Review ArticleClinical presentation and outcomes of adults with bicuspid aortic valves: 2020 update☆
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)
- et al.
Incidence of infective endocarditis in patients with bicuspid aortic valves in the community
Mayo Clin Proc
(2016) - et al.
The incidence of congenital heart disease
J Am Coll Cardiol
(2002) - et al.
Population-based cohort studies: still relevant?
J Am Coll Cardiol
(2011) - et al.
Bicuspid aortic valve aortopathy in adults: incidence, etiology, and clinical significance
Int J Cardiol
(2015) The congenitally bicuspid aortic valve. A study of 85 autopsy cases
Am J Cardiol
(1970)- et al.
The bicuspid aortic valve condition: the critical role of echocardiography and the case for a standard nomenclature consensus
Prog Cardiovasc Dis
(2018) - et al.
Characteristic morphologies of the bicuspid aortic valve in patients with genetic syndromes
J Am Soc Echocardiogr
(2018) - et al.
Should the ascending aorta be replaced more frequently in patients with bicuspid aortic valve disease?
J Thorac Cardiovasc Surg
(2004) - et al.
Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve
Ann Thorac Surg
(2007) - et al.
Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves
Am J Cardiol
(2010)
Long-term risk for aortic complications after aortic valve replacement in patients with bicuspid aortic valve versus Marfan syndrome
J Am Coll Cardiol
Nonsyndromic thoracic aortic aneurysm and dissection: outcomes with Marfan syndrome versus bicuspid aortic valve aneurysm
J Am Coll Cardiol
Aortic dissection in patients with genetically mediated aneurysms: incidence and predictors in the GenTAC registry
J Am Coll Cardiol
Contemporary etiologies, mechanisms, and surgical approaches in pure native aortic regurgitation
Mayo Clin Proc
Improvement in the assessment of aortic valve and aortic aneurysm repair by 3-dimensional echocardiography
JACC Cardiovasc Imaging
Two decades of experience with root remodeling and valve repair for bicuspid aortic valves
J Thorac Cardiovasc Surg
Bicuspid aortic valve stenosis: favorable early outcomes with a next-generation transcatheter heart valve in a multicenter study
JACC Cardiovasc Interv
Long-term durability of transcatheter aortic valve prostheses
J Am Coll Cardiol
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
The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: full online-only version
J Thorac Cardiovasc Surg
Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD)
J Am Coll Cardiol
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
Coarctation of the aorta: lifelong surveillance is mandatory following surgical repair
J Am Coll Cardiol
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
Incidence of aortic complications in patients with bicuspid aortic valves
JAMA.
Bicuspid aortic valve: identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon)
Circulation.
Cited by (16)
Distal aortic progression following acute type A aortic dissection repair among patients with bicuspid and tricuspid aortic valves
2023, Journal of Thoracic and Cardiovascular SurgeryComparison Between Bicuspid and Tricuspid Aortic Regurgitation: Presentation, Survival, and Aorta Complications
2022, JACC: AsiaCitation Excerpt :Most prior studies on the incidence of AD focus on patients with genetic aortopathy, including Marfan syndrome, BAV, and nonsyndromic thoracic aneurysms,12,14 but not patients with significant aortic valve disease. Although patients with BAV are frequently complicated by aortopathy, the notion that BAV patients had lower risk (≤1%) of AD than those with Marfan syndrome or other genetic-associated aortic aneurysms12,14,15 is well recognized. The current study also found a low rate of AD among BAV-AR, but the overall cumulative incidence of AD, driven by TAV-AR patients, was high in this study (3.7% ± 1.0% at 10 years; incidence rate of 48.6 [95% CI: 29.7-75.4] per 10,000 person-year).
The role of mechanical valves in the aortic position in the era of bioprostheses and TAVR: Evidence-based appraisal and focus on the On-X valve
2022, Progress in Cardiovascular DiseasesCitation Excerpt :The steady process of degradation occurs as the immune system attacks the foreign material in the aorta.34 The most common congenital heart condition is bicuspid aortic valve,35 and its most common complication is aortic valve dysfunction (stenosis > regurgitation) requiring AVR in over 50% of patients.36 Bicuspid aortic valve patients with hemodynamically significant aortic stenosis are almost 2 decades younger (mean age 63 versus 78 years) than their tricuspid counterparts.37
Long-term outcome of bicuspid aortic valve disease
2024, European Heart Journal Cardiovascular ImagingBicuspid aortic valve: long-term morbidity and mortality
2023, European Heart Journal
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