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Ann Thorac Surg 1998;65:277
© 1998 The Society of Thoracic Surgeons

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How To Do It

Supravalvular Aortic Stenosis: A Modification of Extended Aortoplasty

Division of Cardiothoracic Surgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA

Accepted for publication July 28, 1997.

Dr Delius, 1616A John Colloton Pavilion, University of Iowa Hospitals and Clinics, Iowa City, IA 52242.

	Abstract

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A modification of the Doty extended aortoplasty for supravalvular aortic stenosis has been recently adopted. This modification, which entails placement of an additional patch in the left coronary sinus, results in a more symmetric aortic root. This technique has been applied with success to 3 patients.

	Introduction

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Supravalvular aortic stenosis is an uncommon form of aortic stenosis. This results in not only a hemodynamically significant stenosis but also distortion of the valve commissures and, consequently, abnormal aortic valve geometry and coaptation of the aortic leaflets. We have routinely performed an extended aortoplasty as originally described by Doty and associates [1]. This technique, however, only reconstructs the right and noncoronary sinuses. The remaining left coronary sinus still has significant distorted anatomy, and also has the potential risk of limited inflow into the left coronary sinus and subsequent ischemia. Reconstruction of three sinuses has been advocated by Brom [2] and Myers and associates [3] and may allow for significant improvement in the aortic valve geometry and function [2][3]. We describe a variation of the Doty technique that also allows easy reconstruction of all three sinuses.

	Material and Methods

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Three patients with the discrete type of supravalvular aortic stenosis underwent repair at the University of Iowa Hospitals and Clinics using a modification of the extended aortoplasty described by Doty and associates [1]. All patients survived the operation and had no postoperative complications. Long-term follow-up revealed low postoperative gradients and no evidence of aortic insufficiency.

Patient 1
A 13-year-old girl presented with angina and exercise intolerance. Left ventricular hypertrophy was present by electrocardiographic criteria. Cardiac catheterization revealed discrete supravalvular aortic stenosis with a gradient of 55 mm Hg. Operative correction was performed using the Doty extended aortoplasty and, in addition, a patch across the ridge in the left coronary sinus. The patient had a routine uncomplicated postoperative course. On postoperative day 3 the aortic gradient was measured to be 3 mm Hg by echocardiography. Follow-up 14 months after the operation revealed no evidence of cardiomegaly or left ventricular hypertrophy by chest roentgenography and electrocardiography and no gradient by echocardiography.

Patient 2
A 31-year-old man with Williams’ syndrome presented with exercise intolerance. Chest roentgenography and electrocardiography revealed cardiomegaly and left ventricular hypertrophy. Cardiac catheterization revealed discrete supravalvular aortic stenosis with a gradient of 70 mm Hg. Operative correction was performed using the Doty extended aortoplasty and left coronary sinus patch. One year later an echocardiogram demonstrated no gradient and no evidence of aortic insufficiency.

Patient 3
A 2-month-old infant presented with congestive heart failure due to severe aortic stenosis. An echocardiogram demonstrated stenosis at the valvular and supravalvular level. Chest roentgenography and electrocardiography revealed cardiomegaly and biventricular hypertrophy. Cardiac catheterization demonstrated a gradient of 84 mm Hg across the left ventricular outflow tract. At operation significant sinotubular stenosis was noted. The aortic valve was trileaflet and very dysplastic. An aortic valvotomy was performed and the supravalvular stenosis was managed as described. A postoperative echocardiogram demonstrated a gradient of 29 mm Hg across the left ventricular outflow tract, largely at the valvular level. The patient is clinically well 4 months after operation. An echocardiogram revealed a gradient of 18 mm Hg across the left ventricular outflow tract.

Operative Technique
Through a median sternotomy the ascending aorta is cannulated near the takeoff of the inominate artery. A single atrial cannula is inserted and cardiopulmonary bypass is initiated. A left ventricular vent is inserted. Cardiac arrest is achieved using cold antegrade cardioplegic solution. An inverted Y-shaped incision is made in the ascending aorta, with the two arms of the Y extending across the supravalvular ring into the noncoronary and right sinuses of Valsalva. The incisions cross the fibrosing ring 90 degrees apart (Fig 1). The incision into the right coronary sinus is placed to the left side of the right coronary ostium (Fig 2). A longitudinal incision is made across the ridge in the left coronary sinus, just to the right of the left coronary ostium (Fig 2, inset). An oval 0.4-mm Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) patch is cut and sewn in place using a running monofilament suture (Fig 3A). A second Gore-Tex fabric patch is fashioned into a pantaloon shape and sewn into the original aortotomy with running monofilament suture (Fig 3B).

View larger version (65K): [in this window] [in a new window]   End-on view of incisions through supravalvular ridge.

View larger version (103K): [in this window] [in a new window]   An inverted Y incision is first made on the anterior ascending aorta. The inset demonstrates the location of the incision made across the supravalvular ridge above the left coronary sinus.

View larger version (112K): [in this window] [in a new window]   (A) An oval patch is sewn in the left coronary sinus. (B) Final appearance of ascending aorta after extended aortoplasty.

	Comment

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To improve the geometry of the aortic sinuses, reconstruction of all three sinuses has been proposed [2][3]. To accomplish this, we have recently incorporated an additional incision across the supravalvular fibrosing ring in the left coronary sinus of Valsalva. Insertion of an oval patch at this site allows better alignment of the commissures of the aortic valve, further improving the geometry of the valve. Hopefully, this will improve long-term results and decrease the need for further operations.

Long-term follow-up will be needed to determine if the repair described improves the aortic valve anatomy significantly enough to decrease the incidence of postoperative aortic stenosis and insufficiency and result in fewer reoperations.

	References

Top Abstract Introduction Material and Methods Comment References

 

1. Doty DB, Polansky DB, Jenson CB Supravalvular aortic stenosis: repair by extended aortoplasty. J Thorac Cardiovasc Surg 1977;74:362-371.[Abstract]
2. Kirklin JW, Barratt-Boyes BG Cardiac surgery, 2nd ed. New York: Churchill Livingstone, 1993:1228-1231.
3. Myers JL, Waldhausen JA, Cyran SE, Gleason MM, Weber HS, Bayler BG Results of surgical repair of congenital supravalvar aortic stenosis. J Thorac Cardiovasc Surg 1993;1055:281-288.
4. McGoon DC, Mankin HT, Vlad P, Kirklin JW The surgical treatment of supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1961;41:125-133.
5. Delius RE, Steinberg JB, L’Ecuyer T, Doty DB, Behrendt DM Long-term follow-up of extended aortoplasty for supravalvular stenosis. J Thorac Cardiovasc Surg 1995;109:155-163.[Abstract/Free Full Text]

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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): John B. Steinberg Ralph E. Delius Douglas M. Behrendt Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Steinberg, J. B. Articles by Behrendt, D. M. Search for Related Content PubMed PubMed Citation Articles by Steinberg, J. B. Articles by Behrendt, D. M.