Supra-valvular Stenosis of the Aorta

Kreel, Isadore; Reiss, Raphael; Strauss, Lotte; Blumenthal, Sidney; Baronofsky, Ivan D.

doi:10.1097/00000658-195904000-00011

Cited by 63 publications

(8 citation statements)

References 3 publications

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“…In other reports, the valve has been imperfect, with involvement of coronary orifices, and these eases have ended fatally.3 4 With improved valve prostheses now available, however, consideration should be given to valve replacement in addition to correction of the supravalvular stenosis in such cases.…”

Section: Discussionmentioning

confidence: 99%

Successful Surgical Correction of Supravalvular Aortic Stenosis

DeBakey

Beall

1963

Circulation

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Stenotic lesions in the aortic valve region may be acquired or congenital, and may be valvular, subvalvular, or supravalvular in location. As more of these patients are offered the benefits of surgical correction, cases of supravalvular aortic stenosis are being reported with increasing frequency. At least seven previous cases have been corrected successfully and another is reported. The diagnosis of supravalvular aortic stenosis usually cannot be made on clinical grounds alone. Definitive diagnostic technics are available, however, and allowed accurate delineation of the lesion in the case reported. Surgical correction consists of enlarging the aortic lumen in the area of supravalvular constriction. This usually is possible by longitudinal aortotomy through the area of constriction followed by patch angioplasty, as employed in this case in conjunction with modified endarterectomy. In most instances of supravalvular aortic stenosis, the valve itself is perfectly normal, and total correction is possible if the correct diagnosis is made.

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Section: Discussionmentioning

confidence: 99%

Successful Surgical Correction of Supravalvular Aortic Stenosis

DeBakey

Beall

1963

Circulation

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“…The aortic roots of the 23 control neonates were histologically normal. The normal invagination or plica at the upper margin of the sinuses of Valsalva29 30 was observed in some sections of each of these aortae, indicating that the plica does not extend around the entire inner circumference. The medial thickening that accounted for this invagination appeared moderately exaggerated ( fig.…”

Section: Anatomic Observations Adult Femalesmentioning

confidence: 94%

“…Three groups of five females each were fed the stock diet and in similar fashion were given intramuscular vitamin D for 30 born to a control mother and to a vitamin Ddeficient mother was sacrificed. All of the remaining offspring were sacrificed at 3 months of age.…”

Section: Friedman Robertsmentioning

confidence: 99%

Vitamin D and the Supravalvar Aortic Stenosis Syndrome

Friedman

Roberts

1966

Circulation

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RECENT REPORTS have indicated that idiopathic infantile hypercalcemia, a disease related to deranged vitamin D metabolism, -4 may be a feature of the nonfamilial, congenital supravalvar aortic stenosis syndrome.5 8 Other manifestations of this syndrome include mental retardation, a peculiar "elfin" facies, narrowing of peripheral pulmonary and systemic arteries, strabismus, inguinal herniae, and dental abnormalities.9 Congenital supravalvar aortic stenosis and peripheral pulmonary arterial stenosis also occur in a familial and a sporadic form unassociated with the other features of the syndrome.10-13 It is not known if the multiplesystem involvement in the supravalvar aortic stenosis syndrome is genetically determined or whether some or all of the features are related to a derangement of maternal or fetal vitamin D metabolism, or a combination of the two.

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“…In those hitherto described, the stricture has consisted of either a fibrous membrane directly adjacent to the cusps or a true stenosis at the level of the upper border of the sinus of Valsalva where, as mentioned by Kreel, Reiss, Strauss, Blumenthal, and Baronofsky (1959), a slight, circular inward bulge in the aortic wall is normally present. Formerly, attempts at operative treatment of supravalvular aortic stenosis met with failure.…”

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confidence: 91%

The Surgical Treatment of Supravalvular Aortic Stenosis Based on Two Cases

Nordström¹,

Silander²

1962

Thorax

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An increasing number of cases of supravalvular aortic stenosis have been reported in recent years. In those hitherto described, the stricture has consisted of either a fibrous membrane directly adjacent to Operation was performed on September 16, 1958, under extracorporeal circulation with the heart-lung machine of Crafoord and Senning. A few centimetres above the aortic valve the wall of the ascending aorta was abnormal; the vessel was partly occluded by a thickening, of which the main part protruded into the lumen of the aorta. As much as possible of this obstruction was removed, making an opening in the lumen of the aorta slightly wider than an index finger. The aortic cusps were normal. The aortic wall was then sutured. Post-operatively, a systolic murmur of the same type and localization as was heard before operation was present, but it was fainter, of lower frequency, and less harsh. The patient was discharged from hospital in good condition just over three weeks after operation.She was roadmitted to hospital for follow-up examination in September, 1961. She had been asymptomatic since operation, and worked as an assistant hospital nurse on night duty. In 1960 she had given birth to a child. Both pregnancy and delivery were uncomplicated. The heart volume was 400 ml./m.2 of body surface compared with 430 ml./ M.2 pre-operatively. The E.C.G. no longer showed hypertrophy of the left ventricle, and depression of the S-T intervals and T waves was less. At phonocardiography a medium-frequency, diamond-shaped pansystolic murmur was recorded, with a maximum over the second right interspace, but of lower intensity than previously. Angiocardiography showed that a stricture with an inner diameter of 1.5 cm. was still present at the site of the supravalvular stenosis (Fig.

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Supra-valvular Stenosis of the Aorta

Cited by 63 publications

References 3 publications

Successful Surgical Correction of Supravalvular Aortic Stenosis

Successful Surgical Correction of Supravalvular Aortic Stenosis

Vitamin D and the Supravalvar Aortic Stenosis Syndrome

The Surgical Treatment of Supravalvular Aortic Stenosis Based on Two Cases

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