Evaluation of pulse wave velocity in children with bicuspid aortic valve patients with normal cardiac and valve function Pulse wave velocity in bicuspid aortic valve

doi:10.21203/rs.3.rs-3987456/v1

Background: We aimed to analyze aortic elasticity properties in patients with bicuspid aortic valve (BAV) by measuring pulse wave velocity (PWV) using a transthoracic echocardiography device.

Methods: Fifty BAV patients and 50 healthy children were included in this study. Patients were classified into type 1 and type 2 according to the morphology of valve fusion. All patients were evaluated by transthoracic echocardiography. Left ventricular wall thickness, function, aortic strain, distensibility index, stiffness index were evaluated using formulas derived from aortic diameters. Velocity measurements were taken from the ascending and descending aorta using pulse wave (PW) Doppler. Pulse wave velocity was determined as the (total distance)/(transit time).

Results: Pulse wave velocity was found to be significantly higher in the BAV group, whose ventricular wall thickness and functions were similar with the other group (p = 0.000). PWV was significantly higher in type 1 BAV group (p = 0.03). The aortic strain and distensibility index (DI) were lower and the stiffness index (SI) was higher in the BAV group. However, the difference was not statistically significant. The aortic diameters in the BAV group were significantly higher than those in the control group. There was a correlation between PWV and aortic diameter, aortic flow velocity, and aortic valve regurgitation vena cava diameter.

Conclusions; In the evaluation of aortic elasticity properties in the BAV group, the PWV value measurement obtained with the values measured by the transthoracic echocardiographic method is practical, easily accessible, inexpensive, and can be evaluated without the need for additional devices.

aortic strain

bicuspid aortic valve

pulse wave velocity

stiffness index

The most prevalent congenital heart condition is the bicuspid aortic valve (BAV). Roughly 1–2% of the overall population is affected by this (1).

Several classifications have been created based on the fusion morphology of the valves in BAV disease (1, 2). In the traditional categorization, type 1 occurs when the left and right coronary cusps fuse, type 2 occurs when the right and left coronary cusps and the non-coronary cusp fuse, and type 3 occurs when the left coronary cusp and non-coronary cusp fuse. Types 2 and 3 are cases where the raphe cannot be identified. The patients were identified as having type 4 BAV.

The main complications of BAV disease are valve stenosis, valve insufficiency, aortic dilatation, aortic dissection, and endocarditis (3).

In our study, we aimed to evaluate aortic elasticity characteristics using transthoracic echocardiography and pulse wave velocity (PWV) measurements in BAV patients with normal ventricular and valve functions.

The study included 50 patients with BAV and 50 healthy children with similar demographic characteristics. The pediatric cardiologist evaluated all patients after measuring their weight, height, and systolic and diastolic blood pressure (SBP, DBP). We acquired approval for our study from the local ethics committee. (decision number: 530/2023).

Patients with BAV with an aortic valve gradient < 20 mmHg and no or mild aortic regurgitation were evaluated in this study. Patients with additional cardiac pathologies were excluded from this study. Aortic valve insufficiency was considered mild aortic insufficiency with a ceva contracta diameter of < 2 mm (4). In our study, patients with a valve gradient > 20 mmHg were not included. Patients with valvular insufficiency and vena contracta diameter > 2 mm were excluded. Patients with valve insufficiency and vena contracta diameter < 2 mm were classified as valvulopathy (+) BAV and those without valvulopathy (-) BAV.

Usually, there are three coronary cusps known as the left coronary cusp (LCC), right coronary cusp (RCC), and noncoronary cusp (NCC). The patients diagnosed with BAV were categorized based on their coronary cusp fusion into three groups: LCC-RCC fusion type 1, RCC-NCC fusion type 2, and NCC-LCC fusion type 3 (1, 2).

The aortic annulus diameter was measured at mid-systolic, sinus of Valsalva diameter, sinotubular junction diameter, and ascending aorta diameter were measured at the end of diastole. Patients with diameters above z-score > + 2 were considered to have aortopathy (+) (www.parameterz.blogspot.com)⁵. Patients with BAV were grouped as aortopathy (+) or aortopathy (-).

The same pediatric cardiologist evaluated all patients, conducting anamnesis and a thorough physical examination. Transthoracic echocardiography was taken with the patients lying in the left lateral position ((Vivid S60, 9; General Electric Healthcare, GE Vingmed, Norway) (6).

Left ventricular wall thickness, left ventricular diameter, systolic function, and left ventricular long-axis M-mode echocardiography findings were measured (7, 8).

Filling velocities (E, A, E/A) and intervals (DT) were measured with pulse wave (PW) doppler from the mitral inflow flow (9).

Velocity was measured at the aortic valve level using PW Doppler in the suprasternal view. Flow samples were taken from the ascending and descending aortas using PW Doppler. Patients with an aortic valve peak velocity of < 2.5 m/s were included in the study. In simultaneous electrocardiography (ECG), the time from the beginning of the QRS wave to the region where the flow trace began was calculated (Fig. 1). The difference between the times measured in the descending aorta and ascending aorta is referred to as the transit time. The ratio of this distance to the transit time is called the pulse wave velocity and is calculated by measuring the distance between the measured locations in the ascending aorta and the descending aorta (Fig. 2) (10, 11).

The M-mode was used to calculate the systolic and diastolic aortic diameters in the long axis of the left ventricle, 3 cm above the aortic valve. These diameters were used to calculate the formulas for aortic strain, aortic stiffness index, and aortic distensibility. Aortic strain (AS, %)= [systolic aortic diameter (ADs)- diastolic aortic diameter (ADd) / diastolic aortic diameter *100], stiffness indeksi (SI) = [ln (SBP/DBP)/AS], distensibility indeks (DI) = [2*AS/(SBP-DBP)] (12).

Mean ± standard deviation or median (minimum-maximum) are used to express continuous variables, while percentages are used to express categorical variables. The analysis used either a Student's T test or Mann-Whitney test based on the normal distribution of continuous variables, and the chi-square test for categorical variables. The correlation analysis was performed using Pearson's and Spearman's tests, taking into consideration the homogeneity of the parameter distribution. The statistical significance level was defined as P < 0.05.

Artificial intelligence (AI)– assisted technologies (such as Large Language Models [LLMs], chatbots, or image creators) were not used in the production of submitted work.

In our study, 72% of patients with BAV had type 1 BAV, and 28% had type 2 BAV. We had no type 3 patients. Aortopathy and valvulopathy were present in 16% and 44% of patients with BAV, respectively. Among the aortopathy patients, 100% were type 1, 72% of valvulopathy patients were type 1, and 28% were type 2. 72% of BAV patients, 72% of type 1 patients, 71% of type 2 patients, 100% of patients with aortopathy, and 81% of patients with valvulopathy were male (Table 1).

No surgical or interventional procedures were performed in any of the patients with BAV. There were no extracardiac anomalies.

The median age of the BAV group was 8.33 years (3.33-16.00), and the median age of the control group was 8.66 years (3.66–16.16). Age, weight, height, body surface area (BSA), SBP, DBP, and PP values were similar in both groups, and no statistical differences were detected (Table 1).

Table 1. Comparison table of demographic data, transthoracic echocardiographic measurements and aortic elasticity parameters of the BAV group and the control group.

Parameter BAV group (n=50) Control group (n=50) p-value

Gender (M/F,%) 72, 28 73, 27 1.00³

Age (year) 8.33 (3.33-16.00) 8.66 (3.66-16.16) 0.77²

Weight (kg) 22 (14-77) 35.50 (14.40-68.00) 0.42²

Height (cm) 133.52 (± 25.39) 137.76 (± 24.68) 0.54¹

BSA (m²) 0.86 (0.63-1.96) 1.17 (0.63-1.87) 0.53²

SBP (mmHg) 108.76 (± 13.58) 110.07 (± 14.52) 0.74¹

DBP (mmHg) 61.96 (± 8.53) 61.88 (± 10.40) 0.98¹

EF (%) 70.00 (± 5.83) 70.19 (± 6.78) 0.91¹

FS (%) 39.25 (± 4.47) 39.38 (± 5.61) 0.92¹

LVEDd (mm) 40.91 (± 7.36) 39.60 (± 6.96) 0.52¹

E (m/s) 1.07 (0.87-1.42) 1.01 (0.84-1.31) 0.06²

A (m/s) 0.70 (± 0.10) 0.64 (± 0.14) 0.14¹

E/A ratio 1.63 (± 0.26) 1.68 (± 0.40) 0.58¹

DT (ms) 123.12 (± 28.48) 120.53 (± 15.94) 0.69¹

Peak aortic velocity (m/s) 1.46 (± 0.35) 1.20 (0.22) 0.003¹

AAo-i (mm/m²) 16.24 (± 2.91) 13.69 (± 2.29) 0.001¹

SV-i (mm/m²) 20.87 (± 3.35) 17.48 (± 3.01) 0.000¹

STJ-i (mm/m²) 18.14 (± 3.25) 14.10 (± 2.63) 0.000¹

Ascending Ao-i (mm/m²) 18.73 (± 3.52) 13.88 (± 2.46) 0.000¹

ADs-i (mm/m²) 21.10 (12.78-28.53) 15.66 (12.27-23.96) 0.000²

ADd-i (mm/m²) 18.74 (± 3.54) 13.88 (± 2.46) 0.000¹

AAo z-score 0.37 (± 1.06) -0.60 (± 0.93) 0.000¹

SV z-score 0.03 (± 1.21) -1.10 (± 0.89) 0.000¹

STJ z-score 0.69 ((-2.31)-(2.06)) -1.14 ((-2.38)-(0.32)) 0.000²

Ascending Ao-z score 0.25 ((-2.00)-(1.82)) -1.62 ((-2.00)- (-1.16)) 0.000²

Aortic strain (%) 14 (± 8) 18 (± 8) 0.07¹

Stiffness index 4.45 (1.59-15.05) 3.42 (1.12-14.23) 0.07²

Distensibility 0.004 (0.001-0.014) 0.007 (0.002-0.017) 0.07²

PWV (m/s) 4.82 (1.97-14.29) 2.78 (1.48-6.96) 0.000²

1: student T test, 2: Mann-Whitney U test, 3: chi-square test, BSA: body surface area, SBP: systolic blood pressure, DBP: diastolic blood pressure, EF: ejection fraction, FS: fraction short, LVEDd: left ventricle end-diastolic diameter, E: early diastolic velocity, A: late diastolic velocity, DT: deceleration time of E velocity, AAo: aortic anulus, SV: sinus of valsalva, STJ: sino-tubuler junction, ADs: systolic aortic diameter, ADd: diastolic aortic diameter, PWV: pulse wave velocity,

Left ventricular wall thickness and diameter (IVSd, LVPWd, LVEDd) and systolic (EF, FS) and diastolic functions (E, A, E/A ratio, DT) were at similar values in both groups (Table 1).

The BAV group showed significantly higher values for the aortic annulus index (AAo-i), sinus of valsalva index (SV-i), sinotubular junction index (STJ-i), and ascending aorta index (Ascending Ao-i) as well as z-scores compared to the control group (p = 0.001, 0.000, 0.000, 0.000, respectively).

The aortic systolic diameter index (ADs-i) and diastolic aortic diameter index (ADd-i) values were significantly higher in the BAV group compared to the control group (p = 0.000 for both).

While aortic strain and distensibility were significantly lower in the BAV group than in the control group, the stiffness index was higher in the BAV group; however, these values were statistically insignificant.

The BAV group exhibited a significantly higher pulse wave velocity (PWV) compared to the control group (p = 0.000). The BAV group had a significantly higher peak aortic velocity compared to the control group (p = 0.003).

The PWV value was significantly higher in type 1 BAV patients compared to type 2 BAV patients when grouped according to valve morphology (p = 0.03). The PWV value was determined to be noticeably elevated in patients with both type 1 and type 2 BAV compared to the control group (p = 0.000 and 0.01, respectively) (Table 2).

Table 2

Comparison of aortic diameters and aortic elasticity parameters of BAV groups and control groups.
Parameters	Type 1 BAV group (n = 36)	Type 2 BAV group (n = 14)	Control group (n = 50)	p-value type 1 vs type 2 BAV	p-value type 1 BAV vs control	p-value type 2 BAV vs control
PWV (m/s)	6.21 (2.53–14.29)	3.76 (1.97–5.68)	2.78 (1.48–6.96)	0.03²	0.000²	0.01²
Peak aortic velocity (m/s)	1.47 (± 0.37)	1.43 (± 0.30)	1.20 (± 0.22)	0.79¹	0.004¹	0.03¹
AAo-i (mm/m²)	16.09 (± 2.83)	16.65 (± 3.31)	13.69 (± 2.29)	0.67¹	0.003¹	0.01¹¹
SV-i (mm/m²)	21.07 (± 3.30)	20.36 (± 3.70)	17.48 (± 3.01)	0.64¹	0.000¹	0.04¹
STJ-i (mm/m²)	18.13 (± 3.42)	18.18 (3.00)	14.10 (± 2.63)	0.97¹	0.000¹	0.001¹
Ascending aorta-i (mm/m²)	18.58 (3.47)	19.10 (± 3.85)	13.87 (± 2.46)	0.74¹	0.000¹	0.01
Aortic strain (%)	13 (± 2)	15 (± 3)	18 (± 1)	0.66¹	0.07¹	0.38¹
Stiffness indeks (SI)	4.41 (1.59–15.05)	4.45 (1.89–10.23)	3.42 (1.14–12.23)	0.46²	0.08²	0.31²
Distensibility index (SI) (10^− 6 cm² dyne^− 1)	0.005 (0.001–0.013)	0.004 (0.002–0.014)	0.007 (0.002–0.017)	0.50²	0.06²	0.42²
1: student T test, 2: Mann-Whitney U test,

Aortic strain, stiffness index, and distensibility values were similar in patients with type 1 and type 2 BAV and were statistically insignificant. Aortic diameters in the type 1 and 2 BAV groups were similar and statistically insignificant (Table 2).

Aortic strain and distensibility values were found to be lower in BAV patients with aortopathy, compared to those without aortopathy. Additionally, the stiffness index and PWV values were higher in the aortopathy (+) BAV group. Nevertheless, this discrepancy did not show statistical significance. The aortic diameters showed no significant difference between the BAV groups with and without aortopathy. The group with aortopathy had a higher PWV value compared to the group without aortopathy, but the difference was not considered statistically significant.

BAV patients were separated based on whether they had valvulopathy. The group with valvulopathy (+) showed lower aortic strain and distensibility values compared to the group without valvulopathy (-). Additionally, the valvulopathy (+) group demonstrated higher stiffness index and PWV values. Nevertheless, it had no statistical significance. The aortic diameters were comparable between the groups with valvulopathy (+) and without valvulopathy (-) in BAV. The occurrence of PWD was found to be comparable in both the groups with and without BAV-related valvulopathy, and no significant statistical disparity was observed (Table 3).

Table 3

Comparison of aortic diameters and aortic elasticity parameters of groups with and without aortopathy and valvulopathy.
Parameters	Aortopathy(+) BAV (n = 8)	Aortopathy(-) BAV (n = 42)	Valvulopathy (+) BAV (n = 22)	Valvulopathy (-) BAV (n = 28)	p-value aortopathy (+) BAV vs aortopathy (-) BAV	p-value valvulopathy (+) BAV vs valvulopathy (-) BAV
PWV (m/s)	7.19 (5.83–7.83)	4.24 (1.97–14.29)	5.82 (1.97–7.83)	4.00 (2.53–14.29)	0.07²	0.24²
Peak aortic velocity (m/s)	1.46 (± 0.18)	1.46 (± 0.37)	1.34 (± 0.28)	1.55 (± 0.37)	0.98¹	0.13¹
AAo-i (mm/m²)	16.81 (± 3.03)	16.13 (± 2.95)	15.68 (± 2.89)	16.66 (± 2.96)	0.68¹	0.40¹
SV-i (mm/m²)	22.32 (± 4.61)	20.60 (± 3.13)	20.76 (± 4.20)	21.00 (± 2.67)	0.35¹	0.82¹
STJ-i (mm/m²)	20.09 (± 5.11)	17.77 (± 2.80)	18.08 (± 4.20)	18.19 (± 2.43)	0.43¹	0.94¹
Ascending aorta-i (mm/m²)	20.23 (± 4.69)	18.44 (± 3.31)	18.03 (± 4.35)	19.28 (± 2.73)	0.36¹	0.39¹
Aortic strain (%)	9 (± 5)	14 (± 8)	13 (± 7)	14 (± 9)	0.25¹	0.81¹
Stiffness index (SI)	8.45 (3.24–12.63)	3.56 (1.59–15.05)	5.26 (1.89–12.63)	3.50 (1.59–15.05)	0.26²	0.82²
Distensibility index (SI) (10^− 6 cm² dyne^− 1)	0.003 (0.001–0.007)	0.006 (0.001–0.014)	0.004 (0.001–0.014)	0.007 (0.001–0.013)	0.22²	0.56²
1: student T test, 2: Mann-Whitney U test,

PWV values were positively correlated with the STJ, ascending Ao, systolic and diastolic aortic diameters, peak aortic velocity, and vena contracta values (Table 4).

Ascending Ao diameter showed a negative correlation with aortic strain and distensibility and a positive correlation with stiffness index (Table 4).

Peak aortic velocity and vena contracta diameter did not correlate with aortic diameters, and aortic strain, distensibility, and stiffness index, which are aortic elasticity parameters, showed a positive correlation with PWV value.

The most basic result of our study is that in BAV patients with normal ventricular function and normal or near-normal valve function, aortic elasticity properties are reduced, and PWV is increased by transthoracic echocardiography, without the need for additional devices.

There are three types of BAV morphologies according to the valve morphology classification (2). In our study, 72% of the patients with BAV had type 1 BAV, and 28% had type 2 BAV. None of the patients had type 3 disease. The male-to-female ratio was 2.5. These findings are consistent with those of previous studies (1, 13).

Valvulopathy is more common in patients with type 2 BAV patients (13). Contrary to the literature, valvulopathy was more common in type 1 patients in our study. This is because when the study was designed, patients without valve stenosis were included in the study.

Structurally and functionally, the arterial system consists of large, elastic, and muscular arteries. Large elastic arteries store ejected blood during systole and ensure constant blood flow throughout the systolic and diastolic cycles. Muscular arteries modulate pressure waves by changing its tone (14). Aortic stiffness has a strong predictive value independent of the risk of cardiovascular diseases (15). A relationship exists between the central aortic stiffness and arterial stiffness.

Aortic elasticity can be evaluated using many methods, such as transthoracic echocardiography (4), magnetic resonance imaging (MRI) (16), and pulse wave velocity (PWV) measurement (17). PWV is the gold standard for evaluating arterial stiffness (18). PWV can be measured regionally, locally, invasively, or non-invasively, depending on the measurement technique. In non-invasive PWV evaluation, time, special device requirements, special equipment, and use of contrast in invasive measurements are the limitations of PWV (19, 20). Non-invasive and invasive PWV measurements yielded similar results (10).

The formulas derived from the diameters obtained from the M-mode measurement of the aorta with transthoracic echocardiography are the aortic strain, distensibility index (DI), and stiffness index (SI). Aortic strain is the degree of change in the aortic diameter during systole. Formulas for DI and SI are derived from aortic strain and systolic and diastolic blood pressure formulas (12). SBP and DBP may not accurately represent the pressure of the aorta, as they are measured from the periphery. DI and SI measurements have limitations.

The mechanism of aortic dilatation in BAV may be due to impaired wall stress due to aortic valve insufficiency (21) or turbulence occurring in aortic valve stenosis (22, 23). The risk of aortic dilatation has also been reported to increase regardless of valve function (24). Aortic dilatation has been reported in 15.1–39.1% of isolated BAV patients (25). According to the current study, 16% of the participants showed aortic dilatation. In our study, although BAV patients did not have aortic valve stenosis, did not have aortic valve regurgitation, or had mild aortic valve regurgitation, aortic diameters were significantly higher in BAV patients. The absence of a connection between the size and velocity of the aorta and the diameter of the vena contracta indicates that there are other factors at play in causing aortic enlargement. Research on adults has revealed that complications in patients with BAV are linked to changes in the elasticity of the aorta, regardless of its diameter (26).

Aortic diameters were increased in patients with BAV, which is consistent with the literature. In BAV disease, valve hemodynamics changes as a result of abnormal valve movements (22). Consequently, valve narrowing, insufficiency, and aortic dilatation may develop as a result of turbulent flow (22, 23). In our study, the correlation of aortic diameters with each other and vena contracta diameter with aortic diameters showed that valve hemadynamics can improve even when intact. Changes in aortic elasticity and valve hemodynamics may have contributed to this situation.

According to a report, patients have shown high aortic strain, DI, and SI (4). We found that the BAV group had high aortic strain, DI, and SI in our study. Nevertheless, there were no statistically significant differences identified. Similarly, the study found that ventricular dysfunction was present, despite normal valve function. In our research, we found that patients with BAV showed no signs of ventricular dysfunction, even though they had similar patient characteristics. Valvulopathy is most commonly reported as type 2 in patients with BAV (13). This finding could be attributed to the small number of type 2 patients in our study.

This study is the first to evaluate PWV in the ascending and descending aorta of pediatric patients with BAV. Although not requiring special equipment and easy bedside measurements are seen as advantages, the limitation of aortic PWV is that the distance between the descending and ascending aorta is difficult to measure with existing devices and is affected by blood viscosity (27).

The correlation of PWV with aortic velocity, valve insufficiency vena contracta diameter, anmeter shows that aortic elasticity is closely related to aortic diameter and valve hemodynamics. The fact that the PWV value is significantly higher in type 1 BAV patients than in type 2 BAV patients shows that aortic elasticity, valve flow velocity, and valve turbulence have different effects in different BAV types.

As the stiffness of the aorta increases, the ability of the aorta to stretch and expand decreases, resulting in an increase in the stiffness index. Our research findings align with existing literature, showing a decrease in aortic strain and distensibility, and an increase in stiffness index among patients with BAV. The BAV patients were separated based on whether they had aortopathy or valvulopathy. The aortopathy (+) and valvulopathy (+) BAV groups showed lower aortic strain and distensibility, and higher stiffness index. Nevertheless, this variation did not have a statistically significant impact. Perhaps the limited occurrence of aortopathies and valvulopathy in patients with BAV may be the cause of this. It is our recommendation that this study is carried out with a larger sample size of patients.

The single center and small number of patients were the main limitations of the study. In the PWD measurement, the distance between the ascending and descending aortas was measured intermittently in the current device. Measurement errors are another limitation of this study. Another limitation is that PWV is affected by the blood viscosity.

The increase in aortic stiffness in patients with BAV was demonstrated by the PWV value with the same echocardiography device, without the need for an additional device. In the evaluation of aortic stiffness, the formulas obtained with values derived from aortic diameters did not correlate with aortic diameters, aortic velocity, aortic jet vena contracta, and PWV correlated with aortic diameter, aortic velocity, and aortic jet vena contracta. We believe that this will provide more accurate information for evaluating the stiffness.

* Conflict of Interest: The authors declare that they have no conflict of interest.
* Funding: There is no funding source.

Ethical approval: This article does not contain any studies with human participants or animals performed by any of the authors. The study was approved by the Ethics Committee of Diyarbakir Gazi Yasargil Training and Research Hospital (Decision no: 2022/171).
* Informed consent: Informed consent was obtained from all individual participants included in the study.

Written informed consent was obtained from all individual participants included in the study and their parents (or legal guardians). All procedures performed involving human participants were in accordance with the ethical standards of the local ethics committee and with the 1964 Helsinki declaration and its later amendments.

Acknowledgements: None.

Declarations: Conflict of interest None.

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No competing interests reported.

Evaluation of pulse wave velocity in children with bicuspid aortic valve patients with normal cardiac and valve function Pulse wave velocity in bicuspid aortic valve

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