Refining current risk stratification guidelines for pregnant women with Fontan circulation: lessons from PROFAT registry.

Background: Pregnancy in women with a Fontan circulation carries increased risk. Given the relative evidence void, pregnancy counseling requires considerable nuance and experience.

Objectives: This study aimed to identify risk factors for maternal and fetal complications and to contrast risk estimates obtained from existing risk stratification tools, including the modified WHO, CARPREG II, and ZAHARA risk scores.

Severe Coronary Vasospasm Complicated by Thyrotoxicosis in Early Pregnancy.

Background: Although rare, thyrotoxicosis can precipitate anginal symptoms secondary to coronary vasospasm.

Case Summary: A woman presented in early pregnancy with new-onset retrosternal chest pain associated with nausea, vomiting, palpitations, and dyspnea. Further investigations revealed non-ST-segment elevation myocardial infarction secondary to coronary vasospasm owing to thyrotoxicosis.

Impact of Paroxysmal Supraventricular Tachycardia on Pregnancy Outcomes.

Background: Paroxysmal supraventricular tachycardia (PSVT) is one of the most common arrhythmias in pregnant women. However, studies investigating the risk of PSVT in pregnancy are lacking. In pregnancy, we aimed to determine the (1) proportion of women presenting with new-onset PSVT, (2) impact of prior PSVT history on episode severity and management, and (3) rate of adverse maternal and fetal or neonatal outcomes associated with PSVT.

Outcome Prediction After Tetralogy of Fallot Repair: A Prospective Clinical and Cardiovascular Magnetic Resonance Study.

Background: Identification of individuals at risk for major adverse cardiovascular events is essential for contemporary management of patients with repaired tetralogy of Fallot. We sought to identify clinical and cardiovascular magnetic resonance imaging (CMR) predictors of adverse clinical outcomes in repaired tetralogy of Fallot.

Methods: Children and adults prospectively enrolled in the CORRELATE (Comprehensive Outcomes Registry Late After Tetralogy of Fallot Repair) registry followed in North American, European, and Asian centers were studied.

Management of Cardiovascular Health Issues in Turner Syndrome: Expert Insights and Expanded Recommendations From the 2024 Guideline Development Team.

Turner syndrome (TS) is frequently complicated by congenital heart disease (CHD). While left-sided lesions such as bicuspid aortic valve (BAV) and coarctation of the aorta are the most common structural heart lesions in TS, other anomalies, such as aortic arch malformations, hypoplastic left heart syndrome (HLHS), persistent left superior vena cava (LSVC), and partial anomalous pulmonary venous return (PAPVR), are also relatively frequent. Standardized mortality is increased threefold in individuals with TS compared to the general population, with cardiovascular complications, including aortic dissection, being the leading cause of death.

Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation.

Background: The Fontan operation is a surgical procedure to palliate single ventricle congenital heart disease. Hepatocellular carcinoma (HCC) is a rare complication of Fontan-associated liver disease (FALD).

Objectives: The authors aim to examine characteristics of individuals with Fontan circulation diagnosed with HCC and to describe tumor characteristics, treatment, and survival outcomes of these patients.

Propensity Score Analysis of Possible Medication Effects on Outcomes in Patients With Systemic Right Ventricles.

Background: Patients with systemic right ventricle (SRV), either d-transposition of the great arteries following an atrial switch procedure or congenitally corrected transposition of the great arteries, develop severe right ventricular dysfunction, prompting appropriate medical therapy. However, the efficacy of beta-blockers and angiotensin receptor blockers or angiotensin-converting enzyme inhibitors (ACEI) in SRV patients is unproven.

Objectives: The objective of this study was to determine the effects of ACEI/ARB and beta-blockers on outcomes in SRV patients after accounting for likely cofounders affecting their use.

Correlation of ECG and cardiac MRI for assessment of ventricular hypertrophy and dilatation in adults with repaired tetralogy of Fallot.

Background: Surgically repaired Tetralogy of Fallot (rTOF) is associated with progressive right ventricular hypertrophy (RVH) and dilation (RVD). Accurate estimation of RVH/RVD is vital for the ongoing management of this patient population. The utility of the ECG in evaluating patients with rTOF with pre-existing right bundle branch block (RBBB) has not been studied.

Fontan circulation and systemic disease - a retrospective cohort analysis over 35 years of follow-up.

Background: The Fontan operation provides lifesaving palliation for individuals with single ventricle (SV) physiology. Given recent concerns of systemic disease (SD) for patients with a Fontan circulation, we sought to (1) quantify the increase in SD incidence associated with the Fontan circulation; (2) identify the risk factor of SD; (3) assess the association between SD and mortality in patients with a Fontan circulation.

Methods: A matched retrospective cohort study design was adopted.

Determinants of Mortality Among US Patients Diagnosed With Malignant Pleural Mesothelioma Over the Past Decade.

Background: Malignant Pleural Mesothelioma (MPM) is a primary pleural tumor with scarce prognostic data estimates given its rarity. This study aims to explore the epidemiologic and survival predictors amongst patients with MPM, extending from the largest and most recent study conducted between 1973 and 2009.

Methods: 3384 patients diagnosed with MPM between 2010 and 2017 were enrolled from the Surveillance, Epidemiology, and End Results (SEER) database.

"We have dealt with so much. There's more coming?": Improving Knowledge About Brain Health in Adults Living With Congenital Heart Disease.

Background: Significant advances in managing congenital heart disease (CHD) have occurred over the past few decades, resulting in a fast-growing adult patient population with distinct needs requiring urgent attention. Research has recently highlighted the prevalence of neurocognitive differences among adults living with CHD. Yet, there is a lack of knowledge about the perspectives of people living with CHD and family members/caregivers on brain health.

The Canadian Women's Heart Health Alliance ATLAS on the Epidemiology, Diagnosis, and Management of Cardiovascular Disease in Women - Chapter 7: Sex, Gender, and the Social Determinants of Health.

Women vs men have major differences in terms of risk-factor profiles, social and environmental factors, clinical presentation, diagnosis, and treatment of cardiovascular disease. Women are more likely than men to experience health issues that are complex and multifactorial, often relating to disparities in access to care, risk-factor prevalence, sex-based biological differences, gender-related factors, and sociocultural factors. Furthermore, awareness of the intersectional nature and relationship of sociocultural determinants of health, including sex and gender factors, that influence access to care and health outcomes for women with cardiovascular disease remains elusive.

Adherence to clinical practice guidelines for pulmonary valve intervention after tetralogy of Fallot repair: A nationwide cohort study.

Objectives: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair.

Methods: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded.

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey.

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.

Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

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.

End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

Background And Aims: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear.

Methods: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained.

Arrhythmic and thromboembolic outcomes in adults with coarctation of the aorta.

Background: Adults with congenital heart disease (ACHD) experience a high prevalence of atrial arrhythmia (AA) and thromboembolic cerebrovascular complications. However, data on AA and associated long-term outcomes are limited in ACHD patients with coarctation of the aorta (CoA).

Objectives: This study aimed to characterize the prevalence and risk factors for AA and thromboembolic complications in adults with CoA.

Two-Dimensional Strain Echocardiographic Parameters and Clinical Outcomes Associated with Significant Atrioventricular Regurgitation in a Single-Center Adult Fontan Population.

Background: Significant atrioventricular valve regurgitation (AVVR) is prevalent in Fontan adults. Two-dimensional speckle-tracking echocardiography allows for evaluation of subclinical myocardial dysfunction and offers technical benefits. We aimed to evaluate the association of AVVR with echocardiographic parameters and adverse outcomes.