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.