• Journal of Chest Surgery
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• v.33 no.9
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• pp.748-751
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• 2000

The diffuse form of supravalvar aortic stenosis represents a surgical challenge when the ascending aorta, aortic arch, proximal descending thoracic aorta and arch arteries are involved. It can be treated by a variety of surgical approaches. We report a case of severe diffuse supravalvar aortic stenosis combined with an aortic valve anomaly and occlusion of the right coronary artery ostium in a 14-year-old boy with Williams syndrome. We enlarged the aortic root(Nick's procedure), ascending aorta, aortic arch, proximal descending thoracic aorta, and innominate artery with patches and replaced aortic valve with 19 mm St. Jude valve. Deep hypothermic circulatory arrest and retrograde cerebral perfusion were used during repair of the arch and arch artery.

• 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.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.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.18 no.4
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• pp.663-666
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• 1985

Whatever a surgeon choose aortic prosthesis in aortic stenosis, it will always provoke some degree of obstruction to flow due to its smaller effective orifice area to tissue annulus. Occasionally, we meet small aortic annulus to his or her body surface area in aortic valve replacement. The small annulus remains a problem in that both tissue and mechanical prosthesis have significant pressure gradients between LV and aorta in resting or exercising states. In these circumstances, diverse surgical procedures, such as tilting disc prosthesis, supraannular position of aortic prosthesis, and enlargement of aortic root [including aortoventriculoplasty, translocation of aortic valve, & double outlet of LV by valved conduit], were applied. We experienced one case of aortic stenosis with small aortic annulus. Systolic pressure gradients between LV & aorta were 90 mmHg. Diameter of annulus was 19 mm. So we performed patch enlargement of aortic root by Manouguian and AVR with St. Jude medical valve 23 mm.

• 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.

• IMMUNE NETWORK
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• v.16 no.1
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• pp.26-32
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• 2016

Aortic valve stenosis is a heart disease prevalent in the elderly characterized by valvular calcification, fibrosis, and inflammation, but its exact pathogenesis remains unclear. Previously, aortic valve stenosis was thought to be caused by chronic passive and degenerative changes associated with aging. However, recent studies have demonstrated that atherosclerotic processes and inflammation can induce valvular calcification and bone deposition, leading to valvular stenosis. In particular, the most abundant cell type in cardiac valves, valvular interstitial cells, can differentiate into myofibroblasts and osteoblast-like cells, leading to valvular calcification and stenosis. Differentiation of valvular interstitial cells can be trigged by inflammatory stimuli from several immune cell types, including macrophages, dendritic cells, T cells, B cells, and mast cells. This review indicates that crosstalk between immune cells and valvular interstitial cells plays an important role in the development of aortic valve stenosis.

• Journal of Veterinary Clinics
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• v.21 no.1
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• pp.83-86
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• 2004

A 4 year old mixed female dog was presented dyspnea, anorexia and syncope. In blood smear test, a lot of heartworm larvae were observed. In thoracic radiography, "reverse D" shape was observed. So, this case was considered with heartworm disease. In echocardiography, marked left heart hypertrophy was presented. In M-mode view, aortic valvular stenosis was found. After mongrel dog died, aortic valvular stenosis was reconfirmed in necropsy. After all, the dog died of pulmonary edema because of aortic valvular stenosis caused left heart failure.t failure.

• 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.21 no.4
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• pp.721-726
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• 1988

Congenital supravalvar aortic stenosis is an obstruction caused by localized or diffuse narrowing of the aortic lumen commencing immediately above the aortic valve. We experienced a case of diffuse supravalvar aortic stenosis involving ascending aorta from just above the sinuses of Valsalva to the proximal l cm of the innominate artery. Supravalvar aortic stenosis in this patient, in contrast to the form seen in infants and children, was not associated with mental retardation, peculiar faces or the syndrome of hypercalcemia. Diagnosis was confirmed by retrograde left heart catheterization and left ventriculography. Surgical correction was performed by the replacement of oval shaped Woven Dacron patch over the narrow segment of aorta under the cardiopulmonary bypass. Blood pressure was controlled sufficiently with some adjunct of Inderal postoperatively. The patient was discharged with much improvement.