Management of adults with bicuspid aortic valve disease
- Alan C Braverman, MD
Alan C Braverman, MD
- Alumni Endowed Professor in Cardiovascular Diseases
- Professor of Medicine
- Washington University School of Medicine
- Director, Marfan Syndrome Clinic
- Director, Inpatient Cardiology Firm
Adults with bicuspid aortic valves require surveillance for aortic valve dysfunction and for disease of the aortic root and/or ascending aorta. Aortic valve replacement is generally required to treat symptomatic aortic stenosis except in selected young adults who may benefit from percutaneous balloon valvuloplasty. Aortic valve replacement is generally the definitive therapy for severe aortic regurgitation. Progressive aortic root and/or ascending aortic dilation (or ascending aortic dissection) is an indication for replacement of the aortic root and/or ascending aorta.
Treatment of adults with bicuspid aortic valve disease will be discussed here. The natural history and clinical manifestations of bicuspid aortic valve disease in adults, management of pregnancy in women with bicuspid aortic valve, and clinical manifestations and management of aortic stenosis in children are discussed separately. (See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults" and "Pregnancy in women with a bicuspid aortic valve" and "Valvar aortic stenosis in children".)
Who and how frequently to monitor — Patients with bicuspid aortic valves should be monitored for progressive aortic valve dysfunction (stenosis and/or regurgitation) as well as for aortic dilation with risk of aneurysm formation and aortic dissection. There are many different phenotypes of bicuspid aortic valve aortic enlargement (bicuspid valve "aortopathy") . The most common site of aortic dilation is at the ascending aorta and monitoring by transthoracic echocardiography is often inadequate at this level unless high right parasternal views are obtained. Echocardiography should be performed at intervals, based upon the lesion requiring greatest frequency of surveillance among aortic stenosis, aortic regurgitation, and dilation of the aorta. The rate of aortic growth in bicuspid aortic valve patients is variable, ranging from 0.2 to 0.9 mm/year, depending upon patient characteristics [1,2]. Higher rates of growth are observed in older adults and those with larger aneurysms [2,3]. Development of significant aortic stenosis is much more common than development of significant aortic regurgitation.
Earlier re-evaluation (beyond the below recommended surveillance) is indicated for changes in symptoms or examination and for hemodynamic assessment related to pregnancy. (See "Pregnancy in women with a bicuspid aortic valve".) Of note, although surveillance of the aorta is based upon aortic diameter, other factors (eg, genetic factors, hemodynamic effects) likely contribute to the risk of acute aortic events (eg, aortic dissection and aortic rupture) .
The following recommendations for surveillance and management for aortic stenosis, aortic regurgitation, and aortic dilatation are based upon the 2014 American Heart Association/American College of Cardiology (AHA/ACC) valve guidelines, the 2008 ACC/AHA adult congenital heart disease guidelines, and the ACC/AHA scientific statement on eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities (table 1A-C) [4,5] and are consistent with the 2011 appropriate use criteria for echocardiography [6-8]:
- Braverman AC. The bicuspid aortic valve and associated aortic disease. In: Valvular Heart Disease, 4th Ed, Otto CM, Bonow RO. (Eds), Saunders/Elsevier, Philadelphia 2013. p.179.
- Hardikar AA, Marwick TH. Surgical thresholds for bicuspid aortic valve associated aortopathy. JACC Cardiovasc Imaging 2013; 6:1311.
- Davies RR, Kaple RK, Mandapati D, et al. Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg 2007; 83:1338.
- Bonow RO, Nishimura RA, Thompson PD, Udelson JE. 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 2015; 66:2385.
- Braverman AC, Harris KM, Kovacs RJ, Maron BJ. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 7: Aortic Diseases, Including Marfan Syndrome: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015; 66:2398.
- 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 2014; 63:e57.
- Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008; 52:e143.
- American College of Cardiology Foundation Appropriate Use Criteria Task Force, American Society of Echocardiography, American Heart Association, et al. ACCF/ASE/AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 Appropriate Use Criteria for Echocardiography. A Report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, American Society of Echocardiography, American Heart Association, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Critical Care Medicine, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance American College of Chest Physicians. J Am Soc Echocardiogr 2011; 24:229.
- Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation 2010; 121:e266.
- Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr 2015; 28:1.
- Gomez D, Al Haj Zen A, Borges LF, et al. Syndromic and non-syndromic aneurysms of the human ascending aorta share activation of the Smad2 pathway. J Pathol 2009; 218:131.
- Forte A, Della Corte A, Grossi M, et al. Early cell changes and TGFβ pathway alterations in the aortopathy associated with bicuspid aortic valve stenosis. Clin Sci (Lond) 2013; 124:97.
- Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC), European Association for Cardio-Thoracic Surgery (EACTS), Vahanian A, et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J 2012; 33:2451.
- Michelena HI, Prakash SK, Della Corte A, et al. Bicuspid aortic valve: identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon). Circulation 2014; 129:2691.
- Wilson W, Taubert KA, Gewitz M, et al. 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 2007; 116:1736.
- Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and ascending aorta guidelines for management and quality measures. Ann Thorac Surg 2013; 95:S1.
- Kari FA, Siepe M, Sievers HH, Beyersdorf F. Repair of the regurgitant bicuspid or tricuspid aortic valve: background, principles, and outcomes. Circulation 2013; 128:854.
- Svensson LG, Al Kindi AH, Vivacqua A, et al. Long-term durability of bicuspid aortic valve repair. Ann Thorac Surg 2014; 97:1539.
- Rosenfeld HM, Landzberg MJ, Perry SB, et al. Balloon aortic valvuloplasty in the young adult with congenital aortic stenosis. Am J Cardiol 1994; 73:1112.
- Sandhu SK, Lloyd TR, Crowley DC, Beekman RH. Effectiveness of balloon valvuloplasty in the young adult with congenital aortic stenosis. Cathet Cardiovasc Diagn 1995; 36:122.
- Bauer T, Linke A, Sievert H, et al. Comparison of the effectiveness of transcatheter aortic valve implantation in patients with stenotic bicuspid versus tricuspid aortic valves (from the German TAVI Registry). Am J Cardiol 2014; 113:518.
- Costopoulos C, Latib A, Maisano F, et al. Comparison of results of transcatheter aortic valve implantation in patients with severely stenotic bicuspid versus tricuspid or nonbicuspid valves. Am J Cardiol 2014; 113:1390.
- Kochman J, Huczek Z, Scisło P, et al. Comparison of one- and 12-month outcomes of transcatheter aortic valve replacement in patients with severely stenotic bicuspid versus tricuspid aortic valves (results from a multicenter registry). Am J Cardiol 2014; 114:757.
- Hayashida K, Bouvier E, Lefèvre T, et al. Transcatheter aortic valve implantation for patients with severe bicuspid aortic valve stenosis. Circ Cardiovasc Interv 2013; 6:284.
- Phan K, Wong S, Phan S, et al. Transcatheter Aortic Valve Implantation (TAVI) in Patients With Bicuspid Aortic Valve Stenosis--Systematic Review and Meta-Analysis. Heart Lung Circ 2015; 24:649.
- Mylotte D, Lefevre T, Søndergaard L, et al. Transcatheter aortic valve replacement in bicuspid aortic valve disease. J Am Coll Cardiol 2014; 64:2330.
- Perlman GY, Blanke P, Dvir D, et al. Bicuspid Aortic Valve Stenosis: Favorable Early Outcomes With a Next-Generation Transcatheter Heart Valve in a Multicenter Study. JACC Cardiovasc Interv 2016; 9:817.
- Della Corte A, Bancone C, Dialetto G, et al. The ascending aorta with bicuspid aortic valve: a phenotypic classification with potential prognostic significance. Eur J Cardiothorac Surg 2014; 46:240.
- Girdauskas E, Disha K, Raisin HH, et al. Risk of late aortic events after an isolated aortic valve replacement for bicuspid aortic valve stenosis with concomitant ascending aortic dilation. Eur J Cardiothorac Surg 2012; 42:832.
- Davies RR, Gallo A, Coady MA, et al. Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg 2006; 81:169.
- Hiratzka LF, Creager MA, Isselbacher EM, et al. Surgery for Aortic Dilatation in Patients With Bicuspid Aortic Valves: A Statement of Clarification From the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2016; 67:724.
- Braverman AC. Guidelines for management of bicuspid aortic valve aneurysms: what's the clinician to do? Curr Opin Cardiol 2014; 29:489.
- Adamo L, Braverman AC. Surgical threshold for bicuspid aortic valve aneurysm: a case for individual decision-making. Heart 2015; 101:1361.
- Wojnarski CM, Svensson LG, Roselli EE, et al. Aortic Dissection in Patients With Bicuspid Aortic Valve-Associated Aneurysms. Ann Thorac Surg 2015; 100:1666.
- Girdauskas E, Disha K, Rouman M, et al. Aortic events after isolated aortic valve replacement for bicuspid aortic valve root phenotype: echocardiographic follow-up study. Eur J Cardiothorac Surg 2015; 48:e71.
- Girdauskas E, Rouman M, Disha K, et al. Aortic Dissection After Previous Aortic Valve Replacement for Bicuspid Aortic Valve Disease. J Am Coll Cardiol 2015; 66:1409.
- Boodhwani M, Andelfinger G, Leipsic J, et al. Canadian Cardiovascular Society position statement on the management of thoracic aortic disease. Can J Cardiol 2014; 30:577.
- Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J 2014; 35:2873.
- Girdauskas E, Disha K, Borger MA, Kuntze T. Risk of proximal aortic dissection in patients with bicuspid aortic valve: how to address this controversy? Interact Cardiovasc Thorac Surg 2014; 18:355.
- Kallenbach K, Sundt TM, Marwick TH. Aortic surgery for ascending aortic aneurysms under 5.0 cm in diameter in the presence of bicuspid aortic valve. JACC Cardiovasc Imaging 2013; 6:1321.
- Verma S, Siu SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med 2014; 370:1920.
- Coady MA, Davies RR, Roberts M, et al. Familial patterns of thoracic aortic aneurysms. Arch Surg 1999; 134:361.
- Shang EK, Nathan DP, Sprinkle SR, et al. Peak wall stress predicts expansion rate in descending thoracic aortic aneurysms. Ann Thorac Surg 2013; 95:593.
- La Canna G, Ficarra E, Tsagalau E, et al. Progression rate of ascending aortic dilation in patients with normally functioning bicuspid and tricuspid aortic valves. Am J Cardiol 2006; 98:249.
- Clouse WD, Hallett JW Jr, Schaff HV, et al. Improved prognosis of thoracic aortic aneurysms: a population-based study. JAMA 1998; 280:1926.
- Coady MA, Rizzo JA, Hammond GL, et al. Surgical intervention criteria for thoracic aortic aneurysms: a study of growth rates and complications. Ann Thorac Surg 1999; 67:1922.
- Davies RR, Goldstein LJ, Coady MA, et al. Yearly rupture or dissection rates for thoracic aortic aneurysms: simple prediction based on size. Ann Thorac Surg 2002; 73:17.
- Pape LA, Tsai TT, Isselbacher EM, et al. Aortic diameter >or = 5.5 cm is not a good predictor of type A aortic dissection: observations from the International Registry of Acute Aortic Dissection (IRAD). Circulation 2007; 116:1120.
- Borger MA, Preston M, Ivanov J, et al. Should the ascending aorta be replaced more frequently in patients with bicuspid aortic valve disease? J Thorac Cardiovasc Surg 2004; 128:677.
- McKellar SH, Michelena HI, Li Z, et al. Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves. Am J Cardiol 2010; 106:1626.
- Who and how frequently to monitor
- How to monitor
- MEDICAL THERAPY
- ENDOCARDITIS PREVENTION
- PHYSICAL ACTIVITY AND EXERCISE
- NONCARDIAC SURGERY
- TYPES OF INTERVENTION
- AORTIC VALVE INTERVENTION
- Rationale for intervention
- Indications for aortic valve intervention
- Role of balloon valvotomy
- Transcatheter aortic valve replacement
- AORTIC ROOT AND ASCENDING AORTA SURGERY
- Our approach
- POSTOPERATIVE OUTCOMES
- MONITORING AFTER AORTIC VALVE SURGERY AND/OR ASCENDING AORTA REPAIR
- SUMMARY AND RECOMMENDATIONS