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Bicuspid Valve-Related Aortic Disease

Rationale and Objectives

Abnormal blood flow with bicuspid aortic valve (BAV) has been characterized with four-dimensional flow magnetic resonance imaging (MRI), but this approach is time consuming and requires technical expertise. We assess the relationship between different leaflets fusion patterns with BAV, eccentric systolic flow, and dilation patterns of the ascending aorta using two-dimensional (2D) phase-contrast (PC) MRI.

Materials and Methods

Fifty-nine patients with BAV who underwent cardiac MRI were identified; 47 had right–left (RL) aortic leaflet fusion and 12 had right-noncoronary (RN) fusion. Flow displacement was calculated, and patients with abnormal displacement (>0.1) were classified as either rightward or leftward. Patterns of aortopathy were determined (0–3), and correlation between leaflet fusion, flow direction, aortopathy type, and other clinical parameters was performed with Pearson correlation, the Fisher exact test and chi-square analysis.

Results

Normal systolic flow was seen in 24% of cases and was significantly correlated with normal aortas ( P = .011). Abnormal flow displacement with RL fusion was strongly associated with rightward deviation (36 of 37 cases), whereas RN fusion skewed leftward (seven of eight cases; P < .01). In patients with aortopathy, RL fusion was strongly associated with type 2 aortopathy and RN with type 3 aortopathy ( P < .01).

Conclusions

Conventional PC MRI can identify abnormal systolic flow and differences in jet orientation with BAV. RL leaflet fusion is associated with rightward flow deviation and type 2 aortopathy, whereas RN fusion is linked to leftward deviation and type 3 aortopathy. The presence and direction of eccentric flow jets may help risk stratify these patients for valve-related aortic disease.

Characterization of eccentric systolic blood flow with bicuspid aortic valve (BAV) has been extensively performed using four-dimensional flow (4D Flow) magnetic resonance imaging (MRI) . Although there is much debate about the significance of these flow patterns, their presence and association with aortic dilation in patients with BAV has been used to support the argument that altered systolic hemodynamics with BAV play a significant role in the aortopathy exhibited by these patients . However, to date, only a few, small cohorts have suggested the prognostic value of MRI flow data in patients with BAV . One reason for this is the relatively slow growth rates of the ascending aorta, typically in the range of ≤1 mm/year . Another reason is the length and complexity of both the 4D Flow acquisition and calculation of flow-related parameters, which have limited its clinical applicability.

Conventional phase-contrast (PC) MRI is often performed in the ascending aortas of patients with BAV to quantify the degree of aortic regurgitation . The calculation is derived from through-plane velocity data for a cross-sectional plane in the tubular ascending aorta, which can be acquired in a single breath-hold. These same data can also be used to assess some of the less-complex findings that have been reported with 4D Flow, such as the presence of eccentric systolic flow and calculation of flow displacement . The aim of this study is to use routine PC data (i.e., two-dimensional [2D] PC MRI) to evaluate the interrelationship between systolic flow, aortopathy, and different aortic leaflets fusion patterns in patients with BAV. We hypothesize that this faster and less technically involved MRI approach to assessing aortic hemodynamics with BAV will reveal important abnormalities of flow that are associated with different types of aortopathy and aortic leaflet fusion patterns.

Materials and methods

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Figure 1, Representative cases of types 0, 2 and 3 aortopathy. Note that type 1 aortopathy was not included because of the limited number of patients with this pattern in our study. Maximum intensity projections of magnetic resonance angiography data demonstrate the pattern of aortopathy, with the associated planar analysis of systolic flow provided for each case. The patient with the normal aorta (type 0) had right–left (RL) aortic leaflet fusion and a flow displacement value of 0.02. The patient with type 2 aortopathy had RL aortic leaflet fusion and a flow displacement value of 0.26 oriented in the right-posterior (RP) quadrant. The patient with type 3 aortopathy had right-noncoronary aortic leaflet fusion and a flow displacement value of 0.24 oriented in the left-posterior (LP) quadrant. LA, left-anterior; RA, right-anterior.

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MRI Technique

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Data Collection and Analysis

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Statistics

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Results

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Table 1

Characteristics of Study Population by Valve Fusion Pattern

Characteristic Right–Left Fusion ( n = 47) Right-Noncoronary Fusion ( n = 12)P Value Age (years), mean ± standard deviation 29.8 ± 12.8 28.1 ± 17.2 .7 Male patients, n (%) 28 (60) 10 (83) .18 Coarctation history, n (%) 24 (51) 3 (25) .11 Aortopathy, n (%) Type 0 20 (43) 4 (33) .56 Type 1 2 (4) 0 .46 Type 2 20 (43) 1 (8) .023 Type 3 5 (10) 7 (58) .0002 Systolic flow, n (%) Normal 10 (22) 4 (33) .38 Rightward 36 (76) 1 (8) <.0001 Leftward 1 (2) 7 (58) <.0001 Aortic stenosis, n (%) ∗ 4 (9) 3 (25) .12 Aortic insufficiency, n (%) ∗ 10 (22) 2 (17) .72

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Aortic Leaflet Fusion Patterns

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Flow Displacement

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Table 2

Associations by Flow Subgroup

Characteristic Normal Flow ( n = 14) Rightward Flow ( n = 37) Leftward Flow ( n = 8) Age (years) 23.8 ± 12.6 32.2 ± 12.5 26.6 ± 19.0 Flow displacement 0.06 ± 0.02 0.21 ± 0.04 ∗ 0.26 ± 0.09 ∗ Aortic shape Type 0 10 (72) † 11 (30) 3 (37) Type 1 1 (7) 1 (3) 0 Type 2 1 (7) 19 (51) † 1 (13) Type 3 2 (14) 6 (16) 4 (50) ‡ Fusion pattern RL 10 (71) 36 (97) † 1 (13) RN 4 (29) 1 (3) 7 (87) †

The values are presented as mean ± standard deviation or n (%).

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Discussion

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Figure 2, Characteristic associations between leaflet fusion pattern, flow, and aortopathy. Right–left aortic leaflet fusion is associated with rightward displacement of systolic flow and type 2 aortopathy (ie, dilation involving the tubular ascending aorta), whereas right-noncoronary aortic leaflet fusion is linked to leftward displacement of systolic flow and type 3 aortopathy (ie, diffuse dilation extending to the transverse arch).

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