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Ann Thorac Surg 1999;67:1147-1148
© 1999 The Society of Thoracic Surgeons


Case Reports

Surgical angioplasty of left main coronary stenosis complicating supravalvular aortic stenosis

Hankei Shin, MDa, Toshiyuki Katogi, MDa, Ryohei Yozu, MDa, Shiaki Kawada, MDa

a Division of Cardiovascular Surgery,Keio University, Tokyo, Japan

Accepted for publication September 8, 1998.

Address reprint requests to Dr Shin, Division of Cardiovascular Surgery, Department of Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan


    Abstract
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 Abstract
 Introduction
 Comment
 References
 
We successfully treated obstruction of the main coronary artery, not aortic valve leaflet adhesion to the intimal shelf, complicating supravalvular aortic stenosis by modifying the Brom aortoplasty. An autologous pericardial patch was used to enlarge the left main coronary artery as well as the stenotic aorta. This modification allows simple and effective restoration of coronary blood flow, while maintaining the Brom procedure’s merit of achieving anatomic geometry of the aortic root in such patients.


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 Abstract
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Several types of surgical repair for supravalvular aortic stenosis have been reported. McGoon and associates [1] successfully performed single patch enlargement of the noncoronary sinus to relieve such stenoses. Doty and associates [2] described an extended aortoplasty reconstructing the right coronary and noncoronary sinuses. Subsequently, they developed a more anatomic repair by adding another incision in the left coronary sinus [3]. Brom [4] has advanced an excellent technique that not only relieves the stenosis but also achieves a more anatomic geometry of the aortic root. We describe a modification of the Brom procedure by which left coronary blood flow is restored without coronary artery bypass grafting in a patient with obstruction of the left coronary ostium complicating supravalvular aortic stenosis.

Through a median sternotomy, the pericardium was resected for later reconstruction of the left main coronary artery and left coronary sinus. After placement of tapes around the aorta and vena cavae, the aorta was cannulated at the level of the brachial artery and bicaval cannulation was performed. Direct introduction of a right-angled cannula into the superior vena cava was used to obtain better exposure of the aortic root. Once cardiopulmonary bypass was initiated, a tape was passed around the main pulmonary artery and dissection between the aorta and the pulmonary artery was carried out as far as the left main coronary artery. To ensure myocardial protection, the right atrium was opened and a retrograde cannula was introduced directly into the coronary sinus. After cardiac arrest was achieved using a combination of antegrade and retrograde cold blood cardioplegia, the aorta was completely transected above the stenotic segment. An incision was made into each sinus across the stenotic ring. The incision was made in the midline of the noncoronary sinus, to the left of the right coronary orifice in the right coronary sinus, and into the left main coronary artery as far as its bifurcation in the left coronary sinus. Patch width was determined as follows: the diameter at the sinus level measured on a preoperative aortogram was 27 mm. According to Kunzelman’s normalization of aortic root dimensions [5], the diameter at the sinotubular junction was expected to be 22 mm. Patch width, excluding margin for suturing, was 12.5 mm to enlarge the 10-mm stenotic segment to 22 mm [ ]. Actually, Hemashield patches (Meadox Medical, Inc., Oakland, NJ) 12.5 mm wide were used to enlarge the noncoronary and right coronary sinuses. To enlarge the left main coronary artery and left coronary sinus, an autologous pericardial patch 18 mm wide was sewn with running polypropylene sutures to avoid residual stenosis of the coronary artery (Fig 1 ). Finally, the size of the transected distal aortic end was adjusted to that of the reconstructed proximal end by making a short longitudinal incision, and the two ends were anastomosed in an end-to-end fashion.



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Fig 1. (A, B) An incision is made into each sinus across the stenotic ring. The incision in the left sinus is extended into the left main coronary artery as far as its bifurcation. (C) Three patches are sewn in to complete the repair.

 
A 17-year-old boy with homozygous familial hypercholesterolemia was referred to us due to anginal pain and dyspnea on exertion. His serum total cholesterol level had been more than 400 mg/dL, despite anticholesterol treatment, up to the age of 16. Since then, the level was controlled within a normal range by adding low-density lipoprotein apheresis once every 2 weeks. Cardiac catheterization revealed supravalvular aortic stenosis, with a 120 mm Hg pressure gradient and mild aortic regurgitation, and a 75% left coronary ostial stenosis.

After this operation, the patient was asymptomatic and returned to a normal school life. Postoperative cardiac catheterization, performed 19 days after the operation, showed the aortic stenosis to be relieved, the reconstructed left main coronary artery widely patent, and no change in the aortic regurgitation.


    Comment
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 Abstract
 Introduction
 Comment
 References
 
Several types of repair for supravalvular aortic stenosis have been proposed to achieve anatomic geometry of the aortic root [24], and favorable long-term results have been reported [6, 7]. In patients whose cases are complicated by obstruction of the left coronary ostium, it is very important to restore the coronary blood flow as well as reconstruct the aortic root. A combination of coronary bypass grafting and repair of the aortic stenosis may be considered, but the longevity of the bypass conduit in a 17-year-old patient with homozygous familial hypercholesteremia is questionable, even if the internal thoracic artery is used. Therefore, we modified the Brom procedure to allow us to enlarge not only the left coronary sinus but also the left main coronary artery. This is a simple and effective method for relief of obstruction of the left main coronary artery complicating supravalvular aortic stenosis while restoring normal anatomic aortic root geometry.


    References
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 Abstract
 Introduction
 Comment
 References
 

  1. McGoon D.C., Mankin H.T., Vlad P., Kirklin J.W. The surgical treatment of supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1961;41:125-133.
  2. Doty D.B., Polansky D.B., Jenson C.B. Supravalvular aortic stenosis: repair by extended aortoplasty. J Thorac Cardiovasc Surg 1977;74:362-371.[Abstract]
  3. Doty BD. Supravalvular aortic stenosis: extended aortoplasty. In: Doty DB, ed. Cardiac surgery: a looseleaf workbook and update service. Chicago: Year Book Medical, 1985:AS-SUPRA 1–3.
  4. Brom A.G. Obstruction to the left ventricular outflow tract. In: Khonsai S., ed. Cardiac surgery: safeguards and pitfalls in operative technique. Rockville, MD: Aspen, 1998:276-280.
  5. Kunzelman K.S., Grande K.J., David T.E., Cochran R.P., Verrier E.D. Aortic root and valve relationships: impact on surgical repair. J Thorac Cardiovasc Surg 1994;107:162-170.[Abstract/Free Full Text]
  6. Delius R.E., Steinberg J.B., L’Ecuyer T., Doty D.B., Behrendt D.M. Long-term follow-up of extended aortoplasty for supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1995;109:155-163.[Abstract/Free Full Text]
  7. Myers J.L., Waldhausen J.A., Cyran S.E., Gleason M.M., Weber H.S., Baylen B.G. Results of surgical repair of congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1993;105:281-288.[Abstract]



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