• Journal of Chest Surgery
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• v.24 no.3
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• pp.280-286
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• 1991

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. Enlargement of the aorta with a diamond-shaped patch of the noncoronary sinus of Valsalva was reported in 1961 by McGoon and associates But this reconstruction is asymmetric and the aortic obstruction may remain. In 1977, Dotty and associates reported the extended aortoplasty, the supravalvular ring was incised at two points in the noncoronary and in the right coronary sinuses of Valsalva closed with a tubular Dacron prosthesis of inverted Y-shape tailored to reconstruct the aorta We experienced three cases of the supravalvular aortic stenosis. The 11-year-old female and 4-year-old male with localized supravalvular aortic stenosis in William`s syndrome were operated with an inverted Y-shaped aortotomy toward the non-coronary sinus and the right coronary sinus and closed with "Hemashield`s collagen impregnated Dacron" tube graft, fashioned into "pantaloon" form patch. The 12-year-old male with localized supravalvular aortic stenosis and mitral insufficiency in William`s syndrome were operated with same procedure as two other patient above-mentioned for relief of supravalvular aortic stenosis and with mitral valve replacement. Postoperative course has been good.ourse has been good.

• Journal of Chest Surgery
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• v.26 no.2
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• pp.135-140
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• 1993

Supravalvular aortic stenosis is an uncommon, congenital narrowing of ascending aorta just above aortic valve. Eleven patients underwent an aortoplasty to relieve supravalvular aortic stenosis at Sejong General Hospital from July 1985 to December 1991. Age ranged from 5 to 14 years(median 9 years). There were 7 male and 4 female patients. Seven patients had characteristics of Williams' syndrome including elfin face and mental retardation. All patients had localized, hourglass type but 4 patients had atypical findings. Preoperative left ventricula-aortic pressure gradient ranged from 40 to 190 mmHg(mean 88 mmHg). To relieve severe supravalvular aortic stenosis, extended aortoplasty was used in 7 patients and standard aortoplasty in 4 patients. Postoperative pressure gradient ranged from 0 to 40 mmHg (mean 16.6 mmHg). Follow-up pressure gradient with Doppler ranged from 0 to 88mmHg(mean 32.5mmHg).

• Journal of Chest Surgery
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• v.18 no.4
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• pp.615-622
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• 1985

Supravalvular aortic stenosis was relatively uncommon form of congenital heart disease. This patient had typical "elfin faces" with mental retardation, and supravalvular aortic stenosis. The diagnosis was confirmed by pressure tracing obtained at retrograde left heart catheterization and aortography. The type of supravalvular aortic stenosis was localized hourglass narrowing, which was treated by insertion of prosthetic gusset placed across the area of narrowing under the cardiopulmonary bypass.ry bypass.

• Journal of Chest Surgery
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• v.23 no.6
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• pp.1146-1151
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• 1990

Supravavular aortic stenosis is a congenital narrowing of the ascending aorta just distal to the level of the origins of the coronary arteries, that may be localized or diffuse. Five patients with supravalvular aortic stenosis were operated upon between July, 1986 arid June, 1990. Four of these patients were William`s syndrome [mental retardation, elfin face], and one was isolated supravalvular aortic stenosis. Preoperative diagnosis of the supravalvular aortic stenosis was made by left side cardiac catheterization and angiocardiography. There are three types of supravalvular aortic stenosis such as membranous, hourglass and hypoplastic. Four of our patients were of hourglass type, and one was hypoplastic type. Patch aortoplasty was performed in all cases. Preoperative systolic gradients ranged from 45 to 1SO mmHg [average 102.6 mmHg]: postoperative gradients ranged from 0 to 75 mmHg [average 39 mmHg]. The patient of hypoplastic type has been suffered from mild exercise intolerance even after the operation, and the postoperative echocardiography revealed the systolic gradient of 100 mmHg [preoperative 180 mmHg]. The results of surgery for hourglass type were excellent. But the patient with hypoplastic form would be benefited from some modifications of the operation.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.680-686
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• 1989

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origin of the coronary arteries. It may occur in several different anatomical form. Three type have been described; the hourglass, the hypoplastic and the membranous type, each term identifying the gross characteristic of the lesion causing by the aortic obstruction. Non cardiovascular condition commonly associated with supravalvular aortic stenosis are mental retardation, facial anomalies, hypercalcemia, etc. The diagnosis can be established preoperatively by left heart catheterization and selective angiography. Recently, we experienced a case of multiple localized supravalvular aortic stenosis involving, just above the sinus Valsalva and just proximal of the innominate artery. The surgical correction which was performed by a vertical incision across the each narrowing of aorta with replacement of diamond shaped double velour Woven Dacron patch under the CPB. He was discharged without any event.

• Journal of Chest Surgery
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• v.28 no.4
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• pp.391-394
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• 1995

We expericenced one case of surgical treatment of congenital supravalvular aortic stenosis associated with Willjams` syndrome using Dory procedure. A 22 years-old male patient was admitted to the cardiology department due to dyspnea,easy fatigue,dizziness. On physical examination,systolic murmur was audibled at fight second parasternal intercostal space and his general appearance was elfin face and mental retardation was noted. Cardiac catheterization was revealed as a supravalvular aortic stenosis and pressure gradient was 45mmHg between the left ventricle and the aorta. Successful surgical treatment was accomplished with Inverted Y procedu~:e using Dacron patch[Doty procedure . Postoperative course was uneventful and he was discharged in good condition.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.591-594
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• 1999

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.

• Journal of Chest Surgery
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• v.27 no.6
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• pp.472-476
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• 1994

The incidence of single coronary artery is extremely rare in a review of congenital anomalies of the coronary arteries. This 27-year-old male patient was referred for the evaluation of cardiac condition showing exertional dyspnea[NYHA class II-III] and chest discomfort for about 1 year. A complete catheterization study including angiogram disclosed large single coronary artery arising from left aortic sinus [Ogden classification L-4] associated with bicuspid aortic valvular stenosis and low grade supravalvular aortic stenosis. Calcified stenotic aortic valve was fully removed with caution and the 19mm St. Jude Medical valve was then implanted in the small nortic annulus. The patient had an uneventful recovery and was discharged on 13th postoperative day.

• Journal of Chest Surgery
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• v.31 no.8
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• pp.763-769
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• 1998

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.

• Journal of Chest Surgery
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• v.49 no.2
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• pp.115-118
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• 2016

Williams syndrome (WS) is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS) and peripheral pulmonary artery stenosis (PPAS) are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.