Abstract
Left ventricular outflow tract obstruction represents a wide spectrum of disease, including subvalvar aortic stenosis, valvar aortic stenosis, supravalvar aortic stenosis, coarctation of the aorta, and interrupted aortic arch. In some cases, these disorders may manifest in the fetal and early neonatal period, requiring early medical management or urgent surgical or catheter-based intervention. Patients presenting later often have chronic left ventricular pressure overload, compensatory hypertrophy with altered ventricular diastolic properties, and arterial hypertension. Most of these conditions are easily diagnosed by physical examination, electrocardiography, chest radiography, and two-dimensional echocardiography with Doppler. Diagnostic catheterization may be helpful if the diagnosis is in question, for precise determination of pressure gradients, or for providing further anatomic detail via angiography. The threshold for invasive therapy varies with the location of the obstruction and its natural history. Catheter-based intervention is commonly utilized for valvar aortic stenosis and coarctation, with success rates varying based on disease type and location. Surgical intervention is indicated for interrupted aortic arch, certain types of valvar aortic stenosis and coarctation deemed not suitable for catheter-based intervention, and for subvalvar and supravalvar aortic stenosis of adequate severity. Lifelong follow-up is generally recommended, as recurrence requiring reintervention may occur.
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References
Hoffman JI, Christianson R. Congenital heart disease in a cohort of 19,502 births with long-term follow-up. Am J Cardiol. 1978;42(4):641–7.
Braunwald E, Goldblatt A, Aygen MM, Rockoff SD, Morrow AG. Congenital aortic stenosis. I. Clinical and hemodynamic findings in 100 patients. II. Surgical treatment and the results of operation. Circulation. 1963;27:426–62.
Wagner HR, Weidman WH, Ellison RC, Miettinen OS. Indirect assessment of severity in aortic stenosis. Circulation. 1977;56(1 Suppl):I20–3.
Bengur AR, Snider AR, Serwer GA, Peters J, Rosenthal A. 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(12):756–61.
Lababidi Z. Aortic balloon valvuloplasty. Am Heart J. 1983;106(4 Pt 1):751–2.
Sanchez GR, Mehta AV, Ewing LL, Brickley SE, Anderson TM, Black IF. Successful percutaneous balloon valvuloplasty of the aortic valve in an infant. Pediatr Cardiol. 1985;6(2):103–6.
Rocchini AP, Beekman RH, Ben Shachar G, Benson L, Schwartz D, Kan JS. Balloon aortic valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Am J Cardiol. 1990;65(11):784–9.
Mosca RS, Iannettoni MD, Schwartz SM, Ludomirsky A, Beekman 3rd RH, Lloyd T, et al. Critical aortic stenosis in the neonate. A comparison of balloon valvuloplasty and transventricular dilation. J Thorac Cardiovasc Surg. 1995;109(1):147–54.
Zeevi B, Keane JF, Castaneda AR, Perry SB, Lock JE. Neonatal critical valvar aortic stenosis. A comparison of surgical and balloon dilation therapy. Circulation. 1989;80(4):831–9.
Fellows KE, Radtke W, Keane JF, Lock JE. Acute complications of catheter therapy for congenital heart disease. Am J Cardiol. 1987;60(8):679–83.
Keane JF, Driscoll DJ, Gersony WM, Hayes CJ, Kidd L, O’Fallon WM, et al. Second natural history study of congenital heart defects. Results of treatment of patients with aortic valvar stenosis. Circulation. 1993;87(2 Suppl):I16–27.
Gerosa G, McKay R, Ross DN. Replacement of the aortic valve or root with a pulmonary autograft in children. Ann Thorac Surg. 1991;51(3):424–9.
Ohye RG, Gomez CA, Ohye BJ, Goldberg CS, Bove EL. The Ross/Konno procedure in neonates and infants: intermediate-term survival and autograft function. Ann Thorac Surg. 2001;72(3):823–30.
Pasquali SK, Shera D, Wernovsky G, Cohen MS, Tabbutt S, Nicolson S, et al. Midterm outcomes and predictors of reintervention after the Ross procedure in infants, children, and young adults. J Thorac Cardiovasc Surg. 2007;133(4):893–9.
Kaine SF, Smith EO, Mott AR, Mullins CE, Geva T. Quantitative echocardiographic analysis of the aortic arch predicts outcome of balloon angioplasty of native coarctation of the aorta. Circulation. 1996;94(5):1056–62.
Quaegebeur JM, Jonas RA, Weinberg AD, Blackstone EH, Kirklin JW. Outcomes in seriously ill neonates with coarctation of the aorta. A multiinstitutional study. J Thorac Cardiovasc Surg. 1994;108(5):841–51. discussion 52–4.
Allen HD, Beekman 3rd RH, Garson Jr A, Hijazi ZM, Mullins C, O’Laughlin MP, et al. Pediatric therapeutic cardiac catheterization: a statement for healthcare professionals from the Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 1998;97(6):609–25.
Kan JS, White Jr RI, Mitchell SE, Farmlett EJ, Donahoo JS, Gardner TJ. Treatment of restenosis of coarctation by percutaneous transluminal angioplasty. Circulation. 1983;68(5):1087–94.
Harrison DA, McLaughlin PR, Lazzam C, Connelly M, Benson LN. Endovascular stents in the management of coarctation of the aorta in the adolescent and adult: one year follow up. Heart. 2001;85(5):561–6.
Chin AJ, Jacobs ML. Morphology of the ventricular septal defect in two types of interrupted aortic arch. J Am Soc Echocardiogr. 1996;9(2):199–201.
Payne RM, Johnson MC, Grant JW, Strauss AW. Toward a molecular understanding of congenital heart disease. Circulation. 1995;91(2):494–504.
Haas F, Goldberg CS, Ohye RG, Mosca RS, Bove EL. Primary repair of aortic arch obstruction with ventricular septal defect in preterm and low birth weight infants. Eur J Cardiothorac Surg. 2000;17(6):643–7.
Kostelka M, Walther T, Geerdts I, Rastan A, Jacobs S, Dahnert I, et al. Primary repair for aortic arch obstruction associated with ventricular septal defect. Ann Thorac Surg. 2004;78(6):1989–93. discussion 93.
Morris CA, Mervis CB. Williams syndrome and related disorders. Annu Rev Genomics Hum Genet. 2000;1:461–84.
Wren C, Oslizlok P, Bull C. Natural history of supravalvular aortic stenosis and pulmonary artery stenosis. J Am Coll Cardiol. 1990;15(7):1625–30.
French JW, Guntheroth WG. An explanation of asymmetric upper extremity blood pressures in supravalvular aortic stenosis: the Coanda effect. Circulation. 1970;42(1):31–6.
Tani LY, Minich LL, Pagotto LT, Shaddy RE. Usefulness of doppler echocardiography to determine the timing of surgery for supravalvar aortic stenosis. Am J Cardiol. 2000;86(1):114–6.
Baltaxe HA, Moller JH, Amplatz K. Membranous subaortic stenosis and its associated malformations. Radiology. 1970;95(2):287–91.
Choi JY, Sullivan ID. Fixed subaortic stenosis: anatomical spectrum and nature of progression. Br Heart J. 1991;65(5):280–6.
Newfeld EA, Muster AJ, Paul MH, Idriss FS, Riker WL. Discrete subvalvular aortic stenosis in childhood. Study of 51 patients. Am J Cardiol. 1976;38(1):53–61.
Wright GB, Keane JF, Nadas AS, Bernhard WF, Castaneda AR. Fixed subaortic stenosis in the young: medical and surgical course in 83 patients. Am J Cardiol. 1983;52(7):830–5.
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(4):818–24.
Rohlicek CV, del Pino SF, Hosking M, Miro J, Cote JM, Finley J. Natural history and surgical outcomes for isolated discrete subaortic stenosis in children. Heart. 1999;82(6):708–13.
Coleman DM, Smallhorn JF, McCrindle BW, Williams WG, Freedom RM. Postoperative follow-up of fibromuscular subaortic stenosis. J Am Coll Cardiol. 1994;24(6):1558–64.
Kitchiner D, Jackson M, Malaiya N, Walsh K, Peart I, Arnold R. 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(6):588–95.
Brauner R, Laks H, Drinkwater Jr DC, Shvarts O, Eghbali K, Galindo A. Benefits of early surgical repair in fixed subaortic stenosis. J Am Coll Cardiol. 1997;30(7):1835–42.
Stewart JR, Merrill WH, Hammon Jr JW, Graham Jr TP, Bender Jr HW. Reappraisal of localized resection for subvalvar aortic stenosis. Ann Thorac Surg. 1990;50(2):197–202. discussion −3.
Suarez de Lezo J, Pan M, Sancho M, Herrera N, Arizon J, Franco M, et al. Percutaneous transluminal balloon dilatation for discrete subaortic stenosis. Am J Cardiol. 1986;58(7):619–21.
Gersony WM. Natural history of discrete subvalvar aortic stenosis: management implications. J Am Coll Cardiol. 2001;38(3):843–5.
Karamlou T, Gurofsky R, Bojcevski A, Williams WG, Caldarone CA, Van Arsdell GS, et al. Prevalence and associated risk factors for intervention in 313 children with subaortic stenosis. Ann Thorac Surg. 2007;84(3):900–6. discussion 6.
Suri RM, Dearani JA, Schaff HV, Danielson GK, Puga FJ. Long-term results of the Konno procedure for complex left ventricular outflow tract obstruction. J Thorac Cardiovasc Surg. 2006;132(5):1064–71.
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Charpie, J.R., Crowley, D.C., Aiyagari, R. (2014). Congenital Heart Disease: Left Ventricular Outflow Tract Obstruction. In: Wheeler, D., Wong, H., Shanley, T. (eds) Pediatric Critical Care Medicine. Springer, London. https://doi.org/10.1007/978-1-4471-6356-5_23
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