Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction

doi:10.1016/j.amjcard.2004.08.098

The American Journal of Cardiology

Volume 95, Issue 2, 15 January 2005, Pages 292-294

https://doi.org/10.1016/j.amjcard.2004.08.098 Get rights and content

The dimensions of the entire aorta at different anatomic levels were measured by transthoracic 2-dimensional echocardiography in 162 consecutive patients with isolated bicuspid aortic valves (BAVs) without significant aortic valve dysfunction. Aortic dilation involved the aortic root and the ascending aorta but was not present in the descending and abdominal aorta. A significant increase in the dimensions of the aortic arch was found in patients with BAVs aged >40 years. Ascending aortic diameter and the extension of aortic dilation were significantly correlated with age, but no correlation was found between aortic dimensions and aortic valve morphology.

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Acknowledgment

We thank Francesco Antonini Canterin, MD, for his critical review and suggestions.

References (18)

R.T. Pachulski et al.
Aortic aneurysm in patients with functionally normal or minimally stenotic bicuspid aortic valve
Am J Cardiol
(1991)
R.T. Hahn et al.
Association of aortic dilation with regurgitant, stenotic and functionally normal bicuspid aortic valves
J Am Coll Cardiol
(1992)
M. Ando et al.
Thoracic aortic aneurysm associated with congenital bicuspid aortic valve
Cardiovasc Surg
(1998)
H.Y. Sabet et al.
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)
J.M. Alegret et al.
Prevalence of and predictors of bicuspid aortic valves in patients with dilated aortic roots
Am J Cardiol
(2003)
G.M. Novaro et al.
Features and predictors of ascending aortic dilatation in association with a congenital bicuspid aortic valve
Am J Cardiol
(2003)
M. Ferencik et al.
Changes in size of ascending aorta and aortic valve function with time in patients with congenitally bicuspid aortic valves
Am J Cardiol
(2003)
R.O. Brandenburg et al.
Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: echocardiographic-anatomic correlation in 115 patients
Am J Cardiol
(1983)
W.C. Roberts
The congenitally bicuspid aortic valve: a study of 85 autopsy cases
Am J Cardiol
(1970)

There are more references available in the full text version of this article.

Cited by (111)

Does the Leaflet Fusion Subtype Affect Pattern and Rate of Growth in BAV Aortopathy?: A Study of 102 BAV Aortopathy Cases With A Literature Review
2021, Heart Lung and Circulation
Bicuspid aortic valves (BAV) and related aortopathy remain an intriguing topic. Not all BAVs get diseased and around 40% would develop aortic dilatation in their lifetime. If haemodynamic theory is to be believed, then leaflet fusion pattern should have an impact. This study sought to compare the association of aortic morphologies and rate of growth in a set of 102 BAV acropathies operated at a single centre, based on the fusion patterns.
Data on aortic valve replacements over a 10-year period was analysed from a prospectively maintained database. Of the 198 BAV undergoing surgery, 102 had aortic dilatation above 40 mm on echocardiogram. These underwent computed tomography (CT) aortograms and were followed up as a part of a database. The impact of leaflet fusion patterns on aortic dilatation pattern and rate was analysed.
Of the 102, two patients had type 0 pathology and one had left-noncoronary (LN) leaflet fusion. Seventy-four (74) had type 1A or left-right (RL) fusion and 25 had type 1B right-noncoronary (RN) fusion. RL fusion had more males, were taller, bigger and had more proportion of aortic stenosis (AS). Aortic diameters, angles and growth rates at root, ascending/descending aorta and arch were not different. Regression analyses for size or growth did not show any significant impact of fusion pattern.
Left-right fusion pattern comprised three-quarters of BAV in this cohort and these patients were bigger, taller and had a greater proportion of males with increased rate of aortic stenosis. Despite these differences, there was no significant impact of fusion pattern on aortic size or rate of growth.
Clinical Outcomes of Adults With Bicuspid Aortic Valve: A European Perspective
2021, Mayo Clinic Proceedings
Citation Excerpt :
Only 5 patients were reoperated for the aortic valve during follow-up. Patients with BAV have larger aortic dimensions than adults with tricuspid aortic valves,1,21,22 with a risk of aortic aneurysms 80-fold higher than in the general population.14 In our study, approximately 40% of patients of the surgical group underwent ascending aorta surgeries, and only 6% of them had had associated replacements of the aortic arch.
To describe the clinical history of patients with a wide age range diagnosed with bicuspid aortic valve (BAV) and no surgical indication and to evaluate the long-term outcome of patients with BAV referred for elective surgery.
Between 2005 and 2017, 350 consecutive patients with no surgical indication (surveillance group, mean age 53±16, 71% men) and 191 with a surgical indication (surgical group, mean age 59±13, 71% men) were prospectively included. Median follow-up was 80 (32 to 115) months.
In the surveillance group, the 5-year and 10-year survival rates were 93±1% and 89±2%, respectively, with a relative survival of patients with BAV compared with an age- and sex-matched control population of 98.7%. During follow-up, the cumulative 10-year incidence of aortic valve and aorta surgery was high; of 35±4%, the incidence of native valve infective endocarditis (IE) of 0.2% per patient-year, and no cases of aortic dissection were observed. In the surgical group, the 5-year and 10-year survival rates were 97±1% and 89±3%, respectively, with a relative survival of 99.4% compared with the general population. The incidence of IE was 0.4% per patient-year, and no cases of aortic dissection were observed.
This regional cohort shows that the 10-year survival rates of patients with BAV and a wide age range, but mostly middle-aged adults, were similar to those of the general population with a very low rate of complications. Adherence to prophylactic surgical indications and younger age might have contributed to this lack of difference.
Aortopathy in Congenital Heart Disease
2020, Cardiology Clinics
Defective NOTCH signaling drives increased vascular smooth muscle cell apoptosis and contractile differentiation in bicuspid aortic valve aortopathy: A review of the evidence and future directions
2019, Trends in Cardiovascular Medicine
Bicuspid aortic valve (BAV) disease remains the most common congenital cardiac disease and is associated with an increased risk of potentially fatal aortopathy including aortic aneurysm and dissection. Mutations in the NOTCH1 gene are one of only a few genetic anomalies identified in BAV disease; however evidence for defective NOTCH signaling, and its involvement in the characteristic histological changes of VSMC apoptosis and differentiation in ascending aortae of BAV patients is lacking. This review scrutinizes the evidence for the interactions of NOTCH signaling, cellular differentiation and apoptosis in the context of aortic VSMCs and provides focus for future research efforts in the diagnosis of BAV aortopathy and prevention of catastrophic complications through NOTCH signaling manipulation.
The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve–related aortopathy: Full online-only version
2018, Journal of Thoracic and Cardiovascular Surgery
Bicuspid aortic valve disease is the most common congenital cardiac disorder, being present in 1% to 2% of the general population. Associated aortopathy is a common finding in patients with bicuspid aortic valve disease, with thoracic aortic dilation noted in approximately 40% of patients in referral centers. Several previous consensus statements and guidelines have addressed the management of bicuspid aortic valve–associated aortopathy, but none focused entirely on this disease process. The current guidelines cover all major aspects of bicuspid aortic valve aortopathy, including natural history, phenotypic expression, histology and molecular pathomechanisms, imaging, indications for surgery, surveillance, and follow-up, and recommendations for future research. It is intended to provide clinicians with a current and comprehensive review of bicuspid aortic valve aortopathy and to guide the daily management of these complex patients.
Aortic Dilatation Associated With Bicuspid Aortic Valve: Relation to Sex, Hemodynamics, and Valve Morphology (the National Heart Lung and Blood Institute-Sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions)
2017, American Journal of Cardiology
This study analyzed the impact of sex, hemodynamic profile, and valve fusion pattern on aortopathy associated with bicuspid aortic valve (BAV). The National Heart Lung and Blood Institute-sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) provided comprehensive information on a large population of well-characterized patients with BAV. Of 969 enrolled patients with BAV, 551 (57%, 77% male) had already undergone valvular and/or aortic surgery. Echocardiographic imaging data were available on 339 unoperated or preoperative participants who formed the basis of this study. BAV function was normal in 45 (14%), with a predominant aortic regurgitation (AR) in 127 (41%) and a predominant aortic stenosis (AS) in 76 (22%). Moderate-severe AR was associated with larger sinus of Valsalva (SOV) diameters compared with normal function and AS (all p <0.01). Moderate-severe AS was associated with a larger ascending aortic (AscAo) diameter compared with normal function (p = 0.003) but not with AR. The SOV diameter was larger in men than in women (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001), whereas AscAo diameters were comparable (3.9 ± 0.9 vs 3.7 ± 0.9 cm, p = 0.08). Right-left commissural fusion was associated with a larger SOV diameter (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001) compared with a right-noncoronary fusion pattern. Predominant AR was more common in men (45% vs 27%, p = 0.004), whereas AS was more common in women (29% vs 18%, p = 0.04). In conclusion, in the GenTAC Registry, AR was associated with diffuse (annular, SOV, and AscAo) enlargement, whereas moderate-severe AS was only associated with AscAo enlargement. Male sex and right-left cusp pattern of cusp fusion were associated with larger SOV diameters and a greater likelihood of AR, whereas women had a higher prevalence of AS.

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Brief reportsAortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction

Section snippets

Acknowledgment

Am J Cardiol

J Am Coll Cardiol

Cardiovasc Surg

Mayo Clin Proc

Am J Cardiol

Am J Cardiol

Am J Cardiol

Am J Cardiol

Am J Cardiol

Brief reports
Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction