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The Toronto Adult Congenital Heart Disease (ACHD) Program at the University Health Network, University of Toronto, began in 1959. It traces its origins to a Paul Wood protégé, Dr. John Evans, and to a long-standing and supportive relationship with Hospital for Sick Children (SickKids), located just across the street. Over the decades, the program has grown to become a major center for training and research in ACHD and one of the largest clinical programs for ACHD care globally. This paper recounts the 65-year history of the program, including some of its key individuals, challenges, milestones, innovations, discoveries, and future aspirations., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Background: 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., Objectives: The aim of this study was to examine outcomes in pregnant women with BPVs and the association with SVD., Methods: 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., Results: 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 age >35 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., Conclusions: 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., Competing Interests: Funding Support and Author Disclosures This study was supported by a grant from the Allan E. Tiffin Trust (Toronto General and Western Hospital Foundation). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Complications 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)., Methods: We 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., Results: After 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/m 2 for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m 2 for RV end-systolic volume indexed(sensitivity 80%, specificity 56%)., Conclusions: Previous 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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Although insufficient maternal cardiac output (CO) has been implicated in poor outcomes in mothers with heart disease (HD), maternal-fetal interactions remain incompletely understood. We sought to quantify maternal-fetal hemodynamics with the use of magnetic resonance imaging (MRI) and explore their relationship with adverse events., Methods: Pregnant women with moderate or severe HD (n = 22; mean age 32 ± 5 years) were compared with healthy control women (n = 21; 34 ± 3 years). An MRI was performed during the third trimester at peak output (maternal-fetal) and 6 months postpartum with return of maternal hemodynamics to baseline (reference). Phase-contrast MRI was used for flow quantification and was combined with T1/T2 relaxometry for derivation of fetal oxygen delivery/consumption., Results: Third-trimester CO and cardiac index (CI) measurements were similar in HD and control groups (CO 7.2 ± 1.5 vs 7.3 ± 1.6 L/min, P = 0.79; CI 4.0 ± 0.7 vs 4.3 ± 0.7 L/min/m, 2 P = 0.28). However, the magnitude of CO/CI increase (Δ, peak pregnancy - reference) in the HD group exceeded that in the control group (CO 46 ± 24% vs 27 ± 16% [P = 0.007]; CI 51 ± 28% vs 28 ± 17% [P = 0.005]). Fetal growth and oxygen delivery/consumption were similar between groups. Adverse cardiovascular outcomes (nonmutually exclusive) in 6 HD women included arrhythmia (n = 4), heart failure (n = 2), and hypertensive disorder of pregnancy (n = 1); premature delivery was observed in 2 of these women. The odds of a maternal cardiovascular event were inversely associated with peak CI (odds ratio 0.10, 95% confidence interval 0.001-0.86; P = 0.04) and Δ,CI (0.02, 0.001-0.71; P = 0.03)., Conclusions: Maternal-fetal hemodynamics can be well characterised in pregnancy with the use of MRI. Impaired adaptation to pregnancy in women with HD appears to be associated with development of adverse outcomes of pregnancy., (Copyright © 2021 Canadian Cardiovascular Society. All rights reserved.)

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 cm 2 at baseline vs 1.7 ± 0.6 cm 2 in the third trimester, p = 0.46). There was an increase in the MG (8 ± 3 vs 11 ± 6 mm Hg, p <0.001) and the RVSP (39 ± 14 vs 47 ± 20 mm Hg, p <0.001) during the third trimester. Adverse CE occurred in 45% (25/56) of pregnancies. CE were associated with baseline MG>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., (Copyright © 2021. Published by Elsevier Inc.)

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., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Introduction The 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., Methods: We 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., Results: There 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)., Conclusions: Among 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: 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/m 2 (range 85-214 mL/m 2 ) and median right ventricular ejection fraction (RVEF) 37% (range 30-51%). All women with cardiovascular complications had an RVEF < 35% (range 32-34%). The association between RVEF < 35% and cardiovascular complications trended towards statistical significance (p = 0.05). No statistically significant differences in CMR measurements were found between those with and without neonatal complications., Conclusions: While the majority of women in this cohort had successful outcomes following pregnancy, important cardiovascular complications were seen in a significant minority, all of whom had an RVEF < 35%. The preliminary findings of our study provide impetus for a larger prospective study to evaluate the prognostic role of CMR in pregnant women with atrial redirection surgery.

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., (© 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

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., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

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., (© 2014 The Authors. Congenital Heart Disease Published by Wiley Periodicals, Inc.)

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., (Copyright © 2014. Published by Elsevier Ltd.)

Aims: The aim of this study was to determine associations between aortic morphometry evaluated by cardiovascular magnetic resonance (CMR) and pregnancy outcomes in women with aortic coarctation (CoA)., Methods: Consecutive women with CoA seen with CMR within 2 years of delivery were reviewed. Aortic dimensions were measured on CMR angiography. Adverse outcomes (cardiovascular, obstetric, and foetal/neonatal) were documented., Results: We identified 28 women (4 with native and 24 with repaired CoA) who had 30 pregnancies. There were 29 live births (1 stillbirth) at mean gestation 38 ± 2 weeks. Mean maternal ages at first cardiac intervention and pregnancy were 6 ± 8 and 29 ± 6 years, respectively. There were nine cardiovascular events (hypertensive complications in five; stroke in two and arrhythmia in two) occurring in seven pregnancies. Minimum aortic dimensions were smaller in women with cardiovascular events (12.1 vs. 14.3 mm, P = 0.001), specifically in those with hypertensive complications (11.6 vs. 14.4 mm, P < 0.001). From receiver operator curve analysis, optimal discrimination for the development of adverse cardiovascular events occurred at the 12 mm diameter threshold [sensitivity 78%, specificity 91%, area under the curve 0.86 (95% CI: 0.685-1)]. All hypertensive events occurred in conjunction with a minimum aortic diameter of 12 mm (7mm/m(2)) or less. No adverse outcomes occurred if minimum diameter exceeded 15 mm., Conclusion: Smaller aortic dimensions relate to increased risk of hypertensive events in pregnant women with CoA. CMR can aid in stratification of risk for women with CoA who are considering pregnancy.

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., (Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.)

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.

Background: Most adults with congenital heart disease (CHD) are interested in discussing matters related to advance care planning (ACP) early in the disease course, yet few such conversations actually occur. We aimed to evaluate factors that impact these discussions between patients and adult CHD providers., Methods: Two hundred adult CHD outpatients completed a survey that included factors that might impact ACP discussions with their doctors. In parallel, forty-eight providers within the Canadian Adult Congenital Heart Network completed a similar online survey. Responses were compared between the groups., Results: Most providers (85%) worried that they were unable to reliably estimate life expectancy and believed that patients were not ready for end-of-life discussions if their estimated life expectancies were beyond 5 years (63%) or beyond 10 years (79%). In contrast, only 24% of patients, independent of disease complexity, thought they were not ready to talk about ACP. Most providers (83%) reported that greater certainty about patients' prognoses would help them discuss ACP. Patients thought that such discussions were best facilitated when they had trust in their doctors (85%) and believed their doctors are good at taking care of patients with CHD (78%)., Conclusion: Despite the fact that challenges to prognostication exist, discussions about ACP should not be reserved for patients with a severely reduced life expectancy. Most patients want these discussions regardless of the complexity of their disease. The trusting and close patient-doctor relationship in adult CHD, often evolving over many years, may provide an excellent platform from which to initiate such discussions., (© 2012 Wiley Periodicals, Inc.)

Objectives: Recommendations for the peripartum obstetric management of women with heart disease have included early induction of labour, shortening the second stage of labour during vaginal delivery, and low threshold for elective Caesarean section, although such techniques may result in complications. The objective of this study was to determine whether a less aggressive approach without routine preterm induction, shortening of the second stage, or Caesarean section adversely affects the mother or neonate., Methods: We examined peripartum obstetric management and its relationship with adverse maternal and neonatal outcomes in 1677 pregnancies: 559 in women with heart disease and 1118 in women without heart disease (control subjects). Logistic regression with propensity matching was used to compare outcomes in women with and without heart disease., Results: Women with heart disease were more likely than control subjects to undergo induction of labour (P < 0.001). Induction of labour tended to be at term and for logistical reasons, not for the indication of maternal heart disease. Assisted vaginal deliveries were more common in women with heart disease (29% vs. 11%, P < 0.001) than in those without, and the second stage of labour was also more prolonged in women with heart disease. Rates of Caesarean section were similar in both groups (P = 0.66). A significant proportion of women with heart disease had unassisted vaginal deliveries. Invasive cardiac monitoring was rarely used. Adverse maternal cardiac events at delivery were rare (2% of pregnancies) and were not associated with mode of delivery. In multivariate analysis, maternal heart disease was not predictive of adverse neonatal events or third- or fourth- degree lacerations. Maternal heart disease was associated with postpartum hemorrhage, but this was not related to assisted delivery or prolonged second stage of labour., Conclusion: This large study has shown that in women with heart disease, avoidance of early induction of labour, rare use of Caesarean section for cardiac indications, and selective use of invasive monitoring produces safe obstetric outcomes.

Congenital heart disease (CHD) is a chronic illness. Few adults with CHD are cured and those with disease of moderate or great complexity remain at risk of premature death. Current adult CHD guidelines recommend that providers encourage their patients to complete advance directives. We evaluated the prevalence of completed advance directives by and the preference for information about life expectancy of outpatients at a large adult CHD program. Two hundred patients with CHD (52% men, 35 ± 15 years old, range 18 to 79, 81% with disease of moderate or great complexity) completed a survey that assessed knowledge of advance directives and nature of and preferences for advance care planning. Only 5% of patients reported that they had completed advance directives; 56% had never heard of them. However, most patients (87%) reported that they would prefer to have an advance directive available if they were dealing with their own dying and were unable to speak for themselves. Patients who had formally identified substitute decision makers (n = 34) were typically older (47 ± 16 vs 33 ± 13 years, p <0.001) and more likely to have partners (30% vs 6%, p <0.001). Most patients (70%) reported that they wanted general information about the average life expectancy for patients with their heart condition. In conclusion, in contrast to recommendations from published guidelines, advance care planning documents are infrequently completed by outpatients. Health care providers caring for patients with CHD should educate their patients about advance directives and assist them in preparing formal end-of-life-planning documents., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: We investigated preferences of adults with congenital heart disease (CHD) and their health care providers regarding end-of-life (EOL) communication., Methods: Adult CHD outpatients and health care providers completed surveys about preferences for and experiences with EOL communication. Responses were compared between patients and providers., Results: Two hundred patients (18-79 years) and 48 CHD health care providers (primarily cardiologists) completed surveys. Only 2 patients (1%) indicated that they had discussed EOL planning with their medical team. In contrast, 50% of providers reported that they typically discuss issues including life expectancy, advance planning, and resuscitation preferences with their outpatients. Seventy-eight percent (156/199) of patients wanted their medical team to raise EOL issues; this preference was independent of disease complexity and socio-demographic factors. In contrast, providers reported that their EOL discussions increase in accordance with disease complexity (p<0.001). Early initiation of EOL discussions, before diagnosis with life-threatening complications, was favored by 62% of patients but only 38% of providers (p<0.001)., Conclusion: Health professionals caring for adults with CHD should explore preferences of their patients for EOL discussions earlier in the disease course, and not only with patients facing life-threatening complications and/or with complex conditions. When EOL discussions do occur, health care providers should attempt to ensure that patients better understand these conversations. Increased attention to EOL issues is proposed in order to improve the care of patients with CHD across the lifespan., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

There are a growing number of women with congenital heart disease reaching adulthood and contemplating and/or undergoing pregnancy. However, pregnancy imposes hemodynamic stress on the heart and this can result in maternal, fetal and neonatal complications. Most women with congenital heart disease do well during pregnancy, but some women with high-risk cardiac lesions will not tolerate the hemodynamic changes of pregnancy. Physicians must be aware of the potential risks for the mother both during and after pregnancy, the risks to the fetus and neonate, and the risks and benefits of medications and procedures used during pregnancy. For women with complex cardiac conditions, management during pregnancy benefits from multidisciplinary care involving cardiologists with expertise in pregnancy, obstetricians with expertise in maternal fetal medicine, neonatologists and obstetric anesthetists, among others. This review will focus on the cardiac risks faced by women with congenital heart disease; particularly those at high risk, and on management strategies to mitigate risk and address cardiac complications.

Adults with congenital heart disease represent a growing patient population. Notwithstanding dramatic improvements in survival and life expectancy over recent decades, many of these patients remain at risk of premature death from progressive heart disease and would benefit from the principles of palliative and end-of-life care. Data on end-of-life care in this patient group are, however, lacking. We report a retrospective study of 48 patients with congenital heart disease who died while admitted to our hospital (mean age at death 37 ± 14 years). We describe circumstances of death, end-of-life discussions, and the provision of end-of-life care. The majority of patients had complex congenital heart disease and were considered to be in the end stage of their disease. Despite this, only a minority of patients had documented end-of-life discussions prior to their terminal admission and most received continuing aggressive medical treatment up to their demise. Advanced palliative and end-of-life care strategies should be developed for and provided to this group of patients, with the dual aims of reduction of unwarranted therapies and enhancement of the quality of death and dying.

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.

There is a growing population of young adults with tetralogy of Fallot. Although surgical approaches have evolved, many adults with repaired tetralogy of Fallot have been left with residual pulmonary regurgitation. Pulmonary regurgitation is an important contributor to a number of late complications including exercise limitations, right heart failure, arrhythmia, and sudden death. Because bioprosthetic valves are used in this population, clinicians must weigh the beneficial effects of pulmonary valve replacement against the associated risks, including subsequent re-operation. In this review, we will appraise the evidence supporting pulmonary valve replacement in the adult with repaired tetralogy of Fallot, as well as the optimal timing and mode of intervention., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objectives: The objectives of this study were to examine: 1) B-type natriuretic peptide (BNP) response to pregnancy in women with heart disease; and 2) the relationship between BNP levels and adverse maternal cardiac events during pregnancy., Background: Pregnancy imposes a hemodynamic stress on the heart. BNP might be a useful biomarker to assess the ability of the heart to adapt to the hemodynamic load of pregnancy., Methods: This was a prospective study of women with structural heart disease seen at our center. Serial clinical data and plasma BNP measurements were obtained during the first trimester, third trimester, and after delivery (>6 weeks)., Results: Seventy-eight pregnant women were studied; 66 women with heart disease (age 31 ± 5 years), and 12 healthy women (age 33 ± 5 years). During pregnancy, the median peak BNP level was higher in women with heart disease compared with control subjects (median 79, interquartile range 51 to 152 pg/ml vs. median 35, interquartile range 21 to 43 pg/ml, p < 0.001). In women with heart disease, those with subaortic ventricular dysfunction had higher BNP levels (p = 0.03). A BNP >100 pg/ml was measured in all women with events during pregnancy (n = 8). Sixteen women had increased BNP levels during pregnancy but did not have clinical events. None of the women with BNP ≤100 pg/ml had events. BNP ≤100 pg/ml had a negative predictive value of 100% for identifying events during pregnancy., Conclusions: Many pregnant women with heart disease have increased BNP levels during pregnancy. Incorporating serial BNP levels in into clinical practice can be helpful, specifically in adjudicating suspected adverse cardiac events during pregnancy., (Copyright © 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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 (>6months 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&emsp14;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 7±2%, 23±5% and 44±10%, respectively (p<0.001)., Conclusions: In women with CHD, pre-pregnancy maternal characteristics can help to identify women at increased risk for LCE. Adverse cardiac events during pregnancy are important and are associated with an increased risk of LCE.

There is a growing population of young women of child-bearing age with complete transposition of the great arteries (TGA) who have had an arterial switch operation (ASO). Pregnancy imposes a hemodynamic stress on the heart and, therefore, adverse cardiac events can occur during this period; however, pregnancy outcomes in this population have not been well studied. We sought to describe cardiac outcomes during pregnancy in women with TGA who had undergone an ASO in childhood. Women were identified from 2 large tertiary care hospitals. A retrospective chart review was performed to determine the prevalence of adverse maternal cardiac events during pregnancy. Overall, 74 women of child-bearing age were identified, 9 of whom had 17 pregnancies. There were 4 miscarriages. Six women (67%) had clinically important valve (n = 5) and ventricular (n = 1) lesions before the index pregnancy. Two women developed cardiac complications during pregnancy; 1 woman with impaired left ventricular systolic function had nonsustained ventricular tachycardia and 1 woman with a mechanical systemic atrioventricular valve developed postpartum valve thrombosis. There were no maternal deaths. In conclusion, young women with TGA from this early cohort repaired with ASO are reaching child-bearing age. A significant proportion have residua and/or sequelae that can confer risk for adverse cardiac events in pregnancy. Co-ordinated care between a congenital heart disease specialist and a high-risk obstetrician should be implemented., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

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., (Copyright (c) 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

There are a number of different anticoagulation options for pregnant women with mechanical heart valves. The purpose of this study was to examine maternal thromboembolic complications in women with mechanical valves treated with low-molecular weight heparin (LMWH) throughout pregnancy. This was a substudy of a larger prospective cohort study of pregnant women with heart disease followed from 1998 to 2008. All pregnant women with mechanical left-sided valves who were treated with LMWH throughout pregnancy were included. Maternal thromboembolic events were defined as valve thrombosis, need for valve replacement, or stroke during pregnancy or postpartum (up to 6 months). Twenty-three pregnancies (17 women) occurred in women treated with LMWH and low-dose aspirin: 15 in women with mechanical mitral valves, 9 in women with mechanical aortic valves, and 1 in a woman with both. There was 1 maternal thromboembolic event (4%), which resulted in maternal and fetal death. Five women (22%) developed other adverse cardiac events during pregnancy. Nine pregnancies (43%) had fetal or neonatal adverse events, 5 of which had favorable outcomes. Three pregnancies were complicated by postpartum hemorrhage. In conclusion, carefully monitored LMWH may be a suitable anticoagulation strategy in pregnant women with mechanical heart valves who are unwilling to use warfarin. However, this group of women remains at risk for maternal cardiac and fetal complications. The occurrence of valve thrombosis resulting in maternal death despite therapeutic anti-Xa levels highlights current limitations with anticoagulation in this population.

Aims: To examine the prevalence of sustained ventricular tachycardia (VT) and sudden death (SD) in adults with atrial repair of transposition of the great arteries (TGA) and to determine associated risk factors., Methods and Results: In a single-centre review, we studied the outcome of 149 adults (mean age 28 +/- 7 years) who had undergone a Mustard operation for TGA. During a mean follow-up of 9 +/- 6 years, sustained VT and/or SD occurred in 9% (13/149) of the cohort. Sustained VT/SD was more likely to occur in patients with associated anatomic lesions [hazard ratio (HR) 4.9, 95% CI 1.5-16.0], with NYHA class >or=III (HR 9.8, 95% CI 3.0-31.6) and with an impaired subaortic right ventricular (RV) ejection fraction (EF) (HR 2.2, 95% CI 1.2-4.0 per 10% decrease in EF). There was an inverse correlation between the RV-EF and both age and QRS duration. Patients with a QRS duration >or=140 ms were at highest risk of sustained VT/SD (HR 13.6, 95% CI 2.9-63.4). Atrial tachyarrhythmia was detected in 66 (44%) patients, but was not a statistically significant predictor of sustained VT/SD in our adult population (HR 2.7, 95% CI 0.6-13.0)., Conclusion: Sustained VT/SD in adults after a Mustard operation for TGA are more common than previously described. Age, systemic ventricular function, and QRS duration are interrelated and are associated with VT/SD. A QRS duration >or=140 ms helps to identify the high risk patient.

Background: Late cardiac outcomes in women with aortic stenosis (AS) who have undergone pregnancy have not been well defined., Methods: We examined 51 consecutive women with congenital AS who underwent 70 pregnancies. Late cardiac events (pulmonary edema, cardiac arrhythmia, cardiac death, cardiac interventions >1 year since baseline evaluation) were the outcome of interest. The frequency of late cardiac events in the postpregnant group were compared to age- and lesion-matched women with congenital AS who have never been pregnant., Results: During the follow-up period (6 + 4 years), 43% of women underwent cardiac interventions which comprised all late cardiac events. Independent baseline predictors of late cardiac events were (1) moderate or severe AS (hazard ratio = 4.5, P = .045) and (2) New York Functional Class II (hazard ratio = 4.6, P = .014). When outcomes in 26 women from the postpregnant group were compared to 26 age- and lesion-matched women who have never been pregnant, the postpregnant group had a higher late cardiac event rate than the never-pregnant group (31% vs 0%, P = .021)., Conclusion: Women with moderate or severe AS and symptomatic during pregnancy are at high likelihood of requiring cardiac interventions late after pregnancy. Women with congenital AS who have undergone pregnancy have a higher frequency of late cardiac events compared to those who have never been pregnant. Late cardiac outcomes after pregnancy should be considered in the counseling of women with AS who are contemplating pregnancy.

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.

Objectives: To examine the results of aortic valve sparing for aortic root aneurysm., Methods: Two hundred twenty consecutive patients who had aortic valve sparing for aortic root aneurysm were prospectively studied with annual clinical assessments and echocardiography. Their mean age was 46 +/- 15 years, 40% had Marfan syndrome, 17% had aortic dissection, and 7% had bicuspid aortic valve. Reimplantation of the aortic valve was performed in 167 patients and remodeling of the aortic root in 53. Aortic cusp repair was performed in 80 patients, and reinforcement of the free margin of one of the cusps with a fine polytetrafluoroethylene (Gore-Tex) suture in 48. The mean follow-up was 5.2 +/- 3.7 years and it was complete., Results: There were 3 operative and 13 late deaths. Patients' survival at 10 years was 88% +/- 3%. Age older than 65 years, advanced functional class, and ejection fraction less than 40% were independent predictors of death. Moderate aortic insufficiency developed in 7 patients and severe insufficiency in 6. Freedom from moderate or severe aortic insufficiency at 10 years was 85% +/- 5% for all patients, but it was 94% +/- 4% after reimplantation and 75% +/- 10% after remodeling (P = .04). Five patients required aortic valve replacement; the freedom from valve replacement at 10 years was 95% +/- 3%. One case of endocarditis developed 11 years postoperatively. At the latest follow-up, 88% of the patients were in functional class I, and 10% were in class II., Conclusions: Aortic valve-sparing operation is associated with low rates of valve-related complications. The probability of late aortic insufficiency was lower after the reimplantation procedure than after remodeling in our experience.

Background: 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., Methods: 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., Results: 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 (p < 0.001 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%, p < 0.001) but VT ablation did not (96% versus 95%, p = 0.50). However, recurrent VT occurred in only 3 patients (10%)., Conclusions: 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.