Results 21 to 30 of about 60,528 (234)

Supravalvular Aortic Stenosis [PDF]

Circulation: Cardiovascular Genetics, 2012
Supravalvular aortic stenosis is a systemic elastin (ELN) arteriopathy that disproportionately affects the supravalvular aorta. ELN arteriopathy may be present in a nonsyndromic condition or in syndromic conditions such as Williams–Beuren syndrome.
Giuseppe, Merla, Nicola, Brunetti-Pierri, Pasquale, Piccolo, Lucia, Micale, Maria Nicla, Loviglio   +4 more
openaire   +2 more sources

Long-term surgical outcomes of congenital supravalvular aortic stenosis: a systematic review, meta-analysis and microsimulation study

European Journal of Cardio-Thoracic Surgery, 2023
OBJECTIVES Congenital supravalvular aortic stenosis (SVAS) is a rare form of congenital outflow tract obstruction and long-term outcomes are scarcely reported.
F. Meccanici, M. Notenboom, Jade Meijssen, Vernon Smit, P. C. van de Woestijne, A. E. van den Bosch, Willem A. Helbing, Ad J.J.C. Bogers, J. Takkenberg, J. Roos-Hesselink   +9 more
semanticscholar   +1 more source

Isolated ostial left main coronary artery stenosis causing ischemic cardiomyopathy in a child with bicuspid aortic valve: Role of echocardiography in diagnosis and follow-up

Annals of Pediatric Cardiology, 2022
Bicuspid aortic valve (BAV) is the most common congenital cardiac defect, commonly associated with valve dysfunction and coarctation of aorta. Rarely, BAV can be associated with abnormalities of the coronary arteries, the most common of which are ectopic
Amad Chohan, Carol J Abraham, Kent Ward, Kumar Ponniah, Anas Salkini, Harold M Burkhart, Arshid Mir   +6 more
doaj   +1 more source

Clinical Results of a Modified Doty’s Technique for Supravalvular Aortic Stenosis

Journal of Clinical Medicine, 2023
This study aimed to assess the early and mid-term results of the modified Doty’s technique compared with the traditional Doty’s technique in patients with congenital supravalvular aortic stenosis (SVAS).
Lizhi Lv, X. Lang, Simeng Zhang, Cheng Wang, Yuanhao Jin, Qiang Wang   +5 more
semanticscholar   +1 more source

Williams-Beuren syndrome with pseudoaneurysm of aortic arch and infective vegetations for modified broms procedure: anesthetic concerns & Echocardiographic illustrations

Annals of Cardiac Anaesthesia, 2023
Williams-Beuren syndrome is a rare genetic malformation with predilection for supravalvular aortic stenosis. Apart from cardiovascular malformation, hypocalcemia, developmental delay, and elfin facies, challenging airway make perioperative management ...
Devishree Das, Neeti Makhija
doaj   +1 more source

Successful percutaneous balloon dilatation of supravalvular aortic membrane

Heart Views, 2019
Supravalvular aortic stenosis is the least common type of left ventricular outflow tract obstruction. Primary balloon dilatation of membranous supravalvular aortic stenosis was performed in a 10-year-old male child with a remarkable reduction in systolic
Gurkirat Singh, Hemant Khemani, Rahul Singla, Narender Omprakash Bansal   +3 more
doaj   +1 more source

Supravalvular aortic arch stenosis: A report of progressive disease and endovascular hybrid repair in a child

Annals of Vascular Surgery - Brief Reports and Innovations, 2022
Objective: This case highlights the clinical consequences of elastin (ELN) arteriopathy, which affects the supravalvular aorta, the branches of the arch and pulmonary arterial branches.
Pradeep Pravinkumar Mistry, Dirk Andries le Roux, Karabo Sekopi Mosiane, Nezerith Cengiz, Karunagaran Govendrageloo, Prof Jayandiran Pillai   +5 more
doaj   +1 more source

Novel ELN mutation in a family with supravalvular aortic stenosis and intracranial aneurysm [PDF]

, 2017
Pathogenic germline mutations in ELN can be detected in patients with supravalvular aortic stenosis. The mutation might occur de novo or be inherited following an autosomal dominant pattern of inheritance.
Hucthagowder, Vishwanathan, Jelsig, Anne Marie, Nissen, Henrik, Ousager, Lilian Bomme, Urban, Zsolt   +4 more
core   +2 more sources

Williams-Beuren syndrome: cardiovascular abnormalities in 20 patients diagnosed with fluorescence in situ hybridization

Arquivos Brasileiros de Cardiologia, 2003
OBJECTIVE: To evaluate the cardiovascular findings and clinical follow-up of patients with Williams-Beuren syndrome. METHODS: We studied 20 patients (11 males, mean age at diagnosis: 5.9 years old), assessed for cardiovascular abnormalities with ...
Sofia Mizuho Miura Sugayama, Regina Lúcia Moisés, Jaqueline Wagënfur, Nana Miura Ikari, Kikue Terada Abe, Cláudio Leone, Clóvis Artur Almeida da Silva, Maria de Lourdes Lopes Ferrari Chauffaille, Ae Kim Chong   +8 more
doaj   +1 more source

Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle

JACC: Case Reports, 2020
Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS.
Taylor E. Katt, MD, Robert L. Spicer, MD, Anji T. Yetman, MD, Ali N. Ibrahimiye, MD, James M. Hammel, MD, Jeffrey A. Robinson, MD   +5 more
doaj   +1 more source