A child of Williams-Beuren syndrome for inguinal hernia repair: Perioperative management concerns
Williams-Beuren syndrome, commonly known as Williams syndrome (WS), is a multi-organ disorder. The principal anomalies of the syndrome are developmental delay, unusual craniofacial dysmorphic features, and cardiovascular anomalies such as valvular or ...
Sangeeta Deka+4 more
doaj +1 more source
Echocardiographic evaluation of velocity ratio, velocity time integral ratio, and pulmonary valve area in dogs with pulmonary valve stenosis. [PDF]
BackgroundVelocity ratio, velocity time integral (VTI) ratio, and pulmonary valve area indexed to body surface area (iPVA) are methods of assessment of pulmonary valve stenosis (PS) severity that are less dependent on blood flow. Studies evaluating these
Bélanger, Catherine+5 more
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Mitral supravalvular ring: a case report [PDF]
Supravalvular mitral stenosis is a rare condition characterized by an abnormal ridge, with one or two orifices, covering and obstructing the mitral valve.
BJ Maron+17 more
core +3 more sources
Oral manifestations of nine individuals with Williams syndrome. A case series
Abstract Aims Williams syndrome (WS) is a congenital developmental disorder characterized, mainly, by distinctive facial features, cardiovascular anomalies, growth delay and a typical neurobehavioral profile. The oral manifestations have not been sufficiently described and, therefore, the aim of the current study was to present the clinical ...
Konstantina Vavetsi+6 more
wiley +1 more source
JAGGED1/NOTCH3 activation promotes aortic hypermuscularization and stenosis in elastin deficiency
Obstructive arterial diseases, including supravalvular aortic stenosis (SVAS), atherosclerosis, and restenosis, share 2 important features: an abnormal or disrupted elastic lamellae structure and excessive smooth muscle cells (SMCs).
Jui M. Dave+15 more
doaj +1 more source
PATHOPHYSIOLOGY, INVESTIGATIONS AND TREATMENT OF PATIENTS WITH PULMONIC STENOSIS: A REVIEW ARTICLE [PDF]
Background: Most patients with pulmonic stenosis have benign disease. While problems and new-onset symptoms in adults are usually non-life-threatening following the newborn period, they might occur in adults and necessitate attention.
ABD AL-RAZIK, ASHRAF HAMADA+5 more
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Williams-Beuren syndrome: a rare presentation of aortic hypoplasia [PDF]
William-Beuren syndrome (WBS) is a rare genetic disorder resulting from a micro-deletion of the elastin gene located on the long arm of chromosome 7 (7q11.23).
Karim, N, Quadros, D.S., Sattar, F.A.A.
core +2 more sources
Updated EUROCAT guidelines for classification of cases with congenital anomalies
Abstract Background Precise and correct classification of congenital anomalies is important in epidemiological studies, not only to classify according to etiology but also to group similar congenital anomalies together, to create homogeneous subgroups for surveillance and research.
Jorieke E. H. Bergman+7 more
wiley +1 more source
A new technological system to revolutionize the management of valvular heart diseases: transcatheter aortic valve for the treatment of aortic stenosis [PDF]
The goal of this study is a technology analysis of a revolution for the management of valvular heart diseases given by new technological system based on transcatheter artificial aortic valve, a delivery catheter and a loading system for the treatment of aortic stenosis (a narrowing of the aortic valve because of calcium build up that restricts blood ...
arxiv
Врожденный надклапанный стеноз аорты: результаты хирургического лечения [PDF]
Цель. Надклапанный стеноз аорты (НСА) – это вариант комплексной аномалии корня аорты. С целью восстановления геометрии, а также сохранения функции аортального клапана были предложены техники симметричной пластики двух- и трех синусов аорты.
Кептанару, Эдуард+6 more
core +1 more source