Background Adults with congenital heart disease (CHD) are at high risk of premature death, making advance care planning (ACP) crucial for aligning care with individual values and goals. Previous ACP research has focused primarily on the United States and Canada, highlighting the need for a global perspective. We aimed to describe the ACP practices, needs and preferences of adults with CHD around the globe and to investigate associations with patient-related factors. Methods This cross-sectional study, part of the APPROACH-IS II project, assessed ACP preferences, needs and practices using patient-reported surveys. Overall, 8,281 patients with CHD (median age 32 years; 54% women; 15% mild, 58% moderate, 27% complex CHD) from 53 centers in 32 countries, spanning 6 continents, were included. Results Over half (55%) of participants reported speaking to their physician about how their health might be in the future and 9% had preferences being documented in a plan. According to 66% of patients, the best time to initiate ACP is early in the disease trajectory. Most patients indicated being relatively comfortable talking to their physician about their future health and about death. ACP varied widely across different countries, with United States and Canada top of the class for most variables. Conclusions When looking at global ACP practices, needs and preferences, much room for improvement of ACP provision could be noticed. Also, a notable variation in ACP was observed worldwide.

Education in pediatric cardiology is evolving in response to increasing clinical complexity, expanding subspecialization, and heightened expectations for demonstrable competence. Parallel advances in digital learning and competency-based medical education have accelerated this transition. This narrative review synthesizes contemporary literature, competency frameworks, and educational initiatives relevant to pediatric and adult congenital cardiology. We review the educational tools available now and in the future that are applicable to Canadian and international training programs. Educational practice has shifted toward blended digital ecosystems incorporating curated e-learning platforms, virtual communities of practice, simulation, extended realities and other emerging technologies. Resource poor regions have access to and can benefit from these emerging tools and technological advancements. For congenital cardiology, educational quality depends less on the availability of new technologies than on their thoughtful integration into coherent curricula supported by faculty development and meaningful assessment. The challenge ahead is alignment, of tools with outcomes, assessment with learning, and education with patient care.

BACKGROUND:Exercise capacity is reduced in adults with congenital heart disease (ACHD) and associated with adverse prognosis. Our objective was to explore the efficacy of high-intensity interval training (HIIT) compared with moderate continuous training (MCT) on improving exercise capacity in this population. METHODS:A 3-arm single centre randomized controlled trial was conducted to evaluate the effect of 12 weeks of exercise training (HIIT vs MCT) vs control in ACHD. The primary outcome was a change in peak oxygen uptake (VO2) between baseline and 12 weeks. Secondary outcomes included exercise-related adverse events, changes in cardiac structure and function, and quality of life scores. RESULTS:Thirty-two ACHD were included, with a mean age of 32 ± 11 (range, 24-39) years, 56% were male with average peak VO2 of 27.8 ± 7.0 mL/kg/min at baseline. Exercise training for 12 weeks with HIIT significantly increased peak VO2 by 3.7 ± 3.8 mL/kg/min (P = 0.001), whereas MCT improved peak VO2 by 2.1 ± 3.1 mL/kg/min (P = 0.01), compared with no change in the control group, -1.3 ± 3.0 mL/kg/min (P = 0.44). No adverse events occurred with exercise training. Cardiac magnetic resonance imaging-derived ventricular volumes and ejection fraction were unchanged after exercise training. However, HIIT increased left ventricular mass by 8% (P = 0.02). Exercise training had no effect on quality of life. CONCLUSIONS:HIIT and MCT improved peak VO2 and were feasible, safe, and did not produce adverse cardiac remodelling in ACHD. HIIT was associated with an adaptive increase in left ventricular mass and should be considered as part of comprehensive cardiac rehabilitation. CLINICAL TRIAL REGISTRATION:NCT07546227.

OBJECTIVE:Accurate assessment of left ventricular (LV) function using three-dimensional echocardiography (3-DE) remains limited by suboptimal image quality and restricted field of view. This study proposes a robotic-arm-assisted acquisition protocol combined with a wavelet-based multi-apical view fusion approach to enhance LV image quality in 3-DE. METHODS:Volunteer scans were acquired using a UR10e robotic arm integrated with a Philips EPIQ 7C ultrasound system to ensure consistent multi-apical 3-DE acquisition. Echocardiographic volumes were converted to NRRD format using 3-D Slicer for visualization and verification of spatial and temporal alignment. Two-view and three-view apical datasets were fused using a wavelet-based approach. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were computed from matched 2-D slices in the end-diastolic phase, and qualitative image assessment was conducted by expert raters using blinded scoring of image clarity, myocardial border continuity and diagnostic confidence. RESULTS:Wavelet-based fusion significantly improved image quality compared to single-view 3-DE, with increased SNR (9.36 ± 5.03 vs. 7.09 ± 4.44, p < 0.0001) and CNR (1.68 ± 0.54 vs. 1.49 ± 0.57, p = 0.0020). Three-view fusion provided additional quantitative improvement over 2-view fusion. Inter-rater agreement on visual assessment confirmed that fused images were consistently rated as equal or superior in quality, with substantial agreement across all scoring categories. CONCLUSION:Wavelet-based fusion of multi-apical 3-DE images acquired with robotic arm assistance significantly enhances image quality for LV assessment, improving both quantitative metrics and visual interpretability, practically with the 3-view fusion. The use of the robotic arm played a key role in ensuring standardized and reproducible probe positioning, which is essential for successful image alignment and fusion. This approach demonstrates the potential to improve the reliability and diagnostic value of 3-DE, and future work should explore incorporating additional views and deep learning methods to further advance robotic-assisted cardiac imaging.

Echocardiography remains a widely used imaging modality for the evaluation of cardiac structure and function. Despite its diagnostic value, conventional manual scanning techniques require sonographers to maintain repetitive postures and apply sustained pressure over extended periods, increasing the risk of work-related musculoskeletal disorders. In recent years, collaborative robots, or cobots, have emerged as a promising solution for applications requiring robots to operate safely alongside human operators. Modern cobot systems are often equipped with integrated force and torque sensors, enabling precise control of contact forces during patient scanning to ensure both safety and comfort. This study investigates the feasibility of a robotic-assisted echocardiography system in a clinical setting, focusing on its potential to reduce physical strain on sonographers while maintaining diagnostic image quality. A patient-based evaluation over 24 participants was conducted to assess system performance, force control accuracy, and image quality compared to conventional manual scanning. The findings aim to provide insights into the integration of robotic assistance in echocardiography workflows, with implications for improving operator ergonomics, patient safety, and imaging quality.

Background There is a global mismatch between the number of adults with congenital heart disease and adult congenital heart disease (ACHD) cardiologists, potentially impacting patient outcomes. This survey explores factors influencing trainees and cardiologists' career choices in ACHD. Methods An online, anonymous global survey on demographics, ACHD as a career choice, encouraging and deterring factors assessed via a 5‐point Likert scale and open‐ended questions on recommending ACHD was distributed through targeted emails and social media. Responses meeting a 75% completion criterion were analyzed using descriptive statistics and thematic analysis. Results Among 811 included responses, most were from women (53.5%), aged 31 to 40 years (48.3%), White individuals (43.3%), non‐Hispanic individuals (85%), from outside the ACHD field (79.0%), and from North America (42.7%). Only 30.9% of non‐ACHD physicians considered specializing in ACHD despite 78.9% recommending it. Encouraging factors included awareness of ACHD significance, mentor influence, and clinical exposure. Key deterrents were inadequate financial incentives, limited job opportunities, and lengthy training. Regional variations showed that longer training duration and lack of compensation incentives were the main deterrents in North America. Suggested solutions included structured training programs, salary improvements, and early ACHD exposure during medical education. Conclusions Less than a third of respondents considered ACHD as a career largely due to inadequate financial incentives, limited job opportunities, and long training duration. Addressing these barriers could enhance recruitment and help meet the growing demands of the ACHD population.

Background Cardiac disease is the leading cause of maternal mortality in developed countries, and myocardial infarction (MI) is an important cause of pregnancy-associated morbidity and mortality. These infrequent, but very serious, events are not optimally described in the medical literature. Objectives This study describes a 15-year consecutive, retrospective cohort of confirmed pregnancy-associated MIs (PAMIs) identified in Alberta, Canada (2003-2017). Methods Utilizing a provincial administrative database, a cohort of women with PAMI were identified using a validated algorithm. Additional cases were identified by reviewing provincial maternal mortality records. Medical record review was conducted on each case with further details obtained via linkage with a provincial coronary heart disease registry. Available angiographic images were also reviewed. Results Forty-three cases of PAMI were identified in Alberta between 2003 and 2017, providing a crude incidence of ∼5.64/100,000 births. Rates of PAMI increased over the study period. Of the identified MIs, 16.3% occurred antepartum (mean gestational age of 18 weeks), while 30.2% were peripartum and 53.4% occurred within 6 months postpartum (at a mean of 7.8 weeks after delivery). The most common mechanism of PAMI was spontaneous coronary artery dissection (44.2%) and this mechanism predominated postpartum. Coronary artery disease was a frequent antepartum cause of MI, whereas demand ischemia was the leading cause of peripartum MI. Maternal mortality was approximately 9%. Conclusions PAMI is an increasing cause of maternal morbidity and mortality in Alberta. Clinicians should have a high index of suspicion for PAMI and ensure optimal management of this dangerous complication of pregnancy.

Background The increasing number of people with adult congenital heart disease (ACHD) leads to greater involvement of non-ACHD cardiologists in their care. Defining and supporting the educational priorities of non-ACHD cardiologists is key to ensure care of this population. Methods Between November 2023 and March 2024, Canadian cardiologists were invited to participate via email and the Canadian Cardiology Society bulletin. They self-identified as ACHD or non-ACHD cardiologists. The survey explored (1) self-identified knowledge gaps of non-ACHD cardiologists, (2) current scope of practice of non-ACHD cardiologists, and (3) ACHD perspectives on the recommended scope of practice of non-ACHD cardiologists. Educational priorities were defined as diagnoses or topics frequently identified as self-identified knowledge gaps or in the current or recommended scope of practice. Results A total of 133 of 1263 Canadian Cardiology Society members participated, of whom 32 were ACHD specialists. Thirty-three percent of non-ACHD cardiologists did not have an ACHD specialist on-site, and 24% indicated that there was no ACHD specialist within 100 km. Non-ACHD cardiologists encountered patients with ACHD in the inpatient (42%) and outpatient settings (42%), whereas ACHD cardiologists predominantly encountered outpatients (81%). Frequent self-identified knowledge gaps included great complexity diagnoses and management of pregnancy, arrhythmias, and heart failure. Recommended and current scope of practice involved simple and moderate diagnoses. Conclusions Targeted educational resources for non-ACHD cardiologists should initially focus on the identified educational priorities, including simple and some moderate diagnoses, a primer on great complexity diagnoses, acute management of heart failure and arrhythmias, and a primer on pregnancy in patients with ACHD.

Background Patients with complex congenital heart disease unfit for biventricular repair are often palliated with Fontan circulation (FC), which obviates the need for a subpulmonary ventricle. This approach has led to high survival rates, with over 80% of patients expected to live beyond 30 years. Despite increasing patient numbers and existing guidelines, there are no standardized hemodynamic parameters defining Fontan failure, resulting in considerable variability in management practices. This pilot project aimed to assess real-world practices in FC patient management, particularly in surveillance, defining Fontan circulatory failure, and determining treatment thresholds. Methods A cross-sectional survey with 10 multiple-choice questions and optional free-form responses was distributed globally to congenital heart disease cardiologists through 2 academic centers. Conducted from January to December 2021, the survey targeted clinicians managing adult FC patients. Results Of 310 invited cardiologists, 170 (55%) participated, including 27% from the United States, 22% from Canada, and 20% from the United Kingdom. Respondents included pediatric (37%), adult congenital (47%), and dual trained adult/pediatric cardiologists (14%), mostly in academic settings (94%). Variability existed in defining FC failure, with markers such as protein-losing enteropathy (74%), fatigue/dyspnea (62%), and elevated Fontan pressure (58%) commonly cited. Responses differed on defining elevated Fontan pressure, with 53% selecting >15 mm Hg, 33% >18 mm Hg, and 14% >20 mm Hg. Ninety-one percent prescribed pulmonary vasodilators, though indications and thresholds varied. Conclusion Substantial heterogeneity in FC patient management currently exists, underscoring the need for standardized hemodynamic parameters.

BACKGROUND:The global prevalence of congenital heart disease (CHD) is increasing. Research on patient-reported outcomes (PROs) predominantly originates from high-income countries, resulting in an incomplete understanding of the true global burden of CHD from the patient perspective. Therefore, we described perceived health, psychological distress and quality of life (QoL) in a large sample of adults with CHD from the globe and explored the relationship between PROs and the income level of the countries. METHODS:Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease-International Study II (APPROACH-IS II) represents an international cross-sectional investigation of PROs in 8415 patients from 53 centres across 32 countries. Patients completed questionnaires to measure perceived health status (RAND-12 Health Survey; EuroQOL-5D Visual Analog Scale); depressive symptoms (Patient Health Questionnaire-8, PHQ-8); anxiety (Generalized Anxiety Disorder Scale-7) and QoL (Linear Analog Scale). Gross National Income per capita in US dollars was used for stratifying countries according to income levels. RESULTS:Large intercountry disparities in PROs were observed. Switzerland demonstrated the highest mean scores for physical functioning, self-rated health and QoL, while Senegal had the lowest scores. Patients from Malta demonstrated the highest mean scores for mental health, and Senegal had the lowest scores. With regard to depressive symptoms and anxiety, Pakistan had the lowest mean scores, while Turkey had the highest scores. Patients from high-income nations reported significantly better physical functioning, mental functioning and QoL. CONCLUSION:Large intercountry disparities in PROs were observed. APPROACH-IS II is a pioneering international endeavour that comprehensively evaluated PROs among adults with CHD, drawing participants from nations with different income levels. TRIAL REGISTRATION NUMBER:NCT04902768.

The perfect alignment of 3D echocardiographic images captured from various angles has improved image quality and broadened the field of view. This study proposes an accelerated sequential Monte Carlo (SMC) algorithm for 3D-3D rigid registration of transthoracic echocardiographic images with significant and limited overlap taken from apical window that is robust to the noise and intensity variation in ultrasound images. The algorithm estimates the translational and rotational components of the rigid transform through an iterative process and requires an initial approximation of the rotation and translation limits. We perform registration in two ways: the image-based registration computes the transform to align the end-diastolic frame of the apical nonstandard image to the apical standard image and applies the same transform to all frames of the cardiac cycle, whereas the mask-based registration approach uses the binary masks of the left ventricle in the same way. The SMC and exhaustive search (EX) algorithms were evaluated for 4D temporal sequences recorded from 7 volunteers who participated in a study conducted at the Mazankowski Alberta Heart Institute. The evaluations demonstrate that the mask-based approach of the accelerated SMC yielded a Dice score value of 0.819 +/- 0.045 for the left ventricle and gained 16.7x speedup compared to the CPU version of the SMC algorithm.

INTRODUCTION:Person-centred care (PCC) is widely recommended by the World Health Organisation and other leading healthcare organisations. Although individuals with congenital heart disease (CHD) require lifelong follow-up, it remains unclear whether healthcare systems worldwide provide PCC to this population. This study investigated one key component of PCC, autonomy support, using patient-reported experiences in a global sample of adults with CHD. METHODS:The study was part of the international cross-sectional APPROACH-IS-II. Data were obtained from 8367 adults with congenital heart disease across 53 centres in 32 countries. Perceived autonomy support was measured using a modified version of the Health Care Climate Questionnaire. A general linear mixed model was used to analyse the data. RESULTS:Autonomy support scores ranged from 27.9 (SD ± 9.4) to 37.7 (SD ± 6.3) on a six - 42 point scale. A significant clinical difference in perceived autonomy was observed, with calculated effect sizes using Cohen's D exceeding eight in several countries. Higher autonomy scores were associated with having a high school diploma and older age. Patient characteristics accounted for 1.4 % of the variance, while geographical location explained 7.5 %. A large proportion of the variance remained unexplained. CONCLUSION:This study highlights significant global differences in perceived autonomy support from healthcare providers among adults with CHD. Education and age were associated with higher levels of perceived autonomy support. The experience of PCC is challenged by diverse expectations of individuals and families, healthcare providers' beliefs and values, institutional policies, and broader sociocultural contexts.

Cardiac disease is the leading cause of maternal mortality in developed countries, and myocardial infarction (MI) is an important cause of pregnancy-associated morbidity and mortality. These infrequent, but very serious, events are not optimally described in the medical literature. This study describes a 15-year consecutive, retrospective cohort of confirmed pregnancy-associated MIs (PAMIs) identified in Alberta, Canada (2003-2017). Utilizing a provincial administrative database, a cohort of women with PAMI were identified using a validated algorithm. Additional cases were identified by reviewing provincial maternal mortality records. Medical record review was conducted on each case with further details obtained via linkage with a provincial coronary heart disease registry. Available angiographic images were also reviewed. Forty-three cases of PAMI were identified in Alberta between 2003 and 2017, providing a crude incidence of ∼5.64/100,000 births. Rates of PAMI increased over the study period. Of the identified MIs, 16.3% occurred antepartum (mean gestational age of 18 weeks), while 30.2% were peripartum and 53.4% occurred within 6 months postpartum (at a mean of 7.8 weeks after delivery). The most common mechanism of PAMI was spontaneous coronary artery dissection (44.2%) and this mechanism predominated postpartum. Coronary artery disease was a frequent antepartum cause of MI, whereas demand ischemia was the leading cause of peripartum MI. Maternal mortality was approximately 9%. PAMI is an increasing cause of maternal morbidity and mortality in Alberta. Clinicians should have a high index of suspicion for PAMI and ensure optimal management of this dangerous complication of pregnancy.