Surgical strategies and outcomes of congenital supravalvular aortic stenosis
Hongli Liu MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorBotao Gao MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorQi Sun MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorXinwei Du MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorYanjun Pan MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorZhongqun Zhu MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorCorresponding Author
Xiaomin He MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Correspondence
Xiaomin He MD, PhD and Jinghao Zheng MD. PhD, Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, 1678 Dongfang Road, Shanghai 200127, China.
Email: [email protected] (X.H); [email protected] (J.Z)
Search for more papers by this authorCorresponding Author
Jinghao Zheng MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Correspondence
Xiaomin He MD, PhD and Jinghao Zheng MD. PhD, Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, 1678 Dongfang Road, Shanghai 200127, China.
Email: [email protected] (X.H); [email protected] (J.Z)
Search for more papers by this authorHongli Liu MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorBotao Gao MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorQi Sun MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorXinwei Du MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorYanjun Pan MD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorZhongqun Zhu MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Search for more papers by this authorCorresponding Author
Xiaomin He MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Correspondence
Xiaomin He MD, PhD and Jinghao Zheng MD. PhD, Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, 1678 Dongfang Road, Shanghai 200127, China.
Email: [email protected] (X.H); [email protected] (J.Z)
Search for more papers by this authorCorresponding Author
Jinghao Zheng MD, PhD
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Correspondence
Xiaomin He MD, PhD and Jinghao Zheng MD. PhD, Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, 1678 Dongfang Road, Shanghai 200127, China.
Email: [email protected] (X.H); [email protected] (J.Z)
Search for more papers by this authorAbstract
Background
Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus upon the optimal approach. This study reviewed our institutional experience in the management of SVAS.
Methods
Ninety patients undergoing surgery for SVAS were identified between 2009 and 2016. Based on surgical techniques, patients were divided into three groups: McGoon repair (n = 63), Doty repair (n = 24), and Brom repair (n = 3). Median follow-up was 38.5 months (range, 4 months-7.5 years). Patient status, cumulative event-free survival rate, and risk factors for adverse clinical outcomes were assessed.
Results
The early mortality rate was 3.3%. There was one late death and two reinterventions. No differences were observed among three surgical groups. Event-free survival was 98.4% at 3 years and 96.5% at 5 years. Diffuse-type SVAS and a preoperative gradient greater than 60 mmHg were risk factors for adverse cardiac remodeling within 6 months post-surgery. Residual aortic stenosis was associated with male gender, preoperative aortic valve stenosis, and a preoperative peak gradient greater than 90 mmHg. Eleven patients (out of 30) who underwent concomitant pulmonary artery patching had a residual pulmonary gradient greater than 40 mmHg.
Conclusions
Surgical repair of SVAS can be safely achieved using different techniques, with similar midterm mortality and reintervention rates. Higher preoperative gradient is associated with worse clinical results. Issues regarding surgical timing and concomitant pulmonary artery stenosis need to be further addressed.
REFERENCES
- 1 Williams JC, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation. 1961; 24: 1311–1318.
- 2 Stamm C, Friehs I, Ho SY, et al. Congenital supravalvar aortic stenosis: a simple lesion? Eur J Cardiothorac Surg. 2001; 19: 195–202.
- 3 Merla G, Brunetti-Pierri N, Piccolo P, et al. Supravalvular aortic stenosis: elastin arteriopathy. Circ Cardiovasc Genet. 2012; 5: 692–696.
- 4 O'Connor WN, Davis JB, Jr., Geissler R, et al. Supravalvular aortic stenosis. Clinical and pathologic observations in six patients. Arch Pathol Lab Med. 1985; 109: 179–185.
- 5 Giddins NG, Finley JP, Nanton MA, et al. The natural course of supravalvar aortic stenosis and peripheral pulmonary artery stenosis in Williams's syndrome. Br Heart J. 1989; 62: 315–319.
- 6 Wang CC, Hwu WL, Wu ET, et al. Outcome of pulmonary and aortic stenosis in Williams-Beuren syndrome in an Asian cohort. Acta Paediatr. 2007; 96: 906–909.
- 7 Rastelli GC, McGoon DC, Ongley PA, et al. Surgical treatment of supravalvular aortic stenosis. Report of 16 cases and review of literature. J Thorac Cardiovasc Surg. 1966; 51: 873–872.
- 8 Doty DB, Polansky DB, Jenson CB. Supravalvular aortic stenosis. Repair by extended aortoplasty. J Thorac Cardiovasc Surg. 1977; 74: 362–371.
- 9 Brom AG. Obstruction of the left ventricular outflow tract. In: Khonsari S, ed. Cardiac Surgery: Safeguards and Pitfalls in Operative Technique. 1988; Rockville, MD: Aspen Publishers, p. 276–280.
- 10 Myers JL, Waldhausen JA, Cyran SE, et al. Results of surgical repair of congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg. 1993; 105: 281–287. discussion 87–88.
- 11 Seo D, Shin H, Park J, et al. Modified simple sliding aortoplasty for supravalvar aortic stenosis. Ann Thorac Surg. 2007; 83: 2248–2250.
- 12 Hazekamp MG, Kappetein AP, Schoof PH, et al. Brom's three-patch technique for repair of supravalvular aortic stenosis. J Thorac Cardiovasc Surg. 1999; 118: 252–258.
- 13 Metton O, Ben AW, Calvaruso D, et al. Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results? Ann Thorac Surg. 2009; 88: 588–593.
- 14 Kaushal S, Backer CL, Patel S, et al. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty. Ann Thorac Surg. 2010; 89: 1371–1377.
- 15 Scott DJ, Campbell DN, Clarke DR, et al. Twenty-year surgical experience with congenital supravalvar aortic stenosis. Ann Thorac Surg. 2009; 87: 1501–1507. Discussion 07–08.
- 16 Lev M. Autopsy diagnosis of congenitally malformed hearts. Springfield, IL: Thomas Publishers; 1953. p. 195.
- 17Huang G. Pediatric Echocardiography. Shanghai Science and Technology Publishers, 2015. p. 574 (in Chinese).
- 18 Kantor PF, Lougheed J, Dancea A, et al. Presentation, diagnosis, and medical management of heart failure in children: Canadian Cardiovascular Society guidelines. Can J Cardiol. 2013; 29: 1535–1552.
- 19 Kavarana MN, Riley M, Sood V, et al. Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique. Ann Thorac Surg. 2012; 93: 1274–1278. Discussion 78–79.
- 20 Deo SV, Burkhart HM, Dearani JA, et al. Supravalvar aortic stenosis: current surgical approaches and outcomes. Expert Rev Cardiovasc Ther. 2013; 11: 879–890.
- 21 Fricke TA, d'Udekem Y, Brizard CP, et al. Surgical repair of supravalvular aortic stenosis in children with Williams syndrome: a 30-year experience. Ann Thorac Surg. 2015; 99: 1335–1341.
- 22 Brown JW, Ruzmetov M, Vijay P, Turrentine MW. Surgical repair of congenital supravalvular aortic stenosis in children. Eur J Cardiothorac Surg. 2002; 21: 50–56.
- 23 Stamm C, Kreutzer C, Zurakowski D, et al. Forty-one years of surgical experience with congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg. 1999; 118: 874–885.
- 24 Deo SV, Burkhart HM, Schaff HV, et al. Late outcomes for surgical repair of supravalvar aortic stenosis. Ann Thorac Surg. 2012; 94: 854–859.
- 25 Kramer P, Absi D, Hetzer R, et al. Outcome of surgical correction of congenital supravalvular aortic stenosis with two- and three-sinus reconstruction techniques. Ann Thorac Surg. 2014; 97: 634–640.
- 26 Mitchell MB, Goldberg SP. Supravalvar aortic stenosis in infancy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011; 14: 85–91.
- 27 Zybach-Benz RE, Aeschbacher BC, Schwerzmann M. Impact of left ventricular hypertrophy late after aortic valve replacement for aortic stenosis on cardiovascular morbidity and mortality. Int J Cardiol. 2006; 109: 41–47.
- 28 Greutmann M, Tobler D, Sharma NC, et al. Cardiac outcomes in adults with supravalvar aortic stenosis. Eur Heart J. 2012; 33: 2442–2450.
- 29 Brady TM. The role of obesity in the development of left ventricular hypertrophy among children and adolescent. Curr Hypertens Rep. 2016; 18: 1–7.
- 30 Buddhe S, Du W, Walters HL, 3rd, et al. Predictors of left ventricular remodeling after aortic valve replacement in pediatric patients with isolated aortic regurgitation. Congenit Heart Dis. 2013; 8: 167–173.
- 31 Xu Z, Quiuxia S, Mei J. Long-term outcomes after mechanical aortic valve replacement with aortic root enlargement in adolescents. J Card Surg. 2017; 32: 133–137.
- 32 Stamm C, Friehs I, Moran AM, et al. Surgery for bilateral outflow tract obstruction in elastin arteriopathy. J Thorac Cardiovasc Surg. 2000; 120: 755–763.
- 33
Gandy L,
Tweddell S,
Pelech N. How we approach peripheral pulmonary stenosis in Williams-Beuren syndrome.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu.
2009;
12: 118–121.
10.1053/j.pcsu.2009.01.012 Google Scholar
- 34 Patel ND, Kenny D, Gonzalez I, et al. Single-center outcome analysis comparing reintervention rates of surgical arterioplasty with stenting for branch pulmonary artery stenosis in a pediatric population. Pediatr Cardiol. 2014; 35: 419–422.
- 35 Minakata K, Nishimura K, Nomoto S, et al. Surgical repair for supravalvular aortic stenosis: intermediate to long-term follow-up. J Card Surg. 1997; 12: 398–402.
- 36 Kawamoto N, Hoashi T, Kagisaki K, et al. Myers' 3-sinus reconstruction for supravalvular aortic stenosis involving left coronary ostium: report of a case. Surg Today. 2015; 45: 1064–1066.
- 37 Shiohama T, Fujii K, Ebata R, et al. Coronary ostium occlusion by coronary cusp displacement in Williams syndrome. Pediatr Int. 2016; 58: 487–490.
