Bicuspid Aortic Valve Differential Diagnoses

1. Keith JD. Bicuspid aortic valve. Keith JD, Rowe RD, Vlad P, eds. Heart Disease in Infancy and Childhood. New York: MacMillan Publishing Co, Inc.; 1978. 728-35.

2. Hajar R. Da Vinci's anatomical drawings the heart. Heart Views. 2002. 3(2):12. [Full Text].

3. Titus JL, Edwards JE. The aortic root and valve: Anatomy and congenital anomalies. Emery RW, Arom KV. The Aortic Valve. Philadelphia: Hanley & Belfus, Inc.; 1991. 1-8.

4. Duran AC, Frescura C, Sans-Coma V, et al. Bicuspid aortic valves in hearts with other congenital heart disease. J Heart Valve Dis. 1995 Nov. 4(6):581-90. [QxMD MEDLINE Link].

5. Ellison JW, Yagubyan M, Majumdar R, et al. Evidence of genetic locus heterogeneity for familial bicuspid aortic valve. J Surg Res. 2007 Sep. 142(1):28-31. [QxMD MEDLINE Link].

6. Foffa I, Ait Ali L, Panesi P, et al. Sequencing of NOTCH1, GATA5, TGFBR1 and TGFBR2 genes in familial cases of bicuspid aortic valve. BMC Med Genet. 2013 Apr 11. 14:44. [QxMD MEDLINE Link]. [Full Text].

7. Roberts WC. Morphologic aspects of cardiac valve dysfunction. Am Heart J. 1992 Jun. 123(6):1610-32. [QxMD MEDLINE Link].

8. Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol. 1970 Jul. 26(1):72-83. [QxMD MEDLINE Link].

9. Sadee AS, Becker AE, Verheul HA, et al. Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation. Br Heart J. 1992 Jun. 67(6):439-41. [QxMD MEDLINE Link].

10. Sabet HY, Edwards WD, Tazelaar HD, Daly RC. Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. Mayo Clin Proc. 1999 Jan. 74(1):14-26. [QxMD MEDLINE Link].

11. Della Corte A, Bancone C, Quarto C, et al. Predictors of ascending aortic dilatation with bicuspid aortic valve: a wide spectrum of disease expression. Eur J Cardiothorac Surg. 2007 Mar. 31(3):397-404; discussion 404-5. [QxMD MEDLINE Link].

12. de Sa M, Moshkovitz Y, Butany J, David TE. Histologic abnormalities of the ascending aorta and pulmonary trunk in patients with bicuspid aortic valve disease: clinical relevance to the ross procedure. J Thorac Cardiovasc Surg. 1999 Oct. 118(4):588-94. [QxMD MEDLINE Link].

13. [Guideline] Bonow RO. Bicuspid aortic valves and dilated aortas: a critical review of the ACC/AHA practice guidelines recommendations. Am J Cardiol. 2008 Jul 1. 102(1):111-4. [QxMD MEDLINE Link].

14. Schaefer BM, Lewin MB, Stout KK, et al. The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape. Heart. 2008 Dec. 94(12):1634-8. [QxMD MEDLINE Link].

15. Fazel SS, Mallidi HR, Lee RS, et al. The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch. J Thorac Cardiovasc Surg. 2008 Apr. 135(4):901-7, 907.e1-2. [QxMD MEDLINE Link].

16. Pham T, Martin C, Elefteriades J, Sun W. Biomechanical characterization of ascending aortic aneurysm with concomitant bicuspid aortic valve and bovine aortic arch. Acta Biomater. 2013 Aug. 9(8):7927-36. [QxMD MEDLINE Link].

17. Matsuyama S, Nishida T, Ushijima T, Tominaga R. Long-term results after treatment of the ascending aorta for bicuspid aortic valve patients. Surg Today. 2016 Jun. 46(6):729-34. [QxMD MEDLINE Link].

18. Beaton AZ, Nguyen T, Lai WW, et al. Relation of coarctation of the aorta to the occurrence of ascending aortic dilation in children and young adults with bicuspid aortic valves. Am J Cardiol. 2009 Jan 15. 103(2):266-70. [QxMD MEDLINE Link].

19. Ren X, Zhang M, Liu K, et al. The significance of aortic valve calcification in patients with bicuspid aortic valve disease. Int J Cardiovasc Imaging. 2016 Mar. 32(3):471-8. [QxMD MEDLINE Link].

20. Ward C. Clinical significance of the bicuspid aortic valve. Heart. 2000 Jan. 83(1):81-5. [QxMD MEDLINE Link].

21. Kitchiner D, Jackson M, Walsh K, et al. The progression of mild congenital aortic valve stenosis from childhood into adult life. Int J Cardiol. 1993 Dec 31. 42(3):217-23. [QxMD MEDLINE Link].

22. Khan W, Milsevic M, Salciccioli L, Lazar J. Low prevalence of bicuspid aortic valve in African Americans. Am Heart J. 2008 Sep. 156(3):e25. [QxMD MEDLINE Link].

23. Tutar E, Ekici F, Atalay S, Nacar N. The prevalence of bicuspid aortic valve in newborns by echocardiographic screening. Am Heart J. 2005 Sep. 150(3):513-5. [QxMD MEDLINE Link].

24. Chui MC, Newby DE, Panarelli M, Bloomfield P, Boon NA. Association between calcific aortic stenosis and hypercholesterolemia: is there a need for a randomized controlled trial of cholesterol-lowering therapy?. Clin Cardiol. 2001 Jan. 24(1):52-5. [QxMD MEDLINE Link].

25. Beppu S, Suzuki S, Matsuda H, et al. Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves. Am J Cardiol. 1993 Feb 1. 71(4):322-7. [QxMD MEDLINE Link].

26. Brook MM. Pediatric bacterial endocarditis. Treatment and prophylaxis. Pediatr Clin North Am. 1999 Apr. 46(2):275-87. [QxMD MEDLINE Link].

27. Parai JL, Masters RG, Walley VM, et al. Aortic medial changes associated with bicuspid aortic valve: myth or reality?. Can J Cardiol. 1999 Nov. 15(11):1233-8. [QxMD MEDLINE Link].

28. Michelena HI, Khanna AD, Mahoney D, et al. Incidence of aortic complications in patients with bicuspid aortic valves. JAMA. 2011 Sep 14. 306(10):1104-12. [QxMD MEDLINE Link].

29. Lamas CC, Eykyn SJ. Bicuspid aortic valve--A silent danger: analysis of 50 cases of infective endocarditis. Clin Infect Dis. 2000 Feb. 30(2):336-41. [QxMD MEDLINE Link].

30. Cripe L, Andelfinger G, Martin LJ, et al. Bicuspid aortic valve is heritable. J Am Coll Cardiol. 2004 Jul 7. 44(1):138-43. [QxMD MEDLINE Link].

31. Wessels MW, Berger RM, Frohn-Mulder IM, et al. Autosomal dominant inheritance of left ventricular outflow tract obstruction. Am J Med Genet A. 2005 Apr 15. 134(2):171-9. [QxMD MEDLINE Link].

32. Hallidie-Smith KA, Karas S. Cardiac anomalies in Williams-Beuren syndrome. Arch Dis Child. 1988 Jul. 63(7):809-13. [QxMD MEDLINE Link].

33. Gelb BD, Zhang J, Sommer RJ, Wasserman JM, Reitman MJ, Willner JP. Familial patent ductus arteriosus and bicuspid aortic valve with hand anomalies: a novel heart-hand syndrome. Am J Med Genet. 1999 Nov 19. 87(2):175-9. [QxMD MEDLINE Link].

34. Sachdev V, Matura LA, Sidenko S, et al. Aortic valve disease in Turner syndrome. J Am Coll Cardiol. 2008 May 13. 51(19):1904-9. [QxMD MEDLINE Link].

35. Zuberbuhler JR. Aortic stenosis. Clinical Diagnosis in Pediatric Cardiology. London, England: Churchill Livingstone; 1981. 75-82.

36. [Guideline] Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents; National Heart, Lung, and Blood Institute. Expert panel on integrated guidelines for cardiovascular health and risk reduction in children and adolescents: summary report. Pediatrics. 2011 Dec. 128(Suppl 5):S213-56. [QxMD MEDLINE Link]. [Full Text].

37. Brandenburg RO Jr, Tajik AJ, Edwards WD, et al. Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: echocardiographic-anatomic correlation in 115 patients. Am J Cardiol. 1983 May 15. 51(9):1469-73. [QxMD MEDLINE Link].

38. McKusick VA. Aortic Valve Disease 1; AOVD1. Online Mendelian Inheritance in Man, OMIM. 1986 Jun 04. [Full Text].

39. [Guideline] 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 Oct 9. 116(15):1736-54. [QxMD MEDLINE Link].

40. Rao PS. Balloon aortic valvuloplasty. Indian Heart J. 2016 Sep - Oct. 68(5):592-5. [QxMD MEDLINE Link]. [Full Text].

41. Stefani L, Galanti G, Toncelli L, et al. Bicuspid aortic valve in competitive athletes. Br J Sports Med. 2008 Jan. 42(1):31-5; discussion 35. [QxMD MEDLINE Link].

42. De Mozzi P, Longo UG, Galanti G, Maffulli N. Bicuspid aortic valve: a literature review and its impact on sport activity. Br Med Bull. 2008. 85:63-85. [QxMD MEDLINE Link].

43. [Guideline] 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 Feb 16. 67(6):724-31. [QxMD MEDLINE Link]. [Full Text].

44. [Guideline] Erbel R, Aboyans V, Boileau C, et al, for the ESC Committee for Practice Guidelines. 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 Nov 1. 35(41):2873-926. [QxMD MEDLINE Link]. [Full Text].

45. [Guideline] Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and ascending aorta guidelines for management and quality measures. Ann Thorac Surg. 2013 Jun. 95(6 Suppl):S1-66. [QxMD MEDLINE Link]. [Full Text].

46. [Guideline] Baddour LM, Wilson WR, Bayer AS, et al. Infective endocarditis in adults: diagnosis, antimicrobial therapy, and management of complications: a scientific statement for healthcare professionals from the American Heart Association. Circulation. 2015 Oct 13. 132(15):1435-86. [QxMD MEDLINE Link]. [Full Text].

47. [Guideline] Habib G, Lancellotti P, Antunes MJ, et al. 2015 ESC guidelines for the management of infective endocarditis: The Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J. 2015 Nov 21. 36(44):3075-128. [QxMD MEDLINE Link]. [Full Text].

48. [Guideline] Nishimura RA, Otto CM, Bonow RO, et al, for the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 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 Jun 10. 63(22):e57-185. [QxMD MEDLINE Link]. [Full Text].

49. Freedom RM, Mawson JB, Yoo SJ. Left ventricular outflow tract obstruction. Congenital heart disease: Textbook of angiocardiography. Armonk, NY: Futura Publishing Company, Inc; 1997. 787-847.

50. Rodrigues I, Agapito AF, de Sousa L, et al. Bicuspid aortic valve outcomes. Cardiol Young. 2017 Apr. 27(3):518-29. [QxMD MEDLINE Link].

51. Herrmann JL, Stram AR, Brown JW. Ross procedure: how to do it and how to teach it. World J Pediatr Congenit Heart Surg. 2019 Sep. 10(5):624-7. [QxMD MEDLINE Link].

52. Attinger-Toller A, Bhindi R, Perlman GY, et al. Mid-term outcome in patients with bicuspid aortic valve stenosis following transcatheter aortic valve replacement with a current generation device: a multicenter study. Catheter Cardiovasc Interv. 2020 May 1. 95(6):1186-92. [QxMD MEDLINE Link].

53. Kochman J, Zbronski K, Koltowski L, et al. Transcatheter aortic valve implantation in patients with bicuspid aortic valve stenosis utilizing the next-generation fully retrievable and repositionable valve system: mid-term results from a prospective multicentre registry. Clin Res Cardiol. 2020 May. 109(5):570-80. [QxMD MEDLINE Link]. [Full Text].

Duraisamy Balaguru, MBBS, MRCP, FACC, FAAP, FSCAI Physician in Pediatric Medicine, Division of Pediatric and Congenital Cardiology, Massachusetts General Hospital, Massachusetts General Hospital for Children, Harvard Medical School

Duraisamy Balaguru, MBBS, MRCP, FACC, FAAP, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Stroke Association, Council on Lifelong Congenital Heart Disease and Heart Health in the Young (AHA), International Society of Invasive Cardiology in Congenital Heart Disease, Pediatric Cardiac Intensive Care Society, Society for Cardiovascular Angiography and Interventions, World Society for Pediatric and Congenital Heart Surgery

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD Emeritus Professor of Pediatrics, University of Pennsylvania School of Medicine

Disclosure: Nothing to disclose.

Syamasundar 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

Syamasundar 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

Disclosure: Nothing to disclose.

Paul 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

Paul 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

Disclosure: Nothing to disclose.

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