Prevalence and Outcomes of Bicuspid Aortic Valve in Patients With Aneurysmal Sub-Arachnoid Hemorrhage: A Prospective Neurology Registry Report.

Background Intracranial aneurysms are reported in 6%-10% of patients with bicuspid aortic valve (BAV), and routine intracranial aneurysm surveillance has been advocated by some. We assessed the prevalence and features of the most important patient-outcome: aneurysmal sub-arachnoid hemorrhage (aSAH), as compared with controls without aSAH, and tricuspid aortic valve (TAV) with aSAH. Methods and Results Adult patients with accurate diagnosis of aSAH and at least one echocardiogram between 2000 and 2019 were identified from a consecutive prospectively maintained registry of aSAH admissions. Controls without a diagnosis of SAH were age- and sex-matched. BAV prevalence was confirmed echocardiographically. Severity of aSAH was categorized using modified Fisher and World Federation of Neurological Scale. Neurologic outcome was assessed using modified Rankin score. A total 488 aSAH cases and 990 controls were identified and BAV status was confirmed. Prevalence of BAV in patients with aSAH was 1.2% (6/488) versus 3.5% (35/990) in controls, P=0.01. BAV+aSAH were noted to be younger than TAV+aSAH (56±11 versus 68±14; P=0.03) with smaller aneurysms (5±2 versus 7±4; P=0.31). The severity of aSAH was lesser in BAV+aSAH than TAV (modified Fisher grade>2 50% versus 74%; P=0.19, World Federation of Neurological Scale grade>3 17% versus 36%; P=0.43). BAV+aSAH had less severe neurologic disability (modified Rankin score 3%-6 33% versus 49% in TAV; P=0.44) and comparable in-hospital mortality rates (P=0.93). BAV had lower odds for aSAH on multivariate analysis (odds ratio 0.23[CI 0.08-0.65]; P=0.01). Conclusions Prevalence of BAV was 3 times lower in the aSAH registry than in controls without aSAH. BAV+aSAH had clinically smaller aneurysms, clinically smaller bleeds, and better neurologic outcome as compared with TAV+aSAH, which needs to be confirmed in larger studies. These findings argue against routine surveillance for intracranial aneurysms in patients with BAV without aortic coarctation.

Aortic Stenosis Progression, Cardiac Damage, and Survival: Comparison Between Bicuspid and Tricuspid Aortic Valves.

OBJECTIVES: This study sought to compare aortic stenosis (AS) progression rates, AS-related cardiac damage (AS-CD) indicator incidence and determinants, and survival between patients with tricuspid aortic valve (TAV)-AS and those with bicuspid aortic valve (BAV)-AS. BACKGROUND: Differences in AS progression and AS-CD between patients with BAV and patients with TAV are unknown. METHODS: We retrospectively studied consecutive patients with baseline peak aortic valve velocity (peakV) ≥2.5 m/s and left ventricular ejection fraction ≥50%. Follow-up echocardiograms (n = 4,818) provided multiparametric AS progression rates and AS-CD. RESULTS: The study included 330 BAV (age 54 ± 14 years) and 581 patients with TAV (age 72 ± 11 years). At last echocardiogram (median: 5.9 years; interquartile range: 3.9 to 8.5 years), BAV-AS exhibited similar peakV and mean pressure gradient (MPG) as TAV-AS, but larger calculated aortic valve area due to larger aortic annulus (p < 0.0001). Multiparametric progression rates were similar between BAV-AS and TAV-AS (all p ≥ 0.08) and did not predict age-/sex-adjusted survival (p ≥ 0.45). Independent determinants of rapid progression were male sex and baseline AS severity for TAV (all p ≤ 0.024), and age, baseline AS severity, and cardiac risk factors (age interaction: p = 0.02) for BAV (all p ≤ 0.005). At 12 years, patients with TAV-AS had a higher incidence of AS-CD than BAV-AS patients (p < 0.0001), resulting in significantly worse survival compared to BAV-AS (p < 0.0001). AS-CD were independently determined by multiple factors (MPG, age, sex, comorbidities, cardiac function; all p ≤ 0.039), and BAV was independently protective of most AS-CD (all p ≤ 0.05). CONCLUSIONS: In this cohort, TAV-AS and BAV-AS progression rates were similar. Rapid progression did not affect survival and was determined by cardiac risk factors for BAV-AS (particularly in patients with BAV <60 years of age) and unmodifiable factors for TAV-AS. AS-CD and mortality were significantly higher in TAV-AS. Independent determinants of AS-CD were multifactorial, and BAV morphology was AS-CD protective. Therefore, the totality of AS burden (cardiac damage) is clinically crucial for TAV-AS, whereas attention to modifiable risk factors may be preventive for BAV-AS.

Prognostic Risk Stratification of Patients with Moderate Aortic Stenosis.

BACKGROUND: Currently risk stratification of moderate aortic stenosis (AS) is still incipient. The aim of this study was to identify prognostic factors in patients with moderate AS. METHODS: The prognosis of patients with moderate AS (1 < aortic valve area ≤ 1.5 cm2) stratified by left ventricular ejection fraction (LVEF; 50%), stroke volume index (SVI; 35 mL/m2), and elevated E/e' ratio (average, 14) was compared with that of the age- and sex-matched general population. RESULTS: Of 696 patients (median age, 77 years; aortic valve area 1.3 cm2; 57% men), 279 (40%) died during a median follow-up period of 3.4 years. Mortality was higher in patients with moderate AS than reference (mortality ratio, 2.43; 95% CI, 2.17-2.72). LVEF < 50%, SVI < 35 mL/m2, and elevated E/e' ratio were present in 113 (17%), 54 (8%), and 330 (54%) patients; mortality ratios were 3.89 (95% CI, 3.07-4.85), 6.40 (95% CI, 4.57-8.71), and 2.58 (95% CI, 2.21-3.00), respectively. Even if LVEF or SVI was preserved, the mortality ratio was more than twice than reference (P < .001), but elevated E/e' ratio could discriminate additional patients at higher risk (hazard ratio [HR], 2.71; 95% CI, 1.88-3.91). Two hundred one patients (29%) underwent aortic valve replacement at a median of 2.3 years after the diagnosis of moderate AS. LVEF < 50% (HR, 2.98; 95% CI, 1.39-6.56), SVI < 35 mL/m2 (HR, 3.34; 95% CI, 1.02-10.90) and elevated E/e' ratio (HR, 2.73; 95% CI, 1.26-5.94) were all associated with worse prognosis even if aortic valve replacement was performed. CONCLUSIONS: In patients with moderate AS, those with decreased LVEF and/or SVI are at high risk. Even if these parameters are preserved, patients with elevated E/e' ratios are at intermediate risk. Further investigation is warranted to assess whether earlier intervention could improve outcomes and reduced cardiac-related death among patients at high and intermediate risk.

Coronary Artery Disease in Adults With Coarctation of Aorta: Incidence, Risk Factors, and Outcomes.

Background Premature coronary artery disease ( CAD ) is common in patients with coarctation of aorta ( COA ), but there are limited data about any direct relationship (or lack thereof) between COA and CAD . We hypothesized that atherosclerotic cardiovascular disease risk factors, rather than COA diagnosis, was the primary determinant of CAD occurrence in patients with COA . Methods and Results This is a retrospective study of 654 COA patients and a control group of 876 patients with valvular pulmonic stenosis and tetralogy of Fallot to determine prevalence and independent risk factors for CAD . There was no evidence of a difference in the unadjusted CAD prevalence between the COA and control groups (7.8% versus 6.3%, P=0.247), but premature CAD was more common in COA patients (4.4% versus 1.8%, P=0.002). In the analysis of a propensity-matched cohort of 126 COA and 126 control patients, there was no evidence of a difference in overall CAD prevalence (6.3% versus 5.6% versus P=0.742) and premature CAD prevalence (4.8% versus 3.2%, P=0.518). The multivariable risk factors for CAD were hypertension (odds ratio [ OR ] 2.14; 95% CI 1.36-3.38), hyperlipidemia ( OR 3.33; 95% CI 2.02-5.47), diabetes mellitus ( OR 1.98; 95% CI 1.31-3.61), male sex ( OR 2.05; 95% CI 1.33-3.17), and older age per year ( OR 1.06; 95% CI 1.04-1.07). Conclusions After adjusting for atherosclerotic cardiovascular disease risk factors, we did not find evidence of a difference in CAD risk between the patients with COA and other patients with congenital heart disease.

Disparities in Cardiac Care of Women: Current Data and Possible Solutions.

PURPOSE OF REVIEW: Cardiovascular disease remains the leading cause of death in women. The goal of this review is to address known disparities in cardiovascular care with regard to diagnosis and treatment of heart disease in women. RECENT FINDINGS: Gender-specific differences in regard to the incidence, treatment, and outcomes of common cardiovascular pathology are increasingly recognized. Particular attention to ischemic heart disease, arrhythmia, congestive heart failure, and structural heart disease are reviewed in this article. There is a clear racial and ethnic discrepancy among women which is particularly concerning with a progressively diverse patient population. Medical and surgical treatment differences between men and women must be addressed by providers in order to optimize long-term outcomes among all patients. Understanding the unique cardiovascular risk profile and barriers to optimal treatment outcomes in women is imperative to eliminate the current disparities in cardiovascular disease.

Role of diastolic function indices in the risk stratification of patients with mixed aortic valve disease.

Aims: Determine the role of diastolic function indices in pre-operative and post-operative risk stratification in patients with moderate mixed aortic valve disease (MAVD). Methods and results: A retrospective study was conducted of asymptomatic patients with moderate MAVD (a combination of moderate aortic stenosis and moderate aortic regurgitation) and an ejection fraction of 50% or more who were followed up at Mayo Clinic from 1 January 2004, to 31 December 2013. A pre-requisite for inclusion in the study was assessment of diastolic function involving at least three of the following indices: tissue Doppler early diastolic velocity (e'), mitral inflow early velocity (E), tricuspid regurgitation velocity, and left atrial volume index. Primary endpoints were aortic valve replacement (AVR) or cardiac death while secondary endpoints were cardiovascular adverse events (CAEs) after AVR. We defined CAEs as stroke, heart failure hospitalization, severe left ventricular dysfunction, and cardiac death. There were 214 patients (age 61 ± 8 years, men 146 [68%]) followed for 6.1 ± 2.3 years during which 162 (76%) AVRs and 11 (5%) cardiac deaths occurred. The multivariable risk factors for cardiac death or AVR were relative wall thickness (RWT) > 0.42 [hazard ratio (HR), 1.88 [95% CI, 1.28-2.59]; P = 0.001] and average E/e' >14 (HR, 1.94 [95% CI, 1.29-3.01]; P = 0.02). Freedom from CAE after AVR was significantly lower in the patients with baseline RWT >0.42 or mean E/e' >14 than the other patients: 79% (95% CI 74-83%) vs. 94% (95% CI 89-98%) at 3 years (P = 0.03). Conclusion: The presence of RWT >0.42 or E/e' >14 identifies a high-risk patient subset whose risk for cardiovascular morbidities persists even after AVR.

Early Prosthetic Valve Dysfunction Due to Bioprosthetic Valve Thrombosis: The Role of Echocardiography.

OBJECTIVES: The purpose of this study was to review the institutional practice of surveillance transthoracic echocardiography (TTE) for diagnosing early prosthetic valve dysfunction (PVD). BACKGROUND: Bioprosthetic valve thrombosis (BPVT) is an important cause of PVD, and guidelines do not recommend routine TTE during the first 5 years after valve implantation. METHODS: The authors performed a retrospective case-control study of all suspected (imaging diagnosis) or confirmed (histopathological diagnosis) cases of BPVT from January 1997 through December 2016. Patients were matched 1:2 (age, sex, prosthesis position) to patients whose prostheses were explanted because of structural failure (SF). PVD was defined as a 50% increase above baseline gradient at valve implantation and classified as early (≤5 years) or late (>5 years) after implantation. RESULTS: There were 94 BPVT (51 suspected, 43 confirmed) and 188 SF cases; patient age 61 ± 9 years; men 61 (65%). The prosthesis positions were aortic 56%; mitral 26%; tricuspid 15%; and pulmonary 3%. Early PVD was more common in the BPVT versus SF group: 83 of 94 (88%) versus 20 of 188 (11%) (p < 0.001). Time from implantation to PVD was shorter for BPVT than SF: 26 months (interquartile range [IQR]: 12 to 43 months) versus 74 months (IQR: 48 to 102 months) (p < 0.001). At the initial PVD diagnosis, 81% of BPVT and 90% of SF patients were asymptomatic. However, BPVT patients had rapid symptomatic deterioration, requiring intervention sooner after PVD diagnosis: 6 months (IQR: 4 to 7 months) versus 51 months (IQR: 22 to 55 months) (p < 0.001). CONCLUSIONS: Most patients with PVD due to BPVT were asymptomatic at initial diagnosis, which was made based on routine surveillance TTE, often performed before 5 years. BPVT, an acute disease process, requires timely diagnosis because patient conditions rapidly deteriorate. Further studies are needed to determine whether routine surveillance TTE should be considered for patients with bioprosthetic valves to identify pre-symptomatic features of BPVT in order to provide effective, appropriate therapy.

Functional assessment of anomalous right coronary artery using fractional flow reserve: An Innovative Modality to Guide Patient Management.

Anomalous origin of a coronary artery is a recognized cause of sudden cardiac death (SCD). To date, there is no standard test to predict which patients are at increased risk for SCD. Fractional flow reserve (FFR) is an invasive technique used to qualify focal obstructive coronary lesions. We present a case where FFR was used to guide therapy in a young patient with anomalous right coronary artery (ARCA) when standard noninvasive testing showed ischemic discrepancy. © 2016 Wiley Periodicals, Inc.