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ERROR: type should be string, got "https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvODk0MDk1LW92ZXJ2aWV3\nPediatric Valvar Aortic Stenosis\nAuthor: Howard S Weber, MD, FSCAI; Chief Editor: Syamasundar Rao Patnana, MD more...\nSections Pediatric Valvar Aortic Stenosis\nHistologic Findings\nBalloon Aortic Valvuloplasty\nSurgical Aortic Valvotomy\nSurgical Aortic Valve Replacement\nInotropic Agents\nLoop Diuretics\nAortic valve stenosis results from minor to severe degrees of aortic valve maldevelopment. This stenosis causes mild to severe obstruction of the left ventricular outflow that may be associated with other left heart obstructive lesions, varying degrees of left heart hypoplasia, or extracardiac malformations, including genetic disorders. This article focuses on the presentation, clinical features, and therapeutic options associated with aortic valve stenosis.\nAn estimated 10%-15% of patients with aortic valve stenosis present with the condition when they are younger than 1 year due to severe stenosis. The remainder of patients may present later in childhood or in adulthood due to progressive obstruction. Adult patients with bicuspid aortic valves may develop significant stenosis or insufficiency after the valve becomes calcified (as seen in the image below), in the fourth, fifth, or sixth decade of life.\nValvular calcification of aortic stenosis is seen with cardiac fluoroscopy during catheterization.\nBicuspid aortic valves without stenosis or insufficiency in adult patients can be associated with progressive pathologic enlargement of the aortic root of uncertain etiology. This may require surgical root replacement once the aorta reaches a specific diameter.\nIn the neonate, transthoracic echocardiography provides complete diagnostic and hemodynamic information. In older patients, transthoracic echocardiography is usually diagnostic; in rare cases, however, a large adolescent patient may require transesophageal echocardiography to clearly delineate the left ventricular outflow tract and to detail the valve anatomy. Cardiac catheterization is usually performed in anticipation of balloon aortic valvuloplasty (see Workup).\nBalloon aortic valvuloplasty is considered the initial treatment of choice in pediatric patients with congenital aortic valve stenosis. Surgical repair or replacement of an aortic valve is primarily reserved for patients in whom balloon valvuloplasty has failed with severe stenosis or have significant valve insufficiency in association with progressive left ventricular dilation or deterioration of left ventricular systolic function (see Treatment).\nGo to Aortic Stenosis, Pediatric Supravalvar Aortic Stenosis, and Pediatric Subvalvar Aortic Stenosis for more complete information on these topics.\nIn patients with aortic valve stenosis, most commonly the valve is bicuspid with a single fused commissure and an eccentrically placed orifice. A third or rudimentary commissure may sometimes be apparent.\nLess commonly, the valve is unicuspid and dome shaped, particularly in the neonate. Rarely, the valve has three unseparated cusps, with the stenosis being centrally located. Secondary calcification of the valve is extremely rare in childhood, and at times, the aortic valve anulus may also be underdeveloped or hypoplastic in association with mitral and left ventricular hypoplasia, adding to the severity of left ventricular outflow tract (LVOT) obstruction.\nPure aortic valve stenosis results in compensatory ventricular hypertrophy over time proportional to the degree of obstruction. Mild-to-moderate degrees of obstruction are usually well tolerated, with minimal hypertrophy and normal left ventricular function. As stenosis progresses, often in association with periods of rapid somatic growth, hypertrophy increases along with wall stress. With severe hypertrophy and valvar obstruction, myocardial ischemia may result from the combination of limited cardiac output, reduced coronary perfusion, and increased myocardial oxygen consumption. A small, fixed, cross-sectional area of the aortic valve can limit the ability to increase cardiac output with exercise. This may result in exercise-induced syncope or sudden death.\nSevere obstruction in utero may lead to variable degrees of left-sided heart hypoplasia, endomyocardial fibroelastosis, reduced ventricular function, and significant mitral valve insufficiency.\nYetman et al described neonatal patients in whom rapid progression of aortic stenosis occurred within 6 months of diagnosis. [1] These patients often had well-preserved ventricular function but, during rapid growth, could exhibit fairly dramatic increases in the aortic valve gradient, requiring intervention. The typical absence of symptoms in this age group, does not correlate with the severity of aortic valve obstruction.\nSpontaneous development of significant aortic insufficiency in the absence of stenosis is less common and may result in ventricular dilation. This could be associated with a recent episode of bacterial endocarditis. Rarely, left ventricular dysfunction and symptomatic congestive heart failure occur unless stenosis is reduced and/or insufficiency is relieved.\nCauses of aortic valve stenosis are multifactorial, although studies suggest a higher rate of recurrence of left ventricular outflow tract obstructive lesions in the offspring than other forms of congenital heart disease. The recurrence risk in offspring of an affected father is approximately 3% but is approximately 15% in offspring of an affected mother. Abnormal fetal hemodynamics are theorized to contribute to the development of aortic valve stenosis and other left-sided heart obstructive lesions.\nSimilarly, other forms of left heart obstructive disease may occur repeatedly within families (eg, hypoplastic left heart syndrome in a child whose older sibling had coarctation of the aorta). A definite genetic defect for aortic valve stenosis has not been identified, but the presence of a bicuspid aortic valve has been documented in multiple family members and is a common congenital heart defect in patients with Turner syndrome (monosomy X).\nThe crude incidence of congenital heart defects is approximately 8 per 1000 live births. Aortic valve stenosis accounts for 3%-5% of all congenital heart defects. Authorities estimate a bicuspid aortic valve is present in as many as 1% of the general population, although accurate figures regarding prevalence of this abnormality are difficult to obtain, because many cases remain undetected and these valves function well for many decades.\nSome studies have documented a higher prevalence of aortic valve stenosis in white children than in black and Hispanic children. There is a strong male sex predilection in aortic valve stenosis: the male-to-female ratio is 4:1.\nThe prognosis for patients with congenital aortic valve stenosis depends on the valve anatomy and its response to intervention. The severity of the disorder is widely varied, ranging from no symptoms in patients with a bicuspid aortic valve and no stenosis to critical illness in neonates.\nMortality is higher in patients presenting with severe or critical aortic valve stenosis during the first year of life, specifically in the neonatal period, although this risk has decreased significantly over the past 20 years. Mortality is in part influenced by associated congenital cardiac anomalies, which occur in as many as 20% of patients. These include coarctation of aorta, ventricular septal defect, mitral valve stenosis or insufficiency, and left ventricular hypoplasia. Undetected, severe aortic valve stenosis is a known cause of sudden death and accounts for approximately 1% of all causes of sudden death in young people.\nA retrospective cohort study in 245 patients with congenital valvular aortic stenosis by Ten Harkel et al found that mortality was high for patients who were diagnosed in infancy but was almost absent in those who were diagnosed after infancy. [2] Interventions were required more often in children diagnosed at a younger age and/or with higher gradients.\nEducation is indicated for affected families regarding signs of progressive aortic stenosis, heart failure, and low cardiac output. Education regarding good dental care and avoidance of risk factors for endocarditis is imperative.\nOther goals of parent education include setting reasonable goals for exercise participation that allow for a healthy lifestyle but downplay long-term goals for competitive athletics or strenuous isometrics at a high level until significant relief of aortic valve disease is accomplished by aortic valve repair (balloon valvuloplasty versus surgical) or replacement.\nCompetitive aerobic sports are prohibited in patients with severe aortic valve stenosis, whereas patients with mild stenosis can participate if they have normal electrocardiogram findings, exercise tolerance, and no history of exercise induced chest pain, syncope, or tachyarrhythmias. Patients with moderate aortic valve stenosis may also participate in low-to-moderate dynamic competitive sports, if certain conditions are met. [3]\nSimilarly, emphasize that treatment for aortic valve disease is a lifelong process and is best introduced early and reinforced often to ensure that patients with aortic valve stenosis continue to receive ongoing follow-up care as they make the transition from an adolescent lifestyle at home to the independence of young adulthood.\nYetman AT, Rosenberg HC, Joubert GI. Progression of asymptomatic aortic stenosis identified in the neonatal period. Am J Cardiol. 1995 Mar 15. 75(8):636-7. [Medline].\nTen Harkel AD, Berkhout M, Hop WC, Witsenburg M, Helbing WA. Congenital valvular aortic stenosis: limited progression during childhood. Arch Dis Child. 2009 Jul. 94(7):531-5. [Medline].\nMaron BJ, Zipes DP. Introduction: eligibility recommendations for competitive athletes with cardiovascular abnormalities-general considerations. J Am Coll Cardiol. 2005 Apr 19. 45(8):1318-21. [Medline].\nKuebler JD, Shivapour J, Yaroglu Kazanci S, et al. Longitudinal assessment of the Doppler-estimated maximum gradient in patients with congenital valvar aortic stenosis pre- and post-balloon valvuloplasty. Circ Cardiovasc Imaging. 2018 Mar. 11(3):e006708. [Medline].\nFratz S, Gildein HP, Balling G, et al. Aortic valvuloplasty in pediatric patients substantially postpones the need for aortic valve surgery: a single-center experience of 188 patients after up to 17.5 years of follow-up. Circulation. 2008 Mar 4. 117(9):1201-6. [Medline].\nEgito ES, Moore P, O'Sullivan J, et al. Transvascular balloon dilation for neonatal critical aortic stenosis: early and midterm results. J Am Coll Cardiol. 1997 Feb. 29(2):442-7. [Medline].\nMcElhinney DB, Lacro RV, Gauvreau K, et al. Dilation of the ascending aorta after balloon valvuloplasty for aortic stenosis during infancy and childhood. Am J Cardiol. 2012 Sep 1. 110(5):702-8. [Medline].\nMagee AG, Nykanen D, McCrindle BW, Wax D, Freedom RM, Benson LN. Balloon dilation of severe aortic stenosis in the neonate: comparison of anterograde and retrograde catheter approaches. J Am Coll Cardiol. 1997 Oct. 30(4):1061-6. [Medline].\nAustoni P, Figini A, Vignati G, Donatelli F. Emergency aortic balloon valvotomy in critical aortic stenosis of the neonate. Pediatr Cardiol. 1990 Jan. 11(1):59-60. [Medline].\nLababidi Z. Aortic balloon valvuloplasty. Am Heart J. 1983 Oct. 106(4 Pt 1):751-2. [Medline].\nAlekyan BG, Petrosyan YS, Coulson JD, Danilov YY, Vinokurov AV. Right subscapular artery catheterization for balloon valvuloplasty of critical aortic stenosis in infants. Am J Cardiol. 1995 Nov 15. 76(14):1049-52. [Medline].\nBeekman RH, Rocchini AP, Andes A. Balloon valvuloplasty for critical aortic stenosis in the newborn: influence of new catheter technology. J Am Coll Cardiol. 1991 Apr. 17(5):1172-6. [Medline].\nFischer DR, Ettedgui JA, Park SC, Siewers RD, del Nido PJ. Carotid artery approach for balloon dilation of aortic valve stenosis in the neonate: a preliminary report. J Am Coll Cardiol. 1990 Jun. 15(7):1633-6. [Medline].\nHausdorf G, Schneider M, Schirmer KR, Schulze-Neick I, Lange PE. Anterograde balloon valvuloplasty of aortic stenosis in children. Am J Cardiol. 1993 Feb 15. 71(5):460-2. [Medline].\nO'Laughlin MP, Slack MC, Grifka R, Mullins CE. Prograde double balloon dilation of congenital aortic valve stenosis: a case report. Cathet Cardiovasc Diagn. 1993 Feb. 28(2):134-6. [Medline].\nRao PS, Jureidini SB. Transumbilical venous, anterograde, snare-assisted balloon aortic valvuloplasty in a neonate with critical aortic stenosis. Cathet Cardiovasc Diagn. 1998 Oct. 45(2):144-8. [Medline].\nWeber HS, Mart CR, Myers JL. Transcarotid balloon valvuloplasty for critical aortic valve stenosis at the bedside via continuous transesophageal echocardiographic guidance. Catheter Cardiovasc Interv. 2000 Jul. 50(3):326-9. [Medline].\nKallio M, Rahkonen O, Mattila I, Pihkala J. Congenital aortic stenosis: treatment outcomes in a nationwide survey. Scand Cardiovasc J. 2017 Oct. 51(5):277-83. [Medline].\nRao PS. Role of interventional cardiology in the treatment of neonates - part II - balloon angioplasty/valvuloplasty. Congenit Cardiol Today. 2008 Jan. 6(1):1-14. [Full Text].\nRao PS. Neonatal catheter interventions. Vijayalakshmi, IB, ed. Cardiac Catheterization and Imaging from Pediatrics to Geriatrics. New Delhi, India: Jaypee Brothers Medical Pub; 2015. 388-432.\nTurley K, Bove EL, Amato JJ, et al. Neonatal aortic stenosis. J Thorac Cardiovasc Surg. 1990 Apr. 99(4):679-83; discussion 683-4. [Medline].\nMcCrindle BW, Blackstone EH, Williams WG, et al. Are outcomes of surgical versus transcatheter balloon valvotomy equivalent in neonatal critical aortic stenosis?. Circulation. 2001 Sep 18. 104(12 Suppl 1):I152-8. [Medline].\nSiddiqui J, Brizard CP, Galati JC, et al. Surgical valvotomy and repair for neonatal and infant congenital aortic stenosis achieves better results than interventional catheterization. J Am Coll Cardiol. 2013 Dec 3. 62(22):2134-40. [Medline].\nRao PS. Balloon aortic valvuloplasty. Indian Heart J. 2016 Sep - Oct. 68(5):592-5. [Medline].\n[Guideline] Feltes TF, Bacha E, Beekman RH 3rd, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation. 2011 Jun 7. 123(22):2607-52. [Medline]. [Full Text].\nNishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2017 Jul 11. 70(2):252-89. [Medline].\nBoggs W. New guidelines support expanded use of TAVR. Reuters Health Information. March 24, 2017.\n[Guideline] Baltimore RS, Gewitz M, Baddour LM, et al. Infective endocarditis in childhood: 2015 update: a scientific statement from the American Heart Association. Circulation. 2015 Oct 13. 132(15):1487-515. [Medline]. [Full Text].\nHoward S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital\nHoward S Weber, MD, FSCAI is a member of the following medical societies: Society for Cardiovascular Angiography and Interventions\nDisclosure: Received income in an amount equal to or greater than $250 from: Abbott Medical .\nPaul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital\nPaul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions\nJohn W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital\nJohn W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions\nSyamasundar Rao Patnana, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital\nSyamasundar Rao Patnana, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research\nJuan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine\nJuan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation\nDisclosure: Received honoraria from Actelion for speaking and teaching.\nencoded search term (Pediatric Valvar Aortic Stenosis) and Pediatric Valvar Aortic Stenosis"