Tricuspid Aortic Valve Group Research Articles

The significance of bicuspid aortic valve after surgery for acute type A aortic dissection

ObjectiveDecision-making concerning the extent of the repair of acute type A aortic dissection (ATAAD) includes functional and anatomical assessment of the aortic valve. We hypothesized that bicuspid aortic valve (BAV) does not impact outcome after surgery for ATAAD. We therefore evaluated the outcome after ATAAD surgery in relation to the presence of BAV, acute aortic regurgitation (AR), and surgical approach, using the Nordic Consortium for Acute Type A Aortic Dissection database. MethodsEight participating Nordic centers collected data from 1122 patients undergoing ATAAD surgery during the years 2005 to 2014. Early complications, reoperations and survival were compared between patients with BAV and tricuspid aortic valves (TAV) before and after propensity score matching for sex, age, AR, organ malperfusion, hemodynamic instability, and site of the tear. Mean follow-up (range) for patients with TAV and BAV was 3.1 years (0-10.4 years) and 3.2 years (0-9.0 years), respectively. ResultsAltogether, 65 (5.8%) of the patients had BAV. Root replacement was more frequently performed in the BAV as compared with the TAV group (60% vs 23%, P < .001). Survival, however, did not differ significantly between patients with BAV or TAV, either before (P = .230) or after propensity score-matching (P = .812). Even so, in cohort as a whole, patients presenting with AR had less favorable survival. ConclusionsEarly and mid-term survival did not differ significantly between patients with BAV and TAV.

Early and long-term results of aortic valve sparing aortic root reimplantation surgery for bicuspid and tricuspid aortic valves

To evaluate early and long-term clinical outcomes following aortic valve sparing aortic root reimplantation surgery in patients with leaking bicuspid and tricuspid aortic valves. The study consisted of 92 consecutive adult patients (tricuspid aortic valve group = 63 and bicuspid aortic valve group = 29) who underwent aortic valve sparing aortic root reimplantation surgery with or without aortic cusp repair for dilatation of the aortic root and/or aortic valve regurgitation at our institution from April 2004 to October 2016. Clinical outcomes were investigated using Kaplan-Meier and log-rank tests between groups. The follow-up was 100% complete with a mean time of 5.3 ± 3.3 years. The 30-day in-hospital mortality was 3.1% in tricuspid aortic valve group and 3.4% in bicuspid aortic valve group patients. The overall survival rates at 10 years did not differ between bicuspid aortic valve and tricuspid aortic valve patient groups (96.6 ± 3.3% vs. 90.3 ± 4.2%, p = 0.3). Freedom from recurrent aortic valve regurgitation (>2+) at 10 years was 90.5 ± 4.1% in tricuspid aortic valve group and 75.7 ± 8.7% in bicuspid aortic valve group (p = 0.06). Freedom from aortic valve reoperation at 10 years was 100% in tricuspid aortic valve group and 83.9 ± 7.4% in bicuspid aortic valve group (p = 0.002). Aortic valve sparing aortic root reimplantation surgery is a safe and efficient technique, providing acceptable long-term survival with low rates of valve-related complications in both tricuspid aortic valve and bicuspid aortic valve patient groups. However, aortic valve reoperation rates at 10 years follow-up were higher in bicuspid aortic valve group patients compared to tricuspid aortic valve group patients.

Ascending aortic dilatation rate after transcatheter aortic valve replacement in patients with bicuspid and tricuspid aortic stenosis: A multidetector computed tomography follow-up study.

Current data is lacking about the progression of ascending aortic dilatation after transcatheter aortic valve replacement (TAVR) in aortic stenosis (AS) patients with bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV). This study aims to assess the ascending aortic dilatation rate (mm/year) after TAVR in patients with BAV versus TAV using a multidetector computed tomography (MDCT) follow-up and to determine the predictors of ascending aortic dilatation rate. Severe AS patients undergoing TAVR from March 2013 to March 2018 at our center with MDCT follow-ups were included. BAV and TAV were identified using baseline MDCT. Baseline and follow-up MDCT images were analyzed, and the diameters of ascending aorta were measured. Study end point is ascending aortic dilatation rate (mm/year). Furthermore, factors predicting ascending aortic dilatation rate were also investigated. Two hundred and eight patients were included, comprised of 86 BAV and 122 TAV patients. Five, 4, 3, 2, and 1-year MDCT follow-ups were achieved in 7, 9, 30, 46, and 116 patients. The ascending aortic diameter was significantly increased after TAVR in both BAV group (43.7±4.4 mm vs. 44.0±4.5 mm; P<0.001) and TAV group (39.1±4.8 mm vs. 39.7±5.1 mm; P<0.001). However, no difference of ascending aortic dilatation rate was found between BAV and TAV group (0.2±0.8 mm/year vs. 0.3±0.8 mm/year, P=0.592). Multivariate linear regression revealed paravalvular leakage (PVL) grade was independently associated with ascending aortic dilatation rate in the whole population and BAV group, but not TAV group. No aortic events occurred during follow-ups. Ascending aortic size continues to grow after TAVR in BAV patients, but the dilatation rate is mild and comparable to that of TAV patients. PVL grade is associated with ascending aortic dilatation rate in BAV patients post-TAVR.

Aortic Arch Branching Patterns in Bicuspid and Tricuspid Aortic Valve Patients

The aortic arch and its branches form during the third week of embryogenesis. The most common human pattern has the innominate artery, the left common carotid artery and the left subclavian artery all as separate branches. Large imaging studies have shown 70% of people have a normal branching pattern with 20% having a common origin of the innominate artery and left common carotid artery, but these studies were performed without reference to the anatomy of the aortic valve (bicuspid versus tricuspid).Bicuspid aortic valve (BAV) is the commonest congenital cardiac malformation and as the arch branching patterns are developmental in origin we decided to see if the frequency of arch variants in BAV and tricuspid aortic valve (TAV) patients differed, as this has not previously been looked at. We examined Computerised Tomographic aortograms and echocardiograms of BAV and TAV patients to assess the aortic arch branching pattern and any possible association with the valve morphology. 28 BAV and 57 TAV patients were assessed. For BAV the branching patterns were: 86% normal (24/28) and 14% abnormal (4/28), and for TAV: 70% normal (40/57) and 30% abnormal (17/57). Although this is a small study our TAV group demonstrated comparable normal/abnormal arch variants as the published literature, but the BAV group appears to have fewer arch variants. This is the first study in the literature to look at the arch branching variants when consideration of the aortic valve morphology (BAV versus TAV) is taken into account.

Targeted next-generation sequencing identified ADAMTS5 as novel genetic substrate in patients with bicuspid aortic valve.

Bicuspid Aortic Valve (BAV) is the most common congenital heart disease, affecting >1% of the general population. Up to date, three genes, NOTCH1, GATA5 and SMAD6, have been linked to the isolated form of BAV. However, potential genetic determinants remain largely unknown in most BAV patients. Targeted next-generation sequencing of 7 BAV candidate genes (NOTCH1, GATA5, SMAD6, NOS3, ADAMTS5, Alk2 and SMAD2) was performed in 32 BAV patients. Additional 35 BAV patients and 238 tricuspid aortic valve (TAV) patients, consisting of 107 patients from the transcatheter aortic valve implantation (TAVI) registry and 131 patients from the coronary artery disease (CAD) registry, were selected for further genotyping. We found 2 rare non-synonymous variants in 2/7 genes in 3 BAV patients: one was NOTCH1:c.4297G>A and the other one was ADMTS5:c.935C>A that shared by two patients. NOTCH1:c.4297G>A has not been reported previously. ADMTS5:c.935C>A was predicted to be pathogenic by all applied algorithms. Alignment of protein sequences from all available species revealed that ADMTS5:p.Arg312Leu, produced by ADMTS5:c.935C>A, is located in a highly conserved region. The minor allele frequency of ADMTS5:c.935C>A in BAV patients was significantly higher than the matched population in TAV group (0.015 vs. 0, P=0.048). Our results suggested that ADMTS5:c.935C>A are potentially associated with BAV. Further studies, such as large sample case-control replication test and functional research, are needed to explore the role of this rare variant in the development of BAV.

Evidence of subannular and left ventricular morphological differences in patients with bicuspid versus tricuspid aortic valve stenosis: magnetic resonance imaging-based analysis.

Prospective analysis of left ventricular (LV) morphological/functional parameters in patients with bicuspid versus tricuspid aortic valve (TAV) stenosis undergoing aortic valve replacement (AVR) surgery. A total of 190 consecutive patients with BAV ( n = 154) and TAV stenosis ( n = 36) (mean age 61 ± 8years, 65% male) underwent AVR ± concomitant aortic surgery from January 2012 through May 2015. All patients underwent preoperative cardiac magnetic resonance imaging in order to evaluate: (i) left ventricular outflow tract (LVOT) dimensions, (ii) length of anterior mitral leaflet (AML), (iii) end-systolic and end-diastolic LV wall thickness, (iv) LV area, (v) LV end-systolic and end-diastolic diameters (LVESD, LVEDD), (vi) LV end-diastolic and end-systolic volumes (LVEDV, LVESV) and (vii) maximal diameter of aortic root. These parameters were compared between the two study groups. The LVOT diameter was significantly larger in BAV patients (21.7 ± 3 mm in BAV vs 18.9 ± 3 mm in TAV, P < 0.001). Moreover, BAV patients had significantly longer AML (24 ± 3 mm in BAV vs 22 ± 4 mm in TAV, P = 0.009). LVEDV and LVESV were significantly larger in BAV patients (LVEDV: 164.9 ± 68.4 ml in BAV groups vs 126.5 ± 53.1 ml in TAV group, P = 0.037; LVESV: 82.1 ± 57.9 ml in BAV group vs 52.9 ± 25.7 ml in TAV group, P = 0.008). A strong linear correlation was found between LVOT diameter and aortic annulus diameter in BAV patients ( r = 0.7, P < 0.001), whereas significantly weaker correlation was observed in TAV patients ( r = 0.5, P = 0.006, z = 1.65, P = 0.04). Presence of BAV morphology was independently associated with larger LVOT diameters (OR 9.0, 95% CI 1.0-81.3, P = 0.04). We found relevant differences in LV morphological/functional parameters between BAV and TAV stenosis patients. Further investigations are warranted in order to determine the cause of these observed differences.

Functional Aortic Root Parameters and Expression of Aortopathy in Bicuspid Versus Tricuspid Aortic Valve Stenosis

BackgroundThe correlation between bicuspid aortic valve (BAV) disease and aortopathy is not fully defined. ObjectivesThis study aimed to prospectively analyze the correlation between functional parameters of the aortic root and expression of aortopathy in patients undergoing surgery for BAV versus tricuspid aortic valve (TAV) stenosis. MethodsFrom January 1, 2012 through December 31, 2014, 190 consecutive patients (63 ± 8 years, 67% male) underwent aortic valve replacement ± proximal aortic surgery for BAV stenosis (n = 137, BAV group) and TAV stenosis (n = 53, TAV group). All patients underwent pre-operative cardiac magnetic resonance imaging to evaluate morphological/functional parameters of the aortic root. Aortic tissue was sampled during surgery on the basis of the location of eccentric blood flow contact with the aortic wall, as determined by cardiac magnetic resonance (i.e., jet sample and control sample). Aortic wall lesions were graded using a histological sum score (0 to 21). ResultsThe largest cross-sectional aortic diameters were at the mid-ascending level in both groups and were larger in BAV patients (40.2 ± 7.2 mm vs. 36.6 ± 3.3 mm, respectively, p < 0.001). The histological sum score was 2.9 ± 1.4 in the BAV group versus 3.4 ± 2.6 in the TAV group (p = 0.4). The correlation was linear and comparable between the maximum indexed aortic diameter and the angle between the left ventricular outflow axis and aortic root (left ventricle/aorta angle) in both groups (BAV group: r = 0.6, p < 0.001 vs. TAV group r = 0.45, p = 0.03, z = 1.26, p = 0.2). Logistic regression identified the left ventricle/aorta angle as an indicator of indexed aortic diameter >22 mm/m2 (odds ratio: 1.2; p < 0.001). ConclusionsComparable correlation patterns between functional aortic root parameters and expression of aortopathy are found in patients with BAV versus TAV stenosis.

Cardiac Mechanics in Isolated Bicuspid Aortic Valve Disease With Normal Ejection Fraction: A Study of Various Valvular Lesion Types.

Aortic stenosis (AS) and aortic regurgitation (AR) are associated with congenital isolated bicuspid aortic valve (BAV) disease. The chronic pressure overload of AS and the volume overload of AR are known to impair the left ventricular function. This study assessed whether two-dimensional speckle tracking echocardiography (2D-STE) is capable of detecting the myocardial dysfunction associated with BAV caused by various aortic valve lesions in patients retaining normal ejection fraction (EF).Thirty-two isolated BAV patients and 20 healthy tricuspid aortic valve (TAV) volunteers were recruited. BAV patients were divided into 4 subgroups based on aortic valvular lesion types: normal function (NF) group, isolated AS group, isolated AR group, and a group who had both AS&AR. Myocardial strain and degree of twist were analyzed and compared between the BAV and TAV groups, as well as between valvular lesion groups and the NF group.Compared with healthy TAV controls, global radial strain (GRS), global circumferential strain (GCS), global longitudinal strain (GLS), and twist angle absolute values were lower in the BAV group (P < 0.05). The AS, AR, and AS&AR groups all demonstrated a significant decrease in GRS and GCS when compared with the TAV group. The AS and AS&AR groups demonstrated lower GLS than the TAV group, and the smallest degree of twist was detected in the AR group. There were no significant differences between the NF and TAV groups. The AR and AS&AR groups demonstrated significant differences in multiple parameters of cardiac mechanics compared with the NF group.2D-STE is able to detect altered cardiac mechanics associated with aortic lesion types in BAV patients with normal EF compared with normal TAV controls, and so can provide valuable information for clinical decision-making.

Abstract 19684: Abnormal Aortic Wall Properties in Children with Isolated Bicuspid Aortic Valve

Introduction: Abnormal aortic wall properties have been reported in patients with isolated bicuspid aortic valve (IBAV) even in the absence of significant aortic stenosis or regurgitation. Hypothesis: We sought to assess aortic distensibility (DIS) and stiffness index (SI) in children with IBAV compared to age group-matched subjects with normal tricuspid aortic valves (TAV) and to determine whether these abnormalities in the aortic wall properties correlate with bicuspid valve morphology or left ventricular systolic or diastolic function. Methods: Children ages 8-18 years with an IBAV and age group-matched controls with a TAV were prospectively enrolled. Subjects with greater than mild stenosis or mild regurgitation were excluded. Using echo, aortic valve morphology, aortic root (AoR) and ascending aorta (AAo) diameters and z-scores were determined. Left ventricular shortening fraction (LVSF), DIS and SI were measured using M-mode echo. Diastolic function was determined using mitral valve septal E/Ea. Blood pressure (BP) was measured at the time of echo. Results: Nineteen had IBAV and 17 had TAV. There were no significant differences in age, weight, height or BP between the two groups. In the IBAV group, 11 had right-left type (R/L) and 8 had right-non type (R/N). There was no significant difference in AoR z-scores between groups. The IBAV group had larger AAo z-scores (2.48±1.9 vs. -0.02±0.98, p&lt;0.0001), decreased DIS (9.6±4 vs. 12.3±3.1 cm2 dynes-1 x 10-6, p&lt;0.05) and increased SI (21.4±9.2 vs. 14.4±3.8, p=0.007) compared to the TAV group. There were no differences in these variables between the R/L or R/N subgroups. No correlation was seen between aortic wall properties and ventricular function in the IBAV group. By multivariate regression, presence of an IBAV (coefficient = -2.4, p=0.03), LVSF (coefficient = -0.35, p=0.01) and age-adjusted systolic BP (coefficient = -0.13, p=0.03) were independently associated with DIS. Similarly, presence of an IBAV (coefficient = 6.7, p=0.005) and age (coefficient=0.85, p=0.02) were independently associated with SI. Conclusions: Children with IBAV have decreased DIS and SI even without hemodynamic abnormalities. Long-term studies to determine the impact of these findings on cardiovascular risk are needed.

Valve-sparing root reimplantation and leaflet repair in a bicuspid aortic valve: Comparison with the 3-cusp David procedure

Valve-sparing root reimplantation (VSRR) in tricuspid aortic valve (TAV) patients is well established, but in bicuspid aortic valve (BAV) patients, it has been less widely adopted. We assessed whether valve type affects midterm outcomes with VSRR. A retrospective review was performed of 186 patients who underwent an aortic valve-sparing root reimplantation operation between 2004 and 2013. Of these, 129 patients underwent elective VSRR with the David V technique. Outcomes were compared in this cohort by valve type: TAV (n=89) versus BAV (n=40). Demographics were similar in the 2 groups. BAV patients had a higher degree of aortic insufficiency (AI) at presentation (P<.05), and an enlarged preoperative annulus (30 ± 4 vs 28 ± 6 mm, P=.06). All BAV patients required primary leaflet repair (6% in the TAV group; P<.01). Postoperative mortality (0), stroke (0% vs 1%), and pacemaker requirement (0% vs 5%) were similar. Postoperative freedom from AI grade ≥2+ was 100% in the entire cohort, and transvalvular gradients were similar. At follow-up, a 1-year echocardiogram showed higher peak and mean transvalvular gradients in the BAV group (P<.01). One TAV group patient died froman unknown cause. The 5-year actuarial freedom from aortic valve reoperation was 100% versus 97%± 3% (P=.6). Three patients in the entire cohort have had AI grade >2+ on follow-up (n=1 in the BAV group; n=2 in the TAV group). Even though BAV patients present with higher AI grade and require concomitant primary valve repair, the VSRR David V technique offers excellent midterm outcomes with both the BAV and TAV valve types.

Type A aortic dissection in patients with bicuspid aortic valves: clinical and pathological comparison with tricuspid aortic valves

ObjectiveBicuspid aortic valve (BAV) is associated with a higher risk of type A aortic dissection (AD) compared with tricuspid aortic valve (TAV). We sought to study differences between patients with...

Long-term prognosis of ascending aortic aneurysm after aortic valve replacement for bicuspid versus tricuspid aortic valve stenosis

The bicuspid aorta is thought to have a higher risk of progressive dilation after aortic valve replacement with a subsequently increased risk of adverse aortic events. Our aim was to compare the risk of late aortic events after isolated aortic valve replacement surgery for bicuspid versus tricuspid aortic valve stenosis with concomitant mild to moderate dilatation of the proximal aorta. A total of 325 consecutive patients (60% males; mean age, 59.5 ± 10 years) with aortic valve stenosis and concomitant ascending aortic dilatation of 40 to 50 mm underwent isolated aortic valve replacement from 1995 through 2000. A total of 153 patients (47%) were diagnosed with bicuspid aortic valve stenosis (bicuspid aortic valve group), whereas the remaining 172 patients (53%) had tricuspid aortic valve stenosis (tricuspid aortic valve group). Follow-up (3566 patient-years) was 100% complete. Adverse aortic events were defined as the need for proximal aortic surgery or the occurrence of aortic dissection/rupture or sudden death during follow-up. Overall survival was 78 ± 4% in the bicuspid aortic valve group versus 55 ± 6% in the tricuspid aortic valve group (P=.006) at 15 years postoperatively, but age-adjusted survival was not significantly different between groups (P=.4). A total of 5 patients (3%) in the bicuspid aortic valve group versus 9 patients (5%) in the tricuspid aortic valve group underwent proximal aortic surgery during follow-up. Aortic dissection occurred in 3 patients in the tricuspid aortic valve group and in no bicuspid aortic valve patients. Fifteen-year freedom from adverse aortic events was 93 ± 3% in the bicuspid aortic valve group versus 82 ± 6% in the tricuspid aortic valve group (P=.2). Patients with bicuspid and tricuspid aortic valve stenosis with concomitant mild to moderate ascending aortic dilatation are at comparably low risk of adverse aortic events 15 years after isolated aortic valve replacement.

Increased Ascending Aortic Wall Stress in Patients With Bicuspid Aortic Valves

Patients with bicuspid aortic valves (BAV) are at increased risk of ascending aortic dilatation, dissection, and rupture. We hypothesized that ascending aortic wall stress may be increased in patients with BAV compared with patients with tricuspid aortic valves (TAV). Twenty patients with BAV and 20 patients with TAV underwent electrocardiogram-gated computed tomographic angiography. Patients were matched for diameter. The thoracic aorta was segmented, reconstructed, and triangulated to create a mesh. Utilizing a uniform pressure load of 120 mm Hg, and isotropic, incompressible, and linear elastic shell elements, finite element analysis was performed to predict 99th percentile wall stress. For patients with BAV and TAV, aortic root diameter was 4.0 ± 0.6 cm and 4.0 ± 0.6 cm (p = 0.724), sinotubular junction diameter was 3.6 ± 0.8 cm and 3.6 ± 0.7 cm (p = 0.736), and maximum ascending aortic diameter was 4.0 ± 0.8 cm and 4.1 ± 0.9 cm (p = 0.849), respectively. The mean 99 th percentile wall stress in the BAV group was greater than in the TAV group (0.54 ± 0.06 MPa vs 0.50 ± 0.09 MPa), though this did not reach statistical significance (p = 0.090). When normalized by radius, the 99 th percentile wall stress was greater in the BAV group (0.31 ± 0.06 MPa/cm vs 0.27 ± 0.03 MPa/cm, p = 0.013). Patients with BAV, regardless of aortic diameter, have increased 99 th percentile wall stress in the ascending aorta. Ascending aortic three-dimensional geometry may account in part for the increased propensity to aortic dilatation, rupture, and dissection in patients with BAV.

Differential Protein Kinase C Isoform Abundance in Ascending Aortic Aneurysms From Patients With Bicuspid Versus Tricuspid Aortic Valves

It is recognized that different events contribute to the initiation of ascending thoracic aortic aneurysms (ATAAs) in patients with bicuspid aortic valves (BAV) versus patients with tricuspid aortic valves (TAV), but the molecular signaling pathways driving aneurysm formation remain unclear. Protein kinase C (PKC) is a superfamily of kinases which differentially mediate signaling events that lead to altered gene expression and cellular function, and may regulate downstream mediators of vascular remodeling. The present study tested the hypothesis that ATAA development in patients with BAV versus TAV proceeds by independent signaling pathways involving differential PKC signaling. ATAA samples were collected from BAV (n=57) and TAV (n=55) patients and assessed for 10 different PKC isoforms by immunoblotting. Results were expressed as a percent change in abundance (mean+/-SEM) from a nonaneurysmal control group (100%, n=21). Correlation analysis was performed, and relationships between PKC and matrix metalloproteinase abundance were reported. In the BAV group, classic and novel PKC isoforms (PKC-alpha, betaI, gamma, epsilon, theta) were increased, whereas PKC-eta and atypical PKC-zeta were decreased. In the TAV group, classic and novel isoforms were decreased and atypical PKC-zeta was elevated. Positive correlations between PKC and matrix metalloproteinase abundance were identified. Differential PKC isoform abundance was observed in ATAA samples from patients with BAV versus TAV, suggesting independent molecular signaling pathways may be operative. Induction of independent transcriptional programs may result and may provide a mechanistic foundation for developing selective diagnostic/therapeutic strategies for patients with ATAAs secondary to BAV or TAV.

Expression of fibrillin-1 in congenital bicuspid aortic valves

To detect the expression of fibrillin-1 in congenital bicuspid aortic valves, and to investigate the molecular mechanism of congenital bicuspid aortic valves. Specimens of aortic valve were obtained from 12 pediatric patients with congenital bicuspid aortic valve, 11 boys and 1 girl, aged 16.7 (10 - 18), including 5 cases of aortic stenosis (AS), 8 of aortic insufficiency (AI), and 1 of AS and AI, undergoing valve replacement, 8 children who died accidentally without cardiovascular system and collagen system diseases, 6 boys and 2 girls, aged 9.1 (1 - 17), collected in autopsy [normal (tricuspid) aortic valve controls], and 18 pediatric patients of rheumatic valvular heart disease with diseased tricuspid aortic valves who underwent aortic valve replacement, 13 boys and 5 girls, aged 16.5 (12 - 18) (rheumatic valvular heart disease controls). HE staining and light microscopy were conducted. Immunohistochemistry was used to detect the expression of fibrillin-1 in the aortic valves. Microscopy showed that the tissue structure of the congenital bicuspid aortic valves was unclear with hyperplasia of fibrous tissue. The grey degree value of fibrillin-1 of the congenital bicuspid aortic valve group was 170 +/- 10, significantly lower than those of normal aortic valve group and diseased tricuspid aortic valve group (126 +/- 8 and 73 +/- 16 respectively, both P < 0.05). There were not significant difference in the grey degree value of fibrillin-1 among the patients of congenital bicuspid aortic valves with AS, AI, and AS + AI (167 +/- 6, 171 +/- 8, and 168 +/- 6 respectively). The expression of fibrillin-1 is significantly reduced in congenital bicuspid aortic valves which may contribute to the morphological changes of the aortic valve leaflets and their resultant functional failure in congenital bicuspid aortic valves.

Progression Rate of Ascending Aortic Dilation in Patients With Normally Functioning Bicuspid and Tricuspid Aortic Valves

Dilation of the ascending aorta (AA), which is disproportionate to associated valvular lesions, is a relatively well-recognized phenomenon in patients with a bicuspid aortic valve (BAV). The aim of this study was to evaluate the rate of changes in the AA dimensions and the outcome in patients with AA dilation and BAVs compared with patients with AA dilation and tricuspid aortic valves (TAVs). Serial transesophageal echocardiograms (>12 months apart) were performed in 113 consecutive patients (BAV, n=27 and TAV, n=86) with AA diameters of >or=40 and <or=60 mm, respectively, without associated significant aortic valve stenosis or regurgitation. Baseline diameters at the sinuses of Valsalva (SV), the sinotubular junction (STJ), and the tubular tract (TT) were similar in both groups (41.3+/-5.3 vs 42.3+/-6.3 mm, p=NS, 37.8+/-5.2 vs 38.7+/-5.5 mm, p=NS, and 47.3+/-5.3 vs 45.9+/-5.1, p=NS, respectively). During an average 3-year follow-up, the rate of AA diameter progression was similar for the BAV and TAV groups (0.86+/-0.81 vs 0.82+/-1.1 mm/year, p=NS for the SV; 1.06+/-1.6 vs 0.63+/-1.1 mm/year, p=NS for the STJ; and 0.81+/-1.1 vs 0.75+/-1.1 mm/year, p=NS for the TT, respectively). Three patients in the TAV group experienced cardiac death (2 died suddenly and 1 after emergency surgery for AA dissection); there was no occurrence of cardiac death in the BAV group. In conclusion, the rate of progression of AA aneurysms was similar in patients with BAVs and in those with TAVs. Furthermore, patients with BAVs did not have increased rates of AA related complications compared with patients with TAVs.

Usefulness of MRI in the follow-up of patients with repaired aortic coarctation and bicuspid aortic valve

Background The long-term outcome of repaired aortic coarctation may be complicated by dilatation of the ascending aorta notably in patients with bicuspid aortic valve. Magnetic resonance imaging was used to compare the size of the ascending aorta in patients with bicuspid or tricuspid aortic valve. Methods In 50 patients with a repair of aortic coarctation, the size of the ascending aorta was measured in a bicuspid aortic valve group ( n=11) and a tricuspid aortic valve group ( n=39). The aortic diameter was measured at the level of the sinus of Valsalva and at the widest part of the ascending aorta using magnetic resonance imaging. Results The mean age of patients at surgical repair was respectively 2.2±3.3 years for the bicuspid aortic valve group and 2.5±3.5 years for the tricuspid aortic valve group ( p=NS) and the mean age at the time of the magnetic resonance imaging was 10.2±4.7 years and 9.3±5.9 years ( p=NS) respectively. A significant difference in the aortic diameter was found between the bicuspid aortic valve group and the tricuspid aortic group both at the level of sinus of Valsalva (34.8±8.2 mm, 19.5±4.4 mm, respectively, p<0.01) and at the level of the ascending aorta (36.8±7.2 mm, 16.9±3.4 mm, respectively, p<0.01). Conclusions The occurrence of ascending aortic dilatation is significantly associated with the presence of a bicuspid aortic valve. This requires long-term follow-up, which can be effectively performed by magnetic resonance imaging.

Dilatation of the aorta in pure, severe, bicuspid aortic valve stenosis

Background Aortic complications are more frequent after bicuspid aortic valve (BAV) replacement (AVR), than tricuspid aortic valve replacement. We studied the size of the proximal thoracic aorta in patients with BAV undergoing AVR for pure, severe aortic stenosis, looking for dilatation in comparison with patients with a matched tricuspid aortic valve (TAV) and normograms of aortic size. Methods Aortic root and ascending aortic diameter measurements were taken at 3 levels, from electrocardiographic-gated multidetector row computed tomograms, in 28 patients with pure, severe aortic stenosis before AVR. The patients were divided in 2 groups (BAV, n = 10; TAV, n = 18). Patients with greater than mild aortic regurgitation or who were scheduled for aortic root replacement were excluded. Results Although patients in the BAV group were younger ( P <.0001) and less likely to have hypertension ( P <.005), their aortic diameters were larger than those of patients in the TAV group at all levels measured (aortic sinus, 41.1 ± 8.1 mm vs 33.8 ± 3.3 mm; sino-tubular junction, 39.0 ± 7.8 mm vs 31.1 ± 3.8 mm; right pulmonary artery level, 42.8 ± 7.1 mm vs 33.7 ± 4.3 mm; P <.005 for all). Whereas 60% (6/10) of patients in the BAV group had ≥1 aortic diameter measurements greater than the 95th age-adjusted percentile, 0% (0/18) of patients in the TAV group did. Conclusions Patients with BAV undergoing AVR with pure, severe aortic stenosis commonly have moderate dilatation of the thoracic aorta, whereas matched patients with a TAV do not. This finding may contribute to the increased frequency of aortic complications seen in follow up of patients with a BAV after AVR.

Aortic complications after bicuspid aortic valve replacement: long-term results

Background Bicuspid aortic valve (BAV) is a risk factor for aortic dissection and aneurysm. We studied patients with BAV and tricuspid aortic valve (TAV) to evaluate long-term changes in the ascending aorta after aortic valve replacement (AVR). Patients and methods One hundred consecutive patients were allocated into two groups according to the presence of BAV (group A, 50 patients) or TAV (group B, 50 patients). Mean age was 51 ± 12 years in group A, and 50 ± years 12 in group B. No patients had hypertension or Marfan’s syndrome. Until July 2001, mean follow-up was 234 ± 47 months in group A and 241 ± 43 months in group B. Results Five patients (10%, CL 5.7 to 13.9) in group A suffered late acute aortic dissection. Acute aortic dissection (5 vs 0, p = 0.0001) and sudden death (7 vs 0, p = 0.0001) occurred more frequently in patients with BAV. All survivors were assessed by echocardiogram. The mean diameter of the ascending aorta was 48.4 mm in group A and 36.8 mm in group B. Three patients in group A were operated on because of ascending aorta aneurysm more than 6 cm in diameter. Conclusions As a result of our experience, we recommend a policy of prophylactic replacement of even a seemingly normal and definitely a mildly enlarged ascending aorta in cases of BAV at the moment of AVR, and consideration of a similar approach for any other cardiac surgical procedure in patients with BAV.