Ann Thorac Surg 1996;61:1251-1253
© 1996 The Society of Thoracic Surgeons
Case Report
Surgical Treatment of Diffuse Supravalvar Aortic Stenosis
Thierry A. Folliguet, MD,
Loic Macé, MD,
Patrice Dervanian, MD,
Antoine Casasoprana, MD,
Suzel Magnier, MD,
Jean-Yves Neveux, MD
Department of Cardio-Vascular and Pediatric Cardiac Surgery, Centre Chirurgical Marie-Lannelongue, Le Plessis Robinson, France
Accepted for publication October 26, 1995.
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Abstract
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Diffuse supravalvar aortic stenosis can be treated by a variety of surgical approaches. In this case of severe diffuse supravalvar aortic stenosis in a child, we used the combination of an apicoaortic conduit followed 6 years later by aortic valve replacement, replacement of the ascending aorta and aortic arch, and an ascending to thoracic descending aorta bypass graft.
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Introduction
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Supravalvar aortic stenosis (SVAS) is a relatively rare cause of congenital left ventricular outflow obstruction. It is a complex anomaly with thickening of the media and intimal hyperplasia resulting in reduction of the aortic circumference at the sinus rim. Operation to relieve the left ventricular outflow tract obstruction in the localized form is usually acomplished by placing a diamond-shaped patch across the sinus rim in the noncoronary sinus of Valsalva, or by an extended aortoplasty dividing the supravalvular stenosing ring twice at the noncoronary sinus of Valsalva and the right sinus just anterior to the commissure between the right and left coronary cusps [1, 2]. Operations to correct the diffuse form of SVAS are less well defined. We report here a case of diffuse SVAS treated by a combination of surgical approaches.
A 9-year-old boy with Williams syndrome and diffuse SVAS involving the ascending aorta and aortic arch underwent surgical relief of severe aortic stenosis by creation of an apicoaortic shunt with an 18-mm porcine-valved Dacron conduit. We placed the conduit on the descending thoracic aorta.
At age 14 years a progressive diastolic murmur led to a complete hemodynamic and echocardiographic work-up, which revealed a 40 mm Hg gradient between the left ventricle and the conduit associated with 2+ aortic insufficiency. Due to the good physical tolerance of the child we decided to wait. A year later chest roentgenography showed a marked cardiomegaly (Fig 1
), and echocardiography revealed a significant increase in left ventricular dimension and a shortening fraction of 0.14, with 3+ aortic insufficiency. Angiography revealed a normal-sized aortic annulus with severe aortic regurgitation and normally positioned coronary arteries, hypoplasia of the ascending aorta starting above the aortic annulus, involving the entire aortic arch and extending to the beginning of the descending aorta, and moderate stenosis of the origin of the inominate artery and of the left carotid artery (Fig 2). The left ventricle was hypokinetic and dilated, with mild mitral regurgitation. The apicoaortic conduit was patent with a normally functioning valve.
The patient underwent aortic valve replacement with a 21-mm bileaflet mechanical prosthesis, placement of a 24-mm supracoronary conduit extending on the aortic arch with reanastomosis of the arch vessels under deep hypothermia, and removal of the apicoaortic conduit. No associated procedure was performed on the arch vessels because the stenosis was not hemodynamically significant. Total circulatory arrest time was 20 minutes and aortic cross-clamp time was 80 minutes. After coming off bypass, pressures recorded in the ascending and descending thoracic aorta revealed a 30 mm Hg gradient. We then decided to insert a 16-mm Dacron graft between the supracoronary aorta prosthesis, placed in a lateral fashion, and the end of the previously implanted Dacron graft on the descending thoracic aorta (Fig 3). This was performed off cardiopulmonary bypass, before protamine infusion.
The patient subsequently did well. A year later chest roentgenography revealed a normal-sized heart (Fig 4), and echocardiography showed a well-functioning prosthesis and no gradient on the various conduits, with a shortening fraction of 0.34. A computed tomographic scan showed no evidence of hematoma or aneurysm around the prosthesis.
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Comment
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Anatomically three types of SVAS have been described: the hourglass type, the membranous type, and the hypoplastic type. Surgically, however, two types are present: a localized form and a diffuse form. Treatment of the diffuse type is most difficult, with several surgical options available, including patch aortoplasty, left ventricular apex to aorta conduits, aortic resection with graft replacement, and extensive endarterectomy of the ascending aorta and arch with patch aortoplasty. Apicoaortic shunts for left ventricular outflow obstruction have been used by various groups with adequate results [3, 4]. Long-term survivors are mostly seen in the adult population, with reoperation necessary in case of infection, stenosis, or disruption of the conduit.
In this case we used a combination of different surgical approaches. The creation of an apicoaortic shunt was originally performed due to the extremely small ascending aorta at the time, with plan to wait for aortic growth. Subsequently we were able to reconstruct the ascending aorta and arch with a conduit. Extensive endarterectomy with Dacron patch aortoplasty is an option we reserve for cases of diffuse supravalvar aortic stenosis due to luminal narrowing. In this case, only the aortic annulus and proximal ascending aorta grew to adequate size; the rest of the ascending aorta, arch, and the proximal descending thoracic aorta remained hypoplastic. Not to leave any residual gradient between the arch and the descending thoracic aorta, which may further compromise left ventricular function, we placed another shunt between the ascending aorta prosthesis and the descending thoracic aorta. We realize that these operations are only palliative, because the course of this disease is progressive.
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Footnotes
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Address reprint requests to Dr Folliguet, Department of Cardio-Vascular and Pediatric Cardiac Surgery, Centre Chirurgical Marie-Lannelongue, 133 Ave de la Resistance, 92350 Le Plessis Robinson, France.
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References
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- Doty DB, Polansky DB, Jenson CB. Supravalvular aortic stenosis. Repair by extended aortoplasty. J Thorac Cardiovasc Surg 1977;74:362–71.[Abstract]
- Braunstein PW Jr, Sade RM, Crawford FA Jr, Oslizlok PC. Repair of supravalvar aortic stenosis: cardiovascular morphometric and hemodynamic results. Ann Thorac Surg 1990;50:700–7.
- Sweeney MS, Walker WE, Cooley DA, Reul GJ. Apicoaortic conduits for complex left ventricular outflow obstruction: 10 year experience. Ann Thorac Surg 1986;42:609–11.[Abstract]
- Sharma BK, Fujiwara H, Hallman GL, Ott DA, Reul GJ, Cooley DA. Supravalvar aortic stenosis: a 29-year review of surgical experience. Ann Thorac Surg 1991;51:1031–9.
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Abstract
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