No abstract available.
Aortic Stenosis, Supravalvular*

No abstract available.
Aortic Stenosis, Supravalvular*

No abstract available.
Aortic Stenosis, Supravalvular*

Adult ; Aortic Stenosis, Supravalvular ; Child ; Female ; Humans ; Mothers

Supravalvular aortic stenosis is an uncommon, congenital narrowing of the ascending aorta which originates just distal to the level of the ostium of the coronary artery. We conducted a successful surgical treatment in a 39 year- old female patient with a congenital supravalvular aortic stenosis and aortic regurgitation who did not show signs of William's syndrome. After we performed an inverted Y-shaped aortotomy toward the noncoronary sinus and right coronary sinus, pantaloon shaped prosthetic patch(Vascutek, Ino, USA) was used to repair the narrowing sinotubular junction. The aortic valve was replaced concommittently using Sorin Bicarbon 19mm. Her postoperative course was uneventful. The patient discharged at 9th postoperative day in good health.
Aorta ; Aortic Stenosis, Supravalvular* ; Aortic Valve ; Aortic Valve Insufficiency* ; Coronary Sinus ; Coronary Vessels ; Female ; Humans

Supravalvular aortic stenosis is a rare cause of left ventricular outflow obstruction in adults. It occurs as an isolated defect sporadically or as a hereditary basis with an autosomal dominant trait without further phenotypical anomalies, or as a part of the Williams syndrome with mental retardation and multiple anomalies. In this report, we present a case of williams syndome associated with megacoronary artery.
Adult ; Aortic Stenosis, Supravalvular ; Arteries* ; Humans ; Intellectual Disability ; Ventricular Outflow Obstruction ; Williams Syndrome*

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta whitch originates just distal to the level of the origins of the coronary arteries, and whitch includes a wide spectrum of pathologic changes. A 27 years-old-female was admitted because of further evaluation of known some heart disease. Clinical diagnosis of supravalvular aortic stenosis was made by echocardiography and angiography. We presented a case of supravalvular aortic stenosis with a review of literature.
Angiography ; Aorta ; Aortic Stenosis, Supravalvular* ; Congenital Abnormalities ; Coronary Vessels ; Diagnosis ; Echocardiography ; Heart Diseases

BACKGROUND: Supravalvular aortic stenosis is a rare form of congenital cardiac anomaly involving ascending aorta distal to coronary orifice. MATERIALS AND METHODS: We operated 12 cases of supravalvular aortic stenosis between July 1986 and March 1997. Age ranged from 4 to 17 (mean 10.2) years and 11 of them were male. Nine patients had clinical features of Williams syndrome. We experienced two types of supravalvular aortic stenosis, including 10 hour glass type and 2 diffuse type. RESULTS: Preoperative transaortic pressure gradient ranged from 40 to 180 (mean 92) mmHg by cardiac catheterization. Pulmonary stenosis was associated in 5 and 2 of them required angioplasty. Operative techniques included 6 standard aortoplasty with elliptical patch, 4 extended aortoplasty with inverted Y shaped patch, and 2 modified Brom's repair. There were no operative deaths. Postoperative echocardiographic evaluation was done at a mean interval of 12 months. Grade I or II aortic regurgitation was found in 3 cases. Postoperative cardiac catheterization revealed a mean transaortic pressure gradient of 26 (range 0 to 75) mmHg. A mean pressure drop was 78 (range 30 to 114) mmHg. All patients were followed up for a mean of 40 (range 1 to 67) months with uneventful clinical course. CONCLUSIONS: Our data proved the low mortality and excellent hemodynamic improvement after surgical relief of supravalvular aortic stenosis in children.
Angioplasty ; Aorta ; Aortic Stenosis, Supravalvular* ; Aortic Valve Insufficiency ; Aortic Valve Stenosis ; Cardiac Catheterization ; Cardiac Catheters ; Child ; Echocardiography ; Glass ; Hemodynamics ; Humans ; Male ; Mortality ; Pulmonary Valve Stenosis ; Williams Syndrome

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origins of the coronary arteries. It may be localized or diffuse, and includes a wide spectrum of pathologic changes. A 16 years old school boy was admitted because of exertional dyspnea for 6 years. Clinical diagnosis of supravalvular and valvular aortic stenosis with bicuspid aortic valves and myocardial hypertrophy was made by echocardiography and angiocardiography. Surgical correction was performed successfully. We presented a case of supravalvular and valvular aortic stenosis with a review of literatures.
Adolescent ; Angiocardiography ; Aorta ; Aortic Stenosis, Supravalvular ; Aortic Valve ; Aortic Valve Stenosis* ; Bicuspid ; Congenital Abnormalities ; Coronary Vessels ; Diagnosis ; Dyspnea ; Echocardiography ; Humans ; Hypertrophy ; Male

Twenty patients with congenital aortic stenosis excluding valvular stenosis were studied beween April 1980 and April 1984 at Seoul National University Hospital. The clinical and radiologic findings with the emphasis on the cineangiographies were analyzed separately according to the type of aortic stenosis as subaortic and supravalvular aortic stenosis. The summaries of the analysis are as follows: 1. Among the 20 cases, 12 cases were subaorticstenosis and 8 cases were supravalvular stenosis. 2. The anatomic types of subaoritc stenosis were the discrete membranous type (8 cases), the fibromuscular type (2 cases) and the tunnel type (2 cases). 3. The obstruction of subaoritic types was usually severe, and the median left ventricular to aortic systolic pressure gradient was 60mmHg, and associated cardiac defects were found in 10 cases (84%), an incidence greater than that reported in mostother large series. 4. The anatomic types of 8 cases of supravalvular aortic stenosis were mainly focal type (7cases ) and 1 case of diffuse hypoplastic type. 5. The median left ventricular to aortic systolic pressure gradient of supravalvular stenosis was 75mmHg, and associated anomalies were seen in 6 cases(75%) with 3 cases of suspected mental retardation and facial abnormalities without definite hypercalcemia. 6. For accurate diagnosis of congenital aortic stenosis, retrograde left ventricular angiograms obtainend in axial pojections are of crucial importance in demonstration of anatomic types of stenosis and associated anomalies. And supravalvular aortic injection is sometimes helpful to outline the anatomy of the valve and to evaluate the degree of aortic regurgitation.
Aortic Stenosis, Supravalvular ; Aortic Valve Insufficiency ; Aortic Valve Stenosis ; Blood Pressure ; Cineangiography ; Constriction, Pathologic ; Diagnosis ; Humans ; Hypercalcemia ; Incidence ; Intellectual Disability ; Seoul