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Maternal cardiovascular disease is a leading cause of maternal death worldwide and recently, maternal mortality has increased secondary to cardiovascular causes. Maternal admissions to critical care encompass 1%-2% of all critical care admissions, and although not common, the management of the critically ill pregnant patient is complex. Caring for the critically ill pregnant cardiac patient requires integration of pregnancy-associated physiologic changes, understanding pathophysiologic disease states unique to pregnancy, and a multidisciplinary approach to timing around delivery as well as antenatal and postpartum care. Herein we describe cardiorespiratory changes that occur during pregnancy and the differential diagnosis for cardiorespiratory failure in pregnancy. Cardiorespiratory diseases that are either associated or exacerbated by pregnancy are highlighted with emphasis on perturbations secondary to pregnancy and appropriate management strategies. Finally, we describe general management of the pregnant cardiac patient admitted to critical care., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

This Atlas chapter summarizes sex- and some gender-associated, and unique aspects and manifestations of cardiovascular disease (CVD) in women. CVD is the primary cause of premature death in women in Canada and numerous sex-specific differences related to symptoms and pathophysiology exist. A review of the literature was done to identify sex-specific differences in symptoms, pathophysiology, and unique manifestations of CVD in women. Although women with ischemic heart disease might present with chest pain, the description of symptoms, delay between symptom onset and seeking medical attention, and prodromal symptoms are often different in women, compared with men. Nonatherosclerotic causes of angina and myocardial infarction, such as spontaneous coronary artery dissection are predominantly identified in women. Obstructive and nonobstructive coronary artery disease, aortic aneurysmal disease, and peripheral artery disease have worse outcomes in women compared with men. Sex differences exist in valvular heart disease and cardiomyopathies. Heart failure with preserved ejection fraction is more often diagnosed in women, who experience better survival after a heart failure diagnosis. Stroke might occur across the lifespan in women, who are at higher risk of stroke-related disability and age-specific mortality. Sex- and gender-unique differences exist in symptoms and pathophysiology of CVD in women. These differences must be considered when evaluating CVD manifestations, because they affect management and prognosis of cardiovascular conditions in women., (© 2021 The Authors.)

Women have unique sex- and gender-related risk factors for cardiovascular disease (CVD) that can present or evolve over their lifespan. Pregnancy-associated conditions, polycystic ovarian syndrome, and menopause can increase a woman's risk of CVD. Women are at greater risk for autoimmune rheumatic disorders, which play a role in the predisposition and pathogenesis of CVD. The influence of traditional CVD risk factors (eg, smoking, hypertension, diabetes, obesity, physical inactivity, depression, anxiety, and family history) is greater in women than men. Finally, there are sex differences in the response to treatments for CVD risk and comorbid disease processes. In this Atlas chapter we review sex- and gender-unique CVD risk factors that can occur across a woman's lifespan, with the aim to reduce knowledge gaps and guide the development of optimal strategies for awareness and treatment., (© 2021 The Authors.)

Women of child-bearing age comprise a large proportion of the patients followed by inherited arrhythmia clinics. Despite being a rare and dangerous diagnosis, cardiac and obstetric care providers should know that catecholaminergic polymorphic ventricular tachycardia (CPVT) is not a contraindication to pregnancy. In fact, pregnancy was not associated with an increased risk of CPVT-associated arrhythmias in a recent large cohort study, and most guideline-based anti-arrhythmic drug treatments are life-saving and carry a low risk of teratogenesis. In principle, the potential for CPVT destabilization may be more likely to occur after anti-arrhythmic drugs are decreased or stopped during pregnancy, when an implantable cardioverter defibrillator (ICD) shock exacerbates catecholamine release, or if adrenaline surges are triggered by labor and delivery. Therefore, all pregnant women should be followed by a cardio-obstetrics team with extensive knowledge of CPVT diagnosis, as well as arrhythmia risk stratification fand management. This multidisciplinary care should begin preconception and involve counseling on preimplantation genetic testing, choosing safe and effective anti-arrhythmic drugs, stopping contraindicated medications, optimal programming of ICDs, and planning for the brief hyper-adrenergic period of labor and delivery. The latest data on pregnancy in CPVT is reviewed here and the optimal care for this rare and complex patient population outlined.

Background: Pregnancies in women with regurgitant valve lesions are generally considered low risk, but this has not been well studied., Objectives: This study determined the frequency of adverse cardiac events (CEs) in pregnant women with moderate or severe regurgitant valve lesions., Methods: Maternal and fetal outcomes in women with moderate or severe chronic valve regurgitation enrolled in a prospective multicenter study on pregnancy outcomes were examined. Adverse CEs included heart failure, sustained arrhythmias, cardiac arrest, or death. A multivariate logistic regression model was used to identify determinants of CEs in women at the highest risk., Results: Outcomes of 430 pregnancies in women with moderate or severe regurgitant lesions were examined: 145 with mitral regurgitation (MR), 101 with pulmonary regurgitation (PR), 71 with multivalve disease, 73 with tricuspid regurgitation (TR), and 40 with aortic regurgitation (AR). Most women had associated congenital or acquired heart disease. Adverse CEs occurred in 13% of pregnancies: 27% of pregnancies with multivalve disease; 15% with MR; 15% with TR; 5% with AR; and 3% with PR. Maternal mortality was rare. In women with MR, TR, or multivalve disease (n = 289), left ventricular systolic dysfunction (p = 0.001), pulmonary hypertension (p = 0.005), and cardiac events before pregnancy (p < 0.001) were important determinants of CEs during pregnancy., Conclusions: Women with AR and PR are at low risk for cardiac complications during pregnancy. While many women with MR, TR, and multivalve regurgitation do well during pregnancy, additional clinical variables help stratify those at highest risk. This new information will enhance the quality and precision of preconception counseling and pregnancy planning., 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). Dr. Silversides was supported by the Miles Nadal Chair in Pregnancy and Heart Disease (Mount Sinai Hospital Foundation). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications., Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes., Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined., Results: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not., Conclusions: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Total cavopulmonary connection (TCPC) is associated with a lower risk of incident atrial arrhythmias as compared to atriopulmonary Fontan, but the risk of recurrent atrial arrhythmias is unknown in this population. The purpose of this study was to determine the incidence and risk factors for recurrent atrial arrhythmias and thromboembolic complications in patients with TCPC., Methods: This is a retrospective multicenter study conducted by the Alliance for Adult Research in Congenital Cardiology (AARCC), 2000-2018. The inclusion criteria were TCPC patients (age > 15 years) with prior history of atrial arrhythmia., Results: A total of 103 patients (age 26 ± 7 years; male 58 [56%]) met inclusion criteria. The mean age at initial arrhythmia diagnosis was 13 ± 5 years, and atrial arrhythmias were classified as atrial flutter/tachycardia in 85 (83%) and atrial fibrillation in 18 (17%). The median duration of follow-up from the first episode of atrial arrhythmia was 14.9 (12.1-17.3) years, and during this period 64 (62%) patients had recurrent atrial arrhythmias (atrial flutter/tachycardia 51 [80%] and atrial fibrillation 13 [20%]) with annual incidence of 4.4%. Older age was a risk factor for arrhythmia recurrence while the use of a class III anti-arrhythmic drug was associated with a lower risk of recurrent arrhythmias. The incidence of thromboembolic complication was 0.6% per year, and the cumulative incidence was 4% and 7% at 5 and 10 years respectively from the time of first atrial arrhythmia diagnosis. There were no identifiable risk factors for thromboembolic complications in this cohort., Conclusions: Although TCPC provides superior flow dynamics and lower risk of incident atrial arrhythmias, there is a significant risk of recurrent arrhythmias among TCPC patients with a prior history of atrial arrhythmias. These patients may require more intensive arrhythmia surveillance as compared to other TCPC patients., (© 2021 The Author(s).)

Background: The impact of Fontan circuit thrombus is poorly understood. The objectives of this study were to determine (1) the incidence of Fontan circuit thrombus and proportion of silent thrombus; (2) any association between Fontan circuit thrombus and markers of Fontan circulatory dysfunction; and (3) the association of Fontan circuit thrombus with adverse cardiac outcomes., Methods: We conducted a retrospective review of adult patients who underwent the Fontan procedure (aged > 18 years) followed at St. Paul's Hospital who underwent cardiac computed tomography or magnetic resonance imaging assessment (n = 67). Fontan circulatory dysfunction markers included clinical heart failure, N-terminal pro-brain natriuretic peptide, ventricular dysfunction, atrioventricular valvular regurgitation, refractory arrhythmias, declining exercise capacity, and hepatic/renal dysfunction. Adverse cardiac outcomes were death, heart transplantation, or surgery for Fontan revision or atrioventricular valve replacement., Results: Fontan circuit thrombus was present in 15 of 67 patients (22%): 41% (7/17) classic/modified Fontan and 16% (8/50) total cavopulmonary connection. Incidence was 36% among those suspected to have Fontan circuit thrombus; 14% in those with no clinical/echocardiographic suspicion; and clinically silent in 40% diagnosed with Fontan thrombus. The time from Fontan surgery to Fontan circuit thrombus diagnosis was 22 ± 6 years in the classic/modified group vs 14 ± 8 years in the total cavopulmonary connection group (P = 0.03. Fontan circuit thrombus was associated with adverse cardiac outcomes (27% [4/15] vs 8% [4/52], P = 0.02), but there was no difference in Fontan circulatory dysfunction markers., Conclusion: Given the incidence of Fontan circuit thrombus and association with adverse cardiac outcomes, routine surveillance of the Fontan circuit should strongly be considered. The identification of thrombus should lead to anticoagulation implementation/optimization, along with screening/intervention for reversible Fontan circulatory issues in an attempt to prevent adverse cardiac outcomes., (Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Pregnant women with mechanical valves are considered a high-risk pregnancy. They carry an increased risk of both maternal and foetal complications. This includes maternal valve thrombosis, foetal embryopathy, and haemorrhage. Cardiac surgery is generally avoided during pregnancy, and is used when there are no other alternative options. Cardiopulmonary bypass (CPB) during pregnancy is associated with high foetal mortality. Maternal mortality in the setting of CPB however, is not dissimilar to a non-pregnant woman., Case Summary: We present the case of a 29-year-old woman with Ebstein's anomaly who developed thrombosis of her mechanical tricuspid valve at 4 weeks' gestation. This was suspected to be likely due to sub-therapeutic anticoagulation at the time of presentation. She underwent a tricuspid valve replacement during the first trimester of pregnancy after failing medical therapy, with overall favourable maternal and foetal outcomes., Discussion: Valve thrombosis during pregnancy is a devastating complication. There is limited data surrounding the best management strategy of valve thrombosis in pregnancy. Cardiac surgery with CPB is reserved for cases refractory to appropriate medical therapy. Though maternal mortality is largely unaffected, foetal mortality with CPB remains high. The management of pregnant women undergoing CPB is unique and extremely challenging. It requires a meticulous, multidisciplinary approach to improve overall outcomes., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Many adults with single-ventricle congenital heart disease who have undergone a Fontan procedure have abnormal pulmonary function resembling restrictive lung disease. Whether this contributes to ventilatory limitations and increased dyspnea has not been comprehensively studied. We recruited 17 Fontan participants and 17 healthy age- and sex-matched sedentary controls. All participants underwent complete pulmonary function testing followed by a symptom-limited incremental cardiopulmonary cycle exercise test with detailed assessments of dyspnea and operating lung volumes. Fontan participants and controls were well matched for age, sex, body mass index, height, and self-reported physical activity levels (all P > 0.05), although Fontan participants had markedly reduced cardiorespiratory fitness and peak work rates ( P < 0.001). Fontan participants had lower values for most pulmonary function measurements relative to controls with 65% of Fontan participants showing evidence of a restrictive ventilatory defect. Relative to controls, Fontan participants had significantly higher breathing frequency, end-inspiratory lung volume (% total lung capacity), ventilatory inefficiency (high ventilatory equivalent for CO 2 ), and dyspnea intensity ratings at standardized absolute submaximal work rates. There were no between-group differences in qualitative descriptors of dyspnea. The restrictive ventilatory defect in Fontan participants likely contributes to their increased breathing frequency and end-inspiratory lung volume during exercise. This abnormal ventilatory response coupled with greater ventilatory inefficiency may explain the increased dyspnea intensity ratings in those with a Fontan circulation. Interventions that enhance the ventilatory response to exercise in Fontan patients may help optimize exercise rehabilitation interventions, resulting in improved exercise tolerance and exertional symptoms. NEW & NOTEWORTHY This is the first study to comprehensively characterize both ventilatory and sensory responses to exercise in adults that have undergone the Fontan procedure. The majority of Fontan participants had a restrictive ventilatory defect. Compared with well-matched controls, Fontan participants had increased breathing frequency, end-inspiratory lung volume, and ventilatory inefficiency. These abnormal ventilatory responses likely form the mechanistic basis for the increased dyspnea intensity ratings observed in our Fontan participants during exercise.

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.)

Adults with congenital heart disease (ACHD) represent one of the fastest expanding groups of patients in contemporary practice with unique and often complex management pathways. ACHD have a higher risk of thromboembolism not only from their complex physiology and anatomy but also from substrates such as valvular heart disease, atrial arrhythmias, cyanosis, and ventricular dysfunction, resulting in significant morbidity and mortality. Thromboembolic event rates in ACHD have been shown to be related to Congestive Heart Failure, Hypertension, Age (≥75 years), Diabetes, Stroke/Transient Ischemic Attack, Vascular Disease, Age (65-74 years), Sex (Female) (CHA 2 DS 2 -VASc) scores (0.75%, 1.24%, and 2.65% per patient-year for CHA 2 DS 2 -VASc scores of 0, 1, and ≥ 2 respectively), but are likely more strongly associated with disease complexity. However, optimum anticoagulation strategies have not been studied extensively in this group, mostly because of their heterogeneity and complex clinical profiles. Individual comorbidities, disease severity, and the presence of prosthetic material must be taken into consideration when determining the ideal anticoagulation strategy in pregnant patients. With improving survival and expanding treatment options, the requirement of thromboprophylaxis is steadily increasing, however, safety, choice, and duration of anticoagulation strategies in this group has not been well studied. In this review we aim to highlight the current understanding of antithrombotic therapy in the ACHD population, with discussion regarding special considerations in unique clinical situations relevant to ACHD., (Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

The Fontan procedure is increasingly being used to palliate univentricular physiology. It is a complex anatomic and physiologic repair that can fail at any age, often leaving heart transplantation as the only remaining solution. A meta-analysis was performed to achieve the aim of systematically evaluating the existing evidence for survival after heart transplantation in patients who have undergone a Fontan palliation. MEDLINE, Embase, PubMed, and Web of Science were searched for original research studies. The primary outcome was mortality at 1 and 5 years after transplantation. Five hundred eighty-two records were screened, after the removal of duplicates, 12 retrospective observational studies were selected for inclusion in our meta-analysis. This encompassed a total of 351 Fontan patients undergoing heart transplantation. Mean age was 14 years (range 7 to 24 years) and 65% were men. One- and 5-year survival rates after heart transplantation were found to be 80.3% (95% CI 75.9% to 84.2%) and 71.2% (95% CI 66.3% to 75.7%), respectively. No significant association was found between age, gender, and pulmonary pressures and 1-year mortality. In conclusion, in the largest analysis to date, we found that heart transplantation in younger patients after Fontan procedure has an acceptable early and mid-term mortality. It is comparable to published mortality data of heart transplantation for other forms of congenital heart disease. Heart transplantation in the younger failing Fontan population appears to be a reasonable option when all other avenues have been exhausted and appropriate screening has taken place., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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.)

Starr-Edwards ball-in-cage prosthetic heart valves, although durable, are associated with a particularly high rate of thromboembolic complications. This valve is seldom used in North America, and is certainly not the valve of choice in a woman of childbearing age. Few reports exist from the 1970s of thrombotic complications in pregnant women with Starr-Edwards prostheses, and the optimal management strategy for such valves is unclear. Here, the case is reported of a 31-year-old woman with a Starr-Edwards prosthesis in the mitral position who was transferred to the authors' center at six weeks' gestation with pulmonary edema. Transthoracic echocardiography demonstrated thrombosis of the prosthetic valve, with a mean gradient of 23 mmHg. When treated initially with intravenous heparin and furosemide the patient improved significantly; however, the optimal management going forward was unclear. Here, the options for anticoagulation during pregnancy and for management in the event of valve thrombosis are reviewed. In the absence of any clear guidelines, a thorough discussion of the various risks and benefits with the patient is necessary, but ultimately any consideration of the risk to the mother is paramount.

Cardiovascular diseases are the major cause of death and a significant cause of disability in the Western world and more recently threaten to pose an increasing health burden on developing nations. People with pre-existent vascular disease are those at highest risk for adverse cardiovascular outcomes and require aggressive secondary preventive therapies. Large strides have been made in the development of pharmacologic agents that intervene on various pathways implicated in atherogenesis, thus offering the ability to greatly impact on disease progression and to prevent events. Compelling data derived primarily from randomized controlled trials have shown the benefits of aspirin (or antiplatelet agents) and angiotensin converting enzyme (ACE) inhibitors (A), beta-blockers and blood pressure (B) and cholesterol-lowering drugs (C), particularly statins, in preventing recurrent events and improving survival. Taken together these data are the foundation for the simple, but important advice for secondary prevention - the ABCs. In addition, the evidence for the central role of lifestyle factors as determinants of risk has lead to increased efforts towards developing interventions aimed at modifying lifestyle patterns. Today, the biggest challenge remains in the implementation of proven effective therapies. Our focus should turn to educating physicians and patients alike regarding available therapies and their indications. In addition systematic, sustainable and globally applicable approaches to the secondary prevention of cardiovascular diseases need to be developed to truly realize the vast potential benefits of existing therapies.

Ambulatory blood pressure (ABP) monitoring is emerging as a useful tool in the diagnosis and management of hypertension. It is particularly useful in certain settings, such as the identification of patients with 'white coat hypertension', the evaluation of patients with unexplained symptoms or end-organ damage, the study of circadian variations in BP and the management of patients with refractory hypertension (as assessed by office BP measurements), and as a guide to antihypertensive treatment. Recent data suggest that ABP monitoring may be superior to office BP measurements in the assessment of risk of hypertensive end-organ damage and clinical events. However, further evidence that the use of ABP monitoring can improve patients' clinical outcomes is needed and would make the use of this promising technique more compelling and more widely accepted.