CanCHD Study of Hematopoietic Cancers in Children With and Without Genetic Syndromes.

BACKGROUND: Individuals with genetic syndromes can manifest both congenital heart disease (CHD) and cancer attributable to possible common underlying pathways. To date, reliable risk estimates of hematopoietic cancer (HC) among children with CHD based on large population-based data remain scant. This study sought to quantify the risk of HC by the presence of genetic syndrome among children with CHD. METHODS AND RESULTS: Data sources were the Canadian CHD database, a nationwide database on CHD (1999-2017), and the CCR (Canadian Cancer Registry). Standardized incidence ratios were calculated for comparing HC incidences in children with CHD with the general pediatric population. A modified Kaplan-Meier curve was used to estimate the cumulative incidence of HC with death as a competing risk. A total of 143 794 children (aged 0-17 years) with CHD were followed up from birth to age 18 years for 1 314 603 person-years. Of them, 8.6% had genetic syndromes, and 898 HC cases were observed. Children with known syndromes had a substantially higher risk of incident HC than the general pediatric population (standardized incidence ratio, 13.4 [95% CI, 11.7-15.1]). The cumulative incidence of HC was 2.44% (95% CI, 2.11-2.76) among children with a syndrome and 0.79% (95% CI, 0.72-0.87) among children without a syndrome. Acute myeloid leukemia had a higher cumulative incidence during early childhood than acute lymphoblastic leukemia. CONCLUSIONS: This is the first large population-based analysis documenting that known genetic syndromes in children with CHD are a significant predictor of HC. The finding could be essential in informing risk-stratified policy recommendations for cancer surveillance in children with CHD.

Authorship Correction: Voice-Based Screening for SARS-CoV-2 Exposure in Cardiovascular Clinics (VOICE-COVID-19-II): Protocol for a Randomized Controlled Trial.

[This corrects the article DOI: 10.2196/41209.].

Voice-assisted Artificial Intelligence-enabled Screening for Severe Acute Respiratory Syndrome Coronavirus 2 Exposure in Cardiovascular Clinics: Primary Results of the VOICE-COVID-19-II Randomized Trial.

BACKGROUND: Voice-assisted artificial intelligence-based systems may streamline clinical care among patients with heart failure (HF) and caregivers; however, randomized clinical trials are needed. We evaluated the potential for Amazon Alexa (Alexa), a voice-assisted artificial intelligence-based system, to conduct screening for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a HF clinic. METHODS AND RESULTS: We enrolled 52 participants (patients and caregivers) from a HF clinic who were randomly assigned with a subsequent cross-over to receive a SARS-CoV-2 screening questionnaire via Alexa or health care personnel. The primary outcome was overall response concordance, as measured by the percentage of agreement and unweighted kappa scores between groups. A postscreening survey evaluated comfort with using the artificial intelligence-based device. In total, 36 participants (69%) were male, the median age was 51 years (range 34-65 years) years and 36 (69%) were English speaking. Twenty-one participants (40%) were patients with HF. For the primary outcome, there were no statistical differences between the groups: Alexa-research coordinator group 96.9% agreement and unweighted kappa score of 0.92 (95% confidence interval 0.84-1.00) vs research coordinator-Alexa group 98.5% agreement and unweighted kappa score of 0.95 (95% confidence interval 0.88-1.00) (P value for all comparisons > .05). Overall, 87% of participants rated their screening experience as good or outstanding. CONCLUSIONS: Alexa demonstrated comparable performance to a health care professional for SARS-CoV-2 screening in a group of patients with HF and caregivers and may represent an attractive approach to symptom screening in this population. Future studies evaluating such technologies for other uses among patients with HF and caregivers are warranted. NCT04508972.

Attitudes About Artificially Intelligent Interactive Voice Response Systems Using Amazon Alexa in Cardiovascular Clinics: Insights from the VOICE-COVID-19 Study.

The acceptability of artificially intelligent interactive voice response (AI-IVR) systems in cardiovascular research settings is unclear. As a result, we evaluated peoples' attitudes regarding the Amazon Echo Show 8 device when used for electronic data capture in cardiovascular clinics. Participants were recruited following the Voice-Based Screening for SARS-CoV-2 Exposure in Cardiovascular clinics study. Overall, 215 people enrolled and underwent screening (mean age 46.1; 55% females) in the VOICE-COVID study and 58 people consented to participate in a post-screening survey. Following thematic analysis, four key themes affecting AI-IVR acceptability were identified. These were difficulties with communication (44.8%), limitations with available interaction modalities (41.4%), barriers with the development of therapeutic relationships (25.9%), and concerns with universality and accessibility (8.6%). While there are potential concerns with the use of AI-IVR technologies, these systems appeared to be well accepted in cardiovascular clinics. Increased development of these technologies could significantly improve healthcare access and efficiency.

Voice-Based Screening for SARS-CoV-2 Exposure in Cardiovascular Clinics (VOICE-COVID-19-II): Protocol for a Randomized Controlled Trial.

BACKGROUND: The COVID-19 pandemic has disrupted the health care system, limiting health care resources such as the availability of health care professionals, patient monitoring, contact tracing, and continuous surveillance. As a result of this significant burden, digital tools have become an important asset in increasing the efficiency of patient care delivery. Digital tools can help support health care institutions by tracking transmission of the virus, aiding in the screening process, and providing telemedicine support. However, digital health tools face challenges associated with barriers to accessibility, efficiency, and privacy-related ethical issues. OBJECTIVE: This paper describes the study design of an open-label, noninterventional, crossover, randomized controlled trial aimed at assessing whether interactive voice response systems can screen for SARS-CoV-2 in patients as accurately as standard screening done by people. The study aims to assess the concordance and interrater reliability of symptom screening done by Amazon Alexa compared to manual screening done by research coordinators. The perceived level of comfort of patients when interacting with voice response systems and their personal experience will also be evaluated. METHODS: A total of 52 patients visiting the heart failure clinic at the Royal Victoria Hospital of the McGill University Health Center, in Montreal, Quebec, will be recruited. Patients will be randomly assigned to first be screened for symptoms of SARS-CoV-2 either digitally, by Amazon Alexa, or manually, by the research coordinator. Participants will subsequently be crossed over and screened either digitally or manually. The clinical setup includes an Amazon Echo Show, a tablet, and an uninterrupted power supply mounted on a mobile cart. The primary end point will be the interrater reliability on the accuracy of randomized screening data performed by Amazon Alexa versus research coordinators. The secondary end point will be the perceived level of comfort and app engagement of patients as assessed using 5-point Likert scales and binary mode responses. RESULTS: Data collection started in May 2021 and is expected to be completed in fall 2022. Data analysis is expected to be completed in early 2023. CONCLUSIONS: The use of voice-based assistants could improve the provision of health services and reduce the burden on health care personnel. Demonstrating a high interrater reliability between Amazon Alexa and health care coordinators may serve future digital tools to streamline the screening and delivery of care in the context of other conditions and clinical settings. The COVID-19 pandemic occurs during the first digital era using digital tools such as Amazon Alexa for disease screening, and it represents an opportunity to implement such technology in health care institutions in the long term. TRIAL REGISTRATION: ClinicalTrials.gov NCT04508972; https://clinicaltrials.gov/ct2/show/NCT04508972. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/41209.

Tetralogy of Fallot Across the Lifespan: A Focus on the Right Ventricle.

Tetralogy of Fallot is a cyanotic congenital heart disease, for which various surgical techniques allow patients to survive to adulthood. Currently, the natural history of corrected tetralogy of Fallot is underlined by progressive right ventricular (RV) failure due to pulmonic regurgitation and other residual lesions. The underlying cellular mechanisms that lead to RV failure from chronic volume overload are characterized by microvascular and mitochondrial dysfunction through various regulatory molecules. On a clinical level, these cardiac alterations are commonly manifested as exercise intolerance. The degree of exercise intolerance can be objectified and aid in prognostication through cardiopulmonary exercise testing. The timing for reintervention on residual lesions contributing to RV volume overload remains controversial; however, interval assessment of cardiac function and volumes by echocardiography and magnetic resonance imaging may be helpful. In patients who develop clinically important RV failure, clinicians should aim to maintain a euvolemic state through the use of diuretics while paying particular attention to preload and kidney function. In patients who develop signs of cardiogenic shock from right heart failure, stabilization through the use of inotropes and pressor is indicated. In special circumstances, the use of mechanical support may be appropriate. However, cardiologists should pay particular attention to residual lesions that may impact the efficacy of the selected device.

De nombreuses techniques chirurgicales permettent aux patients présentant une tétralogie de Fallot (TF), une forme de cardiopathie congénitale, de survivre jusqu'à l'âge adulte. À l'heure actuelle, l'évolution naturelle de la TF corrigée est caractérisée par une insuffisance ventriculaire droite (VD) progressive attribuable à une régurgitation pulmonaire et à d'autres lésions résiduelles. Les mécanismes cellulaires sous-jacents qui mènent à l'insuffisance VD due à une surcharge volumique chronique sont caractérisés par une dysfonction microvasculaire et mitochondriale faisant intervenir diverses molécules régulatrices. Sur le plan clinique, ces atteintes cardiaques se manifestent par une intolérance à l'effort qui peut être évaluée au moyen d'une épreuve d'effort cardiorespiratoire, ce qui permet de faciliter l'établissement d'un pronostic. Le moment propice pour une réintervention en cas de lésions résiduelles contribuant à la surcharge volumique du ventricule droit demeure controversé; toutefois, il peut être utile d'évaluer régulièrement la fonction et les volumes cardiaques au moyen d'une échocardiographie et de tests d'imagerie par résonance magnétique. En présence d'une insuffisance VD cliniquement importante, les cliniciens doivent tenter de maintenir les patients dans un état euvolémique en utilisant des diurétiques, tout en accordant une attention particulière à la précharge et à la fonction rénale. Si les patients manifestent des signes de choc cardiogénique associé à une insuffisance cardiaque droite, il convient de leur administrer des inotropes et des vasopresseurs pour stabiliser leur état. Dans certains cas, l'utilisation d'un dispositif d'assistance mécanique peut être appropriée. Cependant, les cardiologues doivent être attentifs aux lésions résiduelles, car elles peuvent influencer l'efficacité de ce dispositif.

Adherence to Objective Therapeutic Monitoring and Outcomes in Patients with Inflammatory Bowel Disease with Adalimumab Treatment. A Real-world Prospective Study.

BACKGROUND AND AIMS: Objective monitoring and effective early treatment using a treat-to-target approach are key to improving therapeutic outcomes in IBD patients. This study aimed to assess adherence to objective monitoring (clinical, biomarkers, and endoscopy) and its impact on clinical outcomes. METHODS: A prospective, multicenter study included consecutive IBD patients starting on adalimumab therapy between January 2019 and December 2020. Disease activity, assessed by the Harvey-Bradshaw index (HBI), partial Mayo, C-reactive protein (CRP), fecal calprotectin (FCAL), and endoscopy were evaluated at adalimumab initiation and 3, 6, 9 and 12 months. Therapeutic drug monitoring, changes in treatment, drug sustainability, and clinical outcomes were assessed. RESULTS: 104 IBD patients were enrolled (78.8% CD, median age 34.3 years, disease duration 9 years). During the 12 months follow-up, high adherence to clinical activity assessment was observed in both CD (81.3%- 87.7%) and UC patients (76.5-90.9%). CRP measurement decreased over time in both CD (37.3%-54.9%) and UC (29.4%-50.0%). The adherence to serial FCAL monitoring was low in CD (22.7-31.3%) and UC patients (17.6-56.0%). UC patients had higher adherence to early endoscopic assessment (<6 months) compared to CD patients (40.9% vs. 21.5%). Adherence to early combined clinical and biomarkers resulted in earlier dose optimization in CD and UC (log-rank<0.001), but drug sustainability was not different. The patients with early combined adherence had a significantly higher clinical remission rate at 1 year compared to non-adherence (70.2% vs. 29.8%, p=0.007) but no significant difference in UC patients. CONCLUSIONS: The adherence to early objective monitoring with combined clinical and biomarkers assessment in IBD patients starting adalimumab therapy led to dose optimization and improved 1-year clinical remission in CD but did not change drug sustainability and clinical remission in UC.

Synchronous Health Care Delivery for the Optimization of Cardiovascular and Renal Care in Patients with Type 2 Diabetes.

PURPOSE OF REVIEW: The current care model of type 2 diabetes (T2D) and its complications appears to be "asynchronous" with patient care divided by specialty. This model is associated with low use of guideline-directed medical therapies. RECENT FINDINGS: The use of integrated care models has been well described in the management of patients with T2D; this usually includes an endocrinologist coupled with a nutritionist and nurse. However, physician-based care models are largely "asynchronous," whereby the patient requires multiple different siloed specialties to manage their health care. To date, there has been limited exploration of synchronous care delivery, i.e., whereby multi-comorbid patients with T2D are seen simultaneously by health care providers from endocrinology, cardiology, and nephrology to optimize use of guideline-directed medical therapies (GDMT). Given the rising complexity of patients with T2D, further research is needed on the role of synchronous health care delivery in optimizing the use of GDMT and improving patient outcomes.

Feasibility of Incorporating Voice Technology and Virtual Assistants in Cardiovascular Care and Clinical Trials.

PURPOSE OF REVIEW: With the rising cost of cardiovascular clinical trials, there is interest in determining whether new technologies can increase cost effectiveness. This review focuses on current and potential uses of voice-based technologies, including virtual assistants, in cardiovascular clinical trials. RECENT FINDINGS: Numerous potential uses for voice-based technologies have begun to emerge within cardiovascular medicine. Voice biomarkers, subtle changes in speech parameters, have emerged as a potential tool to diagnose and monitor many cardiovascular conditions, including heart failure, coronary artery disease, and pulmonary hypertension. With the increasing use of virtual assistants, numerous pilot studies have examined whether these devices can supplement initiatives to promote transitional care, physical activity, smoking cessation, and medication adherence with promising initial results. Additionally, these devices have demonstrated the ability to streamline data collection by administering questionnaires accurately and reliably. With the use of these technologies, there are several challenges that must be addressed before wider implementation including respecting patient privacy, maintaining regulatory standards, acceptance by patients and healthcare providers, determining the validity of voice-based biomarkers and endpoints, and increased accessibility. SUMMARY: Voice technology represents a novel and promising tool for cardiovascular clinical trials; however, research is still required to understand how it can be best harnessed.

Neurocognitive disorders amongst patients with congenital heart disease undergoing procedures in childhood.

BACKGROUND: Amongst children with congenital heart disease (CHD), earlier age of repair improves cardiovascular outcomes. The effects of early intervention on neurodevelopment remains unclear. We assessed the association between early life repair, cardiopulmonary bypass (CPB) and the incidence of neurocognitive disorders (NCD) amongst CHD patients. METHODS: We created two retrospective cohorts from the Quebec CHD Database; with data from 1988 to 2010. Complexity of reparative procedures for CHD lesions were used as the proxy of CPB exposure with more complex procedure means longer exposure to CPB. Study Population 1 included pediatric patients with a single reparative procedure and compared patients with complex (long CBP) versus isolated shunt (short CBP) lesions. To assess the effects of CPB alone in Study Population 2 we compared patients with isolated atrial septal defects (ASD) who had surgical (short CBP) versus percutaneous (no CBP) repairs. The primary endpoint for both cohorts was development of an NCD. RESULTS: In Study population 1, 1174 patients underwent complex surgical repair and 1620 had a shunt closure. The incidence of NCDs was 2.45/100 person-years in the complex surgery group, and 2.08/100 person-years in the shunt closure group. The following were associated with increased risk of developing a NCD: surgical complexity (Hazard Ratio [HR] = 1.20, 95% Confidence Interval [CI]: 1.01-1.42), younger age at intervention (AAI) (HR = 1.20, 95% CI: 1.16-1.25), male sex (HR = 1.91, 95% CI: 1.61-2.27), and later calendar year at intervention (HR = 1.06, 95% CI: 1.04-1.07). Study population 2 had 527 isolated ASD patients; 202 underwent surgical repair and 325 had percutaneous closure. The incidence of NCDs was not statistically different between groups. Male sex (HR = 1.77, 95% CI: 1.08-2.89) and younger AAI (HR = 1.15, 95% CI: 1.06-1.25) were associated with increased NCD risk. CONCLUSION: Increased surgical complexity, male sex and younger AAI were associated with increased risk of NCDs in pediatric CHD patients. Surveillance protocols should be considered to identify NCDs in CHD patients after cardiac intervention.