Abstract
Congenital aortic valve disease ranges from newborn critical aortic stenosis and shock to teenage asymptomatic aortic regurgitation and progressive ventricular dilatation. Echocardiography provides essential information about the aortic valve and left ventricle, while cardiac magnetic resonance imaging provides additional information about regurgitant fraction and ventricular volume.
There is general consensus on the management of older patients with aortic valve disease; however, decision-making is more complex for newborns. A team-based approach may provide the best results. Treatment strategies should prioritize quality of life and preservation of left ventricular function. Minimizing re-intervention is an important secondary priority.
Both balloon and open interventions are established and effective treatments for newborns with critical aortic stenosis. Re-intervention rates depend on original morphology. Open valvotomy with tricuspidization may offer the best long-term results.
Older children with aortic stenosis or regurgitation have several options. Repair can restore function and maintain growth potential. Although repair is associated with limited durability, it does preserve all future replacement options. Replacement can be autologous (Ross procedure), mechanical, or bioprosthetic. Limited durability of bioprosthetic valves and aortic homografts renders them secondary replacement options for most children. Younger patients requiring replacement can be excellent candidates for the Ross procedure. The supported Ross procedure may offer excellent long-term durability in the older age group.
This chapter strives to provide the reader with a broad understanding of congenital aortic valve disease by highlighting the “standard” treatment as well as controversies in the surgical management of this disease.
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References
Paget J (1844) On obstruction of the branches of the pulmonary artery. Med Chir Trans 27:162
Spencer FC, Neill CA, Sank L et al (1960) Anatomical variation in 46 patients with congenital aortic stenosis. Am Surg 26:204–216
Labadibi Z (1983) Aortic balloon valvuloplasty. Am Heart J 106:751–752
Samanek M, Slavik Z, Zborilova B et al (1989) Prevalence, treatment and outcome of heart disease in liveborn children: a prospective analysis of 91, 823 live-born children. Pediatr Cardiol 10:205
Donofrio MT, Engle MA, O’Loughlin JE et al (1992) Congenital aortic regurgitation. J Am Coll Cardiol 20:366–372
Hraska V (2016) Neonatal aortic stenosis is a surgical disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 19:2–5
De Kerchove L, Glineur D, Poncelet A et al (2008) Repair of aortic leaflet prolapse: a ten-year experience. Eur J Cardiothorac Surg 34:785–791
Bacha EA, Satou GM, Moran AM et al (2001) Valve-sparing operation for balloon-induced aortic regurgitation in congenital aortic stenosis. J Thorac Cardiovasc Surg 122:162–168
Maron BJ, Haas TS, Ahluwalia A et al (2016) Demographics and epidemiology of sudden deaths in young competitive athletes: from the United States National Registry. Am J Med 129:1170–1177
Bonow RO, Carabello B, de Leon Jr AC (1998) Guidelines for the management of patients with valvular heart disease: executive summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation 98:1949–1984
Wilson W, Taubert KA, Gewitz M et al (2007) Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 116:1736–1754
Bierbach BO, Aicher D, Abu Issa O et al (2010) Aortic root and cusp configuration determine aortic valve function. Eur J Cardiothorac Surg 38:400–406
Nishimura RA, Otto CM, Bonow RO et al (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 Practice Guidelines. J Am Coll Cardiol 63:e57–e185
Fratz S, Chung T, Greil G et al (2013) Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease. J Cardiovasc Magn Reson 15:51
Vergnat M, Asfour B, Arenz C et al (2019) Aortic stenosis of the neonate: a single-center experience. J Thorac Cardiovasc Surg 157:318–326
Eicken A, Georgiev S, Balling G et al (2010) Neonatal balloon aortic valvuloplasty-predictive value of current risk score algorithms for treatment strategies. Catheter Cardiovasc Interv 76:404–401
Colan SD, McElhinney DB, Crawford EC et al (2006) Validation and re-evaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis. J Am Coll Cardiol 47:1858–1865
Hickey EJ, Caldarone CA, Blackstone EH et al (2007) Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. J Thorac Cardiovasc Surg 134:1429–1437
Kovalchin JP, Brook MM, Rosenthal GL et al (1998) Echocardiographic hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis. J Am Coll Cardiol 32:237–244
Brown C, Boshoff D, Eyskens B et al (2009) Hybrid approach as bridge to biventricular repair in a neonate with critical aortic stenosis and borderline left ventricle. Eur J Cardiothorac Surg 35:1080–1082
Bonow RO, Nishimura RA, Thompson PD et al (2015) Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 5: valvular heart disease: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 66:2385–2392
Bhabra MS, Dhillon R, Bhudia S et al (2003) Surgical aortic valvotomy in infancy: impact of leaflet morphology on long-term outcomes. Ann Thorac Surg 76:1412–1416
Brown JW, Ruzmetov M, Vijay P et al (2003) Surgery for aortic stenosis in children: a 40-year experience. Ann Thorac Surg 76:1398–1411
Brown JW, Rodefeld MD, Ruzmetov M et al (2012) Surgical valvuloplasty versus balloon aortic dilation for congenital aortic stenosis: are evidence-based outcomes relevant? Ann Thorac Surg 94:146–153
Siddiqui J, Brizard CP, Galati JC et al (2013) Surgical valvotomy and repair for neonatal and infant congenital aortic stenosis achieves better results than interventional catheterization. J Am Coll Cardiol 62:2134–2140
Olofsson CK, Berggren H, Söderberg B et al (2018) Treatment of valvular aortic stenosis in children: a 20-year experience in a single institution. Interact Cardiovasc Thorac Surg:1–7
McElhinney DB, Lock JE, Keane JF et al (2005) Left heart growth, function, and reintervention after balloon aortic valvuloplasty for neonatal aortic stenosis. Circulation 111:451–458
Torres A, Vincent JA, Everett A et al (2015) Balloon valvuloplasty for congenital aortic stenosis: multi-center safety and efficacy outcome assessment. Catheter Cardiovasc Interv 86:808–820
Boe BA, Zampi JD, Kennedy KF et al (2017) Acute success of balloon aortic Valvuloplasty in the current era: a National Cardiovascular Data Registry Study. JACC Cardiovasc Interv 10:1717–1726
Sullivan PM, Rubio AE, Johnston TA et al (2017) Long-term outcomes and re-interventions following balloon aortic valvuloplasty in pediatric patients with congenital aortic stenosis: a single-center study. Catheter Cardiovasc Interv 89:288–296
Emani SM, Bacha EA, McElhinney DB et al (2009) Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart. J Thorac Cardiovasc Surg 138:1276–1282
Davis CK, Pastuszko P, Lamberti J et al (2011) The hybrid procedure for the borderline left ventricle. Cardiol Young 21:26–30
Hraska V, Schneider M (2013) Critical aortic stenosis with severe left ventricular dysfunction. Eur J Cardiothorac Surg 43:148–149
Polimenakos AC, Sathanandam S, Blair C et al (2010) Selective tricuspidization and aortic cusp extension valvuloplasty: outcome analysis in infants and children. Ann Thorac Surg 90:839–847
Tweddell JS, Pelech AN, Frommelt PC et al (2005) Complex aortic valve repair as a durable and effective alternative to valve replacement in children with aortic valve disease. J Thorac Cardiovasc Surg 129:551–558
Hraska V, Lilje C, Kantorova A et al (2010) Ross-Konno procedure in children. World J Pediatr Congenit Heart Surg 1:28–33
Jacobsen RM, Earing MG, Hill GD et al (2015) The externally supported Ross operation: early outcomes and intermediate follow-up. Ann Thorac Surg 100:631–638
Vergnat M, Asfour B, Arenz C et al (2017) Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy. Eur J Cardiothorac Surg 52:581–587
Maskatia SA, Ing FF, Justino H et al (2011) Twenty-five year experience with balloon aortic valvuloplasty for congenital aortic stenosis. Am J Cardiol 108:1024–1028
Hill GD, Ginde S, Rios R et al (2016) Surgical valvotomy versus balloon valvuloplasty for congenital aortic valve stenosis: a systematic review and meta-analysis. J Am Heart Assoc 8:1–8
d’Udekem Y, Siddiqui J, Seaman CS et al (2013) Long-term results of a strategy of aortic valve repair in the pediatric population. J Thorac Cardiovasc Surg 145:461–469
Maskatia SA, Justino H, Ing FF et al (2013) Aortic valve morphology is associated with outcomes following balloon valvuloplasty for congenital aortic stenosis. Catheter Cardiovasc Interv 81:90–95
Brown JW, Ruzmetov M, Shahriari AP (2010) Modification of the Ross aortic valve replacement to prevent late autograft dilatation. Eur J Cardiothorac Surg 37:1002–1007
McMullan DM, Oppido G, Davies B et al (2007) Surgical strategy for the bicuspid aortic valve: tricuspidization with cusp extension versus pulmonary autograft. J Thorac Cardiovasc Surg 134:90–98
Schäfers HJ, Aicher D, Riodionycheva S et al (2008) Bicuspidization of the unicuspid aortic valve: a new reconstructive approach. Ann Thorac Surg 85:2012–2018
Aszyk P, Thiel C, Sinzobahamvya C et al (2012) Ross-Konno procedure in infants – mid-term results. Eur J Cardiothorac Surg 42:687–694
Charitos EI, Takkenberg JJ, Hanke T et al (2012) Reoperations on the pulmonary autograft and pulmonary homograft after the Ross procedure: an update on the German Dutch Ross Registry. J Thorac Cardiovasc Surg 144:813–821; discussion 21–23
Takkenberg JJ, Klieverik LM, Schoof PH et al (2009) The Ross procedure: a systematic review and meta-analysis. Circulation 119:222–228
Elkins RC, Lane NM, McCue C (2001) Ross operation in children: late results. J Heart Valve Dis 10:736–741
Hörer J, Hanke T, Stierle U (2009) Neoaortic root diameters and aortic regurgitation in children after the Ross operation. Ann Thorac Surg 88:594–600
Tran PK, Tsang VT, Cornejo PR et al (2017) Midterm results of the Ross procedure in children: an appraisal of the subannular implantation with interrupted sutures technique. Eur J Cardiothorac Surg 52:798–804
Etnel JRG, Elmont LC, Ertekin E et al (2016) Outcome after aortic valve replacement in children: a systematic review and meta-analysis. J Thorac Cardiovasc Surg 151:143–52.e3
Bradley SM (2013) Aortic valve insufficiency in the teenager and young adult: the role of prosthetic valve replacement. World J Pediatr Congenit Heart Surg 4:297–402
Saleeb SF, Newburger JW, Geva T et al (2014) Accelerated degeneration of a bovine pericardial bioprosthetic aortic valve in children and young adults. Circulation 130:51–60
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Hraška, V., Woods, R.K., Mitchell, M.E., Foerster, S.R., Goot, B.H. (2020). Congenital Aortic Valve Stenosis and Regurgitation. In: da Cruz, E., Ivy, D., Hraska, V., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4999-6_23-2
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DOI: https://doi.org/10.1007/978-1-4471-4999-6_23-2
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