Aortic dilatation in patients with bicuspid aortic valve

Cecconi, Moreno; Nistri, Stefano; Quarti, Andrea; Manfrin, Marcello; Colonna, Pier Luigi; Molini, E; Perna, Gian Piero

doi:10.2459/01.jcm.0000199777.85343.ec

Cited by 67 publications

(54 citation statements)

References 85 publications

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“…The frequency of pathologic dilatation (in 25% of all patients with BAV) was similar to reported estimates from necropsy and surgical series, where it ranged from 10% to 35% [25]. It was also concordant with the prevalence of pathologic dilatation of ascending aorta reported by other authors in performed echocardiographic studies [4,8].…”

Section: Discussionsupporting

confidence: 79%

Bicuspid aortic valve morphology and its association with aortic diameter: an echocardiographic study

2016

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A b s t r a c tBackground: Bicuspid aortic valve (BAV) is strongly associated with aortopathy. Previous studies have suggested that various types of bicuspid aortic valve morphology may differently affect the aortic dilatation. Aim:To evaluate the impact of BAV cusp fusion morphology (type I -right-left coronary cusp fusion; type II -right-noncoronary cusp fusion) on the diameters of the aorta.Methods: BAV morphology was evaluated retrospectively in a group of 67 consecutive patients with BAV. The control group comprised 1000 randomly selected patients with normal tricuspid aortic valve. Aortic dimensions and other echocardiographic parameters were obtained from the echocardiography database of our department. The diameters of aorta in both BAV subtypes were evaluated at the level of: annulus, the sinus of Valsalva, the sinotubular junction, and the ascending aorta and at the level of the ascending aorta in the control group.Results: Patients with BAV were mainly male (78%), with a mean age of 55.3 ± 16.7 years. The dominant morphology of BAV in the study group was type I (n = 46; 69%). It was associated with increased aortic dimension in comparison to type II BAVs at the level of the sinuses of Valsalva (38.4 ± 5.2 vs. 34.0 ± 4.6 mm, p = 0.002), the sinotubular junction (33.1 ± 5.8 vs. 29.6 ± 5.0 mm, p = 0.035), and the ascending aorta (41.6 ± 7.1 vs. 36.6 ± 6.1 mm, p = 0.006). Indexed aortic diameter was also increased in type I BAV at the level of sinuses of Valsalva (19.6 ± 2.7 vs. 18.1 ± 1.6 mm/m 2 , p = 0.008) and the ascending aorta (21.3 ± 3.4 vs. 19.3 ± 3.4 mm/m 2 , p = 0.048). The dimensions of the ascending aorta exceeding the upper normal range limit based on control-group measurements (44.3 mm) were observed more frequently in type I than in type II (33% vs. 10%, p = 0.044). Aortic regurgitation (moderate or severe) occurred in similar percentages of both BAV subtypes (type I: 37% vs. type II: 33%, p = 0.774). There were also no significant differences in aortic valve area (2.2 ± 1.1 vs. 2.0 ± 1.4 cm 2 , p = 0.163), indexed aortic valve area (1.1 ± 0.6 vs. 1.0 ± 0.6, p = 0.337), peak transvalvular gradient (35.3 ± 20.5 vs. 39.1 ± 28.9 mm Hg, p = 0.862), and mean gradient (18.6 ± 12.3 vs. 22.7 ± 18.2 mm Hg, p = 0.571) and left ventricular ejection fraction (51.8 ± 11.6 vs. 51.8 ± 12.2%, p = 0.978) between type I and type II BAV groups.Conclusions: Type I BAV cusp fusion morphology is more commonly associated with dilatation of the aorta than type II, especially at the level of the sinus of Valsalva and the ascending aorta.

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Section: Discussionsupporting

confidence: 79%

Bicuspid aortic valve morphology and its association with aortic diameter: an echocardiographic study

2016

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“…Interestingly, CAE occurred with and without dilatation of the ascending aorta, another frequent appearance of BAV disease. 15,19,20 By retrospective review of coronary angiograms, CAE was found more than twice as frequently in CMR-confirmed BAV disease compared with TAV disease, and notably, the common coincidence of CAE and BAV disease could be independently confirmed in our in-hospital BAV registry. In this validation study, the sample size of subjects with BAV was more than twice the size of subjects with BAV disease in the initial CMR exploration study.…”

Section: Discussionmentioning

confidence: 91%

“…Most often, root dilatation is observed in younger men with BAV and is independent of the presence or severity of an aortic valve stenosis. 15,20 The aorta in BAV and Marfan syndrome patients and ectatic coronary arteries may share common histopathologic findings, including increased matrix metalloproteinase activity. 16 Moreover, the aortic valve, the proximal part of the coronary arteries, the ascending aorta, and the pulmonary trunk share a common embryonic origin, each developing from the neural crest.…”

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confidence: 99%

Coronary Artery Ectasia Are Frequently Observed in Patients With Bicuspid Aortic Valves With and Without Dilatation of the Ascending Aorta

Meindl

Achatz

Huber

et al. 2016

Circ: Cardiovascular Interventions

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Background— The presence of coronary artery ectasia (CAE) is influenced by genetic factors and related to the presence of aneurysms in other vascular beds. Bicuspid aortic valve (BAV) disease is frequently accompanied by ascending aortic aneurysm. Because the aortic valve and the proximal parts of the coronary arteries share a common embryonic origin, we hypothesized that CAE is associated with BAV disease. Methods and Results— One hundred seventy-seven patients with suspected aortic valve disease (n=94 BAV, n=83 tricuspid aortic valve) underwent both cardiac magnetic resonance imaging and coronary angiography. To confirm the association of CAE with BAV, the frequency of CAE was evaluated in an in-house BAV registry (n=600, n=231 with available coronary angiogram) and compared with the frequency of CAE in the German Myocardial Infarction (MI) Family Study, in which the heritability of CAE was formerly established (n=899). Furthermore, the frequency of CAE was investigated in an observational registry of real-life patients undergoing coronary angiography for clinically indicated reasons (n=3.097) and in a subgroup of the KORA MI study (Cooperative Health Research in the Region of Augsburg), which is a population-based MI registry (n=403). Compared with tricuspid aortic valve disease, CAE occurred more than twice as frequently in cardiac magnetic resonance–confirmed BAV disease (17% versus 44%; P <0.0001) and CAE was observed similarly often in subjects with BAV with (37%) and without (54%, P =0.11) ascending aortic pathology. The common appearance of CAE in patients with BAV could be independently confirmed in the BAV registry (frequency 37%), whereas CAE was found less frequently in family history of positive MI patients (21%), sporadic MI without familial disposition (10%), and rarely in unrelated real-life catheterization patients (6%). Conclusions— To our knowledge, our data show for the first time that ectatic coronary artery disease is a common appearance of BAV disease with and without ascending aortic ectasia.

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“…Aortic dilation (mainly involving the ascending aorta) is one of the most common non-valvular findings in BAV occurring in 35-80% of adult patients 3,4,19,37,38 . It is mainly asymptomatic and often precedes aortic dissection or rupture.…”

Section: Clinical Features Associated With Bavmentioning

confidence: 99%

“…BAV is a multifaceted, heterogeneous disorder ( Figure 1). Its natural history is determined by hemodynamic valvular impairment (present at birth or, more commonly, acquired by aging), and/ or by increased prevalence of aortic abnormalities ranging from reduced aortic elasticity to aortic dilatation/aneurysm/ dissection [1][2][3][4][5][6][7][8][9][10] . Each of the above features can be found in patients with isolated BAV or, in association with multiple congenital heart diseases (e.g.…”

Section: Introductionmentioning

confidence: 99%

Bicuspid aortic valve syndrome and fibrillinopathies: potential impact on clinical approach

Pepe¹,

Cario²,

Sticchi³

et al. 2015

ICFJ

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Bicuspid aortic valve (BAV) is a common heterogeneous disorder whose natural history is determined by hemodynamic valvular impairment and/or increased prevalence of aortic abnormalities ranging from dilatation to aneurysm and dissection. BAV-related aortopathy is frequently associated with relevant aortic pathologic changes leading to structural alterations, characteristic degenerative lesions and histological changes of the aorta very similar to those identified and described in patients with Marfan syndrome (MFS), an inherited connective tissue disorder associated with mutations in fibrillin 1 (FBN1) gene in more than 90% of patients. Recently, a 4-fold increase in the prevalence of BAV in MFS patients has been reported. Subsequently, pathogenetic FBN1 mutations in patients with BAV and aortic dilatation/aneurysm in whom MFS and other more severe type 1 fibrillinopathies were clinically excluded have been identified. In this review we discuss how this evidence, together with that of the wide heterogeneity in pathogenetic mechanisms of BAV-related aortopathy, may impact the clinical management of BAV.

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Aortic dilatation in patients with bicuspid aortic valve

Cited by 67 publications

References 85 publications

Bicuspid aortic valve morphology and its association with aortic diameter: an echocardiographic study

Bicuspid aortic valve morphology and its association with aortic diameter: an echocardiographic study

Coronary Artery Ectasia Are Frequently Observed in Patients With Bicuspid Aortic Valves With and Without Dilatation of the Ascending Aorta

Bicuspid aortic valve syndrome and fibrillinopathies: potential impact on clinical approach

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