We present the case of a 54-year-old woman in which a diffuse congenital supravalvular aortic stenosis (SVAS) was associated with a severe aortic valve incompetence and heavy calcification of the aortic annulus. Repair consisted in resection of the ascending aorta, patch augmentation of the hypoplastic aortic root and annulus, placement of a 20 mm Dacron tubular graft (Vascutek, Renfrewshire, UK) and aortic valve replacement with a mechanical prosthesis (Sorin, Turin, Italy). Follow-up echocardiography demonstrated normal prosthetic valve function and a postoperative three-dimensional computed tomographic scan showed a normal shape of the reconstructed ascending aorta.
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http://dx.doi.org/10.4061/2009/976190 | DOI Listing |
Several techniques for the surgical correction of congenital supravalvular aortic stenosis have been devised. We describe the step-by-step surgical approach of a slide aortoplasty to correct localized supravalvular aortic stenosis in a 3-year-old child with Williams syndrome.
View Article and Find Full Text PDFAm J Physiol Heart Circ Physiol
January 2025
Weatherhead P.E.T. Imaging Center, McGovern Medical School at UTHealth, Houston, Texas, USA.
An increasing number of procedures over the past two decades for aortic stenosis (AS) reflects the combination of an aging population and less invasive transcatheter options. As a result, the hemodynamics of the aortic valve (AV) have gained renewed interest to understand its behavior and to optimize patient selection. We studied the hemodynamic relationship between pressure loss (ΔP) and transvalvular flow (Q) of the normal AV as well as the impact of a variable supravalvular stenosis.
View Article and Find Full Text PDFEur J Med Res
January 2025
Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, 100029, China.
Background: An effective prognostic nomogram to predict the prognosis for supravalvular aortic stenosis (SVAS) patients is lacking.
Methods: A multi-center retrospective study of consecutive SVAS patients with surgery between 2002 and 2020 was conducted. Patients underwent McGoon repairs, Doty repairs, and other repairs.
Int J Surg Case Rep
January 2025
The Internist Cardiologist, Department of Cardiology, Al Watani Hospital, Hama, Syria.
BMC Pregnancy Childbirth
November 2024
The Genetics Laboratory, Longgang District maternity & Child Healthcare Hospital of Shenzhen City (Longgang Maternity and Child Institute of Shantou University Medical College), Shenzhen, Guangdong, China.
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