Tiong Cheng Yeo

Clinical profile and outcome in 52 patients with cardiac pseudoaneurysm

Cardiac pseudoaneurysm is defined as a rupture of the myocardium that is contained by pericardial adhesions or the epicardial wall [1, 2]. Myocardial rupture directly causing death reportedly occurs in 7% to 10% of patients after acute myocardial infarction [3-5]; pseudoaneurysm, a contained rupture, is reportedly a rare complication of myocardial infarction. Pseudoaneurysm can also occur after cardiac surgery, chest trauma, and endocarditis [6-18]. The wall of a pseudoaneurysm consists of fibrous tissue and lacks the structural elements found in a normal cardiac wall. An important clinical feature of pseudoaneurysms is their reported propensity for further rupture [2, 6, 19] and fatal outcome [2, 6, 19-21]. Because of these observations, early surgery is recommended even for asymptomatic patients [6-810, 22, 23]. Knowledge of the clinical profile and natural history of cardiac pseudoaneurysm is limited because of its low incidence. The literature has concentrated predominantly on the cause, pathogenesis, clinical presentation, and surgical outcome of pseudoaneurysms and case reports, collections of case reports, and small patient series are overrepresented. No large clinical experience from a single institution has been reported that describes the diagnosis, clinical profile, and outcome of pseudoaneurysms. We reviewed the clinical profiles and outcomes of 52 consecutive patients with pseudoaneurysm that was diagnosed before death at the Mayo Clinic in Rochester, Minnesota; Scottsdale, Arizona; and Jacksonville, Florida, between January 1980 and September 1996. Methods Patients The echocardiographic, surgical, and medical databases of the Mayo Clinic were searched for patients with pseudoaneurysm diagnosed between January 1980 and September 1996. The diagnosis was based on typical morphologic features shown by each imaging method, including echocardiography [23-32], angiography [33], computed tomography [34-36], and magnetic resonance imaging [23, 37]. Surgical or pathologic confirmation (or both) was available in 42 patients (81%). Although some authors have reported that pseudoaneurysm can occur as a complication of infectious endocarditis, especially of the aortic valve [6, 11], we decided not to include this subset of patients because of the difficulty of differentiating pseudoaneurysm from subannular abscess, even at surgery [7]. After identifying the study group, we reviewed the clinical records, which contained patient demographic characteristics, clinical presentation, results of surgical repair, and vital status at latest follow-up. Follow-up was obtained from the Mayo Clinic records and was supplemented by direct mail and telephone contact. Cause of death was determined on the basis of clinical records, death certificates, and correspondence. Diagnosis of Pseudoaneurysm The typical two-dimensional echocardiographic features of pseudoaneurysm [23-25] include a relatively narrow neck in comparison with the diameter of the aneurysm (Figure 1) and sharp discontinuity of the endocardium at the site at which the aneurysm communicates with the left ventricle. Features of the left ventricle on contrast angiography [33] include a narrow communication between the aneurysm and the left ventricular cavity and a paucity of coronary vessels in the vicinity of the pseudoaneurysm (Figure 2). Pseudoaneurysm was diagnosed on computed tomographic scans (Figure 3) if the myocardial wall ended abruptly at the border of the aneurysm [34-36]. Diagnosis made by magnetic resonance imaging was based on the presence of an aneurysm rimmed only by pericardium with a low signal [37]. Figure 1. Two-dimensional apical four-chamber view of pseudoaneurysm of lateral wall of left ventricle (LV). Figure 2. Right anterior oblique view of left ventricular angiogram showing pseudoaneurysm (arrowheads) in diastole (top) and systole (bottom). Figure 3. Computed tomographic scan of the patient whose pseudoaneurysm is shown in Statistical Analysis Continuous variables are expressed as means SDs. The small sample size prohibited informative statistical comparison between groups of patients. Results Demographic and Clinical Features Using the standard imaging features described in the Methods section, we identified 52 patients with pseudoaneurysm: Thirty-four (65%) were male, and the mean age was 48 28 years (range, 0.5 to 84 years). The pseudoaneurysm was discovered incidentally in 25 asymptomatic patients (48%). Cardiac imaging was performed in 13 of these 25 patients after staged repair of complex congenital heart disease, in 7 patients during follow-up after valve operations, and in 5 patients because of an abnormal electrocardiogram obtained while patients were at rest. Four patients (8%) presented acutely: 3 with acute myocardial infarction and 1 with cardiac tamponade. Other clinical presentations were congestive heart failure in 8 patients (15%), chest pain in 7 (13%), syncope or arrhythmia in 5 (10%), and systemic embolism in 3 (6%). Thirteen patients (25%) had a history of hypertension. Diagnostic Methods and Location of Pseudoaneurysm Pseudoaneurysms were imaged and diagnosed with two-dimensional Doppler echocardiography (38 patients), cardiac catheterization (33 patients), magnetic resonance imaging (5 patients), and computed tomography (4 patients). However, the diagnosis was initially made with two-dimensional echocardiography in 32 patients, cardiac catheterization in 12, magnetic resonance imaging in 4, and computed tomography in 2. In 2 other patients, the diagnosis was made intraoperatively during staged repair of tetralogy of Fallot and repeated aortic valve replacement. The median interval between diagnosis and previous cardiac surgery or myocardial infarction was 9.1 months (range, 0 to 15 years) in all patients, 8.3 months in surgically treated patients, and 13.6 months in medically treated patients. The location of pseudoaneurysms was related to their cause: After myocardial infarction, pseudoaneurysms were located in the inferior or posterolateral wall in 18 of 22 patients (82%); after surgery for complex congenital heart disease, they were located in the right ventricular outflow tract in 13 of 15 patients (87%); after mitral valve replacement, they were located in the posterior subannular region of the mitral valve in 4 of 4 patients (100%); and after aortic valve replacement, they were located in the subaortic region in 3 of 3 patients (100%). Cause As shown in Table 1, pseudoaneurysm in our series was caused by cardiac surgery (30 patients [58%]) and myocardial infarction (22 patients [42%]). Table 1. Cause of Cardiac Pseudoaneurysm Follow-Up and Outcome Complete follow-up to September 1996 was available for 48 patients (92%); the median duration of follow-up was 4 years (range, 3 days to 16.6 years). One patient was lost to follow-up after 14.8 months, 1 was lost after 4 years, 1 was lost after 11.8 years, and 1 was lost after 16.4 years. Forty-two patients (81%) had either elective (38 patients [90%]) or emergent (4 patients [10%]) surgical repair (surgical group); the overall surgical mortality rate was 7% (3 deaths). Surgical or pathologic confirmation (or both) was available for all 42 patients who had surgical repair. The surgical mortality rate was higher among patients who had had myocardial infarction (13% [2 of 16] compared with 4% [1 of 26]). Ten patients (19%) did not have surgery (medical group) for the following reasons: associated medical conditions (4 patients), small pseudoaneurysm (3 patients), and patient refusal to undergo surgery (3 patients). Six of these 10 patients had a pseudoaneurysm after myocardial infarction, and 4 had a pseudoaneurysm after cardiac surgery. Nineteen patients died after a median survival of 2.3 years (range, 3 days to 8.2 years) after diagnosis of pseudoaneurysm. Of these 19 patients, 13 were from the surgical group; the causes of death were noncardiac in 5 patients, congestive heart failure in 3, myocardial infarction in 3, and documented ventricular tachycardia in 2. Six medically treated patients died after a median survival of 2.1 years (range, 11 days to 4.7 years) after diagnosis: Three died of noncardiac causes, 2 died of congestive heart failure, and 1 died of acute myocardial infarction. No further cardiac rupture was documented. The overall survival rate at 2 years was 63%. Table 2 shows the clinical characteristics and outcomes of patients in the two treatment groups. Table 2. Clinical Characteristics and Outcomes of 52 Patients with Cardiac Pseudoaneurysm Treated Medically or Surgically Discussion Cardiac rupture is usually fatal and accounts for 7% to 10% of early deaths after acute myocardial infarction [3-5]. In most cases, the cardiac wall ruptures into the pericardial cavity and causes cardiac tamponade and death. Contained rupture of the heart is recognized less frequently than cardiac rupture, and its incidence is unknown. Contained rupture has been attributed to pericardial adhesions in the area of rupture or a slow extracardiac leak that results in pericardial inflammation and adhesions [8]. Thus, an expanding intrapericardial hematoma, or pseudoaneurysm, is formed. The wall of the pseudoaneurysm consists of fibrous tissue and pericardium and lacks any component of the cardiac wall. Various reports suggest that such a contained rupture has a greater propensity for rupture than a true aneurysm, whose wall contains myocardium [2, 6, 19]. Information on the clinical course of pseudoaneurysms is gleaned from many reports of small series [1, 2, 6-40], and no large clinical series from a single institution has been reported. Acute rupture, surgical survival, and exceptional survival are overrepresented in patients with pseudoaneurysm. To our knowledge, our report describes the largest clinical series of patients with pseudoaneurysm from a single institution. Clinical Presentation The diagnosis of pseudoaneurysm is rarely suggested by clinical signs and sy

New Set of Intravascular Ultrasound-Derived Anatomic Criteria for Defining Functionally Significant Stenoses in Small Coronary Arteries (Results from Intravascular Ultrasound Diagnostic Evaluation of Atherosclerosis in Singapore (IDEAS) Study)

We sought to determine the intravascular ultrasound-derived anatomic criteria for functionally significant lesions in small coronary arteries with a reference segment diameter <3 mm. A fractional flow reserve (FFR) of <0.75, as determined by pressure wire using high-dose (100 to 150 microg) intracoronary adenosine, was used as the reference standard for functional significance. For the 94 patients/lesions involved in the present study, the average reference vessel diameter was 2.72 mm. The FFR was <0.75 in 38 patients (40.4%) and > or =0.75 in 56 patients (59.6%). Logistic regression analysis identified the minimal lumen area, plaque burden, and lesion length as the 3 most important determinants of the FFR. Using classification and regression tree analysis, the best cutoff values for these determinants to discriminate a FFR of <0.75 versus > or =0.75 were a minimal lumen area of < or =2.0 mm(2) (sensitivity 82.35%, specificity 80.77%), plaque burden of > or =80% (sensitivity 87.9%, specificity 78.9%), and lesion length of > or =20 mm (sensitivity 63.6%, specificity 78.9%). A significant increase was found in the area under the receiver operating characteristic curve of the combined parameters (minimal lumen area plus plaque burden plus lesion length) compared to the plaque burden (p = 0.014) and other individual parameters (p <0.001). In conclusion, we found that intravascular ultrasound-derived anatomic criteria are able to predict the functional significance of intermediate lesions in small coronary arteries. A minimal lumen area of < or =2.0 mm(2), plaque burden of > or =80%, and lesion length of > or =20 mm predicted a FFR of <0.75 with good sensitivity and specificity.

Severe obstructive sleep apnea and outcomes following myocardial infarction.

STUDY OBJECTIVE We sought to determine the effect of severe obstructive sleep apnea (OSA) on long-term outcomes after myocardial infarction. We hypothesized that severe OSA was associated with lower event-free survival rate after ST-segment elevation myocardial infarction (STEMI). METHODS A total of 120 patients underwent an overnight sleep study during index admission for STEMI. Severe OSA was defined as apnea hypopnea index (AHI) ≥ 30, and non-severe OSA defined as AHI < 30. RESULTS Among the 105 patients who completed the study, 44 (42%) had severe OSA and 61 (58%) non-severe OSA. The median creatine kinase level and mean left ventricular systolic function were similar between the 2 groups. None of the 105 study patients had received treatments for OSA. Between 1- and 18-month follow-up, the severe OSA group incurred 1 death, 2 reinfarctions, 1 stroke, 6 unplanned target vessel revascularizations, and 1 heart failure hospitalization. In contrast, there were only 2 unplanned target vessel revascularizations in the non-severe OSA group. The incidence of major adverse events was significantly higher in the severe OSA group (15.9% versus 3.3%, adjusted hazard ratios: 5.36, 95% CI: 1.01 to 28.53, p = 0.049). Kaplan-Meier event-free survival curves showed the event-free survival rates in the severe OSA group was significantly worse than that in the non-severe OSA group (p = 0.021, log-rank test). CONCLUSION 42% of the patients admitted with STEMI have undiagnosed severe OSA. Severe OSA carries a negative prognostic impact for this group of patients. It is associated with a lower event-free survival rate at 18-month follow-up.

Recalibration of the Global Registry of Acute Coronary Events risk score in a multiethnic Asian population

BACKGROUND Acute myocardial infarction (AMI) is a leading cause of mortality in Asia. However, quantitative risk scores to predict mortality after AMI were developed without the participation of Asian countries. METHODS We evaluated the performance of the Global Registry of Acute Coronary Events (GRACE) in-hospital mortality risk score, directly and after recalibration, in a large Singaporean cohort representing 3 major Asian ethnicities. RESULTS The GRACE cohort included 11,389 patients, predominantly of European descent, hospitalized for AMI or unstable angina from 2002 to 2003. The Singapore cohort included 10,100 Chinese, 3,005 Malay, and 2,046 Indian patients hospitalized for AMI from 2002 to 2005.Using the original GRACE score, predicted in-hospital mortality was 2.4% (Chinese), 2.0% (Malay), and 1.6% (Indian). However, observed in-hospital mortality was much greater at 9.8% (Chinese), 7.6% (Malay), and 6.4% (Indian). The c statistic for Chinese, Malays, and Indians was 0.86, 0.86, and 0.84, respectively, and the Hosmer-Lemeshow statistic was 250, 56, and 41, respectively. Recalibration of the GRACE score, using the mean-centered constants derived from the Singapore cohort, did not change the c statistic but substantially improved the Hosmer-Lemeshow statistic to 90, 24, and 18, respectively. The recalibrated GRACE score predicted in-hospital mortality as follows: 7.7% (Chinese), 6.0% (Malay), and 5.2% (Indian). CONCLUSION In this large cohort of 3 major Asian ethnicities, the original GRACE score, derived from populations outside Asia, underestimated in-hospital mortality after AMI. Recalibration improved risk estimation substantially and may help adapt externally developed risk scores for local practice.

Left atrial volume is an independent predictor of exercise capacity in patients with isolated left ventricular diastolic dysfunction

Left atrial (LA) volume reflects left ventricular (LV) diastolic properties. The latter is an important determinant of exercise capacity in patients with normal LV systolic function. We hypothesized that LA volume predicts exercise capacity in patients with isolated LV diastolic dysfunction. Echocardiography and treadmill exercise testing were performed in 256 patients with normal LV systolic function (ejection fraction≥50%). Diastolic dysfunction was defined using standard Doppler criteria. LA volume was measured using the ellipsoid method and indexed to the body surface area. 119 patients had LV diastolic dysfunction. They had higher indexed maximum LA volume (LA Vol(max), p=0.004) and lower exercise capacity (p<0.001). Univariate predictors of exercise capacity were age, mitral E/A, E wave deceleration time, ratio of early diastolic mitral inflow velocity to early diastolic mitral annular velocity (E/Ea), and indexed LA Vol(max). On multivariate analysis, only age, mitral E/A and indexed LA Vol(max) were independent predictors of exercise capacity. Notably, the combination of LV diastolic dysfunction and enlarged LA Vol(max) predicted worse exercise intolerance. In conclusion, indexed LA volume is an independent and reliable predictor of exercise capacity in patients with isolated LV diastolic dysfunction.

Differences in late cardiovascular mortality following acute myocardial infarction in three major Asian ethnic groups

Aim: the purpose of this study was to investigate differences in long-term mortality following acute myocardial infarction (AMI) in patients from three major ethnicities of Asia. Methods and results: We studied 15,151 patients hospitalized for AMI with a median follow-up of 7.3 years (maximum 12 years) in six publicly-funded hospitals in Singapore from 2000–2005. Overall and cause-specific cardiovascular (CV) mortality until 2012 were compared among three major ethnic groups that represent large parts of Asia: Chinese, Malay and Indian. Relative survival of all three ethnic groups was compared with a contemporaneous background reference population using the relative survival ratio (RSR) method. The median global registry of acute coronary events score was highest among Chinese, followed by Malay and Indians: 144 (25th percentile 119, 75th percentile 173), 138 (115, 167), and 131 (109, 160), respectively, p<0.0001; similarly, in-hospital mortality was highest among Chinese (9.8%) followed by Malay (7.6%) and Indian (6.4%) patients. In contrast, 12-year overall and cause-specific CV mortality was highest among Malay (46.2 and 32.0%) followed by Chinese (43.0 and 27.0%) and Indian (35.9 and 25.2%) patients, p<0.0001. The five-year RSR was lowest among Malay (RSR 0.69) followed by Chinese (RSR 0.73) and Indian (RSR 0.79) patients, compared with a background reference population (RSR 1.00). Conclusions: We observed strong inter-Asian ethnic disparities in long-term mortality after AMI. Malay patients had the most discordant relationship between baseline risk and long-term mortality. Intensified interventions targeting Malay patients as a high-risk group are necessary to reduce disparities in long-term outcomes.

Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy

Importance Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality. Objective To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality). Design, Setting, and Participants In this large international multicenter registry of patients with BAV treated at tertiary referral centers, 2118 patients with BAV were evaluated. Patients referred for echocardiography from June 1, 1991, through November 31, 2015, were included in the study. Exposures Clinical and echocardiographic data were analyzed retrospectively. The morphologic BAV findings were categorized according to the Sievers and Schmidtke classification. Aortic valve function was divided into normal, regurgitation, or stenosis. Patterns of BAV aortopathy included the following: type 1, dilation of the ascending aorta and aortic root; type 2, isolated dilation of the ascending aorta; and type 3, isolated dilation of the sinus of Valsalva and/or sinotubular junction. Main Outcomes and Measures Association between the presence and location of raphe and the risk of significant (moderate and severe) aortic valve dysfunction and aortic dilation and/or dissection. Results Of the 2118 patients (mean [SD] age, 47 [18] years; 1525 [72.0%] male), 1881 (88.8%) had BAV with fusion raphe, whereas 237 (11.2%) had BAV without raphe. Bicuspid aortic valves with raphe had a significantly higher prevalence of valve dysfunction, with a significantly higher frequency of aortic regurgitation (622 [33.1%] vs 57 [24.1%], P < .001) and aortic stenosis (728 [38.7%] vs 51 [21.5%], P < .001). Furthermore, aortic valve replacement event rates were significantly higher among patients with BAV with raphe (364 [19.9%] at 1 year, 393 [21.4%] at 2 years, and 447 [24.4%] at 5 years) vs patients without raphe (30 [14.0%] at 1 year, 32 [15.0%] at 2 years, and 40 [18.0%] at 5 years) (P = .02). In addition, the all-cause mortality event rates were significantly higher among patients with BAV with raphe (77 [5.1%] at 1 year, 87 [6.2%] at 2 years, and 110 [9.5%] at 5 years) vs patients without raphe (2 [1.8%] at 1 year, 3 [3.0%] at 2 years, and 5 [4.4%] at 5 years) (P = .03). However, on multivariable analysis, the presence of raphe was not significantly associated with all-cause mortality. Conclusions and Relevance In this large multicenter, international BAV registry, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation. The presence of raphe was also associated with increased rates of aortic valve and aortic surgery. Although patients with BAV and raphe had higher mortality rates than patients without, the presence of a raphe was not independently associated with increased all-cause mortality.

Prognostic value of left atrial size in chronic kidney disease

AIMS Patients with chronic kidney disease (CKD) have high cardiovascular risk. Although stress imaging provides accurate risk estimation in this population, it is unknown if combinatorial cardiac imaging adds incremental value. METHODS AND RESULTS We performed transthoracic echocardiography and stress single photon emission computed tomography (SPECT) to assess their value in predicting late cardiovascular disease (CVD) mortality in 200 patients with creatinine clearance <60 mL/min without a history of coronary heart disease. During a median follow-up duration of 3.7 (3.5-4.0) years, there were 25 deaths because of CVD. Older age, abnormal SPECT, and increased indexed left atrial (LA) diameter were associated with CVD mortality on univariate analysis with P = 0.007, 0.01, and 0.004, respectively. In multivariable analysis, indexed LA diameter >24 mm/m(2) was independently predictive of CVD mortality [hazard ratio (HR) 2.75, confidence interval (CI) 1.14-6.59], but abnormal SPECT was not. Each mm/m(2) increase in indexed atrial diameter was associated with an HR 1.20 (95% CI 1.06-1.37). CONCLUSIONS In patients with CKD, the indexed LA diameter predicts CVD mortality independent of an abnormal SPECT result. Consideration should be given to this simple measurement as a prognostic tool in this population.

Visit-to-visit variability in LDL- and HDL-cholesterol is associated with adverse events after ST-segment elevation myocardial infarction: A 5-year follow-up study

INTRODUCTION We evaluated the relationship between visit-to-visit low-density lipoprotein cholesterol (LDL-C) and high-density lipoprotein cholesterol (HDL-C) variability and 5-year clinical outcomes in patients who presented with ST-segment elevation myocardial infarction (STEMI). METHODS 130 patients presenting with STEMI and surviving to discharge were analyzed. Visit-to-visit LDL-C and HDL-C variability was evaluated from 2 months after discharge on the basis of corrected variation independent of mean (cVIM, primary measure), coefficient of variation and standard deviation. Major adverse cardiac event (MACE) included death, myocardial infarction, stroke, unplanned revascularization, and heart failure admission. RESULTS After an average of 62.4 ± 30.5 months follow-up, 41 patients (31.5%) had experienced MACE. Compared with the non-MACE group, the MACE group had a higher visit-to-visit LDL-C variability (cVIM: 0.23 ± 0.11 vs. 0.19 ± 0.08; p = 0.049; coefficient of variation: 0.24 ± 0.12 vs. 0.19 ± 0.00; p = 0.019; standard deviation: 24.1 ± 14.5 vs. 17.6 ± 10.0; p = 0.006), mean follow-up LDL-C (p = 0.033) and a higher prevalence of diabetes mellitus (p = 0.012). After adjusting for mean follow-up cholesterol levels and diabetes mellitus, each 0.01 cVIM increase in LDL-C and HDL-C variability increased the risk of MACE by 3.4% (HR: 1.034; 95% CI: 1.004 to 1.065; p = 0.025) and 6.8% (HR: 1.068; 95% CI: 1.003 to 1.137; p = 0.04), respectively. Results derived from coefficient of variation and standard deviation as measures of cholesterol variability were similar. CONCLUSION This is the first report to show an independent association between visit-to-visit LDL-C and HDL-C variability and long-term MACE in patients presenting with STEMI.

Sex Differences in Phenotypes of Bicuspid Aortic Valve and AortopathyCLINICAL PERSPECTIVE: Insights From a Large Multicenter, International Registry

Background— This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). Methods and Results— Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta. New cases of endocarditis and aortic dissection were recorded. There were no significant sex differences regarding BAV morphology and frequency of normal valve function. When presenting with moderate/severe aortic valve dysfunction, men had more frequent aortic regurgitation than women (33.8% versus 22.2%, P<0.001), whereas women were more likely to have aortic stenosis (34.5% versus 44.1%, P<0.001). Men had more frequently isolated dilatation of the sinus of Valsalva or sinotubular junction (14.2% versus 6.7%, P<0.001) and diffuse dilatation of the aortic root and ascending aorta (16.2% versus 7.3%, P<0.001) than women. Endocarditis (4.5% versus 2.5%, P=0.037) and aortic dissections (0.5% versus 0%, P<0.001) occurred more frequently in men. Conclusions— Although there is a male predominance among patients with BAV, men with BAV had more frequently moderate/severe aortic regurgitation at first presentation compared with women, whereas women presented more often with moderate/severe aortic stenosis compared with men. Furthermore, men had more frequent aortopathy than women.Background— This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). Methods and Results— Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta. New cases of endocarditis and aortic dissection were recorded. There were no significant sex differences regarding BAV morphology and frequency of normal valve function. When presenting with moderate/severe aortic valve dysfunction, men had more frequent aortic regurgitation than women (33.8% versus 22.2%, P <0.001), whereas women were more likely to have aortic stenosis (34.5% versus 44.1%, P <0.001). Men had more frequently isolated dilatation of the sinus of Valsalva or sinotubular junction (14.2% versus 6.7%, P <0.001) and diffuse dilatation of the aortic root and ascending aorta (16.2% versus 7.3%, P <0.001) than women. Endocarditis (4.5% versus 2.5%, P =0.037) and aortic dissections (0.5% versus 0%, P <0.001) occurred more frequently in men. Conclusions— Although there is a male predominance among patients with BAV, men with BAV had more frequently moderate/severe aortic regurgitation at first presentation compared with women, whereas women presented more often with moderate/severe aortic stenosis compared with men. Furthermore, men had more frequent aortopathy than women.