Right thoracotomy for aortic valve replacement in the adolescents with bicuspid aortic valve

Background: In this study, we compared our experience about early and midterm follow‐up outcomes for right anterolateral minithoracotomy (RAMT) vs full sternot‐ omy (FS) in surgical aortic valve replacement (AVR) among adolescents with bicuspid aortic valve (BAV).
Methods: Patients were retrospectively enrolled from January 2008 to December 2017. Inclusion criteria were patients with BAV who had to undergo to AVR. They were divided in two groups: RAMT and FS. The choice of RAMT was based on indi‐ vidual surgeon’s preferences or when expressly requested by patient that was in‐ formed of nonconventional approach.
Results: We enrolled 61 patients, 23 in RAMT group and 38 in FS group. The mean age was 15.6 ± 1.7 years for RAMT group and 16.1 ± 1.5 years for FS group (P = .23). The RAMT group had a higher prevalence of female gender (P = .04). The patients in the RAMT group had longer cardiopulmonary bypass (115.2 ± 18.5 vs 102.2 ± 16.5 min; P = .006) and cross‐clamp time (78.6 ± 18.1 vs 74.3 ± 15.2 min; P = .01). No pa‐ tients required intraoperative conversion to FS. No differences were found in venti‐ lation times, postoperative intensive care unit (ICU), and hospital length of stay for both groups. Follow‐up echocardiograms were available for all patients at median of 5.2 years (range 0.5‐9.6 years, median 5.4 years for RAMT and 5.1 for FS) and no patient required reoperation for aortic prosthesis malfunction.
Conclusions: Our study shows that RAMT is safe and effective as FS. Although the RAMT operation takes slightly more operation time, it is not associated with major adverse effects.     

Elastic properties of aortic wall in patients with bicuspid aortic valve by magnetic resonance imaging

Bicuspid aortic valve (BAV) is frequently associated with aortic wall abnormalities, including dilation of the ascending aorta and even dissection. We propose 2 new indexes of aortic wall biophysical properties, the maximum rates of systolic distension and diastolic recoil (MRSD and MRDR, respectively), in patients with BAV and matched control subjects. We evaluated 53 consecutive young patients with BAV (36 males, mean age 16 ± 4 years) with mild aortic valve disease and a control group of 22 age- and gender-matched healthy volunteers. All subjects underwent a cardiac magnetic resonance imaging study that included phase velocity mapping and cine acquisition at several aortic levels. The MRSD and MRDR were measured in the ascending aorta in both patients with BAV and controls. Of the 53 patients with BAV, 26 had enlarged ascending aortas (dilated BAV), and 27 had a normal aortic diameter (nondilated BAV). Compared to controls, the MRSD was significantly lower in the whole BAV group (4.37 ± 1.1 vs 9.1 ± 2.1), in patients with dilated BAV (4.5 ± 1.1 p <0.0001), and in those with nondilated BAV (4.3 ± 1.0, p <0.0001). The MRDR was greater in the whole BAV group (-4 ± 1.2 vs -7.6 ± 2.7, p <0.0001), in the dilated BAV group (-3.9 ± 1.3, p <0.0001), and in the nondilated BAV group (-4.1 ± 1.2, p <0.0001). A receiver operating characteristic curve analysis of MRSD distinguished BAV from controls with 100% sensitivity and 95% specificity. In conclusion, MRSD and MRDR were slower in the patients with BAV than in the controls, regardless of the dimensions of the ascending aorta.     

Matrix Metalloproteinase‐2 and ‐9 Levels in Patients with Dilated Ascending Aorta and Bicuspid Aortic Valve

Background: Predictors of aortic dilatation are not well‐described in patients with bicuspid aortic valve (BAV). Changes in extracellular matrix composition in the aortic wall may play an important role. Our study aimed to examine the relationship between ascending aortic dilatation and biochemical markers for collagen metabolism, such as matrix metalloproteinase‐2 (MMP‐2) and matrix metalloproteinase‐9 (MMP‐9) levels in patients with BAV. Methods: All patients underwent cardiac echocardiography using a standard protocol, and aortic measurements were made in end‐diastole. One hundred twelve BAV patients with no or mild valvular impairment were recruited and grouped according to the aortic dimensions corrected for body surface area (BSA) and age. There were 54 patients with dilated ascending aorta (Group 1) and 58 patients with nondilated ascending aorta (group 2). The plasma levels of MMP‐2 and MMP‐9 were determined by ELISA. Results: The mean ascending aorta diameter was 4.49 ± 0.49 mm in group 1 and 3.51 ± 0.46 mm in group 2 (P < 0.001). There were no significant difference in gender, BSA, presence of hypertension, diabetes mellitus, hyperlipidemia, and smoking between the 2 groups. Nevertheless, no significant difference was observed in the levels of MMP‐2 and MMP‐9 between the 2 groups. The ascending aorta diameter correlated significantly with age (r = 0.438 P < 0.001). No significant correlation was observed between plasma MMP‐2 and MMP‐9 concentration and ascending aorta diameter, respectively (r = ?0.005 P = 0.58, r = ?0.106 P = 0.07). Multivariate analysis showed that age was independent predictor of aortic dilatation (P ≤ 0.001). Conclusion: Age was an independent predictor of aortic dilatation in patients with BAV, whereas MMP‐2 and 9 levels were not relevant by aortic dilatation.     

升主动脉成形术治疗二叶式主动脉瓣患者升主动脉扩张的效果分析

目的:探讨升主动脉成形术(reduction ascending aortoplasty,RAA)治疗二叶式主动脉瓣(bicuspid aortic valve,BAV)患者升主动脉扩张的临床效果。方法:回顾性分析2006年1月至2015年12月长海医院208例因主动脉瓣病变合并升主动脉扩张行主动脉瓣置换术(aortic valve replacement,AVR)联合RAA治疗患者的临床资料。根据患者主动脉瓣情况,分为BAV组和三叶式主动脉瓣(tricuspid aortic valve,TAV)组;在BAV组中,根据术中升主动脉的目标直径,将患者分为30 mm组和≥30 mm组。结果:BAV组与TAV组的术后死亡率、并发症发生率、二次手术比例和随访升主动脉直径等指标差异无统计学意义(P均0.05);BAV组术中升主动脉目标直径30 mm组的随访升主动脉直径、术后5年和9年升主动脉扩张程度均明显小于术中升主动脉≥30 mm组(P均0.05)。结论:对于BAV合并升主动脉扩张的患者,BAV不是行RAA的不利因素,在RAA术中将升主动脉目标直径控制在30 mm以下,中远期疗效更好。     

Long‐term cardiovascular outcome of Williams syndrome

Objective: Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Eighty patients with Williams syndrome followed up for more than 5 years.
Interventions: Not applicable.
Outcome Measures: Long‐term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post‐interventional courses of disease‐specific intervention, and intervention‐free survival of obstructive cardio‐ vascular lesions.
Results: The median follow‐up duration was 11.0 (5.1‐28.3) years. Among 80 pa‐ tients, supravalvular aortic stenosis (87.5%) was the most common cardiovascular lesion, followed by branch pulmonary stenosis (53.8%), mitral valve prolapse (22.5%), and aortic arch hypoplasia/coarctation (5.0%). During the follow‐up period, the peak flow velocity of supravalvular aortic stenosis did not change on peak Doppler echo‐ cardiography. Initially, severe supravalvular aortic stenosis was aggravated (P < .027). Conversely, the peak velocity of branch pulmonary stenosis decreased (from 3.08 to 1.65 m/s; P < .001) within age 3.2 (0.4‐6.9) years. Even the group with severe branch PS improved over time. Twenty‐two patients (27.5%) with Williams syndrome under‐ went disease‐specific interventions without mortality, mostly for supravalvular aortic stenosis or mitral valve prolapse. No patient in the late‐onset and initially mild sup‐ ravalvular aortic stenosis group needed intervention and 37.5%, 48.4%, and 65.1% in initially moderate and severe supravalvular aortic stenosis groups needed inter‐ vention at age 5, 10, and 20 years, respectively. Unlike the conventional therapeutic concept, the intervention for branch pulmonary stenosis was almost unnecessary.
Conclusions: In Williams syndrome, initially severe supravalvular aortic stenosis worsened over time and most branch pulmonary stenoses, including those in the severe group, improved spontaneously. Most patients with branch pulmonary ste‐ nosis did not require disease‐specific intervention. Surgical repairs for cardiovascular abnormalities in Williams syndrome showed favorable results.     

Screening for thoracic aortic pathology: Clinical practice in a single tertiary center

Objective: The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in com‐ parison with computed tomography (CT) to detect aortic dilation. We also investigated which additional abnormalities of the heart, aorta or smaller arteries were discovered.
Design: All patients ≥15 years who visited our tertiary center in 2012‐2016 for first thoracic aortic screening were retrospectively included. Diameters of the sinus of Valsalva (SoV) and maximum ascending aorta (AA) were compared between TTE and CT. The sensitivity and specificity of TTE to detect aortic dilation (≥40 mm) was as‐ sessed with CT as reference standard. Intracardiac abnormalities found with TTE and arterial abnormalities found with CT were identified.
Results: In total 349 patients (155 men, age 41 ± 15 years, 10% genetic mutation) were included. Screening was performed with TTE only in 35% and with TTE and CT in 65%. Patients who underwent TTE only were younger, had less often hypertension and less often a family history of aortic pathology. Although there was a good correlation be‐ tween TTE and CT, the diameters measured with TTE were typically lower (SoV −1.0, 95%CI −6.6 to 4.7 and AA −0.4, 95%CI −6.5 to 5.8). Sensitivity of TTE for detecting aortic dilation was 61% (SoV) and 57% (AA) and specificity was 96% (SoV) and 100% (AA). Valve abnormalities, ventricular dilation or reduced ventricular function was found with TTE in 26 patients (7%). In 47 patients (13%) ascending aortic dilation was diagnosed and in 10 patients (4%) relevant peripheral arterial abnormalities were identified using CT.
Conclusions: Most often patients received both TTE and CT (65%). Since TTE showed a low sensitivity to detect aortic dilation, CT imaging is advised at least once in pa‐ tients referred for thoracic aortic screening.     

Influence of Aortic Dilation on the Regional Aortic Stiffness of Bicuspid Aortic Valve Assessed by 4-Dimensional Flow Cardiac Magnetic Resonance: Comparison With Marfan Syndrome and Degenerative Aortic Aneurysm

ObjectivesThis study sought to ascertain whether patients with a bicuspid aortic valve (BAV) have an intrinsic alteration in regional aortic stiffness compared with patients with tricuspid aortic valve (TAV) and Marfan syndrome (MFS) patients with similar aortic sizes, as well as to assess the influence of ascending aorta (AAo) dilation on regional stiffness parameters in BAV patients.BackgroundImaging biomarkers as predictors of BAV, MFS, and degenerative AAo aneurysms in TAV patients (DA-TAV) are lacking. Biomechanical characterization has been proposed as a possible tool for further aneurysm stratification.MethodsA total 234 subjects (136 BAV, 44 MFS, and 18 DA-TAV patients and 36 healthy control subjects) were included. The cardiac magnetic resonance protocol comprised 4-dimensional flow to assess AAo and descending aorta (DAo) pulse wave velocities (PWVs) and double-oblique, 2-dimensional, steady-state free-precession cine cardiac magnetic resonance to compute aortic distensibility (AD).ResultsOn adjusted analysis, nondilated BAV patients had similar PWV and AD as healthy control subjects in both AAo and DAo, whereas dilated BAV did not differ from DA-TAV. In contrast, AAo and DAo stiffness in MFS patients was markedly greater than in BAV patients, increasing slightly with dilation severity. AAo PWV showed a biphasic pattern in BAV patients: it first decreased and then increased throughout AAo dilation, with a clear turning point at 50 mm, whereas distensibility did not discern mildly dilated aorta. In multivariate analysis adjusted for clinical and demographic characteristics, only PWV was related to AAo dilation in BAV patients.ConclusionsThe mechanical properties of AAo aneurysms are similar in BAV and TAV patients, whereas MFS patients have a stiffer aorta. Aortic stiffness strongly depends on dilation severity. AAo PWV resulted in a potentially clinically useful biphasic trend with respect to aneurysm diameter, whereas distensibility did not discern mildly dilated aorta. Beyond clinical risk factors, PWV but not AD was independently related to AAo dilation in BAV patients.     

Circulating matrix metalloproteinase patterns in association with aortic dilatation in bicuspid aortic valve patients with isolated severe aortic stenosis

Bicuspid aortic valve (BAV) exhibits a clinical incline toward aortopathy, in which aberrant tensile and shear stress generated by BAV can induce differential expression of matrix metalloproteinases (MMPs) and their endogenous tissue inhibitors (TIMPs). Whether stenotic BAV, which exhibits additional eccentric high-velocity flow jet upon ascending aorta and further worsens circumferential systolic wall shear stress than BAV with echocardiographically normal aortic valve, can lead to unique plasma MMP/TIMP patterns is still unknown. According to their valvulopathy and aortic dilatation status, 93 BAV patients were included in the present study. Group A (n = 37) and B (n = 28) comprised severely stenotic patients with or without ascending aorta dilatation; Group C (n = 12) and D (n = 16) comprised echocardiographically normal BAV patients withor without ascending aorta dilatation. Plasma MMP/TIMP levels (MMP-1, -2, -3, -8, -9, -10, -13 and TIMP-1, -2, -4) were determined via a multiplex ELISA detection system in a single procedure. Among patients with isolated severe aortic stenosis, plasma levels of MMP-2 and -9 were significantly elevated when ascending aortic dilatation was present (p = 0.001 and p = 0.002, respectively). MMP-2, however, remained as the single elevated plasma component among echocardiographically normal BAV patients with dilated ascending aorta (p = 0.027). Multivariate analysis revealed that MMP-2 and MMP-9 could both serve as independent risk factor for aortic dilatation in the case of isolated severe stenosis (p = 0.003 and p = 0.001, respectively), and MMP-2 in echocardiographically normal patients (p = 0.002). In conclusion, BAV patients with isolated severe aortic stenosis demonstrated a distinct plasma MMP/TIMP pattern, which might be utilized as circulating biomarkers for early detection of aortic dilatation.     

Rates and determinants of progressive aortic valve dysfunction in aortic coarctation

Purpose

Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and ascending aortic dilatation. The aim of this study was to evaluate the progression of and predictors for aortic valve dysfunction in CoA.

Methods

96 CoA patients prospectively underwent echocardiography twice between 2001 and 2010. AS was defined as an aortic valve gradient ≥ 20 mm Hg, AR as none/minor, or moderate/severe. Aortic dilatation as an ascending aortic diameter ≥ 37 mm.

Results

All patients (median age 28.0 years, range 17–61 years; male 57%) were followed with a median follow-up of 7.0 years. Sixty patients (63%) had BAV. At baseline 10 patients had AS (10%, 9 BAV), 6 patients AR (6%, 3 BAV) and 11 patients aortic dilatation (11%, 11 BAV). At follow-up 15 patients had AS (15%, 13 BAV) and 12 patients AR. (13%, 8 BAV).Median AS progression was 1.1 mm Hg/5 years (range — 13–28). Determinants for AS at follow-up were age (ß = 0.20, P = 0.01), aortic dilatation (ß = 4.6, P = 0.03), and baseline aortic valve gradient (ß = 0.93, P < 0.001). BAV was predictive for AR. (ß = 0.91, P = 0.049).

Conclusion

Progression of AS in adult CoA patients is mild in this young population. Older age, aortic dilatation and the baseline aortic valve gradient are determinants for AS at follow-up. BAV is predictive for AR. These findings point towards a common embryological pathway of both valvular and aortic disease in CoA.     

Configuration of the ascending aorta in patients with bicuspid and tricuspid aortic valve disease undergoing aortic valve replacement with or without reduction aortoplasty

BACKGROUND AND AIM OF THE STUDY: Patients with bicuspid aortic valves (BAV) tend to develop dilatation of the ascending aorta. The study aim was to analyze differences in aortic root diameter and configuration in patients with bicuspid and tricuspid aortic valve disease. METHODS: A retrospective analysis was conducted of the angiographies of 461 patients allocated to four groups with: (i) BAV disease with (n = 179) and (ii) without (n = 78) dilatation of the ascending aorta; (iii) tricuspid aortic valve disease (TAV) and dilatation of the ascending aorta (n = 154); and (iv) coronary artery disease (CAD), TAV and normal diameter of the ascending aorta (n=50). Diameters and distances in the aortic root region were measured, and the ascending aorta configuration analyzed. RESULTS: The diameter of the ascending aorta in patients with BAV and dilatation was significantly larger than in those with TAV and dilatation (26.6 +/- 5.22 versus 24.4 +/- 3.74 mm/m2, p = 0.002). Distances between aortic valve level and point of maximum diameter of the ascending aorta at the outer and inner curve of the vessel in patients with BAV without dilatation were greater than those of the CAD group (31.1 +/- 5.27 versus 28.0 +/- 4.86 mm/m2, p = 0.002 and for the indexed values 21.6 +/- 4.05 versus 20.0 +/- 2.71 mm/m2, p = 0.011). All patients with BAV and enlargement of the ascending aorta showed asymmetric dilatation of the vessel. CONCLUSION: All patients with BAV had an abnormal configuration of the ascending aorta. In cases with enlargement of the ascending aorta exclusively, asymmetric dilatation at the convexity of the vessel occurred. Patients with BAV and normal ascending aorta diameter showed an elongation of this vessel segment.     

Dilatation of the entire thoracic aorta in patients with bicuspid aortic valve: a magnetic resonance angiography study

BACKGROUND: The presence of a bicuspid aortic valve (BAV) might be associated with a progressive dilatation of the aortic root and ascending aorta. However, involvement of the aortic arch and descending aorta has not yet been elucidated. PATIENTS AND METHODS: Magnetic resonance angiography (MRA) was used to assess the diameter of the ascending aorta, aortic arch, and descending aorta in 28 patients with bicuspid aortic valves (mean age 30 +/- 9 years). RESULTS: Patients with BAV, but without significant aortic stenosis or regurgitation (n = 10, mean age 27 +/- 8 years, n.s. versus control) were compared with controls (n = 13, mean age 33 +/- 10 years). In the BAV-patients, aortic root diameter was 35.1 +/- 4.9 mm versus 28.9 +/- 4.8 mm in the control group (p < 0.01). The diameter of the ascending aorta was also significantly increased at the level of the pulmonary artery (35.5 +/-5.6 mm versus 27.0 +/- 4.8 mm, p < 0.001). BAV-patients with moderate or severe aortic regurgitation (n = 18, mean age 32 +/- 9 years, n.s. versus control) had a significant dilatation of the aortic root, ascending aorta at the level of the pulmonary artery (41.7 +/- 4.8 mm versus 27.0 +/- 4.8 mm in control patients, p < 0.001) and, furthermore, significantly increased diameters of the aortic arch (27.1 +/- 5.6 mm versus 21.5 +/- 1.8 mm, p < 0.01) and descending aorta (21.8 +/- 5.6 mm versus 17.0 +/- 5.6 mm, p < 0.01). CONCLUSIONS: The whole thoracic aorta is abnormally dilated in patients with BAV, particularly in patients with moderate/severe aortic regurgitation. The maximum dilatation occurs in the ascending aorta at the level of the pulmonary artery. Thus, we suggest evaluation of the entire thoracic aorta in patients with BAV.     

Balloon aortic valvuloplasty for severe aortic stenosis as a bridge to transcatheter/surgical aortic valve replacement

Objectives : This study aimed to determine success‐ and complication rates after balloon aortic valvuloplasty (BAV) and the outcome of BAV as a standalone therapy versus BAV as a bridge to transcatheter/surgical aortic valve replacement (T/SAVR). Background : The introduction of transcatheter aortic valve replacement (TAVR) has led to a revival in BAV as treatment for patients with severe aortic stenosis. Methods : A cohort of 472 patients underwent 538 BAV procedures. The cohort was divided into two groups: BAV alone 387 (81.9%) and BAV as a bridge 85 (18.1%) to (n = 65, TAVR; n = 20, surgery). Clinical, hemodynamic, and follow‐up mortality data were collected. Results : There was no significant difference between the two groups in mean age (81.7 ± 8.3 vs. 83.2 ± 10.9 years, P = 0.18), society of thoracic surgeons score (13.1 ± 6.2 and 12.4 ± 6.4, P = 0.4), logistic EuroSCORE (45.4 ± 22.3 vs. 46.9 ± 21.8, P = 0.43), and other comorbidities. The mean increase in aortic valve area was 0.39 ± 0.25 in the BAV alone group and 0.42 ± 0.26 in the BAV as a bridge group, P = 0.33. The decrease in mean gradient was 24.1 ± 13.1 in the BAV alone group vs. 27.1 ± 13.8 in the BAV as a bridge group, P = 0.06. During a median follow up of 183 days [54–409], the mortality rate was 55.2% (n = 214) in the BAV alone group vs. 22.3% (n = 19) in the BAV as a bridge group during a median follow‐up of 378 days [177–690], P < 0.001. Conclusion : In high‐risk patients with aortic stenosis and temporary contraindications to SAVR/TAVR, BAV may be used as a bridge to intervention with good mid‐term outcomes. © 2012 Wiley Periodicals, Inc.     

Balloon aortic valvuloplasty to improve candidacy of patients evaluated for transcatheter aortic valve replacement

Objectives

Evaluate the role of balloon aortic valvuloplasty (BAV) in improving candidacy of patients for transcatheter aortic valve replacement (TAVR).

Background

Patients who are not candidates for TAVR may undergo BAV to improve functional and clinical status.

Methods

117 inoperable or high‐risk patients with critical aortic stenosis underwent BAV as a bridge‐to‐decision for TAVR. Frailty measures including gait speed, serum albumin, hand grip, activities of daily living (ADL); and NYHA functional class before and after BAV were compared.

Results

Mean age was 81.6 ± 8.5 years and the mean Society of Thoracic Surgeons predicted risk of mortality was 9.57 ± 5.51, with 19/117 (16.2%) patients non‐ambulatory. There was no significant change in mean GS post‐BAV, but all non‐ambulatory patients completed GS testing at follow‐up. Albumin and hand grip did not change after BAV, but there was a significant improvement in mean ADL score (4.85 ± 1.41 baseline to 5.20 ± 1.17, P = 0.021). The number of patients with Class IV congestive heart failure (CHF) was significantly lower post BAV (71/117 [60.7%] baseline versus 18/117 [15.4%], P = 0.008). 78/117 (66.7%) of patients were referred to definitive valve therapy after BAV.

Conclusions

When evaluating frailty measures post BAV, we saw no significant improvement in mean GS, however, we observed a significant improvement in non‐ambulatory patients and ADL scores. We also describe improved Class IV CHF symptoms. With this improved health status, the majority of patients underwent subsequent valve therapy, demonstrating that BAV may improve candidacy of patients for TAVR.     

Diastolic velocity half time is associated with aortic coarctation gradient at catheterization independent of echocardiographic and clinical blood pressure gradients

Objective: The most accurate noninvasive parameter to predict whether a patient with aortic coarctation will meet interventional criteria at catheterization remains elusive. We aim to determine the best independent echocardiographic predictors of a coarctation peak‐to‐peak pressure gradient ≥20 mm Hg at catheterization, the ac‐ cepted threshold for intervention.
Design: Retrospective query of our catheterization database from 1/2007 to 7/2016 for the diagnostic code of aortic coarctation was performed. Multiple echocardio‐ graphic measurements and blood pressure gradients prior to cardiac catheterization were collected. Univariate correlation of variables with the continuous catheteriza‐ tion peak were calculated using Spearman’s rho. Univariate association with peak‐to‐ peak gradient at catheterization ≥20 mm Hg was tested using Mann‐Whitney U test and the Pearson chi‐square test or Fisher’s exact test. Multivariable logistic regres‐ sion assessed the independent association of the clinically relevant metrics with gra‐ dient at catheterization ≥20 mm Hg.
Results: Sixty‐eight patients met study criteria (median age 9.25 years), of whom 84% underwent intervention at catheterization. Echocardiographic peak and mean coarctation velocity, indexed systolic and diastolic velocity half times (SVHTi, DVHTi), and blood pressure gradient all had moderate correlation (Spearman’s rho = 0.529‐0.617, P < .001) with the continuous catheterization gradient and were significantly associated with the binary outcome of catheterization peak ≥20 mm Hg (P < .001). Logistic regression found echocardiographic mean systolic gradient (OR 1.213 [95% CI 1.041‐1.414]) and DVHTi (OR 1.039 [95% CI 1.004‐1.074]) indepen‐ dently associate with catheterization peak ≥20 mm Hg after controlling for blood pressure gradient (OR 1.066 [0.987‐1.150]).
Conclusions: Most echocardiographic estimates show moderate correlation with arch gradient at catheterization. Noninvasive four extremity blood pressure gradient is significantly associated with peak‐to‐peak gradient ≥20 mm Hg. DVHTi may pro‐ vide a unique independently associated echocardiographic estimate of coarctation severity. Further study of these variables with larger cohorts may allow for develop‐ ment of predictive models to direct catheterization.     

Dilatation of the ascending aorta and serum alpha 1-antitrypsin level in patients with bicuspid aortic valve

Predictors of aortic dilatation are not well described in patients with bicuspid aortic valves (BAV). This study sought to examine the relationship between proximal aortic dilatation and matrix metalloproteinase-9 (MMP-9) and alpha 1-antitrypsin (α1AT) levels in patients with BAV. All patients underwent echocardiography using a standard protocol, and aortic measurements were taken in end-diastole. We studied 82 patients with BAV and categorized them into two groups according to aortic dimensions corrected for body surface area and age. The plasma level of α1AT was routinely determined using a BN ProSpec analyzer (Siemens Healthcare Diagnostics, Marburg, Germany), and that of MMP-9 were determined by ELISA (RayBiotech Inc. Norcross, GA, USA). Statistical analysis was performed using the Statistical Package for Social Sciences (SPSS; SPSS Inc., Chicago, IL, USA) software for Windows version 12. This study included patients with BAV with no or mild valvular impairment. There were no significant differences between groups in terms of gender, body surface area, associated hypertension, diabetes mellitus, hyperlipidemia, or smoking. The mean ascending aortic diameter was 4.38?±?0.5?mm in group 1 and 3.34?±?0.35?mm in group 2 (p?相似文献   

A larger aortic annulus causes aortic regurgitation and a smaller aortic annulus causes aortic stenosis in bicuspid aortic valve

A bicuspid aortic valve (BAV) often causes aortic stenosis (AS) or regurgitation (AR). In 54 patients with a BAV (48 +/- 16 years), transthoracic and transesophageal echo were performed to measure aortic annulus diameter (AAD), to evaluate the severity of aortic valve disease (AVD) and to calculate the area eccentricity index (AEI) of a BAV defined as a ratio of the larger aortic cusp area to a smaller aortic cusp area. By multiple linear regression analysis, the severity of AR correlated significantly with the AAD (r = 0.38) and AEI (r = 0.35) (P < 0.05) and that of AS correlated significantly with the AAD (r =-0.40) and AEI (r = 0.34) (P < 0.05). Thirty-six patients showed anteroposteriorly (A-P) located BAVs and 18 patients showed right-left (R-L) located BAVs. The AAD was larger in A-P type than in R-L type (15 +/- 3 vs 13 +/- 2 mm/BSA, P < 0.05) and there was no difference in the age and AEI between the two groups. AR was more severe in A-P type than in R-L type while AS was more severe in R-L type than in A-P type (P < 0.05). Twenty-nine patients showed raphes. The AEI was larger in raphe (+) type than in raphe (-) type (1.83 +/- 0.53 vs 1.51 +/- 0.47, P < 0.05) and there was no difference in the AAD and severity of AVD between the two groups. In conclusion, a BAV with larger aortic annulus or A-P located will tend to cause AR while a BAV with smaller aortic annulus or R-L located will tend to cause AS.     

Long‐term performance of bicuspid and quadricuspid aortic valves: Similarities and differences

While bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, quadricuspid aortic valve (QAV) is rare. The usual three‐leaflet aortic valve is characterized by engineering advantages with superior long‐term performance, and thus, degenerative changes and significant functional deterioration appear at advanced age. Aim: Evaluation of long‐term performance, similarities, and differences between QAV and BAV. Methods: Screening of 19 000 consecutive echocardiographic studies was performed. Results: BAV was reported in 131 subjects with a prevalence of 0.7%, while QAV was seen in 11 with a prevalence of 0.06%, P < .00001. Age of BAV patients was younger, 45 ± 20 years vs 62 ± 17 years in QAV, P < .05, with higher proportion of females in those with QAV, 40% vs 30%. Chamber diameters were similar in both groups. Higher atrial contraction‐A‐wave mitral peak velocities and longer E‐wave deceleration times were found in subjects with QAV, P < .05 for both. Dilated ascending aorta was found in 25% of patients with BAV and in 18% of those with QAV, P = .2. Moderate and severe aortic valve stenosis were found in 21% of patients with BAV and in 27% of those with QAV, P = ns. More than moderate aortic regurgitation was found in 15.5% of BAV patients and in 9% of QAV, P = ns. Aortic valve infective endocarditis was found in 1.5% of BAV patients and in 9% of those with QAV. Conclusions: BAV is a common congenital anomaly, while QAV is rare. Similar prevalence of significant valve disease and aortopathy was found in both anomalies, though at younger age in BAV patients.     

Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve

BackgroundBicuspid aortic valve (BAV) is the most common congenital heart disease, often associated with valve dysfunction, coarctation of the aorta, and ascending aorta dilatation. Aortic dilatation might result from abnormal regional hemodynamics or inherent vascular disease. Vascular function in pediatric BAV remains poorly characterized.MethodsA cross-sectional study was performed to evaluate vascular function in 142 children with BAV aged 7-18 years compared with healthy control children. Echocardiography was performed to assess aortic dimensions, BAV function, and vascular function (aortic arch pulse wave velocity [PWV]), carotid intima media thickness, and aortic stiffness and distensibility). Carotid-femoral and carotid-radial PWV were assessed using tonometry. Vascular function was compared for 4 patient groups stratified according to aortic dilatation and a history of coarctation of the aorta. Multivariate regression analysis was performed to determine predictors of aortic dilatation.ResultsChildren with BAV had stiffer and less distensible ascending aortas with higher aortic arch PWV compared with control children. Carotid-femoral and carotid-radial PWV were not increased in patients with BAV, and the vascular assessment of the abdominal aorta was unremarkable. Multivariate regression revealed that aortic arch PWV was the only vascular function parameter that was associated with aortic dilatation.ConclusionsChildren with BAV have differences in vascular function that are confined to their proximal aorta, even in normal functioning BAV. The observed differences in vascular function are likely multifactorial, with contributions from abnormal regional flow and a potential localized primary aortopathy.     

Ascending aortic aneurysm and dissection in young adults with bicuspid aortic valve: Implications for echocardiographic surveillance

Bicuspid aortic valve (BAV) is an independent risk factor for aneurysm and dissection of the ascending aorta. Despite this association, routine imaging of the aorta has not been recommended for patients with BAV. We describe two young men who developed life-threatening aneurysm or dissection of the ascending aorta; one had a normally functioning BAV and the other was 10 years after valve replacement. The pathology of this condition is very similar to that found in the Marfan syndrome. We recommend echocardiographic surveillance of the ascending aorta at regular intervals, and consideration of beta-adrenergic blockade among patients with significant dilation.     

Association of Bicuspid Aortic Valve Morphology and Aortic Root Dimensions: A Substudy of the Aortic Stenosis Progression Observation Measuring Effects of Rosuvastatin (ASTRONOMER) Study

Background: Bicuspid aortic valve (BAV) is the leading cause of aortic stenosis in patients younger than the age of 50. A classification scheme of BAVs is based upon leaflet orientation: Type I (fusion of right and left coronary cusps) and Type II (fusion of right and noncoronary cusps). The correlation between BAV leaflet orientation and aortic root pathology however remains ill defined. Objective: The objective was to describe a potential relationship between BAV leaflet morphology and aortic root measurements in the ASTRONOMER study, a multicenter study to assess the effect of rosuvastatin on the progression of AS. Methods: BAV morphology was classified as Type I or Type II orientation based on the parasternal short‐axis view. Echo measurements including left ventricular and aortic root dimensions were obtained. Results: The study population included 89 patients (56 ± 11 years; 44 males). There were 63 patients with Type I and 26 patients with Type II BAV. Baseline demographics, hemodynamics, and left heart dimensions were similar between both groups. Patients with Type I BAV had larger aortic annulus and ascending root dimensions compared to those patients with Type II BAV (P < 0.05). Conclusion: In patients with mild to moderate aortic stenosis due to a BAV, the presence of Type I valve orientation was associated with significantly greater aortic root parameters compared to Type II valve orientation. (ECHOCARDIOGRAPHY 2010;27:174‐179)