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Discrete supravalvular aortic stenosis in children: Is it necessary to reconstruct the whole aortic root? [Anatol J Cardiol]
Anatol J Cardiol. 2009; 9(4): 311-317

Discrete supravalvular aortic stenosis in children: Is it necessary to reconstruct the whole aortic root?

Ergin Koçyıldırım1, Süleyman Özkan2, Demet Karadağ3, S. Kenan Köse4, Enver Ekici5, Coşkun İkizler6
1Department of Cardiothoracic Surgery, University of Pittsburgh, Children’s Hospital of Pittsburgh, Pittsburgh, PA
2Başkent Universitesi Hastanesi, Kalp Damar Cerrahi Kliniği, Ankara, Türkiye
3Department of Radiology, Medical Faculty, University of Ufuk, Ankara, Turkey
4Department of Biostatistics, Medical Faculty, University of Ankara, Ankara, Turkey
5Department of Pediatric Cardiology, Medical Faculty, University of Ufuk, Ankara, Turkey
6Department of Cardiovascular Surgery, Alkan Hospital, Ankara

Objective: Discrete supravalvular aortic stenosis (SAS) is known to involve the whole aortic root. Some surgeons have therefore changed their approach from relief of obstruction using a single-patch to symmetric reconstruction of the whole aortic root – three-patch technique. The advantages are said to be preserved long-term aortic valve function and allowance for growth. This is unproven. We compare growth and aortic root geometry in patients who have undergone relief of discrete SAS using either single-or three-patch technique. Methods: Twenty-five patients (14 male, 11 female, mean age of 11± 4 years, range 4-18) underwent surgery for discrete SAS. No patients with diffuse SAS were included in this retrospective analysis. Twelve patients had features of Williams syndrome. Five patients had other concomitant procedures. A single-patch was inserted into the longitudinal incision, which passed across the stenosis into the non-coronary sinus in 14. A three-patch technique was used in 11 patients. Changes in aortic root following repair were documented in patients using both echocardiography and magnetic resonance imaging (MRI). Results: There were no operative deaths. The mean preoperative gradient was 66 ±17 mmHg (range 50 – 100 mmHg), which decreased to 14±7 mmHg (range 4-18 mmHg) early postoperatively. The late mean gradient was 15±5 mmHg. There was no significant difference in the incidence of postoperative aortic regurgitation or gradient across the repair between two techniques according to the echocardiograms and MRI findings. Conclusion: According to our study, we cannot demonstrate any benefit in reconstructing the whole aortic root for discrete SAS. A single-patch technique is easy, safe and appears durable.

Keywords: Discrete supravalvar aortic stenosis, left ventricular outflow tract obstruction, surgical procedures


Ergin Koçyıldırım, Süleyman Özkan, Demet Karadağ, S. Kenan Köse, Enver Ekici, Coşkun İkizler. Discrete supravalvular aortic stenosis in children: Is it necessary to reconstruct the whole aortic root?. Anatol J Cardiol. 2009; 9(4): 311-317


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