Cell and gene therapy manufacturing capabilities in Australia and New Zealand.

Cell and gene therapy products are rapidly being integrated into mainstream medicine. Developing global capability will facilitate broad access to these novel therapeutics. An initial step toward achieving this goal is to understand cell and gene therapy manufacturing capability in each region. We conducted an academic survey in 2018 to assess cell and gene therapy manufacturing capacity in Australia and New Zealand. We examined the following: the number and types of cell therapy manufacturing facilities; the number of projects, parallel processes and clinical trials; the types of products; and the manufacturing and quality staffing levels. It was found that Australia and New Zealand provide diverse facilities for cell therapy manufacturing, infrastructure and capability. Further investment and development will enable both countries to make important decisions to meet the growing need for cell and gene therapy and regenerative medicine in the region.

Cardiovascular Magnetic Resonance Provides Evidence of Abnormal Myocardial Strain and Primary Cardiomyopathy in Marfan syndrome.

OBJECTIVE: Marfan syndrome is an autosomal-dominant genetic disorder caused by mutations in the fibrillin-1 gene. The condition is a connective tissue disease that frequently involves the cardiovascular system. The existence of a primary cardiomyopathy in Marfan syndrome, however, is controversial. The aims of this study were to investigate the prevalence of left ventricular dysfunction with both transthoracic echocardiography and cardiovascular magnetic resonance (CMR) in a cohort of Marfan syndrome patients and to investigate patterns of myocardial strain across the cohort. METHODS: We used an institutional database to identify all patients with a firm diagnosis of Marfan syndrome based on Ghent criteria. Inclusion required left ventricular ejection fraction (LVEF) to have been measured by both CMR and transthoracic echocardiography within 12 months of each other. Normal LVEF was defined as a value of >55% when measured by CMR. Velocity vector imaging was used to measure left ventricular longitudinal strain patterns by application of feature tracking to cine magnetic resonance images. Results were compared with data from 20 age-matched control subjects. RESULTS: Sixty-nine Marfan syndrome patients met the inclusion criteria. The mean age was 35.4 ± 15.0 years, and 56.5% were male. The mean LVEF was 59.0% ± 7.0% by CMR and 59.1% ± 5.8% by echo. One-fifth of Marfan syndrome patients (15/69; 21.7%) had reduced function with LVEF ≤55% by CMR, but only 5 of these were identified by echo. Furthermore, echo identified 5 Marfan syndrome patients as having reduced LVEF in the presence of a normal LVEF by CMR. Some Marfan syndrome patients had abnormal longitudinal strain patterns even with LVEF within the reference range. CONCLUSIONS: These data provide support for a primary cardiomyopathy in some Marfan syndrome patients. Cardiovascular magnetic resonance is more sensitive than echo for identifying cases with mild systolic dysfunction. Strain analysis may be more sensitive than simple LVEF assessment for identifying at-risk individuals.

Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review.

OBJECTIVES: Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population. METHODS: We performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death. RESULTS: A total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease. CONCLUSIONS: Women with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

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. 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.

Feasibility and Findings of Including Self-Identified Adult Congenital Heart Disease Patients in the INVESTED Trial.

Background: Adult congenital heart disease (ACHD) patients have significant morbidity and rise in cardiac admissions. Their outcome with high-dose influenza vaccination is unknown in comparison to those without ACHD. Objectives: The purpose of this study was to compare all-cause mortality or cardiopulmonary hospitalizations in self-identified ACHD versus non-ACHD patients receiving high- or low-dose influenza vaccination within the INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure trial. Methods: We prospectively included ACHD patients in the INVESTED (INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure) trial. The primary endpoint was all-cause death or hospitalization for cardiovascular or pulmonary causes. Results: Of the 272 ACHD patients, 132 were randomly assigned to receive high-dose trivalent and 140 to standard-dose quadrivalent influenza vaccine. Compared to the non-ACHD cohort (n = 4,988), ACHD patients were more likely to be younger, women, smokers, have atrial fibrillation, and have a qualifying event of heart failure. The primary outcome was 49.8 events versus 42.8 events per 100 person-years (adjusted HR: 1.17; 95% CI: 0.95-1.45; P = 0.144) in the ACHD group and non-ACHD group, respectively. The interaction between ACHD status and randomized treatment effect was not significant for the primary outcome (P = 0.858). Vaccine-related adverse events were similar in both groups. Conclusions: Patients who self-identify as being ACHD had similar primary outcome of all-cause death or hospitalization for cardiovascular or pulmonary causes compared to non-ACHD cohort. High-dose influenza vaccination was similar to standard-dose influenza vaccination on the primary outcome in patients who self-identify as ACHD.

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. Patients were classified according to presence (+) versus absence (-) of PVR and presence (+) versus absence (-) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. Results: In total, 498 participants (57% male, mean age 32 ± 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 ± 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication-). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR-/indication-). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P < .0001; hazard ratio, 2.78; 95% confidence interval, 2.18-3.55, P < .0001). Conclusions: Although a majority of patients met guideline criteria for PVR, only a minority were referred for intervention. Abnormal cardiovascular imaging was the most common indication for referral. Further research will be necessary to establish the longer-term clinical impact of varying PVR referral strategies.

Physical Activity Promotion Among Individuals With Tetralogy of Fallot.

In the general population, the most cited barriers to physical activity (PA) are time, energy, and motivation. Consequently, despite the significant contribution of PA to health and well-being, many individuals are insufficiently active. Physical inactivity and sedentary lifestyles increase the risk of acquired cardiometabolic disease, a risk that may complicate and is compounded by the anatomic and physiologic features inherent in the patient with repaired tetralogy of Fallot (ToF). Individuals with ToF commonly present with reduced exercise capacity and PA levels. In light of historically PA restrictive management of their heart disease, known reductions in exercise capacity among individuals with ToF are combined with psychosocial barriers to their participation, potentially establishing a cycle of further detraining, inactivity, and disease progression/health decline. To this end, children and young adults with ToF are known to have reduced self-efficacy towards PA, defined as their confidence in their ability to participate. In this review, we apply self-efficacy as an overarching mediator of PA participation and explore trends and determinants of PA participation among individuals with ToF and its subsequent impact on exercise capacity, disease risk, and health-related quality of life. We outline the rationale and strategies aimed at improving PA in children and adults with ToF and highlight current knowledge gaps and future directions in the promotion of PA in the population with ToF.

Dans la population générale, les obstacles à la pratique de l'activité physique (AP) le plus fréquemment mentionnés sont le manque de temps, d'énergie et de motivation. Malgré les bienfaits importants de l'AP pour la santé et le bien-être, de nombreuses personnes ne sont pas suffisamment actives. L'inactivité physique et la sédentarité augmentent le risque de maladies cardiométaboliques acquises, et ce risque pourrait s'aggraver et s'assortir de complications chez les patients présentant une tétralogie de Fallot (TF) réparée, en raison de leurs caractéristiques anatomiques et physiologiques. Une tolérance limitée à l'effort et un faible niveau d'AP sont fréquents chez les patients ayant une TF. Compte tenu des approches thérapeutiques antérieures qui limitaient l'AP, cette réduction de la tolérance à l'effort est combinée à des obstacles psychosociaux qui favorisent l'inactivité et la progression de la maladie ou la détérioration de l'état de santé. Les enfants et les jeunes adultes ayant une TF présentent donc une réduction connue de l'auto-efficacité à cet égard, c'est-à-dire leur niveau de confiance dans leur capacité de participer à des AP. Notre article de synthèse présente l'auto-efficacité comme un médiateur global de la participation à l'AP et nous explorons les tendances et les déterminants de la participation à l'AP chez les personnes présentant une TF. Nous présentons également les répercussions sur la tolérance à l'effort, le risque de maladies et l'indice de la qualité de vie liée à la santé (QVLS). Nous décrivons les raisons qui font valoir l'importance de l'AP chez les enfants et les adultes présentant une TF et des stratégies pour y arriver, en plus de souligner les lacunes dans les connaissances actuelles et de proposer des orientations futures pour la promotion de l'AP chez ces patients.

Access to Specialized Care Across the Lifespan in Tetralogy of Fallot.

Individuals living with tetralogy of Fallot require lifelong specialized congenital heart disease care to monitor for and manage potential late complications. However, access to cardiology care remains a challenge for many patients, as does access to mental health services, dental care, obstetrical care, and other specialties required by this population. Inequities in health care access were highlighted by the COVID-19 pandemic and continue to exist. Paradoxically, many social factors influence an individual's need for care, yet inadvertently restrict access to it. These include sex and gender, being a member of a racial or ethnic historically excluded group, lower educational attainment, lower socioeconomic status, living remotely from tertiary care centres, transportation difficulties, inadequate health insurance, occupational instability, and prior experiences with discrimination in the health care setting. These factors may coexist and have compounding effects. In addition, many patients believe that they are cured and unaware of the need for specialized follow-up. For these reasons, lapses in care are common, particularly around the time of transfer from paediatric to adult care. The lack of trained health care professionals for adults with congenital heart disease presents an additional barrier, even in higher income countries. This review summarizes challenges regarding access to multiple domains of specialized care for individuals with tetralogy of Fallot, with a focus on the impact of social determinants of health. Specific recommendations to improve access to care within Canadian and American systems are offered.

Pour les personnes qui vivent avec la tétralogie de Fallot, des soins spécialisés en cardiopathie congénitale (CC) sont nécessaires pour surveiller et prendre en charge toute complication tardive éventuelle. Toutefois, l'accès à des soins en cardiologie demeure difficile pour de nombreux patients, tout comme aux services en santé mentale, aux soins dentaires, aux soins obstétriques et à d'autres soins spécialisés dont cette population a besoin. Des inégalités dans l'accès aux soins de santé ont été mises en lumière lors de la pandémie de COVID-19 et continuent d'exister. Paradoxalement, de nombreux facteurs sociaux agissent sur les besoins d'une personne en matière de soins, et limitent en même temps son accès à ces soins. Le sexe et le genre, l'appartenance à un groupe racial ou ethnique ayant vécu une exclusion par le passé, un niveau de scolarité ou un niveau socio-économique plus faible, l'éloignement géographique des centres de soins tertiaires, les difficultés de déplacement, une protection inadéquate en matière d'assurance de soins de santé, la précarité professionnelle et des expériences antérieures de discrimination en contexte de soins de santé font partie de ces facteurs, qui peuvent coexister et avoir un effet cumulatif. De plus, de nombreux patients croient être guéris et ne sont pas conscients qu'un suivi spécialisé est nécessaire pour eux. Pour ces raisons, les interruptions des soins sont fréquentes, en particulier au moment de la transition entre les soins pédiatriques et les soins destinés aux adultes. La pénurie de professionnels de la santé formés pour intervenir auprès d'adultes présentant une CC est un obstacle supplémentaire, même dans les pays à revenus élevés. Notre article de synthèse résume les obstacles vécus par les personnes vivant avec la tétralogie de Fallot dans l'accès à plusieurs types de soins spécialisés, en portant une attention particulière aux déterminants sociaux de la santé. Des recommandations concrètes pour améliorer l'accès aux soins en contexte canadien et américain sont présentées.

The Aortic Team Model for the Management of the Distal Arch, Descending Thoracic and Thoracoabdominal Aorta: Appraisal at 3 Years.

BACKGROUND: This study aimed to assess feasibility, logistical challenges, and clinical outcomes associated with the implementation of an Aortic Team model for the management of distal arch, descending thoracic and thoracoabdominal aortic disease. METHODS: An Aortic Team care pathway was implemented in November 2019. Working as a unit, two cardiac surgeons, two vascular surgeons, an interventional radiologist, a cardiologist, and an anesthesiologist collectively determined care decisions via multispecialty presence at an Aortic Clinic. Cardiac and vascular surgeons operated in tandem for open procedures. Interventional radiology participated alongside cardiac and vascular for endovascular procedures. Cardiology aided in medical therapies for heritable and degenerative disease, and had a lead role for genetics and high-risk pregnancy referrals. The model spanned three hospitals. Clinical outcomes at 3 years were assessed. RESULTS: There were 35 descending thoracic and thoracoabdominal surgeries and 77 thoracic endovascular aortic repairs. Endoarch devices were used in 7 cases (Gore Thoracic Branch Endoprosthesis, 4, Terumo RelayBranch, 3) and an endothoracoabdominal device in 4 cases (Cook Zenith t-branch). The Aortic Clinic acquired 456 patients, with yearly increases (54 patients [year 1], 181 patients [year 2], 221 patients [year 3]). For surgery, mortality was 8.6% (3/35), permanent paralysis 5.7% (2/35), stroke 8.6% (3/35), permanent dialysis 0%, and reinterventions 8.6% (3/35). For endovascular cases, mortality was 3.9% (3/77), permanent paralysis 3.9% (3/77), stroke 5.2% (4/77), permanent dialysis 1.3% (1/77), and reinterventions 16.9% (13/77). CONCLUSION: An Aortic Team model is feasible and ensures all treatment options are considered. Conventional open thoracoabdominal procedures showed acceptable outcomes. Endoarch technology shows early promise.

Determining Research Priorities With Teen and Adult Congenital Heart Disease Patients: A Mixed-Methods Study.

Background: Children with congenital heart disease (CHD) are living longer than ever before. This growing cohort of adults with CHD has high medical and psychosocial needs. Also, patients and advocacy groups are justifiably demanding that their voices be heard in all phases of clinical and health services research. Methods: We conducted a first of its kind research priority-setting exercise with teens and adults with moderate-to-complex CHD. Focus groups were held using a fixed, mixed methods, exploratory sequential design. Objectives were to include the patient voice in all phases of the research process, determine the key needs of patients living with CHD, to guide health services research, and identify the "top 10" research priorities of teens and adults living with CHD. Results: Thirty-five patients participated in one of nine 3-hour focus groups where they shared their experiences living with CHD. They expressed a desire for connection with others living with CHD and altruistic motives for participating. Patients with CHD identified a need for information about their disease and prognosis, a need for connection through physical activity and mentorship programmes, and a need for advanced communication with health care teams. Qualitative results correlated well with quantitative ratings to create a patient-derived "top 10" research priorities list. Conclusions: Patients affected by a chronic disease like CHD want to be included in all phases of research. Our research priority-setting exercise in teens and adults with CHD has created a roadmap for clinicians and researchers to investigate issues most important to those living with CHD.

Contexte: Les enfants atteints d'une cardiopathie congénitale vivent plus longtemps que jamais auparavant. Cette cohorte croissante est composée d'adultes atteints de cardiopathie congénitale qui ont des besoins médicaux et psychosociaux importants. Par ailleurs, les patients et les groupes de revendication exigent à juste titre de faire entendre leurs voix lors de toutes les phases des recherches cliniques et de celles sur les services de santé. Méthodologie: Nous avons mené un exercice novateur sur l'établissement des priorités de recherche chez des adolescents et des adultes atteints de cardiopathie congénitale modérée ou complexe. Nous avons organisé des groupes de concertation selon un plan fixe, séquentiel, exploratoire, à méthodes mixtes. Les objectifs étaient de permettre aux patients de se faire entendre lors de toutes les étapes du processus de recherche, de déterminer les besoins clés des patients atteints de cardiopathie congénitale pour orienter les recherches sur les services de santé et d'identifier les 10 principales priorités de recherche chez les adolescents et les adultes atteints de cardiopathie congénitale. Résultats: L'exercice a porté sur 35 patients qui ont participé à l'un des neuf groupes de concertation de trois heures, au cours desquels ils ont fait part de leurs expériences de vie avec une cardiopathie congénitale. Les participants ont indiqué qu'ils souhaitaient former des liens avec d'autres personnes atteintes d'une cardiopathie congénitale et ont donné des motifs altruistes pour participer. Les patients ont reconnu la nécessité d'être informé au sujet de leur maladie et de leur pronostic, de former des liens par le biais de l'activité physique et de programmes de mentorat et de communiquer plus avec les équipes soignantes. Il existe une corrélation étroite entre les résultats qualitatifs et les évaluations quantitatives, ce qui a permis d'établir une liste des 10 principales priorités de recherche des patients. Conclusions: Les patients qui sont aux prises avec une maladie chronique comme la cardiopathie congénitale souhaitent être inclus dans toutes les phases des travaux de recherche. Par ailleurs, l'exercice sur l'établissement des priorités de recherche que nous avons effectué chez les adolescents et les adultes atteints d'une cardiopathie congénitale a permis de créer une feuille de route pour les cliniciens et les chercheurs. En effet, ce plan leur permettra d'étudier les questions les plus importantes pour les personnes qui vivent avec une cardiopathie congénitale.

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. 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.

Contexte: La pandémie de COVID-19 a eu des répercussions sur l'accès aux soins de santé partout au Canada, y compris une diminution des évaluations en personne. La présente étude visait à évaluer l'effet de la pandémie de COVID-19 sur l'accès aux soins de santé chez les adultes atteints de cardiopathie congénitale. Méthodologie: Nous avons communiqué avec tous les centres canadiens de prise en charge de la cardiopathie congénitale chez l'adulte et nous leur avons demandé de recueillir des données sur les consultations externes et le volume des interventions pour les années 2019 et 2020. Un sondage détaillé leur a été transmis sur les volumes de consultations et d'interventions et sur les temps d'attentes avant et après la mise en place de restrictions liées à la pandémie. Les groupes ont été comparés lors d'une analyse statistique descriptive utilisant le test t de Student. Résultats: En 2019, 19 326 consultations pour cause de cardiopathie congénitale chez l'vadulte ont été enregistrées au Canada, dont seulement 296 (1,5 %) ont eu lieu en mode virtuel. Au cours de la première année de la pandémie, 20 532 consultations ont été relevées; 11 412 (56 %) ont été menées virtuellement (p < 0,0001). Aucune différence n'a été observée dans le volume d'interventions (interventions en électrophysiologie, interventions chirurgicales et interventions percutanées) entre 2019 et 2020. Les temps d'attente moyens estimés en mois, avant et pendant la pandémie, étaient les suivants : consultations non urgentes, 5,4 ± 2,6 vs 6,6 ± 4,2 (p = 0,65); interventions chirurgicales, 6,0 ± 3,5 vs 7,0 ± 4,6 (p = 0,47); interventions en électrophysiologie, 6,3 ± 3,3 vs 5,7 ± 3,3 (p = 0,72); et interventions percutanées, 4,6 ± 3,9 vs 4,4 ± 2,3 (p = 0,74). Conclusion: Au cours de la pandémie et de la période où les restrictions de distanciation sociale étaient en vigueur, le recours aux consultations virtuelles dans les cliniques a contribué à la continuité des soins offerts aux adultes atteints de cardiopathie congénitale, puisque 56 % des visites se sont déroulées virtuellement. Le volume d'interventions n'a pas été touché et les temps d'attentes pour les consultations, les interventions percutanées et les interventions chirurgicales ne se sont pas allongés.

The Aortic Team Model and Collaborative Decision Pathways for the Management of Complex Aortic Disease: Clinical Practice Update From the Canadian Cardiovascular Society/Canadian Society of Cardiac Surgeons/Canadian Society for Vascular Surgery/Canadian Association for Interventional Radiology.

Disease of the aortic arch, descending thoracic, or thoracoabdominal aorta necessitates dedicated expertise across medical, endovascular, and surgical specialties. Cardiologists, cardiac surgeons, vascular surgeons, interventional radiologists, and others have expertise and skills that aid in the management of patients with complex aortic disease. No specialty is uniformly expert in all aspects of required care. Because of this dispersion of expertise across specialties, an aortic team model approach to decision-making and treatment is advocated. A nonhierarchical partnership across specialties within an interdisciplinary aortic clinic ensures that all treatment options are considered and promotes shared decision-making between the patient and all aortic experts. Furthermore, regionalization of care for aortic disease of increased complexity assures that the breadth of treatment options is available and that favourable volume-outcome ratios for high-risk procedures are maintained. An awareness of best practice care pathways for patient referrals for preventative management, acute care scenarios, chronic care scenarios, and pregnancy might facilitate a more organized management schema for aortic disease across Canada and improve lifelong surveillance initiatives.

Patient-Reported Outcomes After Tetralogy of Fallot Repair.

BACKGROUND: Comprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS). OBJECTIVES: This study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs. METHODS: This was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years. RESULTS: The study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001). CONCLUSIONS: Strategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF.

Experiences and Impacts of Harassment and Discrimination Among Women in Cardiac Medicine and Surgery: A Single-Center Qualitative Study.

Background: Gender- and sex-based harassment and discrimination are consistently reported by about 50% of women physicians, and the prevalence may be even greater among women in cardiology. An exploration of these experiences and their impacts on women in healthcare is necessary to design interventions, create supports, and facilitate empathy, support, and allyship among leadership. Methods: To understand and describe the experiences of harassment and discrimination among women working in cardiac sciences, to inform the design of interventions and supports, we performed one-on-one, semi-structured interviews with women in the Department of Cardiac Sciences in a single institute. Interviews were coded independently in parallel using thematic analysis and reconciled by trained qualitative researchers. Experiences were categorized as harassment using the Canadian Human Rights Act. Codes were grouped into themes by iterative discussion. Results: There were 15 participants, including trainees, physicians in a variety of cardiac subdisciplines, and nurse practitioners. All participants had experienced sex- or gender-based discrimination at work, though the impact and perception of these experiences varied. Whereas some participants felt that these experiences had little influence on their careers or personal lives, others changed practice specialties or locations due to harassment. Several participants had been sexually assaulted at work. Interviews revealed modifiable barriers to reporting harassment. Conclusions: This qualitative dataset enriches the prevalence data on sex- and gender-based harassment among women working in cardiology by describing the impacts and perceptions of this harassment. Organizations should address commonly described barriers to reporting harassment, including addressing retaliation, and create systems-level supports for those affected by harassment.

Introduction: Environ 50 % des femmes médecins signalent constamment la discrimination et le harcèlement fondés sur le genre et le sexe. Cette prévalence est encore plus grande chez les femmes en cardiologie. L'exploration de ces expériences et de leurs répercussions sur les femmes dans les soins de santé est nécessaire pour concevoir des interventions, créer du soutien, et faciliter l'empathie, le soutien et le concept d'allié chez les dirigeants. Méthodes: En vue de comprendre et de décrire les expériences de harcèlement et de discrimination chez les femmes qui travaillent en sciences cardiaques, d'orienter la conception d'interventions et de soutien, nous avons réalisé des entretiens individuels semi-structurés auprès de femmes du Service des sciences cardiaques d'un seul établissement. Les entrevues ont indépendamment été codifiées en parallèle par l'analyse thématique et rapprochées par des chercheurs formés aux méthodes qualitatives. Les expériences ont été catégorisées en harcèlement conformément à la Loi canadienne sur les droits de la personne. Des échanges itératifs ont permis de regrouper les codes par thèmes. Résultats: Les 15 participantes étaient des stagiaires, des médecins de diverses sous-disciplines de la cardiologie et des infirmières praticiennes. Toutes les participantes avaient subi de la discrimination fondée sur le sexe ou le genre au travail, même si les répercussions et la perception de ces expériences variaient. Alors que quelques participantes ont senti que ces expériences avaient eu peu d'influence sur leur carrière ou leur vie personnelle, d'autres ont changé de spécialité ou de lieu de pratique en raison du harcèlement. Plusieurs participantes ont subi des agressions sexuelles au travail. Les entretiens ont révélé des obstacles au signalement du harcèlement qui sont modifiables. Conclusions: Cet ensemble de données qualitatives enrichit les données sur la prévalence du harcèlement fondé sur le sexe et le genre chez les femmes qui travaillent en cardiologie en décrivant les répercussions et les perceptions de ce harcèlement. Les organisations devraient se pencher sur les obstacles au signalement du harcèlement fréquemment décrits, notamment les représailles, et créer du soutien à l'échelle du système pour les femmes qui sont touchées par le harcèlement.

Caring for the Aging Patient With Adult Congenital Heart Disease: A Review of Cardiac and Noncardiac Comorbidities.

As the demographics of congenital heart disease (CHD) have shifted, there are now more adults living with CHD than children in North America. This presents unprecedented challenges as patients with CHD acquire noncardiac comorbidities and seek care for a variety of reasons, including noncardiac surgery and emergency department (ED) visits. CHD shifts from a one organ problem to a multisystem disease and requires a team of specialists to maintain high-quality longitudinal care. In this review, we summarize the challenges patients with CHD and their providers face as they age. We review the demographics of CHD and health care utilization. We examine the rates of noncardiac comorbidities and the current quality of care received by adult patients with CHD.

Les caractéristiques démographiques des patients atteints d'une cardiopathie congénitale ont changé : à l'heure actuelle, en Amérique du Nord, il y a plus d'adultes qui présentent une cardiopathie congénitale que d'enfants. Cela représente un défi sans précédent lorsque ces patients ont des troubles concomitants non cardiaques et doivent subir une chirurgie non cardiaque, par exemple, ou se rendre aux urgences pour toutes sortes de raisons. On ne parle alors plus de l'atteinte d'un seul organe, mais bien d'une maladie multisystémique. Une équipe pluridisciplinaire sera nécessaire pour maintenir des soins longitudinaux de haute qualité.Dans notre article, nous faisons le point sur les défis que les personnes atteintes d'une cardiopathie congénitale et les dispensateurs de soins doivent affronter à mesure que le patient avance en âge. Nous passons en revue les données démographiques sur les cardiopathies congénitales ainsi que celles sur l'utilisation des services de santé. Nous examinons la fréquence des comorbidités non cardiaques, tout comme la qualité des soins que reçoivent actuellement les patients atteints d'une cardiopathie congénitale.

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Lack of Equity in the Cardiology Physician Workforce: A Narrative Review and Analysis of the Literature.

The gender and racial diversity in the cardiology workforce in Canada does not reflect that of the population we serve. As social awareness of the principles of equity, diversity, and inclusion rises, our profession must rise to meet the challenges they present. We detail contemporary examples of publication bias in the cardiac sciences literature and describe the factors that led to oversight in the peer-review process. We performed a narrative review to summarize the published literature on equity and diversity among cardiac physicians. We also summarize the challenges faced by women and racial-minority physicians when pursuing and thriving in a career in cardiology, and the systemic barriers to their success. In the past decade, social justice movements have advanced. Professionalism standards are changing, and awareness and understanding of these advances in terminology is imperative for all physicians. In this review, we summarize key language and concepts, with cardiology-specific examples, and propose a new paradigm of professionalism.

Au Canada, la diversité des genres et des races au sein de la main-d'œuvre en cardiologie ne reflète pas celle qui existe dans la population que nous servons. La prise de conscience sociale des principes d'équité, de diversité et d'inclusion gagne du terrain, et notre profession doit se montrer à la hauteur des défis qui s'y rattachent. Nous abordons des exemples contemporains de biais de publication dans la littérature cardiologique et décrivons les facteurs qui ont mené à des omissions dans le processus d'examen par les pairs. Une revue narrative de la littérature publiée sur l'équité et la diversité parmi les cardiologues nous a permis de résumer l'information publiée sur le sujet. Nous résumons également les difficultés auxquelles sont confrontés les femmes et les médecins issus des minorités raciales qui choisissent et mènent avec brio une carrière en cardiologie, de même que les obstacles systémiques à leur réussite. Au cours de la dernière décennie, les mouvements de justice sociale ont progressé. Les normes de professionnalisme évoluent, et tous les médecins doivent connaître et comprendre les avancées terminologiques. Dans le présent article, nous résumons les termes et les concepts clés, en y adjoignant des exemples propres au domaine de la cardiologie. Nous proposons aussi un nouveau paradigme de professionnalisme.

The Many Care Models to Treat Thoracic Aortic Disease in Canada: A Nationwide Survey of Cardiac Surgeons, Cardiologists, Interventional Radiologists, and Vascular Surgeons.

BACKGROUND: Several specialties treat thoracic aortic disease, resulting in multiple patient care pathways. This study aimed to characterize these varied care models to guide health policy. METHODS: A 57-question e-survey was sent to staff cardiac surgeons, cardiologists, interventional radiologists, and vascular surgeons at 7 Canadian medical societies. RESULTS: For 914 physicians, the response rate was 76% (86 of 113) for cardiac surgeons, 40% (58 of 146) for vascular surgeons, 24% (34 of 140) for radiologists, and 14% (70 of 515) for cardiologists. Several services admitted type B dissections (vascular 37%, cardiology 31%, cardiac 18%, other 7%), and care was heterogeneous. Ownership of disease management was overestimated relative to the perspective of the other specialties. Type A dissection admissions and treatment were more uniform, but emergent call coverage varied. A 24/7 aortic specialist on-call schedule was present only 4% of the time. "Aortic" case rounds promoted attendance by a broader aortic specialty contingency relative to rounds that were specialty specific. Although 89% of respondents felt an aortic team was best for patient care, only 54% worked at an institution with an aortic team present, and only 28% utilized an aortic clinic. Questions designed to define an aortic team derived 63 different combinations. CONCLUSIONS: Thoracic aortic disease follows a network of undefined and variable care pathways, despite its high-risk population in need of complex treatment considerations. Multidisciplinary aortic teams and clinics exist in low volume, and the "aortic team" remains an obscure construct. A multispecialty initiative to define the aortic team and outline standardized navigation pathways within the health systems hospitals is advocated.

CONTEXTE: La prise en charge de la maladie de l'aorte thoracique peut faire appel à plusieurs spécialités, ce qui a pour effet de multiplier les trajectoires de soins des patients. Cette étude visait à caractériser ces différents modèles de soins afin d'éclairer l'élaboration des politiques de santé. MÉTHODOLOGIE: Un sondage électronique de 57 questions a été envoyé aux chirurgiens cardiaques, aux cardiologues, aux radiologistes interventionnels et aux chirurgiens vasculaires membres de 7 associations médicales canadiennes. RÉSULTATS: Sur un total de 914 médecins, le taux de réponse a été de 76 % (86 sur 113) chez les chirurgiens cardiaques, de 40 % (58 sur 146) chez les chirurgiens vasculaires, de 24 % (34 sur 140) chez les radiologistes et de 14 % (70 sur 515) chez les cardiologues. Plusieurs services avaient admis des cas de dissection aortique de type B (chirurgie vasculaire 37 %, cardiologie 31 %, chirurgie cardiaque 18 %, autre 7 %) et les soins étaient hétérogènes. Les spécialistes surestimaient leur responsabilité de la prise en charge des cas par rapport à celle des autres spécialistes. Les admissions de cas de dissection de type A et leur traitement étaient plus uniformes, mais la présence de spécialistes de garde pouvant traiter les cas urgents était variable. La présence continue d'un spécialiste de l'aorte de garde n'était observée que pendant 4 % du temps. Les séances de discussion de cas « aortiques ¼ favorisaient la participation par une gamme plus large de spécialistes de l'aorte que les discussions axées sur une spécialité donnée. Si 89 % des répondants estimaient qu'une équipe « aortique ¼ était la meilleure option pour les soins aux patients, ils n'étaient que 54 % à travailler dans un établissement disposant d'une telle équipe et 28 % à utiliser les services d'une clinique de l'aorte. En réponse aux questions portant sur les éléments constitutifs d'une équipe aortique, 63 combinaisons différentes de spécialités ont été proposées. CONCLUSIONS: La prise en charge de la maladie de l'aorte thoracique emprunte un dédale de trajectoires de soins non définies et variables, alors que sa population à haut risque a besoin de traitements complexes. Les équipes multidisciplinaires et les cliniques spécialisées dans le traitement de l'aorte sont rares, et la notion d' « équipe aortique ¼ demeure un concept obscur. Nous préconisons une initiative réunissant des spécialistes de différents domaines pour définir les éléments constitutifs d'une équipe aortique et établir des trajectoires de navigation normalisées au sein des hôpitaux du système de santé.

Congenital Heart Disease (CHD) in Seniors: a Retrospective Study Defining a Brand New Cohort.

BACKGROUND: With improved surgical techniques and medical therapy, patients with congenital heart disease (CHD) are now expected to achieve normal life expectancies. As a result, a new cohort of senior patients with adult congenital heart disease (ACHD) is emerging which has not been well characterized. METHODS: This study is a retrospective chart review of patients with moderate to complex CHD over the age of 60 years in Southern Alberta. We examined the number, length, and reasons for hospitalizations, and identified common adult comorbidities. RESULTS: A total of 84 patients with CHD who were 60 years or older were identified. The average age was 67.9 ± 6.6 years, with the majority of patients having moderate CHD. The most common cardiac comorbidities were arrhythmia, hypertension, and heart failure, which were also the most common reasons for hospital admission. There were 1.85 admissions per 10 patient-years, with a median length of stay of 6.0 (3.8-10.5) days. CONCLUSIONS: With advanced age, the ACHD population is at risk of developing significant medical burden from acquired cardiac comorbidities, resulting in hospitalization. This analysis provides insight into disease characteristics of seniors with CHD. Further studies are needed to better understand this population and the association with geriatric syndromes.