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Primary cardiac tumors are rare, with benign lesions outnumbering malignant ones by a margin of over four to one. Amongst the benign cardiac neoplasms, papillary fibroelastomas are reported second only to myxomas. Papillary fibroelastomas commonly rise from heart valves and their size ranges from a few millimeters to well over 2.0 cm in diameter. While myxomas commonly arise from the interatrial septum, we present the rare example of a large papillary fibroelastoma arising from the left side of the interatrial septum. [ABSTRACT FROM AUTHOR]

: ObjectivesThis study was designed to determine the feasibility and accuracy of intracardiac echocardiography (ICE) in guiding percutaneous closure of atrial septal defects (ASD).: BackgroundIntracardiac echocardiography is a novel imaging technique that might be used to guide interventional procedures. The sensitivity and specificity of ICE, compared to standard imaging techniques, in detecting potentially adverse procedural events and guiding remedial action will be an important consideration in its use.: MethodsIn a prospective study, 24 patients underwent device closure of ASD using ICE as the primary echocardiographic imaging modality. Feasibility was expressed as proportion of cases in which complete diagnostic ICE imaging was achieved. Accuracy was expressed as the percent agreement between ICE and simultaneously performed transesophageal echocardiography (TEE).: ResultsHigh-quality ICE images were acquired in all patients, though images were limited in two patients with aneurysmal septa. Intracardiac echocardiography successfully guided closure of 24 out of 25 ASDs (96%) in 23 patients. There was close agreement between ICE and TEE in their assessment of device position and the adequacy of septal capture before device release (98%) and in identifying the presence of significant residual shunts. Intracardiac echocardiography detected all potentially adverse events, including four malpositions, and guided appropriate remedial action.: ConclusionsIntracardiac echocardiography guided device closure of secundum ASDs is feasible in the majority of patients and provides diagnostic data comparable to TEE. These data indicate that ICE may be used to guide routine closure of ASDs in adults without the need for TEE and general anesthesia. [Copyright &y& Elsevier]

Background: Atrial flutter and atrial fibrillation are causes of morbidity in adults with an atrial septal defect. In this study, we attempted to identify risk factors for atrial flutter and fibrillation both before and after the surgical closure of an atrial septal defect. Methods: We searched for preoperative and postoperative atrial flutter or fibrillation in 213 adult patients (82 men and 131 women) who underwent surgical closure of atrial septal defects because of symptoms, a substantial left-to-right shunt (ratio of pulmonary to systemic blood flow, >1.5:1), or both at Toronto Hospital between 1986 and 1997. Results: Forty patients (19 percent) had sustained atrial flutter or fibrillation before surgery. As compared with the patients who did not have atrial flutter or fibrillation before surgery, those who did were older (mean [±SD] age, 59±11 vs. 37±13 years, P<0.001) and had higher mean pulmonary arterial pressures (25.0±9.7 vs. 19.7±8.2 mm Hg, P=0.001). There were no perioperative deaths. After a mean follow-up period of 3.8±2.5 years, 24 of the 40 patients (60 percent) continued to have atrial flutter or fibrillation. The mean age of these patients was greater than that of the 16 who converted to sinus rhythm (P=0.02). New-onset atrial flutter or atrial fibrillation was more likely to have developed at follow-up in patients who were older than 40 years at the time of surgery than in those who were 40 or younger (5 of 67 vs. 0 of 106, P=0.008). Late events (those occurring more than one month after surgery) included stroke in six patients (all but one with atrial flutter or fibrillation, one of whom died) and death from noncardiac causes in two patients. Multivariate analysis showed that older age (>40 years) at the time of surgery (P=0.001), the presence of preoperative atrial flutter or fibrillation (P<0.001), and the presence of postoperative atrial flutter or fibrillation or junctional rhythm (P=0.02) were predictive of late postoperative atrial flutter or fibrillation. Conclusions: The risk of atrial flutter or atrial fibrillation in adults with atrial septal defects is related to the age at the time of surgical repair and the pulmonary arterial pressure. To reduce the morbidity associated with atrial flutter and fibrillation, the timely closure of atrial septal defects is warranted. (N Engl J Med 1999;340:839-46.) [ABSTRACT FROM AUTHOR]

Objective: To evaluate a bedside clinical prediction rule for detecting moderate or severe aortic stenosis.Design: Cross-sectional study with independent comparison to a diagnostic reference standard, doppler echocardiography.Setting: Urban university hospital.Participants: Consecutive hospital inpatients (n = 124) who had been referred for echocardiography.Measurements and Main Results: Participants were examined by a third-year general internal medicine resident and a staff general internist. We hypothesized in advance that absence of a murmur over the right clavicle would rule out aortic stenosis, while the presence of three or four associated findings (slow carotid artery upstroke, reduced carotid artery volume, maximal murmur intensity at the second right intercostal space, and reduced intensity of the second heart sound) would rule in aortic stenosis. Study physicians were unaware of echocardiographic findings. The outcome was echocardiographic moderate or severe aortic stenosis, defined as a valve area of 1.2 cm2 or less, or a peak instantaneous gradient of 25 mm Hg or greater. Absence of a murmur over the right clavicle ruled out aortic stenosis (likelihood ratio [LR] 0.10; 95% confidence interval [CI] 0.01, 0.44). The presence of three or four associated findings ruled in aortic stenosis (LR 40; 95% CI 6.6, 240). If a murmur was present over the right clavicle, but no more than two associated findings were present, then the examination was indeterminate (LR 1.8; 95% CI 0.93, 2.9).Conclusion: A clinical prediction rule, using simple bedside maneuvers, accurately ruled in and ruled out aortic stenosis. [ABSTRACT FROM AUTHOR]

Background: Rheumatic heart disease (RHD) remains as 1 of the major contributors to indirect pregnancy-related mortality and morbidity worldwide and disproportionately affects marginalized populations., Objectives: In this scoping review, the authors sought to explore the socioeconomic, cultural, and health care access-related causes of global disparities in outcomes of pregnancy among individuals with RHD., Methods: We performed a literature search of all studies published between January 1, 1990, and January 1, 2022, that investigated causes for disparate outcomes in pregnant individuals with RHD., Results: Of the 3,544 articles identified, 16 were included in the final analysis. The key reasons for disparate outcomes included lack of secondary antibiotic RHD prophylaxis; late and more severe RHD diagnosis, differences in management and antenatal care access; lack of expert and coordinated multidisciplinary care; suboptimal patient health education; inadequate access to RHD medication, intervention and surgery in pregnancy; and limited financial and economic resources., Conclusions: These findings illustrated using a life-course approach demonstrate opportunities for clinical and public health interventions to improve outcomes in this population., Competing Interests: Dr Silversides is the Editor-in-Chief and Dr Siu is the Senior Guest Editor for cardio-obstetrics for JACC: Advances. Dr D’Souza holds a Canada Research Chair in Maternal Health (CRC-2021-00337). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Crown Copyright © 2024 Published by Elsevier on behalf of the American College of Cardiology Foundation.)

Objective: To investigate fetal/neonatal and obstetric events in pregnancies with both maternal and fetal heart disease., Study Design: From the CARPREG database, singleton pregnancies (>24 weeks) in patients with structural heart disease that underwent fetal/neonatal echocardiograms were selected and separated in two groups: maternal heart disease only (M-HD) and maternal and fetal heart disease (MF-HD). Differences in adverse fetal/neonatal (death, preterm birth, and small for gestational age) and obstetric (preeclampsia/eclampsia) outcomes between groups were analyzed., Results: From 1011 pregnancies, 93 had MF-HD. Fetal/neonatal events (38.7% vs 25.3%, p = 0.006) and spontaneous preterm birth (10.8% vs 4.9%, p = 0.021) were more frequent in MF-HD compared to M-HD, with no difference in obstetric events. MF-HD remained as a significant predictor of fetal/neonatal events after adjustment (OR:1.883; 95% CI:1.182-3.000; p = 0.008)., Conclusions: Pregnancies with MF-HD are at risk of adverse fetal/neonatal events and spontaneous preterm birth. Larger studies are needed to determine their association with preeclampsia., (© 2024. The Author(s).)

Objective: Obesity is one of the most prevalent risk factors for hypertensive disorders in pregnancy (HDP); however, the role of pre-pregnancy cardiometabolic health in the development of these conditions is not well understood. Carotid-femoral pulse wave velocity (PWV) is an established measure of arterial stiffness and cardiovascular health and is validated in pregnancy. Our objective was to examine the obesity-related changes in PWV in pregnant individuals with and without HDP., Methods: Eighty-seven individuals with singleton pregnancies were recruited and classified into two groups: cases (HDP: including pre-existing/chronic hypertension, gestational hypertension, preeclampsia, or intrauterine growth restriction (IUGR); n = 39) and normotensive controls (no HDP or IUGR; n = 48). Patient data, including body mass index (BMI), were collected from patient charts. Measurements of PWV were performed weekly until discharge or delivery (gestational age 24-37 weeks) and placental growth factor (PlGF) was measured at routine blood draws., Results: PWV did not significantly change over gestation for either group. Cases had significantly increased PWV and decreased PlGF compared to normotensive controls. An elevated BMI was associated with higher PWV in both cases and controls. Once grouped based on BMI, PWV was only significantly higher in cases with a BMI ≥ 25 kg/m 2 compared to controls, whereas PlGF was less affected by BMI. As PWV increased, PlGF decreased; however, after controlling for BMI, there was no relationship between PWV and PlGF., Conclusion: PWV measurements in early pregnancy may be useful as an additional independent marker to PlGF for risk-stratifying for HDP, especially in individuals with increased BMI., (Copyright © 2024. Published by Elsevier Inc.)

Background: Atrial fibrillation (AF) may increase the risk of adverse maternal and fetal outcomes among pregnant with rheumatic valvular lesions (RHD). We aimed to assess the rate of occurrence of AF in pregnant with RHD and its impact on cardiac and maternal-fetal outcomes compared to those without it., Methods: The study group consisted of pregnant women with RHD and AF (cases) and a matched comparison group of pregnant women with RHD but without AF (controls) was derived from the database of pregnant women with RHD receiving care at our center between 2011 and 2021. Incidence of composite adverse outcomes(maternal death, heart failure, or thrombo-embolic events) and pregnancy outcomes were compared between them., Results: Seventy-one (5.1%; 95%CI 4.1%-6.4%) pregnant women with RHD had AF during pregnancy and childbirth, most occurring in the late second or early third trimester. New-onset AF was diagnosed in 34 (47.9%) of them. After matching, the incidence of composite outcome was higher in women with AF (77.5% (95%CI 66.3%-85.7%) compared to women without AF (17.3%(95%CI 13.3%-22.1%), P < .001), with seven (9.9%) maternal deaths among cases and two (0.7%) in controls. Heart failure was the most common adverse cardiac event (26.7% vs. 4.2%, P < .001, cases vs controls). Those with AF had higher odds (adjusted OR 56.6 (14.1-226.8)) of adverse cardiac outcomes after adjusting for other risk factors. The frequency of most non-cardiac pregnancy complications was similar in both groups. However, there was a trend towards a higher rate of miscarriage (16.9% vs. 9.9%), small-for-gestational-age babies(16.3 vs. 9.0%), and cesarean rates(31.9% vs. 18.3%) women with AF compared to those who did not experience AF., Conclusions: Atrial fibrillation in pregnancy among women with RHD was associated with an increased risk of maternal morbidity and mortality, with a trend towards an increase in some non-cardiac pregnancy complications compared to those pregnant women without AF. Our study results provide background data for developing and implementing a pregnancy-specific management strategy tailored to middle-income settings., Competing Interests: Conflict of interest All authors declare no conflict of interest in relation to this publication., (Copyright © 2024 Elsevier Inc. All rights reserved.)

This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Background: Patients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012., Methods: A survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012., Results: In Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions., Conclusions: Over the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD., (Copyright © 2023 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: The increasing and potentially preventable cardiac events in pregnant patients have led to calls to enhance multidisciplinary cardio-obstetrics education. To design a multidisciplinary cardio-obstetrics curriculum for general cardiology and obstetrics and gynecology (OBGYN) residents, we need to define educational needs from the perspectives of both cardiology and OBGYN residents. Our study characterizes the educational needs of Canadian cardiology and OBGYN residents., Methods: Canadian cardiology and OBGYN residents were surveyed on clinical exposures, perceived needs for topics, unperceived needs for topics (multiple-choice questions) and preferences for educational formats. High priorities were defined as ≥ 50% of responses indicating a perceived need or ≥ 50% indicating an unperceived need., Results: A total of 154 residents participated (cardiology n = 44, OBGYN n = 110). Residents reported insufficient clinical exposure to nearly all cardiac disorders, with 33% of exposures occurring in multidisciplinary contexts. All topics aside from gestational hypertension were rated as high priority on perceived needs by both specialties. High-priority unperceived needs were congenital heart disease (both specialties), pre-existing acquired heart disease (both specialties), medication safety (OBGYN), peripartum management (OBGYN), and pregnancy-related heart disease (OBGYN). Cardiology and OBGYN residents shared preferences for in-person simulation, virtual simulation, and online modules., Conclusions: Residents in both specialties reported low clinical exposure to most cardiac disorders during pregnancy, identified high-priority perceived needs in multiple topics, and shared 2 high-priority unperceived needs. OBGYN residents identified 3 additional high-priority unperceived needs. These data can inform design of multidisciplinary cardio-obstetrics curricula for general cardiology and OBGYN residents., (© 2023 The Authors.)

Background: The COVID-19 pandemic significantly impacted health care access across Canada with the reduction in in-person evaluations. The aim of the study was to examine the effects of the COVID-19 pandemic on access to health care services among the Canadian population with adult congenital heart disease (ACHD)., Methods: All Canadian adult congenital heart affiliated centres were contacted and asked to collect data on outpatient clinic and procedural volumes for the 2019 and 2020 calendar years. A survey was sent detailing questions on clinic and procedural volumes and wait times before and after pandemic restrictions. Descriptive statistics were used with the Student t -test to compare groups., Results: In 2019, there were 19,326 ACHD clinic visits across Canada and only 296 (1.5%) virtual clinic visits. However, during the first year of the pandemic, there were 20,532 clinic visits and 11,412 (56%) virtual visits ( P < 0.0001). There were no differences in procedural volumes (electrophysiology, cardiac surgery, and percutaneous intervention) between 2019 and 2020. The mean estimated wait times (months) before the pandemic vs the pandemic were as follows: nonurgent consult 5.4 ± 2.6 vs 6.6 ± 4.2 ( P  = 0.65), ACHD surgery 6.0 ± 3.5 vs 7.0 ± 4.6 ( P  = 0.47), electrophysiology procedures 6.3 ± 3.3 vs 5.7 ± 3.3 ( P  = 0.72), and percutaneous intervention 4.6 ± 3.9 vs 4.4 ± 2.3 ( P  = 0.74)., Conclusions: During the pandemic and restrictions of social distancing, the use of virtual clinic visits helped to maintain continuity in ACHD clinical care, with 56% of ACHD visits being virtual. The procedural volumes and wait times for consultation and percutaneous and surgical interventions were not delayed., (© 2023 The Authors.)

Background: Transesophageal echocardiography (TEE) conventional multiplane approach (MPA) and the newly proposed commissural-biplane approach (CBA) are the recommended algorithms for identifying the affected mitral valve (MV) segments in the setting of mitral regurgitation. To date, there are no reports to address the diagnostic performance of CBA. In this study we aim to analyze the diagnostic accuracy of CBA and MPA in comparison with three-dimensional echocardiographic findings in patients with severe mitral regurgitation., Methods: We prospectively enrolled 102 patients with severe mitral regurgitation. All patients underwent systematic TEE assessment of MV before surgical intervention to define the affected MV segments/scallops. The standard MPA includes 4-chamber, 2-chamber, long-axis, and commissural views; CBA was performed by obtaining the bicommissural view and simultaneous biplane imaging of the medial, middle, and lateral MV aspects. The findings of both TEE approaches were compared with three-dimensional TEE data to assess the diagnostic accuracy of MPA and CBA., Results: The mean patient age was (65 ± 11) years, and 37 (36.3%) were female. We found that CBA had an overall diagnostic accuracy between 88% and 97% in identifying the abnormal MV scallops; in contrast, MPA accuracy ranged between 82% and 95%. The CBA and MPA were the least accurate in identifying the P3 scallop-88% and 82% respectively; however, both were the most accurate in assessing the A2 segment-95% and 97%, respectively. The sensitivity of identifying commissural abnormalities was 80% with CBA and 30% with MPA. Three-dimensional TEE was found to have a strong agreement with CBA (averaged kappa of 0.81, P < .0001) and a modest agreement with MPA (averaged kappa of 0.61, P < .0001) in identifying abnormal anterior or posterior segments. On the other hand, three-dimensional TEE had a weak agreement with CBA (kappa of 0.43, P < .0001) and no agreement with MPA (kappa of 0.14, P = .153) in the assessment of commissural involvements., Conclusion: The CBA is more accurate than the MPA in the assessment of MV commissural involvement. Given the accuracy differences of the 2 approaches for specific leaflet/scallops, a comprehensive evaluation using both approaches is recommended for all MV scallop assessments., (Copyright © 2023 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background After pregnancy, patients with preexisting heart disease are at high risk for cardiovascular complications. The primary objective was to compare the incidence of new hypertension after pregnancy in patients with and without heart disease. Methods and Results This was a retrospective matched-cohort study comparing the incidence of new hypertension after pregnancy in 832 patients who are pregnant with congenital or acquired heart disease to a comparison group of 1664 patients who are pregnant without heart disease; matching was by demographics and baseline risk for hypertension at the time of the index pregnancy. We also examined whether new hypertension was associated with subsequent death or cardiovascular events. The 20-year cumulative incidence of hypertension was 24% in patients with heart disease, compared with 14% in patients without heart disease (hazard ratio [HR], 1.81 [95% CI, 1.44-2.27]). The median follow-up time at hypertension diagnosis in the heart disease group was 8.1 years (interquartile range, 4.2-11.9 years). The elevated rate of new hypertension was observed not only in patients with ischemic heart disease, but also in those with left-sided valve disease, cardiomyopathy, and congenital heart disease. Pregnancy risk prediction methods can further stratify risk of new hypertension. New hypertension was associated with an increased rate of subsequent death or cardiovascular events (HR, 1.54 [95% CI, 1.05-2.25]). Conclusions Patients with heart disease are at higher risk for developing hypertension in the decades after pregnancy when compared with those without heart disease. New hypertension in this young cohort is associated with adverse cardiovascular events highlighting the importance of systematic and lifelong surveillance.

Background: Poor translation of clinical practice guidelines (CPGs) into clinical practice is a barrier to the provision of consistent and high-quality evidence-based care. The objective was to systematically review the roles and effectiveness of knowledge brokers (KBs) for translating CPGs in health-related settings., Methods: MEDLINE, Embase, PsycINFO and CINAHL Plus were searched from 2014 to June 2022. Randomised controlled trials (RCTs), controlled and uncontrolled preintervention and postintervention studies involving KBs, either alone or as part of a multicomponent intervention, that reported quantitative postintervention changes in guideline implementation in a healthcare setting were included. A KB was defined as an intermediary who facilitated knowledge translation by acting in at least two of the following core roles: knowledge manager, linkage agent or capacity builder. Specific activities undertaken by KBs were deductively coded to the three core roles, then common activities were inductively grouped. Screening, data extraction, quality assessment and coding were performed independently by two authors., Results: 16 studies comprising 6 RCTs, 8 uncontrolled precomparisons-postcomparisons, 1 controlled precomparison-postcomparison and 1 interrupted time series were included. 14 studies (88%) were conducted in hospital settings. Knowledge manager roles included creating and distributing guideline material. Linkage agent roles involved engaging with internal and external stakeholders. Capacity builder roles involved audit and feedback and educating staff. KBs improved guideline adherence in 10 studies (63%), had mixed impact in 2 studies (13%) and no impact in 4 studies (25%). Half of the RCTs showed KBs had no impact on guideline adherence. KBs acted as knowledge managers in 15 (94%) studies, linkage agents in 11 (69%) studies and capacity builders in all studies., Conclusion: Knowledge manager and capacity builder roles were more frequently studied than linkage agent roles. KBs had mixed impact on translating CPGs into practice. Further RCTs, including those in non-hospital settings, are required., Prospero Registration Number: CRD42022340365., Competing Interests: Competing interests: AJC, ALC and JSB have received grant funding from the Medical Research Future Fund (GA187306). JSB has received grant funding or consulting funds from the National Health and Medical Research Council, Medical Research Future Fund, Victorian Government Department of Health and Human Services, Dementia Australia Research Foundation, Yulgilbar Foundation, Aged Care Quality and Safety Commission, Dementia Centre for Research Collaboration, Pharmaceutical Society of Australia, GlaxoSmithKline Supported Studies Programme, Amgen and several aged care provider organisations unrelated to this work. All grants and consulting funds were paid to the employing institution. TPH, CEO, SK, EJL, SSi, SSa, CJ and MS have no conflicts of interest to declare., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

The number of women of childbearing age with cardiovascular disease (CVD) is growing because of increased survival of children with congenital heart disease. More women are also becoming pregnant at an older age, which is associated with increased rates of comorbidities including hypertension, diabetes, and acquired CVD. Over the past decade the field of cardio-obstetrics has significantly advanced with the development of multidisciplinary cardio-obstetric programs (COPs) to address the increasing burden of CVD in pregnancy. With the introduction of formal COPs, pregnancy outcomes in women with heart disease have improved. COPs provide preconception counselling, antenatal and postpartum cardiac surveillance, and labor and delivery planning. Prepregnancy counselling in a COP should be offered to women with suspected CVD who are of childbearing age. In women who present while pregnant, counselling should be performed in a COP as early as possible in pregnancy. The purpose of counselling is to reduce the risk of pregnancy to the mother and fetus whenever possible. This is done through accurate maternal and fetal risk stratification, optimizing cardiac lesions, reviewing safety of medications in pregnancy, and making a detailed plan for the pregnancy, labor, and delivery. This Clinical Practice Update highlights the COP approach to prepregnancy counselling, risk stratification, and management of commonly encountered cardiac conditions through pregnancy. We highlight "red flags" that should trigger a more timely assessment in a COP. We also describe the approach to some of the cardiac emergencies that the care provider might encounter in a pregnant woman., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Cardiovascular disease is now the leading cause of maternal mortality in the industrialised nations. This public health crisis is driven by a variety of factors, including advancing maternal age, increasing prevalence of diabetes and hypertension and the growing number of adults with congenital heart disease. To meet the needs of this complex and diverse population, the subspecialty of cardio-obstetrics has developed. By its very nature, cardio-obstetrics is a team endeavour and requires contributions from multiple disciplines to deliver optimal care. In this article, we argue that cardio-obstetrics is not a niche issue. The magnitude of the current health challenges makes it imperative that all physicians who care for women of childbearing age have a basic knowledge of how cardiovascular disease can impart risk to women during and beyond pregnancy. We address how to increase awareness within the general medical community so that health care workers are able to recognise potential issues and are aware of how to refer to appropriate specialists. We discuss how to incorporate this within cardiology training so that general cardiologists consider the implications that pregnancy has on their patients. And we reflect on the training of the obstetric cardiologists of tomorrow as this field continues to evolve., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. 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.)

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes., (This article has been co-published with permission in the European Journal of Cardio-Thoracic Surgery, The Annals of Thoracic Surgery, the Journal of Thoracic and Cardiovascular Surgery, and in Radiology: Cardiothoracic Imaging. All rights reserved. © 2021 Jointly between the European Association for Cardio-Thoracic Surgery, The Society of Thoracic Surgeons, the American Association for Thoracic Surgery, and the Radiological Society of North America. The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style.)

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes., (This article has been co-published with permission in the European Journal of Cardio-Thoracic Surgery, The Annals of Thoracic Surgery, the Journal of Thoracic and Cardiovascular Surgery, and in Radiology: Cardiothoracic Imaging. All rights reserved. © 2021 Jointly between the European Association for Cardio-Thoracic Surgery, The Society of Thoracic Surgeons, the American Association for Thoracic Surgery, and the Radiological Society of North America. The articles are identical except for minor stylistic and spelling differences in keeping with each journal’s style.)

Background Women with heart disease are at risk for pregnancy complications, but their long-term cardiovascular outcomes after pregnancy are not known. Methods and Results We examined long-term cardiovascular outcomes after pregnancy in 1014 consecutive women with heart disease and a matched group of 2028 women without heart disease. The primary outcome was a composite of mortality, heart failure, atrial fibrillation, stroke, myocardial infarction, or arrhythmia. Secondary outcomes included cardiac procedures and new hypertension or diabetes mellitus. We compared the rates of these outcomes between women with and without heart disease and adjusted for maternal and pregnancy characteristics. We also determined if pregnancy risk prediction tools (CARPREG [Canadian Cardiac Disease in Pregnancy] and World Health Organization) could stratify long-term risks. At 20-year follow-up, a primary outcome occurred in 33.1% of women with heart disease, compared with 2.1% of women without heart disease. Thirty-one percent of women with heart disease required a cardiac procedure. The primary outcome (adjusted hazard ratio, 19.6; 95% CI, 13.8-29.0; P <0.0001) and new hypertension or diabetes mellitus (adjusted hazard ratio, 1.6; 95% CI, 1.4-2.0; P <0.0001) were more frequent in women with heart disease compared with those without. Pregnancy risk prediction tools further stratified the late cardiovascular risks in women with heart disease, a primary outcome occurring in up to 54% of women in the highest pregnancy risk category. Conclusions Following pregnancy, women with heart disease are at high risk for adverse long-term cardiovascular outcomes. Current pregnancy risk prediction tools can identify women at highest risk for long-term cardiovascular events.

Background: Women with heart disease are at risk for complications during pregnancy. This study sought to examine the effect of maternal obesity on pregnancy complications in women with heart disease., Objectives: The objective was to determine the incidence of adverse cardiac events (CE) in pregnant women with heart disease and obesity., Methods: Adverse CE during pregnancy were examined in a prospective cohort of women with heart disease. CE were a composite of the following: cardiac death/arrest, arrhythmias, heart failure, myocardial infarction, stroke, aortic dissection, and thromboembolic events. Pre-eclampsia and post-partum hemorrhage were also studied. Outcomes were examined according to body mass index (BMI). To identify additional predictors of CE, a baseline risk score (CARPREG [Canadian Cardiac Disease in Pregnancy Study] II score) for predicting cardiac complications was calculated for all pregnancies and included in a multivariable logistic regression model., Results: Of 790 pregnancies, 19% occurred in women with BMI ≥30 kg/m 2 (obesity), 25% in women with BMI 25 to 29.9 kg/m 2 (overweight), 53% in women with BMI 18.5 to 24.9 kg/m 2 (normal weight), and 3% in women with BMI <18.5 kg/m 2 (underweight). Women with obesity were at higher risk of CE when compared with women with normal weight (23% vs. 14%; p = 0.006). In a multivariable model, obesity (odds ratio: 1.7; 95% confidence interval: 1.0 to 2.7) and higher CARPREG II risk scores (odds ratio: 1.7; 95% confidence interval: 1.5 to 1.9) predicted CE. Pre-eclampsia was more frequent in women with obesity compared with those with normal weight (8% vs. 2%; p = 0.001)., Conclusions: Obesity increases the risk of maternal cardiovascular complications in pregnant women with heart disease. This modifiable risk factor should be addressed at the time of preconception counseling., 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. This study was also supported by a generous donation provided by Mrs. Josephine Rogers, Toronto General Hospital. Dr. Silversides is supported by the Miles Nadal Chair in Pregnancy and Heart Disease. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021. Published by Elsevier Inc.)

Women with heart disease are at increased risk for maternal and fetal complications in pregnancy. Therefore, all women with heart disease should undergo evaluation and counseling, ideally before conception, or as early in pregnancy as possible. In this article we will review the role of risk assessment, the history of development of the cardiac risk prediction tools, and the role of current cardiac risk prediction tools.

Background: Women with a prosthetic heart valve are perceived to be at higher risk for adverse outcomes, but their absolute and relative risk of experiencing maternal morbidity and cardiac complications is largely unknown., Objective: The objective of the study was to determine the risk of maternal morbidity and cardiac complications in women with a prior heart valve replacement, compared with matched counterparts without known cardiac disease., Study Design: A retrospective population-based matched cohort study was completed in the province of Ontario, Canada, where there is universal health care. Included were all women of child-bearing age who had bioprosthetic or mechanical replacement of the mitral or aortic valve, April 1994 to March 2016 (valve replacement group). Those in the valve replacement group, and who had at least 1 birth, were 1:4 matched to a community comparison group without heart disease and who also had at least 1 birth. Matching was by maternal age at cohort entry, year of cohort entry, geographic area, income level, and age at first birth. Maternal outcomes included severe maternal morbidity, all-cause mortality, and cardiac morbidity as well as a prolonged hospital length of stay >7 days. Relative risks and 95% confidence intervals were further adjusted for age at birth and immigration status., Results: There were 90 live births among the 64 women in the valve replacement group and 404 live births among the 253 women in the matched community comparison group. There were no stillbirths. Severe maternal morbidity occurred in 13 pregnancies (14.4%) in the valve replacement group and 6 (1.5%) in the community comparison group (adjusted relative risk, 9.73, 95% confidence interval, 3.70-25.59); there were no maternal deaths. The corresponding rates of prolonged hospital length of stay were 37.8% and 18.8% (adjusted relative risk, 2.33, 95% confidence interval, 1.48-3.67)., Conclusion: Pregnant women who had aortic or mitral valve replacement were more likely to experience severe maternal morbidity, as well as prolonged hospital length of stay, than matched counterparts without heart disease. This information can enhance shared decision making about the timing of valve replacement and pregnancy planning in young and middle-aged women. To determine the absolute and relative risk of maternal morbidity and cardiac complications in women with prior heart valve replacement, a retrospective population-based matched cohort study was completed in the province of Ontario, Canada, where there is universal health care. Included were all women of child-bearing age who had bioprosthetic or mechanical replacement of the mitral or aortic valve, April 1994 to March 2016 (valve replacement group). Those in the valve replacement group, and who had at least one birth, were 1:4 matched to a community comparison group without heart disease and who also had at least 1 birth. There were 90 live births among the 64 women in the valve replacement group and 404 live births among the 253 women in the matched community comparison group. Severe maternal morbidity occurred in 13 pregnancies (14.4%) in the valve replacement group and 6 (1.5%) in the community comparison group (adjusted relative risk, 9.73); there were no maternal deaths. The corresponding rates of prolonged hospital length of stay were 37.8% and 18.8% (adjusted relative risk, 2.33). In summary, pregnant women who had an aortic or mitral valve replacement were more likely to experience severe maternal morbidity, as well as prolonged hospital length of stay, than matched counterparts without heart disease., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Pregnancy, which is associated with profound cardiovascular changes and higher risk of thrombosis, increases the risk of cardiovascular complications in women with pre-existing heart disease. A comprehensive history and physical examination, 12-lead electrocardiogram, and transthoracic echocardiogram remain the foundation of assessing cardiac risk during pregnancy in women with heart disease. These are often combined to generate risk scores, which are statistically derived. Several statistically derived risk and 1 lesion-specific classification system are currently available. A suggested clinical approach to risk stratification is first to identify pregnancies in women with cardiac lesions at risk for serious or life-threatening maternal cardiac complications and for the remainder to use the Cardiac Disease in Pregnancy II (CARPREG II) risk score, integrating additional lesion-specific and patient-specific information. Conversely, clinicians can use the modified World Health Organization (mWHO) risk classification system and integrate general risk predictors and patient-specific information. Importantly, cardiac-risk assessment should always incorporate clinical judgement in addition to the use of risk scores or risk-classification systems. As pregnant women with heart disease are also at risk for obstetric and fetoneonatal complications, risk assessment should be performed by a multidisciplinary team, preferably before conception, or as soon as conception is confirmed, and repeated at regular intervals during the course of pregnancy., (Copyright © 2020 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Women with prosthetic heart valves are at higher risk for adverse outcomes during pregnancy. The rates of achieved pregnancy, regardless of the pregnancy outcome, are largely unknown in this group of women., Objective: To determine the rate of pregnancy in women with prior heart valve replacement, and compare that to women without known heart disease., Study Design: A retrospective matched population-based cohort study was done between April 1994 and March 2017, in Ontario, Canada, where universal health care is available. Administrative healthcare databases were used to identify study participants, exposures and outcomes. Each woman of child-bearing age who had a bioprosthetic or mechanical mitral or aortic valve replacement (valve replacement group) was matched to four women without heart disease (community comparison group) -- by age, year of cohort entry, any recent prior pregnancy, geographic area of residence and income level. Starting after the date of cohort entry (defined as the date valve replacement date in the valve replacement group), participants were assessed for a recognized pregnancy, namely, a livebirth, stillbirth, miscarriage or induced abortion. Hazard ratios (HR) and 95% confidence intervals (CI) were further adjusted for age, immigrant status and comorbid medical conditions., Results: 1596 women with a valve replacement were matched with 6378 women in the community comparison group. After a median (interquartile range, IQR) duration of follow-up of 3.1 (1.0-5.6) and 2.7 (1.0-6.0) years, respectively, 98 women in the valve replacement group achieved a recognized pregnancy (0.63 per 100 person-years), compared to 607 women in the community comparison group (0.88 per 100 person-years) - an adjusted HR of 0.72 (95% CI 0.57-0.89). Within the valve replacement group, those with a mechanical valve were less likely to achieve a recognized pregnancy than those with a bioprosthetic valve (adjusted HR 0.57, 95% CI 0.38-0.87)., Conclusion: Women who undergo aortic or mitral valve replacement are less likely to achieve a pregnancy than matched counterparts without heart disease. This information, and the reasons for why this is so, can inform decisions about the timing of valve replacement and pregnancy planning., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Bicuspid aortic valve disease is the most common congenital cardiac disorder, being present in 1% to 2% of the general population. Associated aortopathy is a common finding in patients with bicuspid aortic valve disease, with thoracic aortic dilation noted in approximately 40% of patients in referral centers. Several previous consensus statements and guidelines have addressed the management of bicuspid aortic valve-associated aortopathy, but none focused entirely on this disease process. The current guidelines cover all major aspects of bicuspid aortic valve aortopathy, including natural history, phenotypic expression, histology and molecular pathomechanisms, imaging, indications for surgery, surveillance, and follow-up, and recommendations for future research. It is intended to provide clinicians with a current and comprehensive review of bicuspid aortic valve aortopathy and to guide the daily management of these complex patients., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Bicuspid aortic valve disease is a common congenital cardiac disorder, being present in 1% to 2% of the general population. Associated aortopathy is a common finding in patients with bicuspid aortic valve disease, with thoracic aortic dilation noted in approximately 40% of patients in referral centers. Several previous consensus statements and guidelines have addressed the management of bicuspid aortic valve-associated aortopathy, but none focused entirely on this disease process. The current document is an executive summary of "The American Association for Thoracic Surgery Guidelines on Bicuspid Aortic Valve-Related Aortopathy." All major aspects of bicuspid aortic valve aortopathy, including natural history, phenotypic expression, histology and molecular pathomechanisms, imaging, indications for surgery, surveillance, and follow-up, and recommendations for future research are contained within these guidelines. The current executive summary serves as a condensed version of the guidelines to provide clinicians with a current and comprehensive review of bicuspid aortic valve aortopathy and to guide the daily management of these complex patients., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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.

This Canadian Cardiovascular Society position statement aims to provide succinct perspectives on key issues in the management of thoracic aortic disease (TAD). This document is not a comprehensive overview of TAD and important elements of the epidemiology, presentation, diagnosis, and management of acute aortic syndromes are deliberately not discussed; readers are referred to the 2010 guidelines published by the American Heart Association, American College of Cardiology, American Association for Thoracic Surgery, and other stakeholders. Rather, this document is a practical guide for clinicians managing adult patients with TAD. Topics covered include size thresholds for surgical intervention, emerging therapies, imaging modalities, medical and lifestyle management, and genetics of TAD. The primary panel consisted of experts from a variety of disciplines that are essential for comprehensive management of TAD patients. The methodology involved a focused literature review with an emphasis on updates since 2010 and the use of Grading of Recommendations Assessment, Development, and Evaluation methodology to arrive at specific recommendations. The final document then underwent review by a secondary panel. This document aims to provide recommendations for most patients and situations. However, the ultimate judgement regarding the management of any individual patients should be made by their health care team., (Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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.

Advances in pediatric surgical and interventional techniques and medical care over the past 50 years have revolutionized the care of children with congenital heart disease. Survival to adulthood is now expected and, as such, there is a growing population of adults which is exceeding the pediatric population with congenital heart disease. Noninvasive cardiac imaging with modalities such as echocardiography, computed tomography, and cardiac magnetic resonance imaging are integral to the care of adults with congenital heart disease. These modalities are used for diagnosis, surveillance for complications late after surgery and catheter-based interventions, and in decision-making for medical, interventional, and surgical therapies. In this review we will discuss noninvasive imaging modalities used to assess congenital cardiac lesions, imaging strategies for select congenital lesions, and comment on the future of cardiac imaging in congenital heart disease., (Copyright © 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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.

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.

Since 2006, the Canadian Cardiovascular Society heart failure (HF) guidelines have published annual focused updates for cardiovascular care providers. The 2010 Canadian Cardiovascular Society HF guidelines update focuses on an increasing issue in the western world - HF in ethnic minorities - and in an uncommon but important setting - the pregnant patient. Additionally, due to increasing attention recently given to the assessment of how care is delivered and measured, two critically important topics - disease management programs in HF and quality assurance - have been included. Both of these topics were written from a clinical perspective. It is hoped that the present update will become a useful tool for health care providers and planners in the ongoing evolution of care for HF patients in Canada.

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.

Objective: We sought to examine the frequency of early postoperative complications and preoperative predictors of prolonged hospitalization in adults with repaired tetralogy of Fallot undergoing pulmonary valve replacement., Methods: This was a retrospective study of consecutive adult patients undergoing surgical intervention between 1995 and 2006. A multivariate logistic regression model was used to identify determinants of prolonged hospitalization., Results: One hundred sixteen patients (mean age, 36 +/- 11 years) underwent 118 pulmonary valve replacements. Most (95%) operations included additional procedures, such as pulmonary artery/outflow tract reconstruction or tricuspid valve annuloplasty. The early postoperative mortality (<30 days) was 2.5%. The majority of the patients (60%) had no postoperative complications. The postoperative adverse events included postoperative arrhythmias (19%), respiratory complications (13%), reoperation during admission (13%), renal dysfunction (13%), and myocardial infarction (3%). Postoperative adverse events were associated with prolonged hospitalization (14 +/- 12 vs 7 +/- 3 days, P = .001). In the multivariate analysis age at reoperation of greater than 45 years (odds ratio, 6.1; 95% confidence interval, 1.6-23.6; P = .009), the number of previous sternotomies (odds ratio, 3.8; 95% confidence interval, 1.4-10; P = .007), and the need for urgent surgical intervention (odds ratio, 5.7; 95% confidence interval, 1.1-27.8; P = .03) were predictors of prolonged hospitalization., Conclusion: Pulmonary valve replacement in adults with repaired tetralogy of Fallot has a low mortality risk. The most common early postoperative complications are arrhythmias and respiratory and renal complications. Although most early postoperative complications do not result in long-term sequelae, they are associated with prolonged hospitalization. Patients undergoing urgent interventions, older patients, and those with multiple previous sternotomies are at the highest risk for prolonged hospitalization.

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.

As women with congenital heart disease (CHD) approach childbearing age, issues such as pregnancy and contraception need to be addressed. Women with CHD represent a heterogeneous population. While some have simple cardiac lesions and thus have contraceptive risks that are no different from those of the general population, others have complex, multisystem disease such that incorrect contraception advice can be associated with serious health consequences. Choosing a contraceptive requires consideration of the risk of pregnancy; the available contraception options; their risks, failure rates, and benefits; and the preferences of the woman. This review focuses on contraceptive selection in women with CHD.

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: Adult patients with repaired tetralogy of Fallot (rTOF) often have diminished exercise capacity. The primary objective of this study was to examine whether abnormalities of biventricular function play a role in exercise limitation in patients with rTOF., Methods: This was a retrospective review of 99 adult patients with rTOF. Right ventricular (RV) and left ventricular (LV) function were assessed echocardiographically using the myocardial performance index (MPI). Maximal oxygen consumption (VO(2) Max) was measured during a level 1 cardiopulmonary exercise test., Results: The mean age of the cohort was 34 +/- 11 years (50% females). Although most of the patients reported good functional capacity, the peak Vo(2)max was decreased at 22 +/- 6 mL/kg per minute (66% +/- 13% predicted Vo(2)max for age and sex). The mean RV and LV MPI were 0.30 +/- 0.07 and 0.42 +/- 0.09, respectively. In the multivariate model, higher RV MPI (P = .04) and LV MPI (P = .005) values, representing impaired ventricular function, were associated with diminished Vo(2)max. There was a significant correlation between the RV and LV MPI (r = 0.54, P = .001)., Conclusions: Impairment of RV and LV function, as measured by MPI, is associated with diminished exercise capacity in patients with repaired tetralogy of Fallot. Furthermore, there is a linear relationship between the RV and LV function suggesting that ventricular interactions are contributing to the limited exercise capacity in this group of patients. Strategies aimed at preserving biventricular function or improving adverse ventricular interactions could help to improve functional capacity in these patients.

Objectives: To examine the sexual behaviors and reproductive concerns among patients with moderate to complex congenital heart disease (CHD)., Background: There is a growing need to understand and address the psychosocial issues for older adolescents and young adults with CHD. Emerging sexuality is an issue for this age group and pregnancy for many women with CHD is risky. But, patients' sexual behavior and reproductive concerns have not been studied., Methods: Young adults (19-20 years old; n=212) and adolescents (16-18 years old; n=144) with moderate to complex CHD reported their sexual behaviors and reproductive concerns. Data were compared to normative samples from Canada and the United States., Results: Few adolescents (14%) but many young adults (48%) with CHD were sexually active (at least one partner in the previous 3 months). These rates were lower than those of their healthy peers. Among the sexually active patients, 36% of the young adults and 72% of the adolescents engaged in one or more types of potentially risky sexual behavior (i.e., two or more partners in the past 3 months, questionable birth control, using drugs or alcohol before sex at least sometimes). Women with complex CHD had the highest levels of concern regarding their fertility and risk of genetic transmission of CHD, as well as concerns about adverse effects of pregnancy on their own health., Conclusions: Sexual health should be discussed with adolescents and young adults with CHD. Particular attention should be given to discussing sexual health with women who have complex CHD.

Objective: We sought to determine the incremental diagnostic utility of pediatric cardiac assessment in the offspring of women with congenital heart disease who have had previous fetal echocardiography., Patients and Methods: We prospectively followed pregnant women with congenital heart disease who were receiving care at 2 obstetric and cardiac centers and identified 276 infants who underwent both fetal echocardiography and pediatric cardiac assessment. All of the infants with abnormal fetal echocardiography findings or abnormal pediatric cardiac assessments underwent subsequent confirmatory pediatric echocardiography., Results: In this cohort, congenital heart disease was detected in 22 (8%) of 276 offspring born to women with congenital heart disease. There was concordance between the results of fetal echocardiography and pediatric cardiac assessment in 235 (85%) of 276 offspring (231, both normal; 4, both abnormal) and discordance between the results of fetal echocardiography and pediatric cardiac assessment in 41 (15%) of 276 infants. In the 41 subjects with discordant results, there were normal fetal echocardiography findings but abnormal pediatric cardiac assessments in 35 of 41 (pediatric echocardiography revealed congenital heart disease in 18 of 35 and normal anatomy in 17 of 35) and abnormal fetal echocardiography findings but normal pediatric cardiac assessments in 6 of 41 (pediatric echocardiography findings normal in all 6 of the infants). Fetal echocardiography detected all of the major forms of congenital heart disease. Lesions missed by fetal echocardiography but detected on pediatric cardiac assessment included shunt lesions and minor valvular abnormalities., Conclusions: Although fetal echocardiography can reliably exclude major forms of congenital heart disease, minor congenital heart disease lesions can be missed on fetal echocardiography; however, these can be diagnosed with careful pediatric cardiac assessment. Postnatal pediatric cardiac assessment has incremental diagnostic utility for the detection of congenital heart disease in the offspring of women with congenital heart disease and previous fetal echocardiography.

Objectives: Document the frequency of substance use and oral hygiene among adolescents and young adults with moderate to complex congenital heart disease (CHD)., Background: Patients' knowledge of health behaviors, including substance use and oral hygiene, has been examined among patients with CHD, but patients' actual behavior has not been studied. Understanding patients' behavior is needed to inform interventions that enhance healthy lifestyles, and in turn encourage patients to better care for their own health., Methods: Young adults (19 or 20 years old) and older adolescents (16-18 years old) with moderate or complex CHD (n = 328) reported their substance use (i.e., smoking, marijuana, alcohol, other illicit drugs) and oral hygiene. Rates of these health behaviors were contrasted with comparison samples of peers of the same age., Results: Just over half of the young adults (54%) and over one-quarter of the adolescents (28%) reported significant substance use (i.e., smoking cigarettes on more than 2 days, using marijuana or other illicit drugs at least once, or binge drinking) during the previous 30 days. Rates of significant substance use among the patients with CHD were either comparable to, or lower than, rates in comparison samples of similar aged peers. Only about 15% of the patients with CHD had excellent oral hygiene (i.e., self-reported annual teeth cleaning by dentist, brushing and flossing daily); however, virtually all patients (>95%) brushed daily. Patients with CHD generally had comparable oral hygiene to comparison samples in previously published data., Conclusions: Many older adolescents and young adults with CHD are engaging in behaviors that may compromise their health. The impact of substance use and poor dental hygiene warrants further investigation. Nevertheless, health behaviors should be routinely discussed in this population.