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In women with mitral stenosis (MS), mitral valve gradients and right ventricular systolic pressure (RVSP) can increase in response to the physiologic stress of pregnancy. The prognostic significance of these echocardiographic changes has not been well studied. Pregnancy outcomes and serial echocardiograms were collected in women with MS prospectively recruited as part of a larger study on pregnancy outcomes. Third trimester echocardiograms were compared with baseline echocardiograms. Changes in mitral valve area (MVA), transmitral mean gradient (MG), and RVSP during pregnancy and their relationship to adverse cardiac events (CE) were examined. Fifty-six pregnancies in 47 women with MS were included. The MVA did not change during pregnancy (1.6 ± 0.6 cm2 at baseline vs 1.7 ± 0.6 cm2 in the third trimester, p = 0.46). There was an increase in the MG (8 ± 3 vs 11 ± 6 mm Hg, p 10 mm Hg, baseline RVSP >40 mm Hg, third-trimester MG>10 mm Hg, and RVSP >40 mm Hg. Women with mitral valve MG ≤10 mm Hg who had a normal RVSP at baseline and in the third trimester were at lowest risk for CE (11%) with a negative predictive value of 89%. In conclusion, baseline echocardiographic assessment of MS severity as well as changing echocardiographic parameters during pregnancy can help identify women at risk for cardiac complications during pregnancy.

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.

BackgroundComplications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on outcome after pulmonary valve replacement (PVR).MethodsWe performed a retrospective analysis of 215 patients with PVS who underwent surgical valvotomy or balloon valvuloplasty. Incidence and predictors of reinterventions and complications were identified. Right ventricle (RV) remodelling after PVR was also assessed.ResultsAfter a median follow-up of 38.6 (30.9–49.4) years, 93% of the patients were asymptomatic. Thirty-nine patients (18%) had at least one PVR. Associated right ventricular outflow tract (RVOT) intervention and the presence of an associated defect were independent predictors of reintervention (OR: 4.1 (95% CI 1.5 to 10.8) and OR: 3.6 (95% CI 1.9 to 6.9), respectively). Cardiovascular death occurred in 2 patients, and 29 patients (14%) had supraventricular arrhythmia. Older age at the time of first intervention and the presence of an associated defect were independent predictors of complications (OR: 1.0 (95% CI 1.0 to 1.1) and OR: 2.1 (95% CI 1.1 to 4.2), respectively). In 16 patients, cardiac magnetic resonance before and after PVR was available. The optimal cut-off values for RV volume normalisation were 193 mL/m2 for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m2 for RV end-systolic volume indexed(sensitivity 80%, specificity 56%).ConclusionsPrevious RVOT intervention, presence of an associated defect and older age at the time of first repair were predictors of outcome. More data are needed to guide timing of PVR, and extrapolation of tetralogy of Fallot guidelines to this population is unlikely to be appropriate.

[Extract] The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease” (S1.4-1), which was the first U.S. guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease” (S1.4-2), particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (

Background: Pregnancies in women with regurgitant valve lesions are generally considered low risk, but this has not been well studied. Objectives: This study determined the frequency of adverse cardiac events (CEs) in pregnant women with moderate or severe regurgitant valve lesions. Methods: Maternal and fetal outcomes in women with moderate or severe chronic valve regurgitation enrolled in a prospective multicenter study on pregnancy outcomes were examined. Adverse CEs included heart failure, sustained arrhythmias, cardiac arrest, or death. A multivariate logistic regression model was used to identify determinants of CEs in women at the highest risk. Results: Outcomes of 430 pregnancies in women with moderate or severe regurgitant lesions were examined: 145 with mitral regurgitation (MR), 101 with pulmonary regurgitation (PR), 71 with multivalve disease, 73 with tricuspid regurgitation (TR), and 40 with aortic regurgitation (AR). Most women had associated congenital or acquired heart disease. Adverse CEs occurred in 13% of pregnancies: 27% of pregnancies with multivalve disease; 15% with MR; 15% with TR; 5% with AR; and 3% with PR. Maternal mortality was rare. In women with MR, TR, or multivalve disease (n = 289), left ventricular systolic dysfunction (p = 0.001), pulmonary hypertension (p = 0.005), and cardiac events before pregnancy (p < 0.001) were important determinants of CEs during pregnancy. Conclusions: Women with AR and PR are at low risk for cardiac complications during pregnancy. While many women with MR, TR, and multivalve regurgitation do well during pregnancy, additional clinical variables help stratify those at highest risk. This new information will enhance the quality and precision of preconception counseling and pregnancy planning.

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.

Objective Although heart failure (HF) is the leading cause of premature death in adult congenital heart disease (ACHD), little population-specific data exist. This study reports early experience from a dedicated, sub-specialty adult congenital heart disease-heart failure (ACHD-HF) clinic, aiming to identify risk factors for adverse outcome. Methods Between 2012 and 2015, 126 patients (57% male) attended the ACHD-HF clinic. Baseline and follow-up data were analysed and compared across 4 anatomical/physiological subgroups: cyanotic ACHD, Fontan circulation (1V), biventricular circulation with a subaortic right ventricle (2V-RV) and biventricular circulation with a subaortic left ventricle (2V-LV). Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using multivariable Cox proportional hazard models. Results Mean age at first visit was 38±13years. Patients were grouped as follows: cyanotic ACHD 10%, 1V 24%, 2V-RV 29% and 2V-LV 37%. During a median follow-up of 1.7 (IQR 0.8–2.9) years, 38 patients (30%) reached the primary outcome. Event-free survival was 89%, 78% and 63% at 1, 2 and 3years. Forty (31.7%) patients experienced 69 HF hospitalisations. Between-group differences were noted for systolic function, valvular regurgitation, pacing prevalence and invasive hemodynamics. Multivariable analysis revealed 2V-RV subgroup ( p =0.001), NYHA class ( p =0.002) B-type natriuretic peptide >164pg/ml ( p =0.003) and sodium p =0.036) as independently associated with death, transplant or VAD. Conclusions Our young ACHD-HF patients experienced high adverse event rates during a short period of follow-up. The prognostic markers identified will aid clinicians to stratify short-term risk and thereby guide advanced HF management decisions in ACHD.

Background Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare condition that usually presents in childhood. The aim of this study is to describe its characteristics in unrepaired adults. Methods We conducted a retrospective study of 18 patients with left and right anomalous coronary artery from the PA (ALCAPA and ARCAPA) diagnosed in adulthood. Patients ≥18 years old at diagnosis and assessed at least once in our institution were included. Results Median age at diagnosis was 29.5 years [IQR 24.0–48.8]. Presentation mode varied, with two patients (11%) diagnosed following aborted sudden cardiac death. Diagnosis was most frequently made by coronary angiography (44%) and cardiac computed tomography (28%). The mean left ventricle ejection fraction (LVEF) at presentation was 56 ±10% and moderate or severe mitral regurgitation was present in 5 patients. Surgical or interventional repair was performed in 14 patients (78%), 9 of whom (50%) underwent reimplantation. Median follow-up was 8.5 years [IQR 0.98–13.8]. One patient with severe pulmonary hypertension died during the follow-up. Most patients (79%) were in NYHA functional class 1 at last follow-up with a mean LVEF of 53±12%. Conclusions Anomalous origin of a coronary artery from the PA can present in adulthood in various ways. Multimodality imaging allows the diagnosis and assessment of functional impact. Surgical correction of ALCAPA with establishment of a dual coronary pathway was the most frequent management strategy in our cohort. The majority of patients experienced favorable long-term outcomes.

ObjectivesIndividuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population.MethodsWe performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death.ResultsA total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease.ConclusionsWomen with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

Background: Women with transposition of the great arteries (TGA) following atrial redirection surgery are at risk of pregnancy-associated arrhythmia and heart failure. The cardiovascular magnetic resonance imaging (CMR) characteristics of these women and the relationship of CMR findings to pregnancy outcomes have not been described. Methods: We included 17 women with atrial redirection surgery and CMR within 2 years of delivery. Results: All women were asymptomatic at baseline (New York Heart Association Class 1). CMR studies were completed pre-pregnancy in 3, antepartum/peripartum in 2, and postpartum in 12 women. Three women (3/17, 18%) experienced major cardiovascular events related to pregnancy: cardiac arrest (n = 1) and symptomatic atrial arrhythmia (n = 2). Median gestational age at delivery was 38 weeks (24-39 weeks) and birth weight was 2770 g (2195-3720 g). Complications were seen in 3 offspring (3/17, 18%): death (n = 1) and prematurity (n = 2). CMR characteristics included median right ventricular end diastolic volume 119 mL/m2 (range 85-214 mL/m2) and median right ventricular ejection fraction (RVEF) 37% (range 30-51%). All women with cardiovascular complications had an RVEF

Background In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes. Objectives This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome. Methods All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons. Results A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%. Conclusions Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death.

Objective The objective of this study was to determine outcomes in pregnant women with pre-existing coronary artery disease (CAD) or following an acute coronary syndrome (ACS) including myocardial infarction (MI).Background The physiological changes of pregnancy can contribute to myocardial ischaemia. The pregnancy risk for women with pre-established CAD or a history of ACS/MI is not well studied.Methods This was a retrospective multicentre study. Adverse maternal cardiac, obstetric and fetal/neonatal events were examined. The primary outcome was a composite endpoint of cardiac arrest, ACS/MI, ventricular arrhythmia or congestive heart failure. The prevalence of new or progressive angina during pregnancy was also examined.Results Fifty pregnancies in 43 women (mean age 35 +/- 5 years) were included. Coronary atherosclerosis (40%) and coronary thrombus (36%) were the most common underlying diagnoses. The primary outcome occurred in 10% (5/50) of pregnancies and included one maternal death secondary to cardiac arrest. Other events included ACS/MI (3/50) and heart failure (1/50). New or progressive angina occurred in 18% of pregnancies. Ischaemic complications of any type (new or progressive angina, ACS/MI, ventricular arrhythmia, cardiac arrest) occurred more commonly in women with coronary atherosclerosis compared with those without (50% vs 10%, p=0.003). A high rate of adverse obstetric (16%) and fetal/neonatal (30%) events was observed.Conclusions Pregnant women with pre-existing CAD or ACS/MI before pregnancy are at increased risk of adverse events during pregnancy. Those with coronary atherosclerosis are at highest risk of adverse maternal cardiac events due to myocardial ischaemia during pregnancy.

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.

IntroductionThe adult CHD population is increasing and ageing and remains at high risk for morbidity and mortality. In a retrospective single-centre study, we conducted a comprehensive review of non-elective hospitalisations of adults with CHD and explored factors associated with length of stay.MethodsWe identified adults (⩾18 years) with CHD admitted during a 12-month period and managed by the adult CHD service. Data regarding demographics, cardiac history, hospital admission, resource utilisation, and length of stay were extracted.ResultsThere were 103 admissions of 91 patients (age 37±10 years; 52% female). Of 91 patients, 96% had moderate or complex defects. Of 103 admissions, 45% were through the emergency department. The most common reasons for admission were arrhythmia (37%) and heart failure (28%); 29% of admissions included a stay in the ICU. The mean number of consultations by other services was 2.0. Electrophysiology and anaesthesiology departments were most frequently consulted. After removing outliers, the mean length of stay was 7.9±7.4 days (median=5 days). The length of stay was longer for patients admitted for heart failure (12.2±10.3 days; p=0.001) and admitted directly to the ward (9.6±8.9 days; p=0.009).ConclusionsAmong non-electively hospitalised adults with CHD in a tertiary-care centre, management often entails an interdisciplinary approach, and the length of stay is longest for patients admitted with heart failure. The healthcare system must ensure optimal resources to maintain high-quality care for this expanding patient population.

Background Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. Methods Among 12 644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. Results Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. Conclusions Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.

In women with valvular heart disease, pregnancy-associated cardiovascular changes can contribute to maternal, foetal and neonatal complications. Ideally, a woman with valvular heart disease should receive preconception assessment and counselling from a cardiologist with expertise in pregnancy. For women with moderate- and high-risk valve lesions, appropriate risk stratification and management during pregnancy will optimise outcomes. Pregnancy in women with high-risk lesions, such as severe aortic stenosis, severe mitral stenosis and those with mechanical valves, requires careful planning and coordination of antenatal care by a multidisciplinary team. The purpose of this overview is to describe the expected haemodynamic changes in pregnancy, review pregnancy risks for women with valvular heart disease and discuss strategies for management.

Heart disease, present in 0.5% to 3% of pregnant women, is an important cause of morbidity and the leading cause of death among pregnant women in the developed world. Certain heart conditions are associated with an increased risk of heart failure during pregnancy or the postpartum period; for these conditions, management during pregnancy benefits from multidisciplinary care at a center with expertise in pregnancy and heart disease. This article focuses on cardiac risks and management strategies for women with acquired and congenital heart disease who are at increased risk of heart failure during pregnancy.

To assess the impact of aortic root asymmetry on the relationship between aortic dimensions derived from two-dimensional transthoracic echocardiography (TTE) as compared with cross-sectional cardiovascular magnetic resonance (CMR) imaging in adults with a bicuspid aortic valve (BAV). Maximal CMR cross-sectional aortic measurements at the level of the sinuses of Valsalva, including cusp-commissure, cusp-cusp diameters and aortic root areas, from 68 consecutive patients (65% male) were retrospectively analyzed. The degree of aortic root asymmetry on CMR was expressed using the coefficient of variance of the root diameters in each dimension for an individual (CoeffVi) as compared with the median of the entire population (CoeffVp) and asymmetry was defined as CoeffVi > CoeffVp. Values obtained from CMR were compared with standard root measurements using TTE from contemporary studies (48 patients, 71%). Reproducibility of CMR measurements was assessed using the intra-class correlation coefficient (ICC). Echocardiography systematically underestimated aortic root dimensions in comparison with CMR, particularly in asymmetric roots with cusp-cusp measurements in systole (bias: -4.9 mm). Best agreement between modalities existed in symmetric roots with cusp-commissure measurements in diastole (bias: -0.01 mm). CMR measurements showed excellent intra-reader (ICC ≥ 0.98) and moderate inter-reader (ICC range 0.37-0.95) reproducibility, particularly aortic root area (inter/intra-reader ICC ≥ 0.94). In comparison to cross-sectional CMR diameters, standard TTE measurements consistently underestimates maximum aortic root diameter in adults with a BAV and aortic root asymmetry further decreases the agreement between CMR and TTE. CMR-derived aortic root measurements are reproducible and aortic root area showed the best reproducibility.

Young women with heart disease are increasingly being seen in obstetrical referral centres owing, in large part, to the dramatic improvements in survival of young adults with congenital heart disease in recent years. Although pregnancies in most women with heart disease result in favourable outcomes, there are important exceptions that must be recognized. These exceptions pose a significant mortality risk to the mother and/or the fetus. The present article provides a general framework for the classification of congenital heart lesions in pregnant women as well as a detailed lesion-specific review.

Young women with heart disease are increasingly being seen in obstetrical referral centres owing, in large part, to the dramatic improvements in survival of young adults with congenital heart disease in recent years. Although pregnancies in most women with heart disease result in favourable outcomes, there are important exceptions that must be recognized. These exceptions pose significant mortality risk to the mother and/or the fetus. The present article outlines cardiovascular adaptations to pregnancy, general outcomes and management considerations for practitioners caring for pregnant young women with congenital heart disease. A lesion-specific review is published in a complementary article.

Objective: Women with congenital heart disease (CHD) are at risk for adverse cardiac events during pregnancy; however, the risk of events late after pregnancy (late cardiac events; LCE) has not been well studied. A study was undertaken to examine the frequency and determinants of LCE in a large cohort of women with CHD. Design: Baseline characteristics and pregnancy were prospectively recorded. LCE (>6 months after delivery) were determined by chart review. Survival analysis was used to determine the risk factors for LCE. Setting: A tertiary care referral hospital. Patients: The outcomes of 405 pregnancies were studied (318 women; median follow-up 2.6 years). Main outcome measures: LCE included cardiac death/ arrest, pulmonary oedema, arrhythmia or stroke. Results: LCE occurred after 12% (50/405) of pregnancies. The 5-year rate of LCE was higher in women with adverse cardiac events during pregnancy than in those without (27±9% vs 15±3%, HR 2.2, p=0.02). Women at highest risk for LCE were those with functional limitations/cyanosis (HR 3.9, 95% CI 1.2 to 13.0), subaortic ventricular dysfunction (HR 3.0, 95% CI 1.4 to 6.6), subpulmonary ventricular dysfunction and/or significant pulmonary regurgitation (HR 3.2, 95% CI 1.6 to 6.6), left heart obstruction (HR 2.6, 95% CI 1.2 to 5.2) and cardiac events before or during pregnancy (HR 2.6, 95% CI 1.3 to 4.9). In women with 0, 1 or >1 risk predictors the 5-year rate of LCE was 762%, 2365% and 44610%, respectively (p

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder that affects multiple organ systems, primarily the cardiovascular, ocular and skeletal. It is the most common inherited condition affecting the heart and the aorta, occurring in 1:5000–1:9800 people. There is no ethnic or gender predisposition; 20 to 35% of cases arise fromde novomutations.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

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.

Adult congenital heart disease (ACHD) patients face unique medical and social challenges that may contribute to psychological difficulties. The goals of this study were to identify predictors of symptoms of depression and anxiety and evaluate the prevalence of mood and anxiety disorders among North American ACHD patients.In this cross-sectional study, consecutive patients were recruited from two ACHD outpatient clinics. All patients completed self-report psychosocial measures and a subset was randomly selected to participate in structured clinical interviews. Linear regression models were used to predict symptoms of depression and anxiety.A total of 280 patients (mean age=32 years; 52% female) completed self-report measures. Sixty percent had defects of moderate complexity and 31% had defects of great complexity. Significant predictors of depressive symptoms were loneliness (p0.001), perceived health status (p0.001), and fear of negative evaluation (p=0.02). Predictors of anxiety symptoms were loneliness (p0.001) and fear of negative evaluation (p0.001). Disease severity and functional class did not predict mood or anxiety symptoms. Fifty percent of interviewed patients (29/58) met diagnostic criteria for at least one lifetime mood or anxiety disorder, of whom 39% had never received any mental health treatment.The results confirm an increased risk and under-treatment of mood and anxiety disorders in ACHD patients. Social adjustment and patient-perceived health status were more predictive of depression and anxiety than medical variables. These factors are modifiable and therefore a potential focus of intervention.

Prophylactic aortic root replacement is the main factor explaining the increase in median probability of survival in Marfan syndrome (MFS) patients. In women with MFS, earlier root replacement has been suggested as a strategy to facilitate safe pregnancy. However, in this case report a pregnant patient with MFS who sustained a Type B aortic dissection in her second trimester, with fatal outcome, despite having had a prophylactic aortic root replacement five months prior to conception is described.

Background A study was conducted to determine the long-term results of aortic valve reimplantation to treat aortic root aneurysm. Methods Prospective follow-up with clinical assessments and echocardiography was done of 167 consecutive patients who had reimplantation of the aortic valve as treatment of aortic root aneurysm. Their mean age was 45 ± 15 years, 78% were men, 38% had Marfan syndrome, 14% had aortic dissection, and 7% had bicuspid aortic valve. The aortic valve was reimplanted into a straight Dacron (Dupont, Wilmington, DE) tube in 89 patients and in a Dacron tube with creation of neoaortic sinuses in 78. Aortic cusp repair was performed in 66 patients, and the free margin was reinforced with a fine Gore-Tex suture (W.L. Gore & Assoc, Flagstaff, AZ) in 36. The mean follow-up was 5.1 ± 3.8 years and was 100% complete. Results There were two operative and six late deaths. Survival at 10 years was 92% ± 3%. Moderate aortic insufficiency developed in 3 patients, and severe developed in 2. Freedom from moderate or severe aortic insufficiency was 94% ± 4% at 10 years. Two patients required aortic valve replacement. Freedom from aortic valve replacement was 95% ± 4% at 10 years. At the latest follow-up, 90% of the patients were in New York Heart Association functional class I and 10% were in class II. Conclusions Reimplantation of the aortic valve to treat patients with aortic root aneurysm is associated with excellent long-term survival and low rates of valve-related complications. Reimplantation of the aortic valve is a durable type of aortic valve repair.

Background The mechanistic basis of the proposed relationship between maternal cardiac output and neonatal complications in pregnant women with heart disease has not been well elucidated. Methods and Results Pregnant women with cardiac disease and healthy pregnant women (controls) were prospectively followed with maternal echocardiography and obstetrical ultrasound scans at baseline, third trimester, and postpartum. Fetal/neonatal complications (death, small‐for‐gestational‐age or low birthweight, prematurity, respiratory distress syndrome, or intraventricular hemorrhage) comprised the primary study outcome. One hundred and twenty‐seven women with cardiac disease and 45 healthy controls were enrolled. Neonatal events occurred in 28 pregnancies and were more frequent in the heart disease group as compared with controls (n=26/127 or 21% versus n=2/45 or 4%; P =0.01). Multiple complications in an infant were counted as a single outcome event. Neonatal complications in the heart disease group were small‐for‐gestational‐age/low birthweight (n=18), prematurity (n=14), and intraventricular hemorrhage/respiratory distress syndrome (n=5). Preexisting obstetric risk factors ( P =0.003), maternal cardiac output decline from baseline to third trimester ( P =0.017), and third trimester umbilical artery Doppler abnormalities ( P< 0.001) independently predicted neonatal complications and were incorporated into a novel risk index in which 0, 1, and >1 predictor corresponded to expected complication rates of 5%, 30%, and 76%, respectively. Conclusions Decline in maternal cardiac output during pregnancy and abnormal umbilical artery Doppler flows independently predict neonatal complications. These findings will enhance the identification of higher risk pregnancies that would benefit from close antenatal surveillance.

We evaluated outcomes in patients requiring late reoperation after tetralogy of Fallot (ToF) repair to identify risk factors for arrhythmia and determine whether arrhythmia surgery decreased the risk of subsequent death or recurrent arrhythmia.Review was performed of all ToF patients from 1969 to 2005 undergoing reoperation late (1 year) after repair. Patients with associated lesions, except pulmonary atresia, were included. A total of 249 patients had 278 reoperations. Procedures at initial reoperation included pulmonary valve replacement (PVR) in 217, ablation in 63, and tricuspid valve repair/replacement in 46. Pre-reoperative arrhythmias were present in 75, including supraventricular tachycardia (SVT) in 31, ventricular tachycardia (VT) in 34, and SVT+VT in 10 patients.Median age at reoperation was 23 years (range, 1 to 63). Ten-year survival after reoperation was 93%, and was independent of arrhythmia status (p = 0.86). Arrhythmia patients were characterized by older age at initial repair and at late reoperation, tricuspid and pulmonary regurgitation, and longer QRS duration (p0.001 for all). Risk factors for post-reoperative recurrent arrhythmia were longer QRS duration and not having PVR. Longer QRS duration, with a cut-point of more than 160 msec, was associated with recurrent SVT (p = 0.004). Supraventricular tachycardia ablation improved arrhythmia-free survival (75% versus 33%, p0.001) but VT ablation did not (96% versus 95%, p = 0.50). However, recurrent VT occurred in only 3 patients (10%).Late mortality in patients undergoing reoperation after ToF repair is not impacted by pre-reoperative arrhythmia. Prolongation of QRS identifies patients at risk for recurrent VT and SVT, but recurrent VT is uncommon. Early PVR, and surgical ablation in patients with SVT, decreases arrhythmic risk.

In women with heart disease, sustained arrhythmias can result in an increased risk to the mother and fetus. The purpose of this study was to determine the recurrence rates of arrhythmias during pregnancy in women with cardiac rhythm disorders and examine the impact on fetal and neonatal outcomes. Women with tachyarrhythmias before pregnancy who underwent obstetric care at the Toronto General and Mount Sinai Hospitals from 1990 to 2002 were included. The recurrence rates of arrhythmias were calculated. A multivariate logistic model was used to identify predictors of fetal complications. Seventy-three women had 87 pregnancies; 36 pregnancies were in women with a history of paroxysmal supraventricular tachycardia, 23 with paroxysmal atrial fibrillation or atrial flutter (AF/Afl), 6 with persistent AF/Afl, and 22 with ventricular tachycardia. In the women in sinus rhythm at baseline, 44% (36 of 81 pregnancies) developed recurrences of tachyarrhythmias during pregnancy or in the early postpartum period. The specific recurrence rates during pregnancy in women with a history of supraventricular tachycardia, paroxysmal AF/Afl, and ventricular tachycardia were 50%, 52%, and 27%, respectively. The 6 women in AF/Afl at baseline remained in this rhythm throughout their pregnancy. Adverse fetal events occurred in 17 of the 87 pregnancies (20%). Adverse fetal events occurred more commonly in women who developed antepartum arrhythmias (RR 3.4, 95% confidence interval 1.0 to 11.0, p = 0.045) compared with those who did not. In conclusion, in women with preexisting cardiac rhythm disorders, exacerbation of arrhythmia during pregnancy is common. Recurrence of arrhythmia during the antepartum period increases the risk of adverse fetal complications, independent of other maternal and fetal risk factors.

The timing of pulmonary valve replacement in adult patients with repaired tetralogy of Fallot remains controversial. A magnetic resonance imaging study in 17 adult patients with repaired tetralogy of Fallot reveals a statistically significant decrease in right ventricular (RV) volume (RV end-diastolic volume 163 +/- 34 to 107 +/- 26 ml/m2, p0.001; RV end-systolic volume 109 +/- 27 to 69 +/- 22 ml/m2, p0.001) at a mean follow-up of 21 months after pulmonary valve replacement; whereas RV systolic function remained unchanged (mean RV ejection fraction 32 +/- 7% to 34 +/- 10%, p = 0.12). In no patients with a RV end-diastolic volume170 ml/m2 or a RV end-systolic volume85 ml/m2 before pulmonary valve replacement were RV volumes "normalized" after surgery.

Women with congenital heart disease constitute the majority of cardiac patients encountered in pregnancy, but nevertheless are only infrequently found in the practice of most medical cardiologists and high-risk obstetricians. The patients need guidance regarding contraception and pregnancy, and their various caregivers must share their expertise to promote optimal outcomes. Though maternal mortality is rare except in patients with Eisenmenger syndrome, pulmonary vascular obstructive disease and Marfan syndrome with aortopathy, maternal cardiac complications of pregnancy are more common. As well, women with heart disease are at higher risk for adverse fetal and neonatal outcomes. A review of general and lesion-specific risks and complications of pregnancy and an approach to management are outlined.

This report examines early and intermediate-term outcomes in a contemporary cohort of women with congenital aortic stenosis (AS) who became pregnant. We examined changes in the aortic valve area (AVA) and peak gradients before, during, and after pregnancy in a subgroup of patients in whom serial echocardiography was performed.  Consecutive women with congenital AS receiving obstetric care at the Toronto General and Mount Sinai Hospitals between 1986 and 2000 were included in the study. Congenital AS was defi ned as stenosis secondary to bicuspid aortic valves, subvalvular, or supravalvular lesions. We excluded 22 women with bicuspid aortic valves and low peak aortic gradients (20 mm Hg), prosthetic heart valves, or hypertrophic cardiomyopathy. Five women who experienced miscarriage or underwent termination were also excluded (1 miscarriage, 4 terminations). As part of the ongoing Pregnancy and Heart Disease Research Program at the University of Toronto, detailed clinical data were obtained during the antepartum (baseline), peripartum, and postpartum periods. Thirty-four women who underwent pregnancies during 1986 to 1999 were also included in larger

Background — Pregnant women with heart disease (HD) are at increased risk for cardiac (CV) complications. However, the frequency of neonatal (NE) complications in pregnant women with HD relative to pregnant women without HD has not been examined. Methods and Results — Pregnant women with HD were prospectively monitored during 302 pregnancies. The frequency of NE and CV complications was compared with those in a control group without HD during 572 pregnancies. The frequency of NE complications was higher in the HD group (18% versus 7%; HD versus controls). The NE complication rate was lowest in pregnancies of women age 20 to 35 years who did not smoke during pregnancy, did not receive anticoagulants, and had no obstetric risk factors: 4% in control patients, 5% in HD patients with no cardiac risk factors for NE complications (left heart obstruction, poor functional class, or cyanosis), and 7% in HD patients with ≥1 such risk factor. In contrast, the event rate in pregnancies of controls age 35 years who had obstetric risk factors or multiple gestation or who smoked was 11%. In the HD group, women age 35 years who had obstetric risk factors or multiple gestation, who smoked, or who received anticoagulants experienced an even higher NE complication rate (27% with no cardiac risks for NE events and 33% in the presence of ≥1 cardiac risk factors). The frequency of CV complications was higher in the HD group (17% versus 0%; HD versus controls). Conclusion — Pregnant women with HD are at increased risk for both NE and CV complications. The risk for NE adverse events in pregnant women with HD is highest in those with both obstetric and cardiac risk factors for NE complications.

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

Background: Do adult Fontans with RV-dominant single ventricle physiology (RV) have worse prognosis versus patients with LV-dominance (LV) or unseptated ventricles? Methods: 180 adult Fontans (median age 25) were reviewed. Endpoints relating to Fontan failure and exercise capacity were compared.

Isolated partial anomalous pulmonary venous connection with an intact inter-atrial septum is a relatively uncommon condition, with hemodynamic impact similar to the ones observed in patients with an atrial septal defect. Patients with a single anomalous vein are usually asymptomatic; generally such

Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease.Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029.Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot.Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.

Pregnancy in most women with heart disease has a favourable maternal and fetal outcome. With the exception of patients with Eisenmenger syndrome, pulmonary vascular obstructive disease, and Marfan syndrome with aortopathy, maternal death during pregnancy in women with heart disease is rare.1-4 However, pregnant women with heart disease do remain at risk for other complications including heart failure, arrhythmia, and stroke. Women with congenital heart disease now comprise the majority of pregnant women with heart disease seen at referral centres. The next largest group includes women with rheumatic heart disease. Peripartum cardiomyopathy, though infrequent, will be discussed in view of its unique relation to pregnancy. Two groups of conditions not discussed further are coronary artery disease, infrequently encountered, and isolated mitral valve prolapse, which generally has an excellent outcome. Hormonally mediated increases in blood volume, red cell mass, and heart rate result in a major increase in cardiac output during pregnancy; cardiac output peaks during the second trimester, and remains constant until term. Gestational hormones, circulating prostaglandins, and the low resistance vascular bed in the placenta result in concomitant decreases in peripheral vascular resistance and blood pressure. During labour and delivery, pain and uterine contractions result in additional increases in cardiac output and blood pressure. Immediately following delivery, relief of caval compression and autotransfusion from the emptied and contracted uterus produce a further increase in cardiac output. Most haemodynamic changes of pregnancy resolve by two weeks postpartum.5 ### Congenital heart lesions #### Left to right shunts The effect of increase in cardiac output on the volume loaded right ventricle in atrial septal defect ( ASD), or the left ventricle in ventricular septal defect ( VSD) and patent ductus arteriosus , is counterbalanced by the decrease in peripheral vascular resistance. Consequently, the increase in volume overload is attenuated. In the absence of pulmonary hypertension, pregnancy, labour and delivery are well …

We report the first use of a stented pulmonic valve within the right atrial to right ventricular conduit of a patient who underwent a Bjork modification Fontan for tricuspid atresia. In effect, a functional tricuspid valve was created. This case report demonstrates the feasibility of transcatheter valve replacement in positions other than the pulmonic or aortic valve and that future revision of conduits in the Bjork modification Fontan can be performed percutaneously.

Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital condition seen in the adult population, with most patients developing symptoms in infancy. We describe successful closure of an ALCAPA in an adult using a percutaneous transcatheter approach in a patient with evidence of anterior wall ischemia believed to be the consequence of myocardial steal. A 30-year-old female was noted to have continuous flow in the right ventricular septum on transthoracic echocardiography, with subsequent imaging confirming the presence of an ALCAPA. Myocardial perfusion imaging confirmed anterior wall ischemia, and as a strategy to optimize coronary perfusion pressure, the patient underwent percutaneous ALCAPA closure using an Amplatzer vascular plug. The procedure was well tolerated, with no evidence of anterior ischemia or myocardial dysfunction. Follow up perfusion imaging demonstrated no residual anterior wall ischemia. Transcatheter closure of an ALCAPA is a potentially safe and effective alternative treatment strategy in this patient population.

Background The physiological changes of pregnancy can result in cardiovascular complications in the mother, which in turn may have fetal implications. Prior studies have focused on specific cardiac lesions or identified univariate predictors. There is a need to refine the risk stratification of women with heart disease so they can receive appropriate obstetrical counseling and care. Methods and Results We examined the outcomes of 221 women with heart disease who underwent 276 pregnancies and received their obstetrical care at three Toronto hospitals from 1986 through 1994. Those who underwent therapeutic abortions were excluded. Among the study participants, there were 24 miscarriages and 252 completed pregnancies (pregnancies not ending in miscarriage). Maternal heart failure, arrhythmia, or stroke occurred in 45 completed pregnancies (18%). There were no maternal deaths. Poor maternal functional class or cyanosis, myocardial dysfunction, left heart obstruction, prior arrhythmia, and prior cardiac events were predictive of maternal cardiac complications. These predictors were incorporated into a point score that can be used to estimate the probability of a cardiac complication in the mother. The rate of cardiac complications for a patient with 0, 1, and >1 of the above factors was 3%, 30%, and 66%, respectively. Neonatal complications occurred in 42 completed pregnancies (17%). Neonatal events included death (2), respiratory distress syndrome (16), intraventricular hemorrhage (2), premature birth (35), and small-for-gestational-age birth weight (14). Poor maternal functional class or cyanosis was predictive of neonatal events. Conclusions Despite low maternal and neonatal mortality, pregnancy in women with heart disease is associated with significant cardiac and neonatal morbidity. The probability of maternal cardiac or neonatal events can be predicted from baseline characteristics of the mother.