Ascending aortaCongenital supravalvular aortic sSVASMurmurMitralNarrowing of the aortaSupravalvarEchocardiographyCalcificationCharacteristicInterrupted aortPulmonary artery stenosisCardiovascularDissectionSeptalLeft ventricularUpper extremityFaciesDopplerPulmonicRegurgitationThoracic SurgerySevereWilliamsCommonlyCoronary arteryPatientsInsufficiencyAtherosclerosisConstrictionElastin geneObstructionAbstractFindingsSinusesSinotubularSurgicalSeverityIndicationsDiagnosisInfantsSyndromeVentricleSymptomsPeak
Ascending aorta9
- Further studies have shown that patients with less extensive deletions featuring the Elastin gene also tend to develop supravalvular aortic stenosis Supravalvular aortic stenosis is due to diffuse or discrete narrowing of ascending aorta. (wikipedia.org)
- One presentation was characterized by hypercalcemia plus persistent growth failure, characteristic facial appearance, "mental retardation," heart murmur, and hypertension, while the other was characterized by supravalvular aortic stenosis (narrowing of the ascending aorta above the aortic valve, involving the sinotubular junction) plus a distinctive facial appearance, "mental retardation," "friendly" personality, and growth retardation. (williams-syndrome.org)
- Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. (msdmanuals.com)
- Narrowing in ascending aorta or fibrous diaphragm just above aortic valve). (rimikri.com)
- Background The treatment of dilatation of the ascending aorta associated with aortic valve disease is still controversial. (heartviews.org)
- Replacement of ascending aorta and aortic valve with a composite conduit may be too radical an approach, especially in patients with moderate dilatation. (heartviews.org)
- To repair a dilated ascending aorta with associated aortic valve disease, we devised a technique of aortoplasty. (heartviews.org)
- Contraindications to aortoplasty were: Marfan's disease, bicuspid aortic valve, calcification of ascending aortic wall, aortic dissection, and fusiform aneurysm without aortic valve disease.The aortic valve was replaced with a bileaflet valve and the ascending aorta was repaired with "waistcoat" technique. (heartviews.org)
- Conclusion Plastic reconstruction of dilated ascending aorta with reduction of its diameter and aortic valve replacement can reduce wall stress and the incidence of late aneurysm formation or aortic dissection. (heartviews.org)
Congenital supravalvular aortic s1
- Surgical repair of congenital supravalvular aortic stenosis in children. (medscape.com)
SVAS7
- Supravalvular aortic stenosis (SVAS) is a heart defect that develops before birth. (medlineplus.gov)
- Some people with SVAS also have defects in other blood vessels, most commonly stenosis of the artery from the heart to the lungs ( the pulmonary artery ). (medlineplus.gov)
- If SVAS is not treated, the aortic narrowing can lead to shortness of breath, chest pain, and ultimately heart failure. (medlineplus.gov)
- Aortic narrowing causes the heart to work harder to pump blood through the aorta, resulting in the signs and symptoms of SVAS. (medlineplus.gov)
- Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). (nih.gov)
- The most significant medical problem associated with Williams syndrome is a form of heart disease called supravalvular aortic stenosis (SVAS). (rareguru.com)
- Supravalvar aortic stenosis (SVAS) is the most common heart abnormality, occurring in 75% of people with WS. (rareguru.com)
Murmur2
- The murmur associated with it is systolic murmur and is similar in character to valvular aortic stenosis murmur but commonly present at 1st Intercostal space (ICS) on the right. (wikipedia.org)
- There is a continuous left-to-right shunt of blood throughout systole and diastole, giving the characteristic waxing and waning continuous murmur, as aortic pressures exceed pulmonary artery pressures throughout the cardiac cycle. (bsavalibrary.com)
Mitral2
- Parents are cautioned to keep their muscles and the mitral rim is seen at the back through the av interval measured during spectral doppler for detection of isolated aortic regurgitation. (lowerbricktown.com)
- The aortic valve is located between the ventricular septum and the mitral valve. (medscape.com)
Narrowing of the aorta1
- Supravalvular aortic stenosis is a congenital obstructive narrowing of the aorta just above the aortic valve and is the least common type of aortic stenosis. (wikipedia.org)
Supravalvar4
- Vindhyal MR, Priyadarshni S, Eid F. Supravalvar aortic stenosis. (medscape.com)
- Severe left main coronary artery stenosis with abnormal branching pattern in a patient with mild supravalvar aortic stenosis and Williams-Beuren syndrome. (medscape.com)
- Usefulness of doppler echocardiography to determine the timing of surgery for supravalvar aortic stenosis. (medscape.com)
- Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique. (medscape.com)
Echocardiography5
- Changes in dimensions of the aortic root were demonstrated more clearly by angiography than by echocardiography. (nih.gov)
- According to the American College of Radiology (ACR) in its appropriateness criteria for preintervention planning for transcatheter aortic valve displacement (TAVR), preintervention imaging with echocardiography and CT are essential for procedure planning and device selection, with MR angiography playing a complementary role. (medscape.com)
- Echocardiography is the preferred imaging test for aortic stenosis. (medscape.com)
- Aortic stenosis is seen on 2-dimensional echocardiography. (medscape.com)
- Main coronary artery and coronary ostial stenosis in children: detection by transthoracic color flow and pulsed Doppler echocardiography. (medscape.com)
Calcification4
- Calcification of the aortic valve is found in almost all adults with hemodynamically significant aortic stenosis. (medscape.com)
- Lipid deposition and inflammation lead to thickening of aortic valve structures by fibrosis and calcification initially without causing significant obstruction. (msdmanuals.com)
- It is a degenerative condition in which there is thickening and/or calcification of leaflets of aortic valve. (rimikri.com)
- There are some risk factor like hyperlipidemia, diabetes mellitus, smoking, hypertension are prone to develop aortic calcification. (rimikri.com)
Characteristic2
- Fluorescent in situ hybridisation techniques have revealed that 96% of patients with Williams syndrome, where supravalvular aortic stenosis is characteristic, have a hemizygous deletion of the Elastin gene. (wikipedia.org)
- The incidence of cytoplasmic metachromasia has been studied in cultures of skin fibroblasts derived from 6 cases of the syndrome of supravalvular aortic stenosis, characteristic facies, and mental retardation which in many instances represents the late normocalcaemic stage of the severe form of infantile hypercalcaemia. (bmj.com)
Interrupted aort1
- associated lesions included subAS (n=9), supravalvular AS (n=2), coarctation (n=5), and interrupted aortic arch (n=2). (elsevierpure.com)
Pulmonary artery stenosis2
- Issues regarding surgical timing and concomitant pulmonary artery stenosis need to be further addressed. (nih.gov)
- Wren C, Oslizlok P, Bull C. Natural history of supravalvular aortic stenosis and pulmonary artery stenosis. (medscape.com)
Cardiovascular1
- Bicuspid aortic valve is the most common congenital cardiovascular abnormality. (msdmanuals.com)
Dissection1
- Aortic Dissection Aortic dissection is the surging of blood through a tear in the aortic intima with separation of the intima and media and creation of a false lumen (channel). (msdmanuals.com)
Septal1
- Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. (medscape.com)
Left ventricular2
- Individuals with significant supravalvular AS chronically may develop left ventricular hypertrophy and also are at risk of developing coronary artery stenosis. (wikipedia.org)
- Coarctation of the Aorta Coarctation of the aorta is a localized narrowing of the aortic lumen that results in upper-extremity hypertension, left ventricular hypertrophy, and, if severe, malperfusion of the abdominal. (msdmanuals.com)
Upper extremity1
- An explanation of asymmetric upper extremity blood pressures in supravalvular aortic stenosis: the Coanda effect. (medscape.com)
Facies1
- Supravalvular aortic stenosis coarse, elfin-like facies with prominent bulbar palsies and clear center or your doctor. (albionfoundation.org)
Doppler2
- The antegrade systolic velocity across the narrowed aortic valve, or aortic jet velocity, is measured using continuous-wave Doppler ultrasound. (medscape.com)
- Doppler interrogation of the aortic valve makes use of the modified Bernoulli equation (gradient = 4 × velocity 2 ) to assess the severity of the stenosis. (medscape.com)
Pulmonic1
- Peripheral pulmonic stenosis is common in infancy. (rareguru.com)
Regurgitation1
- Seven of them had aortic regurgitation, 3 aortic stenosis, 1 postendocarditic periprosthetic leak, and 1 calcified bioprosthesis. (heartviews.org)
Thoracic Surgery1
- She was subsequently referred to thoracic surgery for evaluation of superior vena cava stenosis. (ctsnet.org)
Severe3
- [ 2 ] In symptomatic patients with medically treated moderate to severe aortic stenosis, death usually occurs within 5 years. (medscape.com)
- The diagnosis of severe AS is currently based on 3 hemodynamic parameters: maximal jet velocity, mean pressure gradient across the aortic valve, and aortic valve area. (medscape.com)
- All the four patients presented with effort angina and all were found to have obstructive coronary artery disease oncoronary angiogram and two of them had severe supravalvular aortic stenosis. (ijmedicine.com)
Williams3
- Vascular wall remodeling in patients with supravalvular aortic stenosis and Williams Beuren syndrome. (medscape.com)
- [1] J.C.P. Williams observed in four patients an association between supravalvular aortic stenosis and the common physical and mental characteristics of this patient population and stated that it "may constitute a previously unrecognized syndrome" [1] . (physio-pedia.com)
- She had supravalvular aortic stenosis-a life-threatening heart problem often associated with Williams Syndrome. (legacyhealth.org)
Commonly1
- Background - The Ross procedure is commonly used to treat aortic valve disease in pediatric and adult patients. (elsevierpure.com)
Coronary artery2
- Surgical management of congenital obstruction of the left main coronary artery with supravalvular aortic stenosis. (medscape.com)
- In line with the ageing population, the prevalence of coronary artery disease (CAD), lower extremity peripheral arterial disease (PAD), supra-aortic arterial disease (SAD) and renal stenosis (RAS) is increasing. (who.int)
Patients5
- Identification and characterization of seven novel mutations of elastin gene in a cohort of patients affected by supravalvular aortic stenosis. (medscape.com)
- Over years, aortic sclerosis progresses to stenosis in as many as 15% of patients. (msdmanuals.com)
- Tong Y, Zhang P, Li S, Yan J, Feng Z, Zhao J, Guo S, Jin Y, Liu J. Perioperative blood product transfusion of two different perfusion strategies on pediatric patients undergoing aortic arch surgery. (ispmcs.org)
- Most patients develop PCAD and aortic stenosis before the age of 20 years and die before 30 years of age. (ijmedicine.com)
- Methods and Results Between July 1996 and July 1998, 12 patients underwent aortic valve replacement and ascending aortoplasty. (heartviews.org)
Insufficiency1
- Most often, valvular stenosis or insufficiency. (msdmanuals.com)
Atherosclerosis3
- The cause of aortic sclerosis and stenosis is not yet known but is mediated by an inflammatory process that is similar to but distinct from atherosclerosis. (msdmanuals.com)
- Lipoprotein (a) is implicated in the pathogenesis of both aortic stenosis and atherosclerosis. (msdmanuals.com)
- are at increased risk for atherosclerosis, and more recently, psoriasis has been tied to an increased risk of aortic stenosis. (msdmanuals.com)
Constriction2
- Supravalvular AS caused by a discrete congenital membrane or hypoplastic constriction just above the sinuses of Valsalva is uncommon. (msdmanuals.com)
- Rarely, supravalvular (Constriction at the first part of aorta. (rimikri.com)
Elastin gene2
- Supravalvular aortic stenosis is associated with genetic damage at the Elastin gene locus on chromosome 7q11.23. (wikipedia.org)
- 1993). "The elastin gene is disrupted by a translocation associated with supravalvular aortic stenosis" . (wikipedia.org)
Obstruction1
- Aortic stenosis (AS) is the obstruction of blood flow across the aortic valve. (medscape.com)
Abstract1
- abstract： BACKGROUND:EVAR for abdominal aortic aneurysm has an initial survival advantage over OR, but more frequent complications increase costs and long-term aneurysm-related mortality. (shengsci.com)
Findings4
- Chest radiographs may show several significant findings consistent with aortic stenosis. (medscape.com)
- What are the X-ray findings of aortic stenosis? (rimikri.com)
- What are the ECG findings of aortic stenosis? (rimikri.com)
- What are the echocardiogram findings of aortic stenosis? (rimikri.com)
Sinuses1
- Anatomic restoration of the aortic root should ideally take into account all of the deformed components by enlarging all three sinuses of Valsalva at the sinotubular junction. (nih.gov)
Sinotubular1
- Postoperative echocardiographic study showed a significant reduction of both sinotubular and ascending aortic diameters. (heartviews.org)
Surgical2
- We aimed to elucidate the structural basis of supravalvular aortic stenosis in the context of its surgical repair. (nih.gov)
- Akkaya G, Bilen C, Tuncer ON, Atay Y. Requirement for repetitive surgical approaches at supravalvular aortic stenosis. (medscape.com)
Severity2
- Imaging of the aortic valve is critical in establishing a diagnosis, grading severity, and informing the timing of valvular intervention. (medscape.com)
- How will you classify the severity of aortic stenosis? (rimikri.com)
Indications3
- What are the indications of coronary angiography in aortic stenosis? (rimikri.com)
- What are the indications of aortic valve replacement? (rimikri.com)
- Indications for aortoplasty were: echocardiographic aortic diameter between 5 and 6 cm and macroscopic appearance of normal aortic wall. (heartviews.org)
Diagnosis1
- Why is your diagnosis aortic stenosis? (rimikri.com)
Infants1
- Pola dos Reis F, Minamoto H, Bibas BJ, Minamoto FEN, Cardoso PFG, Caneo LF, Pego-Fernandes PM. Treatment of tracheal stenosis with extracorporeal membrane oxygenation support in infants and newborns. (ispmcs.org)
Syndrome3
- Supravalvular aortic stenosis-infantile hypercalcaemia syndrome: in vitro hypersensitivity to vitamin D2 and calcium. (bmj.com)
- Heyde's syndrome is the association between aortic stenosis and occult gastrointestinal bleeding usually from colonic angiodysplasia. (rimikri.com)
- Marfan syndrome, supravalvular aortic stenosis, and Ehlers-Danlos syndrome type IV result from mutations of genes encoding fibrillin-1, elastin, and type III collagen, respectively. (heartviews.org)
Ventricle1
- 3. From the Left Ventricle, the blood passes through the Aortic Valve to the Aorta. (hemopet.org)
Symptoms1
- [ 1 ] Symptoms of aortic stenosis usually develop gradually after an asymptomatic latent period of 10-20 years. (medscape.com)
Peak1
- Residual aortic stenosis was associated with male gender, preoperative aortic valve stenosis, and a preoperative peak gradient greater than 90 mmHg. (nih.gov)