Kardiologie up2date 2020; 16(01): 59-71
DOI: 10.1055/a-0994-0032
Aorten- und Gefäßerkrankungen
Georg Thieme Verlag KG Stuttgart · New York

Die bikuspide Aortenklappe

The bicuspid aortic valve
Stefanos Sakellaropoulos
,
Thomas Baumgartner
,
Andreas Mitsis
,
Xun Yuan
,
Christoph A. Nienaber
Further Information

Publication History

Publication Date:
11 March 2020 (online)

Zusammenfassung

Die bikuspide Aortenklappe ist der häufigste kongenitale Herzfehler und gleichzeitig die häufigste angeborene Fehlbildung der Aortenklappe bei Erwachsenen. In dieser Übersichtsarbeit werden die verschiedenen Aspekte der bikuspiden Aortenklappe einschließlich ihrer Komplikationen unter klinischen Gesichtspunkten dargestellt.

Abstract

The bicuspid aortic valve is the most common congenital heart disease as also the most common congenital malformation of the aortic valve. Due to altered hemodynamics, anatomy and geometry of the valve apparatus there is a high risk of complications, with the most common of them the aortic valve stenosis. Echocardiography is primarily considered as the gold standard for the diagnosis. However computed tomography and magnetic resonance tomography belong to the diagnostic armamentarium for precise diagnosis, risk stratification and defining the therapeutic approaches. Operative aortic valve replacement has been ultimately the gold standard therapy for the bicuspid aortic valve, although in the last years, transaortic valve replacement emerge as an alternative option with excellent results.

Kernaussagen
  • Die bikuspide Aortenklappe ist der häufigste kongenitale Herzfehler mit einer Inzidenz von 0,5 bis 2% und gleichzeitig die häufigste angeborene Fehlbildung der Aortenklappe bei Erwachsenen.

  • Aufgrund des genetischen Hintergrunds ist ein echokardiografisches Screening wertvoll.

  • Die Echokardiografie ist der diagnostische Goldstandard. CT und MRT gehören ebenfalls zum diagnostischen Armamentarium.

  • Aufgrund der Pathogenität treten häufig Komplikationen auf, am häufigsten ist dabei die Aortenklappenstenose.

  • Die Therapieoptionen sind die medikamentöse Behandlung, interventionelle und operative Behandlung, z. B. TAVI und der Aortenklappenersatz.

  • Im Rahmen einer interventionellen Behandlung kann es zu Komplikationen kommen, die meist akut behandelt werden müssen, z. B. hochgradige AV-Blockierung oder schwere Blutung.

  • Nicht nur der Klappenapparat, sondern auch Biomarker und insbesondere die Aorta müssen im Verlauf kontrolliert werden.

 
  • Literatur

  • 1 Fedak PWM, Verma S, David TE. et al. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation 2002; 106: 900-904
  • 2 Glick BN, Roberts WC. Congenitally bicuspid aortic valve in multiple family members. Am J Cardiol 1994; 73: 400-404
  • 3 Martin LJ, Ramachandran V, Cripe LH. et al. Evidence in favor of linkage to human chromosomal regions 18q, 5q and 13q for bicuspid aortic valve and associated cardiovascular malformations. Hum Genet 2007; 121: 275-284
  • 4 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). J Am Coll Cardiol 2008; 52: e1-e121
  • 5 Zhang X, Zhu M, He T. et al. Cardiac mechanics in isolated bicuspid aortic valve disease with normal ejection fraction: a study of various valvular lesion types. Medicine (Baltimore) 2015; 94: e2085
  • 6 Stefani L, De Luca A, Maffulli N. et al. Speckle tracking for left ventricle performance in young athletes with bicuspid aortic valve and mild aortic regurgitation. Eur J Echocardiogr 2009; 10: 527-531
  • 7 Hein S, Arnon E, Kostin S. et al. Progression from compensated hypertrophy to failure in the pressure-overloaded human heart: structural deterioration and compensatory mechanisms. Circulation 2003; 107: 984-991
  • 8 Michelena HI, Della Corte A, Prakash SK. et al. Bicuspid aortic valve aortopathy in adults: incidence, etiology, and clinical significance. Int J Cardiol 2015; 201: 400-407
  • 9 Kilickesmez KO, Abaci O, Kocas C. et al. Dilatation of the ascending aorta and serum alpha 1-antitrypsin level in patients with bicuspid aortic valve. Heart Vessels 2012; 27: 391-397
  • 10 Branchetti E, Bavaria JE, Grau JB. et al. Circulating soluble receptor for advanced glycation end product identifies patients with bicuspid aortic valve and associated aortopathies. Arterioscler Thromb Vasc Biol 2014; 34: 2349-2357
  • 11 Tzemos N, Therrien J, Yip J. et al. Outcomes in adults with bicuspid aortic valves. JAMA 2008; 300: 1317-1325
  • 12 Ward C. Clinical significance of the bicuspid aortic valve. Heart 2000; 83: 81-85
  • 13 Cecconi M, Manfrin M, Moraca A. et al. Aortic dimension in patients with bicuspid aortic valve without significant valve dysfunction. Am J Cardiol 2005; 95: 292-294
  • 14 Russell TA, Quarto C, Nienaber CA. A single-stage hybrid approach for the management of severely stenotic bicuspid aortic valve, ascending aortic aneurysm, and coarctation of the aorta with a literature review. J Cardiol Cases 2018; 17: 183-186
  • 15 Januzzi JL, Isselbacher EM, Fattori R. et al. Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). J Am Coll Cardiol 2004; 43: 665-669
  • 16 Bossone E, Pyeritz RE, OʼGara P. et al. International Registry of Acute Aortic Dissection (IRAD) Investigators. Acute aortic dissection in blacks: insights from the International Registry of Acute Aortic Dissection. Am J Med 2013; 126: 909-915
  • 17 Fenoglio JJ, McAllister HA, DeCastro CM. et al. Congenital bicuspid aortic valve after age 20. Am J Cardiol 1977; 39: 164
  • 18 Bauer M, Pasic M, Meyer R. et al. Morphometric analysis of aortic media in patients with bicuspid and tricuspid aortic valve. Ann Thorac Surg 2002; 74: 58-62
  • 19 Turina J, Turina M, Krayenbühl HP. Die Bedeutung der bikuspiden Aortenklappe für die Entstehung des Aortenklappenvitiums bei Erwachsenen. Schweiz med Wschr 1986; 116: 1518-1523
  • 20 Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 1970; 26: 72-83
  • 21 Sabet HY, Edwards WD, Tazelaar HD. et al. Congenitally bicuspid aortic valves: A surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2715 additional cases. Mayo Clin Proc 1999; 74: 14-26
  • 22 Brooke BS, Habashi JP, Judge DP. et al. Angiotensin II blockade and aortic-root dilation in Marfanʼs syndrome. N Engl J Med 2008; 358: 2787-2795
  • 23 Cowell SJ, Newby DE, Prescott RJ. et al. A randomized trial of intensive lipid-lowering therapy in calcific aortic stenosis. N Engl J Med 2005; 352: 2389-2397
  • 24 Rosenfeld HM, Landzberg MJ, Perry SB. et al. Balloon aortic valvuloplasty in the young adult with congenital aortic stenosis. Am J Cardiol 1994; 73: 1112-1117
  • 25 Yap SC, Drenthen W, Pieper PG. et al. Risk of complications during pregnancy in women with congenital aortic stenosis. Int J Cardiol 2008; 126: 240-246
  • 26 Tzemos N, Silversides CK, Colman JM. et al. Late cardiac outcomes after pregnancy in women with congenital aortic stenosis. Am Heart J 2009; 157: 474-480
  • 27 Johnston KW, Rutherford RB, Tilson MD. et al. Suggested standards for reporting on arterial aneurysms. Subcommittee on Reporting Standards for Arterial Aneurysms, Ad Hoc Committee on Reporting Standards, Society for Vascular Surgery and North American Chapter, International Society for Cardiovascular Surgery. J Vasc Surg 1991; 13: 452-458
  • 28 Grotenhuis HB, Ottenkamp J, Westenberg JJ. et al. Reduced aortic elasticity and dilatation are associated with aortic regurgitation and left ventricular hypertrophy in nonstenotic bicuspid aortic valve patients. J Am Coll Cardiol 2007; 49: 1660-1665
  • 29 Weber M, Hausen M, Arnold R. et al. Diagnostic and prognostic value of N-terminal pro B-type natriuretic peptide (NT-proBNP) in patients with chronic aortic regurgitation. Int J Cardiol 2008; 127: 321-327
  • 30 Golledge J, Tsao PS, Dalman RL. et al. Circulating markers of abdominal aortic aneurysm presence and progression. Circulation 2008; 118: 2382-2392