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Although pregnancy outcomes in women with normally functioning bioprosthetic valves (BPVs) are often good, structural valve dysfunction (SVD) may adversely affect pregnancy outcomes, but this has not been studied.The aim of this study was to examine outcomes in pregnant women with BPVs and the association with SVD.Pregnancy outcomes in women with BPVs were prospectively collected. Adverse maternal cardiac events (CEs) included cardiac death or arrest, sustained arrhythmia, heart failure, thromboembolism, and stroke. Adverse fetal events were also studied. Determinants of adverse events were examined using logistic regression.Overall, 125 pregnancies in women with BPVs were included, 27% with left-sided and 73% with right-sided BPV. SVD was present in 27% of the pregnancies (44% with left-sided BPVs vs 21% with right-sided BPVs; P = 0.009). CEs occurred in 13% of pregnancies and were more frequent in women with SVD compared with those with normally functioning BPVs (26% vs 8%; P = 0.005). CEs were more common in women with left-sided BPVs with SVD vs normally functioning BPVs (47% vs 5%; P = 0.01) but not in women with right-sided BPVs (11% in those with SVD vs 8% in those without SVD; P = 0.67). Left-sided SVD (P = 0.007), maternal age35 years (P = 0.001), and a composite variable of "high-risk" features (P = 0.006) were predictors of CEs. Fetal events occurred in 28% of pregnancies.In this cohort of young women with BPVs, SVD was present in 27% at the first antenatal visit and negatively affected pregnancy outcomes. In particular, SVD of left-sided BPVs was associated with high rates of adverse outcomes.

Maternal morbidity and mortality continue to rise in the United States, with cardiovascular disease as the leading cause of maternal deaths. Congenital heart disease is now the most common cardiovascular condition encountered during pregnancy, and its prevalence will continue to grow. In tandem with these trends, maternal cardiovascular health is becoming increasingly complex. The identification of women at highest risk for cardiovascular complications is essential, and a team-based approach is recommended to optimize maternal and fetal outcomes. This document, the second of a 5-part series, will provide practical guidance from pre-conception through postpartum for cardiovascular conditions that are predominantly congenital or heritable in nature, including aortopathies, congenital heart disease, pulmonary hypertension, and valvular heart disease.

The specialty of cardio-obstetrics has emerged in response to the rising rates of maternal morbidity and mortality related to cardiovascular disease (CVD) during pregnancy. Women of childbearing age with or at risk for CVD should receive appropriate counseling regarding maternal and fetal risks of pregnancy, medical optimization, and contraception advice. A multidisciplinary cardio-obstetrics team should ensure appropriate monitoring during pregnancy, plan for labor and delivery, and ensure close follow-up during the postpartum period when CVD complications remain common. The hemodynamic changes throughout pregnancy and during labor and delivery should be considered with respect to the individual cardiac disease of the patient. The fourth trimester refers to the 12 weeks after delivery and is a key time to address contraception, mental health, cardiovascular risk factors, and identify any potential postpartum complications. Women with adverse pregnancy outcomes are at increased risk of long-term CVD and should receive appropriate education and longitudinal follow-up.

The specialty of cardio-obstetrics has emerged in response to the rising rates of maternal morbidity and mortality related to cardiovascular disease (CVD) during pregnancy. Women of childbearing age with or at risk for CVD should receive appropriate counseling regarding maternal and fetal risks of pregnancy, medical optimization, and contraception advice. A multidisciplinary cardio-obstetrics team should ensure appropriate monitoring during pregnancy, plan for labor and delivery, and ensure close follow-up during the postpartum period when CVD complications remain common. The hemodynamic changes throughout pregnancy and during labor and delivery should be considered with respect to the individual cardiac disease of the patient. The fourth trimester refers to the 12 weeks after delivery and is a key time to address contraception, mental health, cardiovascular risk factors, and identify any potential postpartum complications. Women with adverse pregnancy outcomes are at increased risk of long-term CVD and should receive appropriate education and longitudinal follow-up.

Background: Women with heart disease are at risk for complications during pregnancy. This study sought to examine the effect of maternal obesity on pregnancy complications in women with heart disease. Objectives: The objective was to determine the incidence of adverse cardiac events (CE) in pregnant women with heart disease and obesity. Methods: Adverse CE during pregnancy were examined in a prospective cohort of women with heart disease. CE were a composite of the following: cardiac death/arrest, arrhythmias, heart failure, myocardial infarction, stroke, aortic dissection, and thromboembolic events. Pre-eclampsia and post-partum hemorrhage were also studied. Outcomes were examined according to body mass index (BMI). To identify additional predictors of CE, a baseline risk score (CARPREG [Canadian Cardiac Disease in Pregnancy Study] II score) for predicting cardiac complications was calculated for all pregnancies and included in a multivariable logistic regression model. Results: Of 790 pregnancies, 19% occurred in women with BMI ≥30 kg/m2 (obesity), 25% in women with BMI 25 to 29.9 kg/m2 (overweight), 53% in women with BMI 18.5 to 24.9 kg/m2 (normal weight), and 3% in women with BMI

Maternal and fetal complications are increased in pregnant women with cardiovascular disease (CVD) ([1][1],[2][2]). The most common maternal cardiac complications are heart failure and arrhythmias, and although beta-blockers are vital to protecting pregnant women from these, there are concerns over

Background Identifying women at high risk is an important aspect of care for women with heart disease. Objectives This study sought to: 1) examine cardiac complications during pregnancy and their temporal trends; and 2) derive a risk stratification index. Methods We prospectively enrolled consecutive pregnant women with heart disease and determined their cardiac outcomes during pregnancy. Temporal trends in complications were examined. A multivariate analysis was performed to identify predictors of cardiac complications and these were incorporated into a new risk index. Results In total, 1,938 pregnancies were included. Cardiac complications occurred in 16% of pregnancies and were primarily related to arrhythmias and heart failure. Although the overall rates of cardiac complications during pregnancy did not change over the years, the frequency of pulmonary edema decreased (8% from 1994 to 2001 vs. 4% from 2001 to 2014; p value = 0.012). Ten predictors of maternal cardiac complications were identified: 5 general predictors (prior cardiac events or arrhythmias, poor functional class or cyanosis, high-risk valve disease/left ventricular outflow tract obstruction, systemic ventricular dysfunction, no prior cardiac interventions); 4 lesion-specific predictors (mechanical valves, high-risk aortopathies, pulmonary hypertension, coronary artery disease); and 1 delivery of care predictor (late pregnancy assessment). These 10 predictors were incorporated into a new risk index (CARPREG II [Cardiac Disease in Pregnancy Study]). Conclusions Pregnancy in women with heart disease continues to be associated with significant morbidity, although mortality is rare. Prediction of maternal cardiac complications in women with heart disease is enhanced by integration of general, lesion-specific, and delivery of care variables.

Cancer treatment can lead to cardiac disease in female cancer survivors of child-bearing age. We evaluated the incidence of cardiac decompensation during pregnancy in cancer survivors and evaluated the effect of a cardiotoxicity history on the risk of decompensation. We retrospectively identified

The incidence of pregnancy in women with cardiovascular disease is rising, primarily due to the increased number of women with congenital heart disease reaching childbearing age and the changing demographics associated with advancing maternal age. Although most cardiac conditions are well tolerated during pregnancy and women can deliver safely with favorable outcomes, there are some cardiac conditions that have significant maternal and fetal morbidity and mortality. The purpose of this paper is to review the available published reports and provide recommendations on the management of women with high-risk cardiovascular conditions during pregnancy.

Women with heart disease are at high risk for pregnancy complications. Obesity is an emergent health risk for women, and in the non-cardiac pregnant population, it has been shown to adversely impact the health of mother and baby. Therefore, we sought to determine if obesity increased maternal

Identifying women at high risk is an important aspect of care for women with heart disease.This study sought to: 1) examine cardiac complications during pregnancy and their temporal trends; and 2) derive a risk stratification index.We prospectively enrolled consecutive pregnant women with heart disease and determined their cardiac outcomes during pregnancy. Temporal trends in complications were examined. A multivariate analysis was performed to identify predictors of cardiac complications and these were incorporated into a new risk index.In total, 1,938 pregnancies were included. Cardiac complications occurred in 16% of pregnancies and were primarily related to arrhythmias and heart failure. Although the overall rates of cardiac complications during pregnancy did not change over the years, the frequency of pulmonary edema decreased (8% from 1994 to 2001 vs. 4% from 2001 to 2014; p value = 0.012). Ten predictors of maternal cardiac complications were identified: 5 general predictors (prior cardiac events or arrhythmias, poor functional class or cyanosis, high-risk valve disease/left ventricular outflow tract obstruction, systemic ventricular dysfunction, no prior cardiac interventions); 4 lesion-specific predictors (mechanical valves, high-risk aortopathies, pulmonary hypertension, coronary artery disease); and 1 delivery of care predictor (late pregnancy assessment). These 10 predictors were incorporated into a new risk index (CARPREG II [Cardiac Disease in Pregnancy Study]).Pregnancy in women with heart disease continues to be associated with significant morbidity, although mortality is rare. Prediction of maternal cardiac complications in women with heart disease is enhanced by integration of general, lesion-specific, and delivery of care variables.

Heart disease continues to be the leading cause of nonobstetric maternal morbidity and mortality. Early diagnosis and appropriate care can lead to prevention of complications and improvement of pregnancy outcome. This paper continues the review and provides recommendations for the approach to high-risk cardiovascular conditions during gestation.

Objectives The purpose of this study was to determine the prevalence of valvular aortic stenosis requiring surgery in patients with a pre-existing diagnosis of subaortic stenosis. Background Classic teachings emphasize aortic regurgitation as the most common complication associated with discrete subaortic stenosis. We hypothesized that significant aortic stenosis may also be an important valve lesion associated with this condition. Methods Clinical outcomes in patients with subaortic stenosis were examined. The primary outcome of interest was the prevalence of valvular aortic stenosis requiring surgery (surgical valvotomy or valve replacement). Logistic regression was used to identify variables associated with the need for surgery for aortic stenosis. Results One hundred twenty-one adults with subaortic stenosis (median age 32 years) were evaluated in our clinic. Associated lesions were common: 23% had bicuspid valves and 21% had coarctation of the aorta. Seventy-nine percent of the patients had at least 1 surgical resection of subaortic tissue (median age 12 years). Moderate to severe aortic regurgitation was present in 16% of patients (19 of 121), 3 of whom required surgical intervention in adulthood. Twenty-six percent of patients (32 of 121) required surgery for valvular aortic stenosis. Valve surgery for aortic stenosis was more common in patients with concomitant bicuspid aortic valve disease (p = 0.008), coarctation of the aorta (p = 0.03), and supravalvular stenosis (p = 0.02). Conclusions Valvular aortic stenosis is a surprisingly common finding in patients with discrete subaortic stenosis. Careful clinical follow-up of this population to monitor aortic valve status continues to be warranted even after a successful surgical resection.

A growing number of women seek infertility treatment with assisted reproductive technologies (ART), such as in vitro fertilization (IVF) or intrauterine insemination (IUI), in conjunction with ovarian stimulation treatments [(1)][1]. Although ART improve the chances of successful pregnancy, there

Objectives The aim of this study was to determine the acute and long-term outcome of radiofrequency catheter ablation (RFCA) for intra-atrial re-entrant tachycardia (IART) in adults with congenital heart disease (CHD), and predictors of these outcomes. Background Atrial myopathy can be progressive in CHD and contributes to the substrate for IART. Although the outcome of RFCA for IART has been well described in children and adolescents with CHD, it is unclear whether these results are similar in the adult population. Methods Clinical records of adults with CHD undergoing attempted RFCA of IART were analyzed retrospectively. Multivariate analyses identified clinical and procedural factors that predicted acute and long-term outcomes. Results A total of 193 procedures was performed in 130 patients (mean age 40 ± 13 years); 82 of 118 (69%) initially attempted RFCA were successful, defined as termination of all IART circuits. The use of electroanatomic mapping was associated with a successful RFCA, whereas Fontan palliation and Mustard repair were associated with an unsuccessful RFCA. Median clinical follow-up of 77 patients (≥2 months of follow-up) after a successful RFCA was 3.7 years (range 0.2 to 10.2 years). IART recurrence was noted in 48%, cardioversion/reablation in 42%, and death in 4%. Older age and Fontan palliation were independent predictors of IART recurrence. Conclusions In adults with CHD, acute and long-term outcomes of RFCA for IART are similar to those reported for younger cohorts. Complex atrial surgery limits the success of RFCA, and older age is associated with a higher risk of IART recurrence.

Objectives We sought to determine cardiac outcomes in young adults with complete transposition of the great arteries (TGA) after the arterial switch operation (ASO). Background Although cardiac outcomes in the pediatric population with TGA after ASO have been well described, outcomes in the adult population have not to our knowledge been studied. Methods We determined late survival in all operative survivors with TGA after ASO performed before 1991 at our local pediatric referring hospital. In the subset of adults (n = 65) followed in our adult congenital cardiac clinic, we examined cardiac outcomes in adulthood. Results Survival of the 132 infants discharged from hospital after ASO was 97% (70% confidence interval [CI]: 95.0% to 98.1%) at 20 years. In the 65 patients (mean age 21 ± 3 years, 62% male) followed at our institution, 17% (11 of 65) had at least 1 clinically significant cardiac lesion, including ventricular dysfunction, valvular dysfunction, or arrhythmias. Residual lesions were more common in those who had had cardiac reinterventions in childhood (odds ratio: 10.7, 95% CI: 2.1 to 55). In adulthood, 5 patients (8%) had arrhythmia requiring treatment and 7 patients (11%) required reinterventions (5 reoperations and 2 pacemaker implantations). Intervention for aortic valve regurgitation and aortic root dilation were not observed. Exercise capacity was reduced in most adults (82%) after ASO. Conclusions Although most adults after ASO are well, and few have residual defects, there are subgroups, particularly those who needed further cardiac intervention in childhood, who are at higher risk for ventricular and valve dysfunction and arrhythmias.

The impact of a prior history of cancer treatment related cardiac dysfunction (CTRCD) on cardiac decompensation (CD) during pregnancy in female cancer survivors has not been examined. Our objective was to perform a meta-analysis to quantify the risk of CD during pregnancy in cancer survivors with

Objectives The objectives of this study were to determine adverse outcomes during pregnancy in women with dilated cardiomyopathy (DCM) and to compare their cardiac outcomes with those of nonpregnant women with DCM. Background Women with DCM are at risk for complications during pregnancy, but few studies have examined outcomes in this specific population. Methods This was a substudy of a larger prospective cohort study of outcomes in women with heart disease. Maternal cardiac, obstetric, and fetal outcomes in pregnancy in women with DCM were examined. For comparison, cardiac outcomes in nonpregnant women with DCM (n = 18) matched by age and left ventricular (LV) systolic function were examined. A matched-pair survival analysis was used to compare groups. Results Thirty-six pregnancies in 32 women with DCM were included. Thirty-nine percent (14 of 36) of the pregnancies were complicated by at least 1 maternal cardiac event. In the multivariate analysis, moderate or severe LV dysfunction and/or New York Heart Association functional class III or IV (p = 0.003) were the main determinants of adverse maternal cardiac outcomes during pregnancy. In the subset of women with moderate/severe LV dysfunction, 16-month event-free survival was worse in pregnant women compared with nonpregnant women (28 ± 11% vs. 83 ± 10%, p = 0.02). The adverse neonatal event rate was highest among women with obstetric and cardiac risk factors (43%). Conclusions In pregnant women with DCM the risk of adverse cardiac events is considerable, and pre-pregnancy characteristics can identify women at the highest risk. Pregnancy seems to have a short-term negative impact on the clinical course in women with DCM.

Objectives We sought to determine the prevalence of pulmonary artery thrombosis in patients with Eisenmenger syndrome and to identify individuals at highest risk. Background Eisenmenger syndrome is associated with pulmonary arterial thrombus formation. Both the prevalence and the determinants of pulmonary arterial thrombosis are unknown. Methods This is a review of patients with Eisenmenger syndrome seen at the Toronto Congenital Cardiac Centre for Adults, Canada. Patients underwent a contrast-enhanced computed tomographic (CT) scan of the thorax. Results Forty-nine consecutive patients with Eisenmenger syndrome were seen in our hospital. Fifteen patients did not undergo CT angiograms; therefore, 34 patients (mean age 42 ± 10 years) were included in the study. Responsible shunts included ventricular septal defect (65%), atrial septal defect (15%), patent ductus arteriosus (9%), and other (11%). The prevalence of proximal pulmonary artery thrombus was 21% (7/34) of patients. Evidence of more distal vessel thrombosis was observed in 43% (3/7) of the patients who had visible thrombus in the proximal pulmonary arteries. Patients with thrombus were more likely to be female (86% vs. 37%, p = 0.04) and to have lower oxygen saturations (72% ± 9% vs. 85% ± 6%, p = 0.01). Differences in functional status did not identify patients at highest risk for thrombosis. Conclusions Patients with Eisenmenger syndrome have a substantial risk of pulmonary artery thrombus formation. Women and patients with lower oxygen saturations are at the highest risk of developing thrombosis. In the context of an increased bleeding tendency in these patients, the role of anticoagulation treatment needs to be determined.

ObjectivesThe purpose of this study was to determine if left ventricular (LV) systolic dysfunction was also a predictor of sudden cardiac death (SCD) in adults late after repair of tetralogy of Fallot (TOF).BackgroundPrevious studies looking at risk factors for SCD in adults with repair of TOF have focused on the right ventricle (RV).MethodsA retrospective chart review of patients assessed at the Toronto Congenital Cardiac Centre for Adults was performed. Twelve adult patients with repaired TOF and SCD were identified (SCD group). A total of 125 living adult patients with repaired TOF were randomly selected for comparison (control group).ResultsPatients with SCD were more likely to exhibit moderate or severe pulmonary regurgitation (92% vs. 51%, p = 0.02), have a history of sustained ventricular tachycardia (42% vs. 6%, p < 0.01), and have a QRS ≥180 ms (56% vs. 13%, p = 0.02). Moderate or severe LV systolic dysfunction was also significantly more common in patients with SCD than in the control group (42% vs. 9%, p < 0.01) with a positive predictive value of 29%. The combination of moderate or severe LV systolic dysfunction and QRS ≥180 ms had a positive and negative predictive value for SCD of 66% and 93%, respectively.ConclusionsModerate or severe LV systolic dysfunction is significantly more common in adult patients with repaired TOF and SCD. The combination of QRS ≥180 ms and significant LV systolic dysfunction has high positive and negative predictive value for SCD. The implication of the role of prophylactic antiarrhythmic implantable cardiac defibrillator insertion in these patients needs further elucidating.

Background: Pregnant women with heart disease are at increased risk of complications. At our centre, management algorithms for this population have been implemented since 2001. However, the impact of standardized care on outcomes has not been evaluated. Methods: We prospectively recorded baseline

To the Editor: Activation delay due to right bundle branch block (RBBB) has been implicated in the pathogenesis of right ventricular (RV) dilation and dysfunction in patients with tetralogy of Fallot (ToF) ([1][1]). Accordingly, it has been proposed that RV apical pacing may improve RV function on

The absolute risk of sudden cardiac death (SCD) is largely unknown in adults with congenital heart disease (CHD). This hampers accurate assessment of the individual patient's risk of SCD. We developed a risk scoring system to assess the absolute risk of SCD among adults with various CHD. Predictors

The purpose of this study was to determine the prevalence of valvular aortic stenosis requiring surgery in patients with a pre-existing diagnosis of subaortic stenosis.Classic teachings emphasize aortic regurgitation as the most common complication associated with discrete subaortic stenosis. We hypothesized that significant aortic stenosis may also be an important valve lesion associated with this condition.Clinical outcomes in patients with subaortic stenosis were examined. The primary outcome of interest was the prevalence of valvular aortic stenosis requiring surgery (surgical valvotomy or valve replacement). Logistic regression was used to identify variables associated with the need for surgery for aortic stenosis.One hundred twenty-one adults with subaortic stenosis (median age 32 years) were evaluated in our clinic. Associated lesions were common: 23% had bicuspid valves and 21% had coarctation of the aorta. Seventy-nine percent of the patients had at least 1 surgical resection of subaortic tissue (median age 12 years). Moderate to severe aortic regurgitation was present in 16% of patients (19 of 121), 3 of whom required surgical intervention in adulthood. Twenty-six percent of patients (32 of 121) required surgery for valvular aortic stenosis. Valve surgery for aortic stenosis was more common in patients with concomitant bicuspid aortic valve disease (p = 0.008), coarctation of the aorta (p = 0.03), and supravalvular stenosis (p = 0.02).Valvular aortic stenosis is a surprisingly common finding in patients with discrete subaortic stenosis. Careful clinical follow-up of this population to monitor aortic valve status continues to be warranted even after a successful surgical resection.

The aim of this study was to determine the acute and long-term outcome of radiofrequency catheter ablation (RFCA) for intra-atrial re-entrant tachycardia (IART) in adults with congenital heart disease (CHD), and predictors of these outcomes.Atrial myopathy can be progressive in CHD and contributes to the substrate for IART. Although the outcome of RFCA for IART has been well described in children and adolescents with CHD, it is unclear whether these results are similar in the adult population.Clinical records of adults with CHD undergoing attempted RFCA of IART were analyzed retrospectively. Multivariate analyses identified clinical and procedural factors that predicted acute and long-term outcomes.A total of 193 procedures was performed in 130 patients (mean age 40 ± 13 years); 82 of 118 (69%) initially attempted RFCA were successful, defined as termination of all IART circuits. The use of electroanatomic mapping was associated with a successful RFCA, whereas Fontan palliation and Mustard repair were associated with an unsuccessful RFCA. Median clinical follow-up of 77 patients (≥2 months of follow-up) after a successful RFCA was 3.7 years (range 0.2 to 10.2 years). IART recurrence was noted in 48%, cardioversion/reablation in 42%, and death in 4%. Older age and Fontan palliation were independent predictors of IART recurrence.In adults with CHD, acute and long-term outcomes of RFCA for IART are similar to those reported for younger cohorts. Complex atrial surgery limits the success of RFCA, and older age is associated with a higher risk of IART recurrence.

Objectives: This study sought to examine the relationship between proximal aortic dilation and systemic vascular function in men with bicuspid aortic valve (BAV). Background: Proximal aortic dilation in subjects with BAV is associated with structural and functional abnormalities in the ascending aorta. Methods: We studied 32 men (median age 31 years [range 28 to 32 years]) with nonstenotic BAV categorized into 2 subgroups according to proximal ascending aorta dimensions (nondilated ≤35 mm and dilated ≥40 mm, respectively). Sixteen healthy men were studied as control subjects. Flow-mediated dilation in response to hyperemia (a marker of endothelial dysfunction) and carotid-femoral pulse wave velocity (an index of aortic stiffness) were assessed, and peripheral blood was sampled for matrix metalloproteinases (MMP-2 and -9) and their tissue inhibitors (TIMP-1 and -2), respectively. Cardiac chamber and aortic dimensions were assessed by echocardiography and cardiac magnetic resonance imaging, respectively. Results: Despite the similar severity of aortic stenosis, left ventricular mass, and function, men with dilated aortas had blunted brachial flow-mediated vasodilation to hyperemia (5% [interquartile range (IQR) 4% to 6%] vs. 8% [IQR 7% to 9%] change, p = 0.001), higher carotid-femoral pulse wave velocity (9.3 cm/s [IQR 9 to 10 cm/s] vs. 7 cm/s [IQR 6.9 to 7.4 cm/s], p = 0.001), and significantly higher plasma levels of MMP-2 (1,523 [IQR 1,460 to 1,674] vs. 1,036 [IQR 962 to 1,167], p = 0.001) compared with men with BAV and nondilated aorta. Values for MMP-9, TIMP-1 and -2 levels, and nitroglycerin-induced (endothelium-independent) vasodilation were similar in all 3 groups. Conclusions: Young men with BAV and dilated proximal aortas manifest systemic endothelial dysfunction, increased carotid-femoral pulse wave velocity, and higher plasma levels of MMP-2. These observations could introduce new targets for screening and perhaps for therapeutic intervention. © 2010 American College of Cardiology Foundation.

To the Editor: As increased numbers of patients with congenital heart disease (CHD) survive to adulthood, more women with CHD are reaching reproductive age. Contraception and pregnancy have now become important issues in this population; however, both can be associated with increased risks in women

Bicuspid aortic valve (BAV) disease is the most common congenital cardiac defect. While the BAV can be found in isolation, it is often associated with other congenital cardiac lesions. The most frequent associated finding is dilation of the proximal ascending aorta secondary to abnormalities of the aortic media. Changes in the aortic media are present independent of whether the valve is functionally normal, stenotic, or incompetent. Although symptoms often manifest in adulthood, there is a wide spectrum of presentations ranging from severe disease detected in utero to asymptomatic disease in old age. Complications can include aortic valve stenosis or incompetence, endocarditis, aortic aneurysm formation, and aortic dissection. Despite the potential complications, 2 large contemporary series have demonstrated that life expectancy in adults with BAV disease is not shortened when compared with the general population. Because BAV is a disease of both the valve and the aorta, surgical decision making is more complicated, and many undergoing aortic valve replacement will also need aortic root surgery. With or without surgery, patients with BAV require continued surveillance. Recent studies have improved our understanding of the genetics, the pathobiology, and the clinical course of the disease, but questions are still unanswered. In the future, medical treatment strategies and timing of interventions will likely be refined. This review summarizes our current understanding of the pathology, genetics, and clinical aspects of BAV disease with a focus on BAV disease in adulthood. © 2010 American College of Cardiology Foundation.

One-third of North American adults with congenital heart disease (CHD) have diagnosable mood or anxiety disorders; unfortunately most of these patients do not receive mental health treatment. Despite an increasing awareness of the unique psychosocial concerns of this patient population, there are no