Predictors of Ascending Aortic Dilation in Bicuspid Aortic Valve Disease: A Five-year Prospective Study

Avadhani, Sriya; Martin‐Doyle, William; Shaikh, Amir Y.; Pape, Linda

doi:10.1016/j.amjmed.2014.12.027

Cited by 31 publications

(29 citation statements)

References 20 publications

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“…The growth rate of the ascending aorta in patients with BAV ranges from 0.2 to 1.9 mm/year, a rate much greater than in patients with TAV 24. In 115 patients with BAV (mean age 42±13 years; baseline aortic size 35.5±5/6 mm), the ascending aortic size increased 0.66 mm/year (range 0.2–2.2 mm/year) over 4.8 years 25. In 15% of patients, the aorta grew >1 mm/year 25.…”

Section: How Quickly Does the Bav-associated Aortic Aneurysm Typicallmentioning

confidence: 97%

“…In 115 patients with BAV (mean age 42±13 years; baseline aortic size 35.5±5/6 mm), the ascending aortic size increased 0.66 mm/year (range 0.2–2.2 mm/year) over 4.8 years 25. In 15% of patients, the aorta grew >1 mm/year 25. Oliver et al 26 reported an increase in ascending aorta size of 0.25 mm/year among 244 patients with isolated BAV.…”

Section: How Quickly Does the Bav-associated Aortic Aneurysm Typicallmentioning

confidence: 99%

“…The aortic root phenotype (dilatation at the sinuses as opposed to the ascending aorta) is associated with more rapid aortic dilatation,41 aortic regurgitation42 and may have a genetic component 42. A family history of aortic valve disease may correlate with more rapid BAV aortic growth rate 25. Valve dysfunction at the time of diagnosis is associated with higher risk of cardiovascular events 22.…”

Section: Prognostic Factors In Patients With Bav Aortopathymentioning

confidence: 99%

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Surgical threshold for bicuspid aortic valve aneurysm: a case for individual decision-making

Adamo

Braverman

2015

Heart

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The bicuspid aortic valve (BAV) affects 1-2% of the population and may be associated with important valvular disease and an increased risk of aortic root and/or ascending aortic aneurysm and dissection. BAV aortic aneurysm and dissection occur earlier in life than when these disorders are associated with a tricuspid aortic valve (TAV). Alterations in the aortic media and differences in aortic elastic properties and wall stress also accompany BAV aortopathy. With appropriate follow-up and timely surgery, population studies have documented a survival rate for patients with BAV no different from age-matched controls. Guidelines have previously recommended prophylactic aortic surgery at a smaller aortic aneurysm size for patients with BAV compared with aneurysms in patients with a TAV. Recent guidelines have presented differing indications regarding the appropriate timing of prophylactic surgery for BAV aneurysms, giving the recommendation for surgery when the aortic root and/or ascending aortic exceeds 5.5 cm (unless certain factors are present), the same size for which TAV-associated aortic aneurysm surgery is recommended. We review the pathophysiology of BAV aortopathy, the clinical history of BAV ascending aortic disease, areas of uncertainty and make a case for a patient-centered, individualised decision regarding the optimal timing of aortic aneurysm surgery in BAV disease.

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Section: How Quickly Does the Bav-associated Aortic Aneurysm Typicallmentioning

confidence: 97%

Section: How Quickly Does the Bav-associated Aortic Aneurysm Typicallmentioning

confidence: 99%

Section: Prognostic Factors In Patients With Bav Aortopathymentioning

confidence: 99%

See 1 more Smart Citation

Surgical threshold for bicuspid aortic valve aneurysm: a case for individual decision-making

Adamo

Braverman

2015

Heart

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“…Few studies are available regarding dilatation rates and associated risk factors, showing variable outcomes. [2,19–21] Therefore, the aim of this study was to analyze the dimensions and dilatation rates of different segments of the ascending aorta and its determinants/risk factors, including BAV morphology.…”

Section: Introductionmentioning

confidence: 99%

Clinical and echocardiographic determinants in bicuspid aortic dilatation

et al. 2016

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Bicuspid aortic valve (BAV) disease is associated with aortic dilatation. Timing of follow-up and surgery is challenging. Hence, there is an unmet clinical need for additional risk stratification. It is unclear whether valve morphology is associated with dilatation rates. Therefore, the objective of this study was to examine the association between clinical and echocardiographic determinants (including valve morphology) and aortic dimension and the progression rate of dilatation.Aortic dimensions were assessed on serial echocardiographic images between 1999 and 2014 in a population of 392 patients with BAVs in a tertiary care center in the Netherlands. Analyses using mixed linear models were performed.Mean age of participants was 48 ± 17 years and 69% were male. BAV morphology was associated with aortic dimensions, as well as age, sex, BSA, and valvular dysfunction. Tubular ascending aorta, sinus of Valsalva, and sinotubular junction showed a dilatation rate of 0.32, 0.18, and 0.06 mm/year, respectively. Dilatation rate was not associated with valve morphology.In the present study, there is no association between BAV morphology and aortic dilatation rates. Therefore, morphology is of limited use in prediction of aortic growth. Discovering fast progressors remains challenging.

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“…A prospective adult study of 115 patients with asymptomatic BAV followed over a 5 year period showed that family history of aortic valve disease was associated with a significantly increased risk of increasing ascending aortic size, while no correlations with cusp fusion pattern or aortic valve dysfunction were found. 15 Other studies have shown additional risk factors, including a study of 68 adult patients with BAV showing that aortic insufficiency or stenosis may increase the development of ascending aorta dilatation. 16 Similarly, a large study of children with isolated BAV examined the incidence and rate of dilation of the ascending aorta versus a control population found that the BAV group had a larger ascending aorta initially and it increased in size over the follow-up period.…”

Section: Discussionmentioning

confidence: 99%

Abnormal Longitudinal Growth of the Aorta in Children with Familial Bicuspid Aortic Valve

et al. 2017

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Background Bicuspid aortic valve (BAV) is the most common type of congenital heart defect (CHD) and is associated with clinically significant cardiovascular complications including valve calcification and ascending aortopathy (AscAo), predominantly occurring in adulthood. While a limited number of genetic etiologies for BAV have been defined, family members of affected individuals display BAV along with other left-sided CHD. This has led to guidelines from the American Heart Association and American College of Cardiology that recommend echocardiographic screening of first-degree relatives of affected adults. While potentially beneficial in adults, the yield of such screening in children is unknown. The purpose of this study was to investigate a cohort of children with familial BAV to determine the frequency of development of AscAo, and to identify risk factors that contribute to abnormal aortic growth. Methods: Echocardiograms over a 10 year follow up period were reviewed on 26 patients with familial BAV (22 male, 4 female; 22 with isolated BAV, 6 with BAV and aortic coarctation (CoA)). All had a family history of CHD and were recruited from 2005–2010 as part of a genetics research study. Four aortic segments (annulus, root, sinotubular junction, ascending aorta) on parasternal long axis echocardiographic images were measured by a single observer. The mean age at first echocardiogram was 7.1+/−5.5 years and 13.8+/−6.2 years on the last. Only patients with >2 echocardiograms in the 10 year period were included. Z score measurements of the aorta were plotted over time and based on these the cohort was divided into two groups: Group 1 (abnormal) – Z score for any segment >2 or a change in Z score >2 over follow up; Group 2 (normal) – Z score <2 throughout follow up and change in Z score <2. Results 19 out of 26 children displayed abnormal aortic growth or dilation of the aorta. BAV with right/left cusp fusion was more frequent in Group 1(15/18) versus Group 2(3/7) (p<0.05). There were no significant differences in gender, aortic valve dysfunction, presence of CoA, family history, cardiac function, presence of left ventricular hypertrophy, or medication use between the 2 groups. Conclusions In our longitudinal study of children with familial BAV, the majority display evidence of abnormal growth of the ascending aorta during the follow up period consistent with AscAo and support the extension of current adult guidelines to the pediatric population. While we find that right/left cusp fusion is a risk factor for abnormal aortic growth, additional studies are needed to identify other factors to better select children who require serial screening.

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Predictors of Ascending Aortic Dilation in Bicuspid Aortic Valve Disease: A Five-year Prospective Study

Cited by 31 publications

References 20 publications

Surgical threshold for bicuspid aortic valve aneurysm: a case for individual decision-making

Surgical threshold for bicuspid aortic valve aneurysm: a case for individual decision-making

Clinical and echocardiographic determinants in bicuspid aortic dilatation

Abnormal Longitudinal Growth of the Aorta in Children with Familial Bicuspid Aortic Valve

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