• Journal of Chest Surgery
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• 제24권3호
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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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• 제26권2호
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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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• 제18권4호
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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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• 제23권6호
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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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• 제22권4호
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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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• 제28권4호
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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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• 제32권6호
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• pp.591-594
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• 1999

대동맥판상부 협착증은 발살바동 상부에서부터 협착이 존재하는 비교적 흔치않은 선천성 질환이다. William 증후군없이 선천적으로 대동맥판 상부 협착증과 대동맥 좌관상판엽의 형성부전으로 인한 대동맥판 폐쇄부전증으로 진단받은 39세 여자환 悶“\ulcorner대동맥판막 치환술 및 Vascutek graft를 이용한 판탈롱 대동맥성형술을 시행 하였다. 환자의 술후경과는 좋았고 술후 9일째 퇴원하였다.

• Journal of Chest Surgery
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• 제27권6호
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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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• 제31권8호
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• pp.763-769
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• 1998

배경: 판막상부 대동맥협착증은 상행대동맥의 관상동맥 기시부 상부의 협착을 초래하는 드문 형태의 선천성 기형이다. 대상 및 방법: 본 서울대학교 어린이병원 흉부외과에서는 1986년 7월부터 1997년 3월까지 12예의 판막상부 대동맥협착증 환자들에게 수술적치료를 하였다. 평균나이는 10.2세였고 4세에서 17세까지 분포하였다.11명이 남자환자였고 Williams증후군의 임상양상을 보인 경우는 12예중 9예였다. 두가지형태의 판막상부 대동맥협착증을 경험하였고 10예가 모래시계형(hour glass type)이였고 2예가 미만형(diffuse type) 이었다. 결과: 모든 환자들은 수술 전에 심도자검사를 하였다. 술전 좌심실과 대동맥의 평균압력차이는 92 mmHg였고 40에서 180 mmHg까지 분포하였다. 폐동맥협착은 5예에서 관찰되었으며 그중 2예에서 폐동맥혈관성형 술을 시행하였다. 세 가지 종류의 수술적기법을 시행하였는바 타원형의 포편을 이용한 단순포편대동맥성형술이 6예였고 역Y모양의 포편을 이용한 확장형 대동맥성형술이 4예였고 변형 Brom 기법이 2예였다. 수술사망례는 없었고 수술후 심에코검사를 평균 12개월에 시행하였다. 1도와 2도의 대동맥판폐쇄부전이 3예에서 있었다. 술후 시행한 심도자검사상 좌심실과 대동맥 사이에 평균 26 mmHg (0∼75 mmHg)의 압력차이가 있었고 평균압력차이의 감소는 78 mmHg(30∼114 mmHg)였다. 모든 환자는 평균 40개월(1∼67개월)간의 추적관찰기간동안 특별한 문제없는 임상 경과를 보였다. 결론: 본 연구는 소아의 판막상부 대동맥협착증의 수술적 교정이 술후 낮은 사망률을 보장하면서 양호한 혈역학적 개선을 가져옴을 보여주었다.

• Journal of Chest Surgery
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• 제49권2호
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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.