Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Subvalvar aortic stenosis (subaortic stenosis)

David W Brown, MD
Section Editor
David R Fulton, MD
Deputy Editor
Carrie Armsby, MD, MPH


Left ventricular outflow tract (LVOT) obstructive lesions account for approximately 6 percent of cases of congenital heart disease in children; in one series, the incidence was estimated to be 6 in 10,000 live births [1,2]. Obstruction may occur at valvar (which is by far the most common), subvalvar, and supravalvar levels.

Subvalvar aortic stenosis (also called subaortic stenosis) will be reviewed here. Valvar and supravalvar aortic stenosis are discussed separately. (See "Valvar aortic stenosis in children" and "Supravalvar aortic stenosis".)

Subvalvar aortic stenosis can be induced by a fixed stenosis, or may have a dynamic component that is primarily due to the genetic disease hypertrophic cardiomyopathy (previously called idiopathic hypertrophic subaortic stenosis). Only fixed stenosis will be reviewed here; subvalvar obstruction due to hypertrophic cardiomyopathy is presented separately. (See "Hypertrophic cardiomyopathy: Morphologic variants and the pathophysiology of left ventricular outflow tract obstruction".)


Subvalvar aortic stenosis (AS) is the second most common form of AS. Among children with congenital AS, subvalvar AS accounts for 10 to 14 percent of cases [2,3]. Similar to valvar AS, subvalvar AS is more common in males, who account for 67 to 75 percent of cases [2,3].

Other cardiac malformations occur in 50 to 65 percent of cases [4-6]. One report of 35 patients found the following lesions: patent ductus arteriosus (34 percent), ventricular septal defect (VSD, 20 percent), aortic coarctation (23 percent), pulmonic stenosis (9 percent), and miscellaneous other lesions (14 percent) [4].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Oct 26, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Hoffman JI, Christianson R. Congenital heart disease in a cohort of 19,502 births with long-term follow-up. Am J Cardiol 1978; 42:641.
  2. Kitchiner D, Jackson M, Malaiya N, et al. Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960-91): a study of 313 patients. Br Heart J 1994; 71:588.
  3. Liu CW, Hwang B, Lee BC, et al. Aortic stenosis in children: 19-year experience. Zhonghua Yi Xue Za Zhi (Taipei) 1997; 59:107.
  4. Leichter DA, Sullivan I, Gersony WM. "Acquired" discrete subvalvular aortic stenosis: natural history and hemodynamics. J Am Coll Cardiol 1989; 14:1539.
  5. Newfeld EA, Muster AJ, Paul MH, et al. Discrete subvalvular aortic stenosis in childhood. Study of 51 patients. Am J Cardiol 1976; 38:53.
  6. Choi JY, Sullivan ID. Fixed subaortic stenosis: anatomical spectrum and nature of progression. Br Heart J 1991; 65:280.
  7. Cicini MP, Giannico S, Marino B, et al. "Acquired" subvalvular aortic stenosis after repair of a ventricular septal defect. Chest 1992; 101:115.
  8. Freedom RM, Pelech A, Brand A, et al. The progressive nature of subaortic stenosis in congenital heart disease. Int J Cardiol 1985; 8:137.
  9. Kleinert S, Geva T. Echocardiographic morphometry and geometry of the left ventricular outflow tract in fixed subaortic stenosis. J Am Coll Cardiol 1993; 22:1501.
  10. Gewillig M, Daenen W, Dumoulin M, Van der Hauwaert L. Rheologic genesis of discrete subvalvular aortic stenosis: a Doppler echocardiographic study. J Am Coll Cardiol 1992; 19:818.
  11. Barkhordarian R, Wen-Hong D, Li W, et al. Geometry of the left ventricular outflow tract in fixed subaortic stenosis and intact ventricular septum: an echocardiographic study in children and adults. J Thorac Cardiovasc Surg 2007; 133:196.
  12. Urbach J, Glaser J, Balkin J, et al. Familial membranous subaortic stenosis. Cardiology 1985; 72:214.
  13. Pyle RL, Patterson DF, Chacko S. The genetics and pathology of discrete subaortic stenosis in the Newfoundland dog. Am Heart J 1976; 92:324.
  14. Latson LA. Aortic stenosis: valvar, supravalvar, and fibromuscular subvalvar. In: The Science and Practice of Pediatric Cardiology, Garson A, Bricker JT, Fisher DJ, Neish SR (Eds), Williams and Wilkins, Baltimore 1998. p.1257.
  15. Brauner R, Laks H, Drinkwater DC Jr, et al. Benefits of early surgical repair in fixed subaortic stenosis. J Am Coll Cardiol 1997; 30:1835.
  16. Katz NM, Buckley MJ, Liberthson RR. Discrete membranous subaortic stenosis. Report of 31 patients, review of the literature, and delineation of management. Circulation 1977; 56:1034.
  17. Maizza AF, Ho SY, Anderson RH. Obstruction of the left ventricular outflow tract: anatomical observations and surgical implications. J Heart Valve Dis 1993; 2:66.
  18. Brown JW, Stevens LS, Holly S, et al. Surgical spectrum of aortic stenosis in children: a thirty-year experience with 257 children. Ann Thorac Surg 1988; 45:393.
  19. Maron BJ, Redwood DR, Roberts WC, et al. Tunnel subaortic stenosis: left ventricular outflow tract obstruction produced by fibromuscular tubular narrowing. Circulation 1976; 54:404.
  20. McMahon CJ, Gauvreau K, Edwards JC, Geva T. Risk factors for aortic valve dysfunction in children with discrete subvalvar aortic stenosis. Am J Cardiol 2004; 94:459.
  21. Currie PJ, Hagler DJ, Seward JB, et al. Instantaneous pressure gradient: a simultaneous Doppler and dual catheter correlative study. J Am Coll Cardiol 1986; 7:800.
  22. Bengur AR, Snider AR, Serwer GA, et al. Usefulness of the Doppler mean gradient in evaluation of children with aortic valve stenosis and comparison to gradient at catheterization. Am J Cardiol 1989; 64:756.
  23. Wright GB, Keane JF, Nadas AS, et al. Fixed subaortic stenosis in the young: medical and surgical course in 83 patients. Am J Cardiol 1983; 52:830.
  24. Chaikhouni A, Crawford FA Jr, Sade RM, et al. Discrete subaortic stenosis. Clin Cardiol 1984; 7:289.
  25. de Vries AG, Hess J, Witsenburg M, et al. Management of fixed subaortic stenosis: a retrospective study of 57 cases. J Am Coll Cardiol 1992; 19:1013.
  26. Oliver JM, González A, Gallego P, et al. Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgitation. J Am Coll Cardiol 2001; 38:835.
  27. van der Linde D, Takkenberg JJ, Rizopoulos D, et al. Natural history of discrete subaortic stenosisin adults: a multicentre study. Eur Heart J 2013; 34:1548.
  28. Coleman DM, Smallhorn JF, McCrindle BW, et al. Postoperative follow-up of fibromuscular subaortic stenosis. J Am Coll Cardiol 1994; 24:1558.
  29. Freedom RM. The long and the short of it: some thoughts about the fixed forms of left ventricular outflow tract obstruction. J Am Coll Cardiol 1997; 30:1843.
  30. Douville EC, Sade RM, Crawford FA Jr, Wiles HB. Subvalvar aortic stenosis: timing of operation. Ann Thorac Surg 1990; 50:29.
  31. Gersony WM. Natural history of discrete subvalvar aortic stenosis: management implications. J Am Coll Cardiol 2001; 38:843.
  32. Anderson BR, Tingo JE, Glickstein JS, et al. When Is It Better to Wait? Surgical Timing and Recurrence Risk for Children Undergoing Repair of Subaortic Stenosis. Pediatr Cardiol 2017; 38:1106.
  33. Karamlou T, Gurofsky R, Bojcevski A, et al. Prevalence and associated risk factors for intervention in 313 children with subaortic stenosis. Ann Thorac Surg 2007; 84:900.
  34. Binet JP, Losay J, Demontoux S, et al. Subvalvular aortic stenosis: long-term surgical results. Thorac Cardiovasc Surg 1983; 31:96.
  35. Vogt J, Dische R, Rupprath G, et al. Fixed subaortic stenosis: an acquired secondary obstruction? A twenty-seven year experience with 168 patients. Thorac Cardiovasc Surg 1989; 37:199.
  36. Pickard SS, Geva A, Gauvreau K, et al. Long-term outcomes and risk factors for aortic regurgitation after discrete subvalvular aortic stenosis resection in children. Heart 2015; 101:1547.
  37. Hirata Y, Chen JM, Quaegebeur JM, Mosca RS. The role of enucleation with or without septal myectomy for discrete subaortic stenosis. J Thorac Cardiovasc Surg 2009; 137:1168.
  38. Serraf A, Zoghby J, Lacour-Gayet F, et al. Surgical treatment of subaortic stenosis: a seventeen-year experience. J Thorac Cardiovasc Surg 1999; 117:669.
  39. Dodge-Khatami A, Schmid M, Rousson V, et al. Risk factors for reoperation after relief of congenital subaortic stenosis. Eur J Cardiothorac Surg 2008; 33:885.
  40. Geva A, McMahon CJ, Gauvreau K, et al. Risk factors for reoperation after repair of discrete subaortic stenosis in children. J Am Coll Cardiol 2007; 50:1498.
  41. van der Linde D, Roos-Hesselink JW, Rizopoulos D, et al. Surgical outcome of discrete subaortic stenosis in adults: a multicenter study. Circulation 2013; 127:1184.
  42. Jou CJ, Etheridge SP, Minich LL, et al. Long-term Outcome and Risk of Heart Block After Surgical Treatment of Subaortic Stenosis. World J Pediatr Congenit Heart Surg 2010; 1:15.
  43. Jahangiri M, Nicholson IA, del Nido PJ, et al. Surgical management of complex and tunnel-like subaortic stenosis. Eur J Cardiothorac Surg 2000; 17:637.