Citation: Faruk Akturk (2013) Valvular and Supravalvular Aortic Stenosis Secondary to Familial Hyperlipidemia. View PDF Download PDF Tables & Figures
Supravalvular aortic stenosis is frequently associated with other congenital abnormalities such as pulmonary artery branch stenosis, unusual facies, retarded physical development, abnormal dentition, and mental retardation. Stenosis of the origins of the brachiocephalic vessels may be found in a significant percentage of cases. To determine long-term outcome after operation for supravalvular aortic stenosis, we reviewed the case histories of 80 patients who had repair of the localized form (group A) ( n = 67) or diffuse form (group B) ( n = 13) from 1956 to 1992, including 31 patients with the Williams-Beuren syndrome. Ages ranged from 7 months to 54 years (mean = 12.6 years). Forty-six patients had one or more Fig. 5. M-modeechocardiogram demonstrating supravalvular stenosis with 7 mm reduction in aortic diameter. Prolapse of posteriormitralleaflet into dilated left atrium is clearly visualized. Cross-sectional echocardiography (Fig. 6) demonstrated the areaof SVAS as well as the normal mobility ofthe aortic cusps. The left atrium also appeared enlarged. Congenital supravalvar aortic stenosis (SVAS) is the least common form of left ventricular outflow tract (LVOT) obstruction.1 It is a manifestation of elastin arteriopathy and is the most common surgical lesion associated with this disorder. Elastin arteriopathy is caused by a microdeletion of the elastin precursor gene 7q11.23, resulting in defective elastin production and a variable spectrum Williams syndrome (WS) is a developmental disorder affecting connective tissue and the central nervous system. A common feature of WS, supravalvular aortic stenosis, is also a distinct autosomal Supravalvular aortic stenosis with a chronic type A aortic dissection. Takahiro Ishigaki MD. View Enhanced PDF Access article on Wiley Online Library (HTML view) Download PDF for offline viewing. Logged in as READCUBE_USER. Log out of ReadCube. No abstract is available for this article. Volume 33, Issue 4 We report a 19-year-old male patient with familial homozygous hypercholesterolemia with progressive supravalvular and valvular aortic stenosis that ultimately required aortic root enlargement and aortic valve replacement using a mechanical prosthesis, despite aggressive medical therapy. Surgical importance of this rare condition is highlighted.
Supravalvular aortic stenosis is frequently associated with other congenital abnormalities such as pulmonary artery branch stenosis, unusual facies, retarded physical development, abnormal dentition, and mental retardation. Stenosis of the origins of the brachiocephalic vessels may be found in a significant percentage of cases. To determine long-term outcome after operation for supravalvular aortic stenosis, we reviewed the case histories of 80 patients who had repair of the localized form (group A) ( n = 67) or diffuse form (group B) ( n = 13) from 1956 to 1992, including 31 patients with the Williams-Beuren syndrome. Ages ranged from 7 months to 54 years (mean = 12.6 years). Forty-six patients had one or more Fig. 5. M-modeechocardiogram demonstrating supravalvular stenosis with 7 mm reduction in aortic diameter. Prolapse of posteriormitralleaflet into dilated left atrium is clearly visualized. Cross-sectional echocardiography (Fig. 6) demonstrated the areaof SVAS as well as the normal mobility ofthe aortic cusps. The left atrium also appeared enlarged. Congenital supravalvar aortic stenosis (SVAS) is the least common form of left ventricular outflow tract (LVOT) obstruction.1 It is a manifestation of elastin arteriopathy and is the most common surgical lesion associated with this disorder. Elastin arteriopathy is caused by a microdeletion of the elastin precursor gene 7q11.23, resulting in defective elastin production and a variable spectrum Williams syndrome (WS) is a developmental disorder affecting connective tissue and the central nervous system. A common feature of WS, supravalvular aortic stenosis, is also a distinct autosomal Supravalvular aortic stenosis with a chronic type A aortic dissection. Takahiro Ishigaki MD. View Enhanced PDF Access article on Wiley Online Library (HTML view) Download PDF for offline viewing. Logged in as READCUBE_USER. Log out of ReadCube. No abstract is available for this article. Volume 33, Issue 4
Valvulopathies in Young Athletes Developed and typed by Dr. Ghazi Al-Shumrani (intern). 1 1. Úvod Aberantní fenotyp může vznikat z mnoha různých příčin, například narušením jednoho určit&ea GUIA_013.pdf - Free download as PDF File (.pdf), Text File (.txt) or read online for free. Unless the obstruction is severe, occur at cardiac output will be maintained and the valve, below the valve (double- the clinical symptoms of chambered right ventricle, heart failure will be either subtle or subaortic membrane), or above it… MRCP mcq - Free download as PDF File (.pdf), Text File (.txt) or read online for free. MRCP mcq cardiology emq - Free download as PDF File (.pdf), Text File (.txt) or read online for free.
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