Aortic morphological variability in patients with bicuspid aortic valve and aortic coarctation

Abstract

OBJECTIVES This study aimed to explore aortic morphology and the associations between morphological features and cardiovascular function in a population of patients with bicuspid aortic valve, while further assessing differences between patients with repaired coarctation, patients with unrepaired coarctation and patients without coarctation. METHODS This is a single-centre retrospective study that included patients with available cardiovascular magnetic resonance imaging data and native bicuspid aortic valve diagnosis ( n = 525). A statistical shape analysis was performed on patients with a 3-dimensional magnetic imaging resonance (MRI) dataset ( n = 108), deriving 3-dimensional aortic reconstructions and computing a mean aortic shape (template) for the whole population as well as for the 3 subgroups of interest (no coarctation, repaired coarctation and unrepaired coarctation). Shape deformations (modes) were computed and correlated with demographic variables, 2-dimensional MRI measurements and volumetric and functional data. RESULTS Overall, the results showed that patients with coarctation tended towards a more Gothic arch architecture, with decreased ascending and increased descending aorta diameters, with the unrepaired-aortic coarctation subgroup exhibiting more ascending aorta dilation. Careful assessment of patients with repaired coarctation only revealed that a more Gothic arch, increased descending aorta dimensions and ascending aorta dilation were associated with reduced ejection fraction ( P ≤ 0.04), increased end-diastolic volume ( P ≤ 0.04) and increased ventricular mass ( P ≤ 0.02), with arch morphology distinguishing patients with and without recoarctation ( P = 0.05). CONCLUSIONS A statistical shape modelling framework was applied to a bicuspid aortic valve population revealing nuanced differences in arch morphology and demonstrating that morphological features, not immediately described by conventional measurements, can indicate those shape phenotypes associated with compromised function and thus possibly warranting closer follow-up.

Determinants of aortic growth rate in patients with bicuspid aortic valve by cardiovascular magnetic resonance

“…Our previous cross-sectional study focused exclusively on aortic morphology suggested that patients with reCoA tended to present increased aortic diameters compared with CoA patients with successful repair and no residual narrowing,18 yet current results did not reveal an association between reCoA and aortic growth rate. This may be partly due to a relatively short time window, nevertheless significant increases in aortic size were observed in other patients over this time.…”

“…Results showed that patients with repaired CoA tended to have slower aortic root growth rate compared with patients with isolated BAV. Previous work from our group based on a statistical shape modelling framework18 revealed nuanced differences in arch morphology in BAV patients with/without CoA, suggesting detrimental functional implications for some aortic arch architectures, and with BAV+CoA patients generally presenting with smaller ascending and larger descending aortas compared with isolated BAV. This agrees with observations in the literature that prior CoA repair may protect BAV aorta from rapid dilation 8.…”

“…Taking advantage of a large cohort of BAV patients with and without CoA, this study aims to assess the effect of CoA and other potential determinants on aortic growth rate. Based on a recent study from our group reporting morphological differences in the aortic arch architecture in BAV patients with/without CoA,18 the primary hypothesis is that BAV+CoA patients exhibit slower aortic growth rate, while patients with re-coarctation (reCoA) exhibit increased aortic growth rate. Furthermore, based on differences in aortic growth across BAV fusion types established with echocardiography,19 a secondary hypothesis is that patients with right non-coronary (RN) valve fusion type exhibit increased aortic growth rate.…”

Bicuspid aortic valve: different clinical profiles for subjects with versus without repaired aortic coarctation

“…22 Altered aortic geometry may be an additional differing feature, as previous CoA repair is associated with a 'gothic' aortic arch. 23 Mechanobiological studies on the aortic valve have elucidated the intricate relationship between altered leaflet pressure, stretch and shear stresses and the cellular, proteomic and genomic profiles of aortic valves which may be involved in aortic valve dysfunction. 24 These abnormalities in aortic and aortic valve mechanics, therefore, may potentially contribute to the accelerated valvular dysfunction that we observed in the patients with BAV-CoA.…”

Acute and medium term results of balloon expandable stent placement in the transverse arch—a multicenter pediatric interventional cardiology early career society study

“…Traditionally, treatment of aortic arch pathology was a domain of cardiac surgery. [1][2][3][4][5][6] The advent of transcatheter procedures, along with improvement in equipment, has opened a new field enabling therapy in patients with native or previously repaired coarctation. [7][8][9][10] Percutaneous techniques, such as, balloon dilation and stenting, allow clinicians to safely decrease or abolish most gradients along the aorta, albeit with limitations.…”