.AimTo study the referral preference of PHCP for patient with embolised intravascular foreign bodies.MethodsA questionnaire with three questions was distributed to PHCP of all paediatric subspecialties, including surgery, in two tertiary care centres. The questions were about the total number of patients seen with central line, experience with complications, and preferred specialty for removal of dislodged central lines.ResultsThe questionnaire was distributed to 128 professionals. The response rate was 79% (n=101). Incomplete answers (n=14) were excluded. The grades of responders were senior consultants 18%, junior consultants 38%, and residents 43%. Thirty nine percent of care providers experienced dislodgement or fragmentation of central lines. The majority (82%) prefer to refer the patients for surgical removal.ConclusionsMost PHCP in the selected hospitals prefer to refer patients with embolised foreign bodies in the vascular system for surgical removal. The local health policy should be updated for the use of the alternative percutaneous approach.
Background: Percutaneous transcatheter closure (PTCC) of atrial septal defect (ASD) may convert to a long procedure. We aimed to identify predictors of prolonged procedure. Methods: Under transesophageal echocardiography and fluoroscopy guidance, 81 children with ASD underwent PTCC. Retrospectively, medical charts, echocardiographic recordings, catheterization reports and fluoroscopic films were reviewed. Demographics, echocardiographic measurements of ASD, dimensions of the device and hemodynamic data were collected. Prolonged procedure was defined as the duration from device deployment out of the delivery sheath to its release exceeding 10 minutes. A statistical model was designed using stepwise logistic regression analysis. Receiver operating characteristic curves were plotted to find the best cutoff for significant predictors. Results: The procedure was prolonged in 25 patients. By monovariate analysis, the significant predictors for prolonged procedure were smaller, and younger patients, larger ASD, smaller left atrial (LA) dimensions and device waist ratios to weight, patient's length, and LA dimensions. By multivariate analysis, the significant predictors were deficient septal rim toward superior vena cava (SVC) and device waist diameter in relation to patient's length (best cutoff: < 12 mm and > 0.13, respectively). In three cases (3.7%) the device embolized; retrospectively possibly the cause is small used device
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