Background/Aim: In patients with bicuspid aortic valves, guidelines call for regular follow-up to monitor disease progression and guide timely intervention. We aimed to evaluate how closely these recommendations are followed at a tertiary care center. Methods: This was retrospective cohort study at a tertiary care center. Among 48,504 patients who received echocardiograms between 2013-2018, 245 patients were identified to have bicuspid aortic valve. Bivariate analyses compared patient and echocardiographic characteristics between patients who did and did not receive follow-up by a cardiovascular specialist. Results: The mean age of the cohort was 55.2  15.6 years and 30.2% were female. During a median follow-up of 3.5  2.2 years, 72.7% of patients had at least one visit with a cardiovascular specialist after diagnosis of bicuspid aortic valve by echocardiogram. Patients followed by specialists had a higher proportion of follow-up surveillance by echocardiogram (78.7% vs. 34.3%, p< .0001), or by CT or MRI (41.0% vs. 3.0%, p < .0001), and were more likely to undergo valve or aortic surgery compared with patients not followed by specialists. Patients with moderate to severe valvular or aortic pathology (aortic stenosis/regurgitation, dilated ascending aorta) were not more likely to be followed by a cardiovascular specialist or receive follow-up echocardiograms. Conclusions: Follow-up care for patients with bicuspid aortic valve was highly variable, and surveillance imaging was performed sparsely despite guidelines. There is an urgent need for surveillance and clinical follow-up mechanisms to monitor this patient population with increased risk of progressive valvulopathy and aortopathy.

Background: Bicuspid aortic valve is the most common congenital heart defect and predisposes patients to developing aortic stenosis more frequently and at a younger age than the general population. However, the influence of bicuspid aortic valve on the rate of progression of aortic stenosis remains unclear. Methods: In 236 patients (177 tricuspid aortic valve, 59 bicuspid aortic valve) matched by initial severity of mild or moderate aortic stenosis, we retrospectively analyzed baseline echocardiogram at diagnosis with latest available follow-up echocardiogram. Baseline comorbidities, annualized progression rate of hemodynamic parameters, and hazard of aortic valve replacement were compared between valve phenotypes. Results: Median echocardiographic follow-up was 2.6 (IQR 1.6-4.2) years. Patients with tricuspid aortic stenosis were significantly older with more frequent comorbid hypertension and congestive heart failure. Median annualized progression rate of mean gradient was 2.3 (IQR 0.6-5.0) mmHg/year vs. 1.5 (IQR 0.5-4.1) mmHg/year (p=0.5), and that of peak velocity was 0.14 (IQR 0-0.31) m/s/year vs. 0.10 (IQR 0.04-0.26) m/s/year (p=0.7) for tricuspid vs. bicuspid aortic valve, respectively. On multivariate analyses, bicuspid aortic valve was not significantly associated with more rapid progression of aortic stenosis. In a stepwise Cox proportional hazards model adjusted for baseline mean gradient, bicuspid aortic valve was associated with increased hazard of aortic valve replacement (HR: 1.7, 95% CI [1.0, 3.0], p=0.049). Conclusion: Bicuspid aortic valve may not significantly predispose patients to more rapid progression of mild or moderate aortic stenosis. Guidelines for echocardiographic surveillance of aortic stenosis need not be influenced by valve phenotype.

Background. Query a single institution computed tomography (CT) database to assess the prevalence of aortic arch anomalies in general adult population and their potential association with thoracic aortopathies. Methods. CT chest scan reports of patients aged 50-85 years old performed for any indication at a single health system between 2013 and 2016 were included in the analysis. Characteristics of patients with and without aortic arch anomalies were compared by t-test and Fisher exact tests. Logistic regression analysis was performed to assess for independent risk factors of thoracic aortic aneurysm. Results. Of 21,336 CT scans, 603 (2.8%) described arch anomalies. Bovine arch (n=354, 58.7%) was the most common diagnosis. Patients with arch anomalies were more likely to be female (p<0.001), non-Caucasian(p<0.001), and hypertensive (p<0.001). Prevalence of thoracic aortic aneurysm in arch anomalies group was 10.8% (n=65) compared to 4.1% (n=844) in the non-arch anomaly cohort (p<0.001). The highest prevalence of thoracic aneurysm was associated with right-sided arch combined with aberrant left subclavian configuration (33%), followed by bovine arch (13%), and aberrant right subclavian artery (8.2%). On binary logistic regression, arch anomaly (OR=2.85 [2.16-3.75]), aortic valve pathology (OR 2.93 [2.31-3.73]), male sex (OR 2.38 [2.01-2.80]), and hypertension (OR 1.47 [1.25-1.73]) were significantly associated with increased risk of thoracic aneurysm disease. Conclusions. Reported prevalence of aortic arch anomalies by CT imaging in the older adult population is ~3%, with high association of thoracic aortic aneurysm (OR=2.85) incidence in this subgroup. This may warrant a more tailored surveillance strategy for aneurysm disease in this subpopulation.

Objective: To assess the impact of surgeon experience on the outcomes of degenerative mitral valve disease. Methods: We reviewed all patients who had surgery for degenerative mitral valve disease between 2011-2016. Experienced surgeon was defined as performing  25 mitral valve operations/year. Patient characteristics and outcomes were compared. Multivariable analysis was performed to identify factors associated with MR recurrence. Survival analysis for mortality was done using Kaplan Meier curve and Cox proportional Hazard method. Results: There were 575 patients treated by 9 surgeons for severe mitral regurgitation caused by degenerative mitral valve disease between 2011-2016. Three experienced surgeons performed 77.2% of the operations. Patients treated by less experienced surgeons had worse comorbidity profile and were more likely to have an urgent or emergent operation (P=0.001). Experienced surgeons were more likely to attempt repair (P=0.024), to succeed in repair (94.7% vs 87%, P=0.001), had shorter cross-clamp times (P=0.001), and achieved higher repair rate (81.3% vs 69.7%, P=0.005). Experienced surgeons were more likely to use neochordae (P=0.001) and less likely to use chordae transfer (P=0.001). Surgeon experience was not associated with recurrence (moderate or higher MR) within the first two years after surgery but was an independent risk factor for mortality (HR= 2.64, P=0.002). Conclusions: Techniques of degenerative mitral valve surgery differ with surgeon experience, with higher rates of repair and better outcomes associated with more experienced surgeons.

Objective: Reports of prevalence and clinical significance of bicuspid aortic valve (BAV) disease are variable. We assessed our institutional echocardiography (ECHO) database to understand the reported prevalence of BAV and its potential association with thoracic aortic aneurysm disease (TAAD). Methods: All ECHOs of adult patients (>18 years) performed at a single institution between calendar year 2013 to 2018 were reviewed. BAV patients were categorized by age group (Young age:18-39 years; Middle age:40-65 years; Old age: >65years) to assess for aortic valvulopathy and TAAD. Logistic regression analysis was performed to understand association of BAV with TAAD. Results: Of 48,503 unique patient ECHOs, 245 (0.51%) described a diagnosis of BAV, with 93(40%) concomitant TAAD. Increased association with endocarditis (p=0.01) and severe aortic insufficiency (p=0.005) was seen in the Young group. Ascending aortic diameter was significantly higher in the Middle compared to the Young group (p<0.001), but similar to Old group. On multivariable regression, BSA(OR=7.31(2.27-23.57)) and age (OR=1.02(1.00-1.04)), but not BAV dysfunction (OR1.07(0.51-2.26)) were associated with TAAD. Conclusions: In this large cross-sectional ECHO study, reported BAV prevalence was 0.51%. We found high association of BAV with concomitant TAAD especially in patients greater than 40 years of age. This suggests that more frequent aortic surveillance may be warranted in the middle and old age BAV subjects.