Ann Thorac Surg 1999;67:1167-1169
© 1999 The Society of Thoracic Surgeons
Case Reports
Application of successive principles of repair to correct supravalvular aortic stenosis
René Prêtre, MDa,
Urs Arbenz, MDa,
Paul R. Vogt, MDa,
Marko I. Turina, MD
a Cardiovascular Surgery, University Hospital Zürich, Zürich, Switzerland
Accepted for publication September 27, 1998.
Address reprint requests to Dr Prêtre, Klinik für Herzgefässchirurgie, Universitätsspital, 100 Rämistrasse, 8091 Zürich, Switzerland
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Abstract
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The diffuse form of supravalvular aortic stenosis may extend in the takeoff of the coronary and arch arteries. Relief of the supravalvular stenosis requires a harmonious enlargement of the sinotubular junction to maintain aortic valve competence. The technique we used in a 9-year-old boy involved patch enlargement of all affected structures. Deep hypothermic circulatory arrest and retrograde cerebral perfusion was used during repair of the aortic arch and arch arteries.
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Introduction
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The correction of the diffuse type of congenital supravalvular aortic stenosis may require the application of successive reconstruction principles to the aortic root and ascending aorta, the coronary arteries, the aortic arch, and the arch arteries, as described here.
An asymptomatic 9-year-old boy was referred to our clinic because of a severe and extended form of supravalvular aortic stenosis with additional stenosis at the origin of the left main coronary artery, the innominate, and the left common carotid artery (Fig 1 ). The left ventricle outflow tract, the descending thoracic, and the abdominal aorta, and their branches were normal. Systolic gradient between left ventricle and descending aorta was 120 mm Hg. Chromosome research for Williams-Beuren syndrome was negative.
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Fig 1. Angiogram of the thoracic aorta showing diffuse supravalvular stenosis extending to the descending aorta and stenosis of the innominate and left common carotid artery. The origin of the left main coronary artery, not visible here, was also stenotic.
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The patient was operated under cardiopulmonary bypass (using the external iliac artery as arterial inflow) and deep hypothermia. The heart was arrested with intermittent antegrade and retrograde cold blood cardioplegia. The supravalvular aortic stenosis was sharply delineated, from the sinotubular junction to the descending aorta. The thickened arterial wall extended more than 5 mm in the left coronary ostium, and 15 mm in the innominate and left common carotid arteries. The aortic valve was normal.
During systemic cooling, remodeling of the sinotubular junction and angioplasty of the left main coronary artery were performed. The ascending aorta was cross-clamped and opened longitudinally along its convexity down to the aortic annulus (in the noncoronary sinus). A few millimeters above the sinotubular junction, the anterior half of the aorta was cut transversely and two additional incisions were performed across the fibrotic ridge, in the right and left sinus of Valsalva. Two separate patches of glutaraldehyde-treated autologous pericardium were used to enlarge the sinotubular junction and restore a normal aortic root anatomy (Fig 2 ). Care was applied to obtain a sinotubular junction about the same size of the aortic annulus. With the enlargement of all three sinuses, the commissures of the aortic valve formed an isosceles triangle and the coaptation of the aortic leaflet was excellent. In the left coronary sinus, the incision was extended in the left main coronary artery, beyond the fibrotic thickening of the vessel, and the patch of pericardium used to enlarge to origin of the left main coronary artery. At completion of the aortic root remodeling, deep hypothermia was achieved and circulatory arrest initiated. The aortic cross-clamp was removed and a retrograde, pressure-controlled perfusion of cold blood (with flows between 250 and 300 mL/min) in the superior vena cava instituted. The aortic incision was prolonged over the aortic arch to the descending aorta. Two additional incisions were performed across the origin of the innominate and left common carotid artery and a patch of pericardium was used for enlargement. Finally, the ascending aorta and aortic arch were enlarged with a patch of pericardium (Fig 2). The aorta was deaired, retrograde perfusion discontinued, and cardiopulmonary bypass reestablished. The heart function resumed rapidly. Circulatory arrest, cross-clamp, and cardiopulmonary bypass times were 39, 67, and 160 minutes, respectively. Direct measurement of blood pressure between the aortic root and femoral artery revealed no residual systolic gradient. Postoperative course was uneventful. The patient awoke without neurologic deficit, and was extubated 12 hours after operation. He has remained asymptomatic and, on last control in our clinic 8 months after operation, showed, on echocardiography, a normal anatomy of the aortic root with laminar flow.
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Fig 2. Surgical repair of the supravalvular stenosis with extensive pericardial patching. Insets show the lines of incision and the plasty of the left main coronary artery.
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Comment
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Supravalvular aortic stenosis is the least common form of left ventricle outflow tract obstruction, which, in 85% of patients, appears as a localized form. The individual experience of surgeons with the more challenging diffuse form is, therefore, at best limited to a few patients and, even in the largest published series, the long-term result of the various surgical reparative techniques used is unknown [1–3]. In the patient reported here, four anatomic components were involved in the pathologic process (Fig 1). We choose to tackle them by the successive application of techniques that proved their efficiency and long-term reliability in the individual repair of each component (Fig 2). The sinotubular junction, important to the aortic valve competence, is normally of the same caliber or only slightly larger that the aortic annulus. The enlargement plasty we performed in the three sinuses restored an anatomically normal aortic root with an equidistant positioning of the aortic valve commissures. The enlargement was calibrated to the aortic annulus to avoid excessive dilatation of the sinotubular junction, which, by reducing the coapting surface of the leaflets, could create an aortic valve insufficiency. Because of an associated ostial stenosis, the incision in the left coronary sinus was prolonged in the left coronary artery. The patch used to enlarge the coronary sinus was inferiorly sutured on the arteriotomy borders to relieve the ostial stenosis. The enlargement of the ascending aorta and aortic arch was performed on the convexity of the aorta during a period of deep hypothermic circulatory arrest. Retrograde cerebral perfusion was used to protect the brain during circulatory arrest. The immediate result of the repair was excellent as assessed macroscopically and by operative and postoperative echocardiography (Fig 3 ). The long-term evolution of this repair is, however, less certain because longitudinal and transversal growth of the aorta may be inadequate. Fifty percent of the circumference of the repaired aorta was of original pathologic aortic tissue with unknown potential for growth and the other 50% was of glutaraldehyde-tanned autologous pericardium with no potential for growth.
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Fig 3. Postoperative transthoracic echocardiography showing a normal anatomy of the aortic root and proximal ascending aorta.
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References
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Delius R.E., Steinberg J.B., L’Ecuyer T.B., Doty D.B., Behrendt D.M. 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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Sharma B.K., Fujiwara H., Hallman G.L., Ott D.A., Reul G.L., Cooley D.A. Supravalvar aortic stenosis: a 29-year review of surgical experience. Ann Thorac Surg 1991;51:1031-1039.[Abstract]
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Van Son J.A., Danielson G.K., Puga F.J., et al. Supravalvular aortic stenosis. Long-term results of surgical treatment. J Thorac Cardiovasc Surg 1994;107:103-115.[Abstract/Free Full Text]
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Abstract
Introduction
