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Ann Thorac Surg 2001;72:661-662
© 2001 The Society of Thoracic Surgeons
a The New Children’s Hospital, Royal Alexandra Hospital for Children, Parramatta, New South Wales, Australia
Address reprint requests to Dr
Chard, The New Children’s Hospital, Royal Alexandra Hospital for
Children, Parramatta, New South Wales 2124,
Australia
e-mail: chardric{at}netspace.net.au
As Originally Published in 1993: Localized Supravalvar Aortic Stenosis: A New Technique for Repair by Richard B. Chard, FRACS, and Timothy B. Cartmill, FRACS. Department of Cardiac Surgery, Royal Alexandra Hospital for Children, Sydney, New South Wales, Australia.
The deformity in supravalvar aortic stenosis includes generalized thickening of the tissues of the aortic root with a fairly normal sized aortic valve ring. The thickened tissues may obstruct the origins of the left and right coronary arteries. We have repaired 2 cases with complete excision of the stenosing ring below the level of the commissures of the aortic valve into the sinuses, to the level of the left and right coronary arteries.
Updated in 2001:
We now have long-term follow-up data for 3 patients who have had supravalvar aortic stenosis (AS) corrected by the method described in 1993 [1] which entails (1) complete excision of the stenosing ring below the level of the commissures of the aortic valve into the sinuses, to the level of the left and right coronary arteries, (2) extensive mobilization of the aortic arch, and (3) direct end-to-end anastomosis of the aortic root to the distal ascending aorta.
Patient 1 from our original report underwent correction of supravalvar AS at 6 months of age. Preoperative cardiac catheterization had demonstrated a gradient of 120 mm Hg. Cardiac catheterization at age 19 months demonstrated no supravalvar gradient and trivial aortic regurgitation. The patient subsequently developed significant subvalvar stenosis which was resected at age 5 via a trans-aortic route. However, 3 years later, the subaortic stenosis recurred more extensively, with a tunnel-like obstruction from opposite the papillary muscles to the base of the aortic valve, and at age 8, he again underwent resection and patch augmentation of the left ventricular outflow tract via a right ventriculotomy. Echocardiogram 3 months after the last operation revealed no significant subvalvar obstruction, a 35 mm Hg gradient across the aortic valve, and mild to moderate aortic regurgitation.
Patient 2 from our original report had William’s syndrome and underwent operation for supravalvar AS at age 2 years 5 months. The stenosing ring caused compression of the proximal left main coronary artery. Cardiac catheterization demonstrated a gradient of 100 mm Hg and severe left main coronary ostial obstruction. Follow-up echocardiography at age 7 years 4 months revealed a residual gradient of 25 mm Hg and no significant aortic regurgitation, but marked mitral valve prolapse with moderate mitral regurgitation.
Since our original report, one other patient, a girl aged 12 years whose mother had had surgery for the same condition, has undergone this technique of correction for supravalvar AS. She had a discrete supravalvar stenosing ring with an 8-mm lumen and typical fibrous thickening of the aorta above and below the ring. Preoperative echocardiogram revealed a gradient of 65 mm Hg. At last follow-up age 16 years 9 months, echocardiogram demonstrated no significant gradient, trivial aortic regurgitation, and regression of left ventricular hypertrophy.
Six other patients have had supravalvar AS corrected at our institution since 1993 by other methods. Three had a direct end-to-end interlacing anastomosis using the technique described by Myers and colleagues [2]. All three had Williams’ syndrome. A 14-month-old girl with a preoperative gradient of 70 mm Hg died in hospital. She required reoperation for bleeding at the aortic suture line and subsequently developed low cardiac output syndrome with multiorgan failure. The other two have improved and on echocardiogram have residual gradients of 25 mm Hg and 10 mm Hg respectively. Three other patients have had patch repair for correction of supravalvar AS. Two had diffuse hypoplasia of the aortic arch, in addition to discrete to discrete supravalvar AS. Direct end-to-end anastomosis was not technically feasible and augmentation with a pantaloon pericardial patch was therefore used. One of these patients required grafting of the right coronary artery with a right internal mammary artery owing to distortion of the right coronary origin by the patch. Her outcome was positive with a 20 mm Hg residual supraaortic gradient. The other patient underwent further surgery 2 years later for patch augmentation of the hypoplastic aortic arch. She had a 16 mm Hg gradient across the supraaortic region 5 years after the original operation. The third patient also had repair using pericardial patch augmentation because of right coronary origin above the level of the commissures. At 5 years postoperatively, he has no residual supravalvar obstruction but has a 20 mm Hg gradient across the aortic valve.
In conclusion, supravalvar AS is a condition which is commonly associated with obstruction in other sites along the left ventricular outflow tract and aorta. We have observed such multilevel obstruction developing progressively over follow-up. Two of the 9 patients in this series required reoperation. We believe that repair by direct end-to-end anastomosis using the technique described, can be achieved in selected cases of discrete supravalvar AS. Our follow-up echocardiographic studies suggest that relief of obstruction is sustained up to 8 years. We have had less success with other methods of direct repair and patch repair. Therefore, we continue to use the technique described by us whenever feasible.
References
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