• Journal of Chest Surgery
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• 제33권9호
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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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• 제21권4호
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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.

• Journal of Chest Surgery
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• 제53권3호
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• pp.144-146
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• 2020

Supravalvar aortic stenosis (SVAS) is a rare congenital cardiac disease that usually co-occurs with Williams syndrome. In the adult population, a few SVAS cases have been reported in patients affected by homozygous familial hypercholesterolemia. However, because of the rarity of this disease entity, there is no standard surgical treatment for SVAS. Here, we present a case of successful surgical treatment using an autologous excised aortic patch in a 65-year-old patient with SVAS.

• Journal of Chest Surgery
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• 제26권3호
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• pp.228-233
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• 1993

Patients with anomalous connection of the left coronary artery to the pulmonary artery are at risk for myocardial infarction, and early or sudden death. Between 1986 to 1992, a total of 4 of these patients underwent surgical intervention with various operative techniques at our institution. Age at operation ranged from 2 months to 43 years. Three infant patients had congestive heart failure, 2 of them had mitral regurgitaion, and 1 had ST-T change on elctrocardiogram. Operative techniques included direct coronary artery transfer to the aorta[n=2], intrapulmonary tunnel from the aortopulmonary window[n=1], coronary artery bypass using saphenous vein[n=1]. One deaths occured at 2 weeks after direct coronary arterial transfer due to respiratory failure caused by Respiratory Syncitial virus pneumonia. Supravalvar pulmoanry stenosis occured after intrapulmoanry tunnel. We recommend direct aortic implatation of the anomalous coronary artery at the time of diagnosis. Intrapulmonary tunnel from aortopulmonary window or subclavian-coronary anastomosis could be alternatives in whom aortic implantation is not feasible anatomically.

• Journal of Chest Surgery
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• 제42권3호
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• pp.292-298
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• 2009

배경: 소아 환자의 승모판막 질환에 대한 외과적 치료는 그 모양의 다양성과 동반된 선천성 심장 기형 등으로 인해 기술적, 임상적으로 어렵다. 본 연구는 선천성 승모판막 폐쇄부전증 환아에서 본원에서 시행한 승모판막 성형 수술의 결과를 평가하였다. 대상 및 방법: 1997년 4월부터 2007년 10월까지 승모판막 폐쇄부전증으로 승모판막 수술을 시행한 소아 환자 22명을 대상으로 후향적으로 분석하였다. 수술 당시 환아의 평균 연령은 5.4세였고, 이 중 12개월 미만의 영아는 4명이었다. 동반된 심기형으로는 심실 중격결손이 13예로 가장 많았고, 그 외에 심방 중격결손과 판막 상부 대동맥 판막 협착증(supravalvar aortic valve stenosis)이 각각 1예 있었으며, 승모판막 페쇄부전증만 단독으로 있는 경우는 7예가 있었다. 수술 전 승모판막 페쇄부전증의 정도는 II, III, IV가 각각 4예, 15예, 3예였고, 그 원인으로 판막 탈출증(leaflet prolapse)이 12예, 판막륜 확장(annular dilatation)이 4예, 제한된 판막 움직임(restrictive leaflet motion)이 5예 있었다. 수술 전후의 페쇄부전증 정도와 승모판막륜의 Z-value를 비교함으로써 그 결과를 평가하였다. 결과: 모든 환자에서 판막 성형술이 가능했고, 수술 후 조기 사망과 만기 사망 모두 얼었다. 19예에서 판막륜 축소술(reduction annuloplasty)을 시행하였고, 18예에서 건삭 축소술(shortening of chordae)이나 판막열 봉합술(cleft closure) 등의 판막 성형술(valvuloplasty)은 추가로 시행하였다. 수술 직후 승모판막 폐쇄부전증의 정도는 줄어들었고(0=10, I=5, II=5, III=2), 승모판막륜의 Z-value도 감소하였다($2.2{\pm}2.1$ vs $0.7{\pm}2.3$, p<0.01). 3.68년의 추적기간 동안 3명의 환아가 승모판막 폐쇄부전증으로 재수술을 받았고, 한명은 재성형술을, 나머지 2명은 승모판막 치환술을 받았으며, 퇴원 시 3명의 환아에서 수술 직 후 보다 폐쇄부전증이 좀 더 진행되었다. 결론: 소아의 승모판막 페쇄부전증에서 일차 수술방법으로 적극적인 승모판막 성형술은 효과적이고 믿을만한 방법이다. 좀 더 좋은 수술 결과를 얻기 위해서는 수술 전 환아의 승모판막의 모양과 기능을 면밀히 파악하고, 다양한 수술적 기법을 적용하는 노력이 필요하겠다.