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Ann Thorac Surg 1998;65:532
© 1998 The Society of Thoracic Surgeons

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Case Reports

Reconstruction of Supravalvar Aortic Stenosis With Autologous Pulmonary Artery

Department of Cardiovascular Diseases, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia

Accepted for publication September 9, 1997.

Dr Al-Halees, Department of Cardiovascular Diseases, King Faisal Specialist Hospital & Research Centre, MBC-16, PO Box 3354, Riyadh 11211, Saudi Arabia (e-mail: alhalees@kfshrc.edu.sa).

	Abstract

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Use of autologous tissue in corrective cardiac operations offers many advantages including the potential for growth. We report a surgical technique using autologous pulmonary artery in the repair of supravalvar aortic stenosis in a 6-year-old child. At 30 months’ follow-up, the pulmonary arterial tissue shows no evidence of calcification or dilatation and appears to be growing with the aorta.

	Introduction

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Supravalvar aortic stenosis (SVAS) was described in 1930 by Mancarelli [1] and first corrected more than 40 years ago by sewing a prosthetic gusset into the aortotomy across the stenosed region [2]. Although the localized form of SVAS also has been corrected by resection and end-to-end anastomosis, the most widely accepted method probably is the extended aortoplasty described by Doty and colleagues [3]. A combination of these two surgical methods as described by Brom [4] seems to be gaining wider application. We describe a modification of this technique that uses autologous pulmonary arterial tissue, which may confer the benefit of growth with age.

A 6-year-old boy without Williams syndrome was referred to our center for the treatment of symptomatic localized SVAS. Echocardiography and cardiac catheterization showed left ventricular outflow tract obstruction at the sinus ridge of the aortic valve (Fig 1). The systolic peak gradient across the stenosis was 140 mm Hg without aortic valve regurgitation. The aortic valve itself was trileaflet and nonstenotic. There was no supravalvar pulmonary stenosis and the pulmonary valve was normal. The aortic root measured about 16 mm and the aorta measured 6 to 7 mm at its narrowest point. The pulmonary artery measured 20 mm.

View larger version (110K): [in this window] [in a new window]   Preoperative aortic root angiogram showing supravalvar aortic stenosis.

View larger version (27K): [in this window] [in a new window]   The incision made in the aorta (A), excision and shaping of the pulmonary arterial tube (B), and aortic and pulmonary artery reconstruction (C).

At last follow-up 30 months after operation, the patient was well and asymptomatic. There was no gradient across the area of aortic reconstruction by Doppler echocardiography. The aortic sinuses had a normal configuration. The aorta measured 2 cm at the area of the supravalvar ridge (compared with 1.6 cm immediately after repair). The pulmonary artery showed a mild (20-mm Hg) gradient.

	Comment

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The incidence of SVAS is low and its localized form is much more common and easier to treat than the diffuse variety. The localized form of SVAS is not truly localized, but the abnormality is one of the whole aortic root. In addition to the supravalvar stenosis, there is an abnormality of the aortic valve leaflets and sinuses of Valsalva, with adhesions of a portion of the cusps to the supravalvar stenosis [5]. The ideal treatment, therefore, should be directed not only toward complete removal of the SVAS tissue, but also toward widening for the malformed sinuses and correction of any valvular abnormality that may be present.

The results of surgical treatment of the localized form of SVAS are good in terms of survival and symptoms [3]. Because a single gusset repair across the stenosis into the noncoronary sinus does not relieve adequately the other sinus abnormality, Doty and associates [3] devised an extended aortoplasty to allow a symmetric reconstruction of the aortic root. This technique involves dividing the sinus rim at two points and extending the incision into the right and noncoronary sinuses to restore the aortic root to its normal configuration. The left coronary sinus can be corrected by excision or incision of the thickened supravalvar rim [6]. Complete resection with end-to-end anastomosis has the theoretic advantage of preventing recurrence, although it does not restore the aortic root configuration. The benefits of complete resection and extended aortoplasty were combined by Brom [4]. The surgical technique that we present is similar to that of Brom with the exception that the left coronary sinus was not widened in our patient.

The choice of material for the reconstruction of SVAS has varied from Teflon to polytetrafluoroethylene, Dacron, or pericardium. The aortic root is functionally a dynamic structure that will grow with age in children. The use of prosthetic materials temporarily restores the aortic root configuration but may not support the aortic tissue over time and, in the long run, has the disadvantage of not growing with the aorta. In a modification of Brom’s technique [4], Myers and co-workers [7] performed the reconstruction by direct anastomosis of the scalloped distal aorta into the recess of the proximal aortic segment when feasible. This should be the ideal surgical treatment because it involves autologous tissue.

The spectrum of SVAS morphology is wide. We believed that mobilization and direct anastomosis after adequate resection would be difficult in our patient. Applying the principle of using autologous growing tissue, we used the pulmonary arterial tube because we knew that the patient did not have Williams syndrome or supravalvar pulmonary stenosis. In addition to being compliant and affording ease of implantation, this tissue would permit the aortic root to be dynamic and would allow growth. Furthermore, the possibility of thromboembolism and hemolysis would be lower than it is with the use of synthetic materials. However, the pulmonary arterial tube can be used only in the localized form of SVAS because the amount of tissue harvested from the pulmonary artery remains limited. This technique cannot be recommended for patients with Williams syndrome because they may have supravalvar pulmonary stenosis associated with their SVAS.

At 30 months after operation, the pulmonary arterial tissue used for the reconstruction of SVAS in our patient appears to be without dilatation, calcification, or stenosis. It appears to be growing with the aorta. The pulmonary artery anastomosis has demonstrated no problems. Long-term follow-up is required to confirm these results.

	References

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1. Mancarelli L Stenosi sopravalvolare aortica danello. Arch Ital Anat Stol Path 1930;1:829-841.
2. McGoon DC, Mankin HT, Vlad P, Kirlin JW The surgical treatment of supravalvar aortic stenosis. J Thorac Cardiovasc Surg 1961;41:125-133.
3. Doty DB, Polansky DB, Jenson CG Supravalvar aortic stenosis: repair by extended aortoplasty. J Thorac Cardiovasc Surg 1977;74:362-371.[Abstract]
4. Brom AG Obstruction to left ventricular outflow tract. In: Khansari S, ed. Cardiac surgery: safeguards and pitfalls in operative technique. Rockville, MD: Aspen, 1988:276-280.
5. Flaker G, Teske D, Kilman J, Hosier D, Wooley C Supravalvar aortic stenosis: a 20-year clinical perspective and experience with patch aortoplasty. Am J Cardiol 1983;51:256-260.[Medline]
6. Doty DB Supravalvar aortic stenosis. Ann Thorac Surg 1991;51:886-887.[Medline]
7. Myers JL, Waldhausen JA, Cyran SE, Gleason MM, Weber HS, Baylen BG Results of surgical repair of congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1993;105:281-288.[Abstract]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Zohair Al-Halees Ganga Prabhakar Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Al-Halees, Z. Articles by Galal, O. Search for Related Content PubMed PubMed Citation Articles by Al-Halees, Z. Articles by Galal, O.