Review Article
Clinical presentation and outcomes of adults with bicuspid aortic valves: 2020 update

https://doi.org/10.1016/j.pcad.2020.05.010Get rights and content

Abstract

The congenital bicuspid aortic valve (BAV) is a heterogeneous condition that is better understood considering 3 types of valvulo-aortopathy presentations: A typical-presentation valvulo-aortopathy, a complex-presentation valvulo-aortopathy and an uncomplicated/undiagnosed-presentation. The burden of BAV-related complications has been elucidated in patients with the typical-presentation valvulo-aortopathy and is discussed in this review. These patients enjoy an overall normal expected long-term survival as long as complications are identified and treated in a timely manner. This notion and the fact that the most dreaded complications (infective endocarditis and aortic dissection) are infrequent, represent important reassuring points for the BAV patient. Common complications such as valve dysfunction and aorta dilatation must be the subject of focused research in prevention and treatment. Conversely, BAV patients with complex-presentation valvulo-artopathy, as well as typical valvulo-aortopathy BAV patients who are older, with advanced valvular dysfunction, and possible late-referral to specialized care, may incur a survival penalty as compared to the general population. An evidence-based discussion of these concepts is provided in this review.

Section snippets

General nosology of the congenital BAV condition

Fundamentally, the congenital BAV is a valvulo-aortopathy characterized by significant heterogeneity of its valvular and aorta phenotypic expressions, of its associated disorders, of its complications and its prognosis.6., 7., 8., 9. From the nosology perspective, and in order to reconcile this clinical and prognostic heterogeneity, the BAV clinical-presentation may be categorized (Fig 1) into 3 general groups: 1) complex -presentation valvulo-aortopathy10 where concomitant or associated

Clinical complications in BAV adults with typical valvulo-aortopathy

Table 1 shows 17 BAV cohorts from 16 published manuscripts depicting complications and clinical outcomes of BAV up to 2020.3,8,13., 14., 15., 16., 17., 18., 19., 20., 21., 22., 23., 24., 25., 26.Table 2 shows the most common BAV complications in typical valvulo-aortopathy adults, in order of frequency.

Marfan syndrome and BAV: different diseases

Aortopathy in BAV was originally thought to be Marfan-like because of the similar distribution of fibrillin, fibronectin, and tenascin and vascular smooth muscle cell apoptosis in both.46 Therefore, patients with Marfan syndrome and BAV were recommended to undergo elective surgery for aorta-repair at the same maximal aortic diameter (50 mm) in previous Aorta guidelines.47 Almost 5 decades ago, a landmark natural study reported that patients with Marfan syndrome had poor survival; patients died

Conclusions

BAV is a heterogeneous condition that is better understood considering 3 types of valvulo-aortopathy presentations (Fig 1). The burden of BAV-related complications has been elucidated in patients with the typical-presentation valvulo-aortopathy (Table 1, Table 2), who enjoy a normal expected long-term survival as long as complications are identified and treated in a timely manner. This notion and the fact that the most dreaded complications (IE and aortic dissection) are infrequent, represent

Statement of conflict of interest

There is no conflict of interest of any of the listed authors.

References (52)

  • S. Itagaki et al.

    Long-term risk for aortic complications after aortic valve replacement in patients with bicuspid aortic valve versus Marfan syndrome

    J Am Coll Cardiol

    (2015)
  • A.G. Sherrah et al.

    Nonsyndromic thoracic aortic aneurysm and dissection: outcomes with Marfan syndrome versus bicuspid aortic valve aneurysm

    J Am Coll Cardiol

    (2016)
  • J.W. Weinsaft et al.

    Aortic dissection in patients with genetically mediated aneurysms: incidence and predictors in the GenTAC registry

    J Am Coll Cardiol

    (2016)
  • L.T. Yang et al.

    Contemporary etiologies, mechanisms, and surgical approaches in pure native aortic regurgitation

    Mayo Clin Proc

    (2019)
  • A. Hagendorff et al.

    Improvement in the assessment of aortic valve and aortic aneurysm repair by 3-dimensional echocardiography

    JACC Cardiovasc Imaging

    (2019)
  • U. Schneider et al.

    Two decades of experience with root remodeling and valve repair for bicuspid aortic valves

    J Thorac Cardiovasc Surg

    (2017)
  • G.Y. Perlman et al.

    Bicuspid aortic valve stenosis: favorable early outcomes with a next-generation transcatheter heart valve in a multicenter study

    JACC Cardiovasc Interv

    (2016)
  • D.J. Blackman et al.

    Long-term durability of transcatheter aortic valve prostheses

    J Am Coll Cardiol

    (2019)
  • V. Lad et al.

    Mitral regurgitation due to myxomatous degeneration combined with bicuspid aortic valve disease is often due to prolapse of the anterior leaflet of the mitral valve

    Ann Thorac Surg

    (2009)
  • M.A. Borger et al.

    The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: full online-only version

    J Thorac Cardiovasc Surg

    (2018)
  • J.L. Januzzi et al.

    Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD)

    J Am Coll Cardiol

    (2004)
  • L.F. Hiratzka 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 Thorac Cardiovasc Surg

    (2016)
  • M.L. Brown et al.

    Coarctation of the aorta: lifelong surveillance is mandatory following surgical repair

    J Am Coll Cardiol

    (2013)
  • W.C. Roberts et al.

    Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation

    Circulation

    (2005)
  • H.I. Michelena et al.

    Incidence of aortic complications in patients with bicuspid aortic valves

    JAMA.

    (2011)
  • H.I. Michelena et al.

    Bicuspid aortic valve: identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon)

    Circulation.

    (2014)
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