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

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Pregnancy related changes in Doppler gradients and left ventricular mechanics in women with sub-valvular or valvular aortic stenosis.

BACKGROUND: The aim of our study was to characterize echocardiographic changes during pregnancy in women with known LVOT obstruction or AS compared to the healthy pregnancy controls, and to assess the relationship with pregnancy outcomes. METHODS: We retrospectively studied 34 pregnant patients with congenital LVOT obstruction or AS with healthy age-matched pregnant controls. Patients with other significant valvular lesions, structural heart disease (LVEF < 40%), or prior valve surgery were excluded. All LVOTO/AS patients underwent a minimum of two consecutive echocardiograms between 1 year pre-conception and 1 year postpartum, with at least two studies during the pregnancy. Comprehensive echocardiographic evaluation was performed including speckle-tracking LV global longitudinal strain. RESULTS: A total of 83 echocardiograms from the study group and 34 echocardiograms from the control group were evaluated. Over the range of LVOTO/AS, a significantly greater increase in the AV gradients and LV and LA volumes were observed as compared with the controls. In the sub-group of LVOTO/AS pregnant women with ≥ moderate (n = 8) versus < moderate LVOTO/AS (n = 26), averaged 2nd /3rd trimester LVEF was lower (51 ± 12)% versus (58 ± 4)%, (p = 0.02) and GLS was lower (-19.5 ± 2.8) versus (21.2 ± 2.4), (p = 0.06). Pregnancy was well tolerated despite these changes. CONCLUSION: Among pregnant women with even milder forms of LVOTO/AS, increases in cardiac volumes and AV gradients can be expected over the course of pregnancy. Significant decreases in LV function and mechanics were only observed in women with moderate or greater LVOTO/AS, although still remained in normal range.

Pregnancy outcomes in women with cardiovascular disease: evolving trends over 10 years in the ESC Registry Of Pregnancy And Cardiac disease (ROPAC).

AIMS: Reducing maternal mortality is a World Health Organization (WHO) global health goal. Although maternal deaths due to haemorrhage and infection are declining, those related to heart disease are increasing and are now the most important cause in western countries. The aim is to define contemporary diagnosis-specific outcomes in pregnant women with heart disease. METHODS AND RESULTS: From 2007 to 2018, pregnant women with heart disease were prospectively enrolled in the Registry Of Pregnancy And Cardiac disease (ROPAC). Primary outcome was maternal mortality or heart failure, secondary outcomes were other cardiac, obstetric, and foetal complications. We enrolled 5739 pregnancies; the mean age was 29.5. Prevalent diagnoses were congenital (57%) and valvular heart disease (29%). Mortality (overall 0.6%) was highest in the pulmonary arterial hypertension (PAH) group (9%). Heart failure occurred in 11%, arrhythmias in 2%. Delivery was by Caesarean section in 44%. Obstetric and foetal complications occurred in 17% and 21%, respectively. The number of high-risk pregnancies (mWHO Class IV) increased from 0.7% in 2007-2010 to 10.9% in 2015-2018. Determinants for maternal complications were pre-pregnancy heart failure or New York Heart Association >II, systemic ejection fraction <40%, mWHO Class 4, and anticoagulants use. After an increase from 2007 to 2009, complication rates fell from 13.2% in 2010 to 9.3% in 2017. CONCLUSION: Rates of maternal mortality or heart failure were high in women with heart disease. However, from 2010, these rates declined despite the inclusion of more high-risk pregnancies. Highest complication rates occurred in women with PAH.

Ventilatory and sensory responses to incremental exercise in adults with a Fontan circulation.

Many adults with single-ventricle congenital heart disease who have undergone a Fontan procedure have abnormal pulmonary function resembling restrictive lung disease. Whether this contributes to ventilatory limitations and increased dyspnea has not been comprehensively studied. We recruited 17 Fontan participants and 17 healthy age- and sex-matched sedentary controls. All participants underwent complete pulmonary function testing followed by a symptom-limited incremental cardiopulmonary cycle exercise test with detailed assessments of dyspnea and operating lung volumes. Fontan participants and controls were well matched for age, sex, body mass index, height, and self-reported physical activity levels (all P > 0.05), although Fontan participants had markedly reduced cardiorespiratory fitness and peak work rates ( P < 0.001). Fontan participants had lower values for most pulmonary function measurements relative to controls with 65% of Fontan participants showing evidence of a restrictive ventilatory defect. Relative to controls, Fontan participants had significantly higher breathing frequency, end-inspiratory lung volume (% total lung capacity), ventilatory inefficiency (high ventilatory equivalent for CO2), and dyspnea intensity ratings at standardized absolute submaximal work rates. There were no between-group differences in qualitative descriptors of dyspnea. The restrictive ventilatory defect in Fontan participants likely contributes to their increased breathing frequency and end-inspiratory lung volume during exercise. This abnormal ventilatory response coupled with greater ventilatory inefficiency may explain the increased dyspnea intensity ratings in those with a Fontan circulation. Interventions that enhance the ventilatory response to exercise in Fontan patients may help optimize exercise rehabilitation interventions, resulting in improved exercise tolerance and exertional symptoms. NEW & NOTEWORTHY This is the first study to comprehensively characterize both ventilatory and sensory responses to exercise in adults that have undergone the Fontan procedure. The majority of Fontan participants had a restrictive ventilatory defect. Compared with well-matched controls, Fontan participants had increased breathing frequency, end-inspiratory lung volume, and ventilatory inefficiency. These abnormal ventilatory responses likely form the mechanistic basis for the increased dyspnea intensity ratings observed in our Fontan participants during exercise.

Creatinine versus cystatin C to estimate glomerular filtration rate in adults with congenital heart disease: Results of the Boston Adult Congenital Heart Disease Biobank.

BACKGROUND: Glomerular filtration rate is a key physiologic variable with a central role in clinical decision making and a strong association with prognosis in diverse populations. Reduced estimated glomerular filtration rate (eGFR) is common among adults with congenital heart disease (ACHD). METHODS: We conducted a prospective cohort study of outpatient ACHD ≥18 years old seen in 2012-2017. Creatinine and cystatin C were measured; eGFR was calculated using either the creatinine or cystatin C Chronic Kidney Disease-Epidemiology Collaboration equation (CKD-EPICr and CKD-EPICysC, respectively). Survival analysis was performed to define the relationship between eGFR and both all-cause mortality and a composite outcome of death or nonelective cardiovascular hospitalization. RESULTS: Our cohort included 911 ACHD (39 ±â€¯14 years old, 49% female). Mean CKD-EPICr and CKD-EPICysC were similar (101 ±â€¯20 vs 100 ±â€¯23 mL/min/1.73 m2), but CKD-EPICr estimates were higher for patients with a Fontan circulation (n = 131, +10 ±â€¯19 mL/min/1.73 m2). After mean follow-up of 659 days, 128 patients (14.1%) experienced the composite outcome and 31 (3.4%) died. CKD-EPICysC more strongly predicted all-cause mortality (eGFR <60 vs >90 mL/min/1.73 m2: CKD-EPICysC unadjusted HR = 20.2 [95% CI 7.6-53.1], C-statistic = 0.797; CKD-EPICr unadjusted HR = 4.6 [1.7-12.7], C-statistic = 0.620). CKD-EPICysC independently predicted the composite outcome, whereas CKD-EPICr did not (CKD-EPICysC adjusted HR = 3.0 [1.7-5.3]; CKD-EPICr adjusted HR = 1.5 [0.8-3.1]). Patients reclassified to a lower eGFR category by CKD-EPICysC, compared with CKD-EPICr, were at increased risk for the composite outcome (HR = 2.9 [2.0-4.3], P < .0001); those reclassified to a higher eGFR class were at lower risk (HR = 0.5 [0.3-0.9], P = .03). CONCLUSIONS: Cystatin C-based eGFR more strongly predicts clinical events than creatinine-based eGFR in ACHD. Creatinine-based methods appear particularly questionable in the Fontan circulation.

Pregnancy in women with heart disease: risk assessment and management of heart failure.

Heart disease, present in 0.5% to 3% of pregnant women, is an important cause of morbidity and the leading cause of death among pregnant women in the developed world. Certain heart conditions are associated with an increased risk of heart failure during pregnancy or the postpartum period; for these conditions, management during pregnancy benefits from multidisciplinary care at a center with expertise in pregnancy and heart disease. This article focuses on cardiac risks and management strategies for women with acquired and congenital heart disease who are at increased risk of heart failure during pregnancy.

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

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

Les profils de facteurs de risque, les facteurs sociaux et environnementaux, le tableau clinique, le diagnostic et le traitement des maladies cardiovasculaires montrent des différences importantes entre les femmes et les hommes. Il est plus probable que les femmes expérimentent des problèmes de santé complexes et multifactoriels, qui sont souvent en relation avec les disparités dans l'accès aux soins, la prévalence des facteurs de risque, les différences biologiques entre les sexes, les facteurs liés au genre et les facteurs socioculturels. De plus, la sensibilisation à la nature et à la relation intersectionnelles des déterminants socioculturels de santé, notamment les facteurs liés au sexe et au genre, qui influencent l'accès aux soins et les résultats cliniques des femmes atteintes d'une maladie cardiovasculaire demeure insaisissable. La présente revue résume la littérature qui porte sur les facteurs de risque liés au sexe et au genre peu reconnus qui se recoupent aux facteurs psychosociaux, économiques et culturels dans le diagnostic, le traitement et les résultats cliniques en lien avec la santé cardiovasculaire des femmes.

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

Background: Significant advances in managing congenital heart disease (CHD) have occurred over the past few decades, resulting in a fast-growing adult patient population with distinct needs requiring urgent attention. Research has recently highlighted the prevalence of neurocognitive differences among adults living with CHD. Yet, there is a lack of knowledge about the perspectives of people living with CHD and family members/caregivers on brain health. We sought to explore their perspectives to guide future research and clinical endeavours. Methods: Using the principles of integrated knowledge translation and qualitative interpretive description, we conducted 2 focus groups with 7 individuals with CHD and their family members as part of a virtual forum on brain health in CHD. Data analysis followed the principles of interpretive description. Results: A lack of understanding about overall brain health and neurocognitive differences in adult CHD was identified. To increase overall knowledge about brain health, initiatives should (1) focus on the individual living with CHD, involving family members and peers; (2) use social media and health care encounters for knowledge exchange; and (3) ensure a "balancing act" in the information provided to avoid feelings of worry and uncertainty about the future while simultaneously empowering people living with CHD. Conclusions: There is a pressing need for better education about brain health among individuals living with CHD. Our findings can guide clinicians in developing programmes of care and (re)design health services that address the brain-heart axis and neurocognitive differences in CHD.

Contexte: Des progrès significatifs ont été réalisés au cours des dernières décennies dans la prise en charge des cardiopathies congénitales, et il en résulte une croissance rapide de la population des patients adultes dont les besoins distincts requièrent une attention urgente. Des études récentes ont mis en évidence la prévalence des différences neurocognitives chez les adultes atteints de cardiopathies congénitales (CC). À ce jour, les connaissances sur les points de vue des personnes qui vivent avec la CC, des membres de leurs familles et de leurs aidants au sujet de la santé du cerveau sont insuffisantes. Notre objectif était d'explorer ces points de vue afin d'orienter les recherches à venir et les initiatives cliniques. Méthodologie: En nous basant sur les approches d'application des connaissances intégrées et de description interprétative (DI) qualitative, nous avons organisé deux groupes de discussion avec 7 personnes atteintes de CC et les membres de leur famille, dans le cadre d'un forum virtuel sur la santé du cerveau dans les CC. L'analyse des données a été réalisée selon les principes de la DI. Résultats: Un manque de connaissances au sujet de la santé du cerveau en général et des différences neurocognitives chez les personnes atteintes de CC a été observé. Afin d'améliorer le niveau des connaissances générales sur la santé du cerveau, les initiatives doivent : (1) être centrées sur les personnes qui vivent avec la CC, avec la participation des membres de leur famille et d'autres patients; (2) tirer profit des médias sociaux et des rencontres en contexte de soins de santé pour le partage de connaissances; et (3) veiller à maintenir un équilibre, puisqu'il convient d'outiller les personnes atteintes de CC sans toutefois causer de sentiment d'inquiétude ou d'incertitude quant à l'avenir. Conclusions: Nous avons constaté le besoin urgent d'améliorer les connaissances des personnes qui vivent avec une CC au sujet de la santé du cerveau. Les résultats obtenus pourront guider les cliniciens dans l'élaboration de programmes de soins et la conception (ou la refonte) de services de soins de santé qui intègrent l'axe cerveau-cœur et les différences neurocognitives associées aux CC.

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.

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

BACKGROUND: Adults with congenital heart disease (ACHD) experience a high prevalence of atrial arrhythmia (AA) and thromboembolic cerebrovascular complications. However, data on AA and associated long-term outcomes are limited in ACHD patients with coarctation of the aorta (CoA). OBJECTIVES: This study aimed to characterize the prevalence and risk factors for AA and thromboembolic complications in adults with CoA. METHODS: We conducted a retrospective cohort study in a tertiary ACHD care center and included consecutive CoA patients older than 18 years old with more than one year of follow-up. RESULTS: Two hundred seventy patients with CoA were followed for 7.2 ± 3.95 years. The mean age was 35.3 ± 11.1 and 55.2% were male. Patients had a mean of 2.1 ± 1.8 cardiovascular surgical or transcatheter procedures. Thirty-five patients (13%) had AA. Ten subjects (3.8%) had a thromboembolic cerebrovascular event, of which four (1.4%) had AA. In univariate analysis, age (p = 0.005) and total intracardiac interventions (p = 0.007) were associated with the presence of AA. Age (p = 0.021), history of heart failure (p = 0.022), and dyslipidemia (p = 0.019) were associated with thromboembolism. In multivariate analysis, age (p < 0.001) and intracardiac interventions (p = 0.007) were associated with AA. CONCLUSIONS: The rate of AA is higher in adults with CoA than in the general population but lower than in other ACHD. Increasing age and intracardiac interventions were associated with AA. The rate of thromboembolic events was low. Some traditional risk factors for stroke may apply. Larger studies are needed to validate predictors for stroke in this population.

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.

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.

COVID-19-Related Thrombotic and Bleeding Events in Adults With Congenital Heart Disease.

Background: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications. Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients. Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression. Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P < 0.0001) and were in more advanced physiological stage (P = 0.032) and NYHA functional class (P = 0.01), had lower baseline oxygen saturation (P = 0.0001), and more frequently had a history of atrial arrhythmia (P < 0.0001), previous hospitalization for heart failure (P < 0.0007), and were more likely hospitalized for COVID-19 (P < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P < 0.0001) were independently associated with increased risk of TE/bleeding complications. Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants.

A case of Starr-Edwards valve thrombosis in pregnancy.

Starr-Edwards ball-in-cage prosthetic heart valves, although durable, are associated with a particularly high rate of thromboembolic complications. This valve is seldom used in North America, and is certainly not the valve of choice in a woman of childbearing age. Few reports exist from the 1970s of thrombotic complications in pregnant women with Starr-Edwards prostheses, and the optimal management strategy for such valves is unclear. Here, the case is reported of a 31-year-old woman with a Starr-Edwards prosthesis in the mitral position who was transferred to the authors' center at six weeks' gestation with pulmonary edema. Transthoracic echocardiography demonstrated thrombosis of the prosthetic valve, with a mean gradient of 23 mmHg. When treated initially with intravenous heparin and furosemide the patient improved significantly; however, the optimal management going forward was unclear. Here, the options for anticoagulation during pregnancy and for management in the event of valve thrombosis are reviewed. In the absence of any clear guidelines, a thorough discussion of the various risks and benefits with the patient is necessary, but ultimately any consideration of the risk to the mother is paramount.

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

Background: Significant atrioventricular valve regurgitation (AVVR) is prevalent in Fontan adults. Two-dimensional speckle-tracking echocardiography allows for evaluation of subclinical myocardial dysfunction and offers technical benefits. We aimed to evaluate the association of AVVR with echocardiographic parameters and adverse outcomes. Materials and Methods: Fontan adults (≥18 years) with lateral tunnel or extracardiac connection actively followed at our institution were retrospectively reviewed. Patients with AVVR on most recent transthoracic echocardiogram (≥grade 2 as per American Society of Echocardiography guidelines) were matched with Fontan controls. Echocardiographic parameters, including global longitudinal strain (GLS), were measured. The composite outcome of Fontan failure included Fontan conversion, protein losing enteropathy, plastic bronchitis, and New York Heart Association Class III/IV. Results: Sixteen patients (14%, mean age 28.4 ± 7.0 years) with predominantly moderate AVVR (81%) were identified. The mean duration of AVVR was 8.1 ± 5.8 months. There was no significant reduction in ejection fraction (EF) (51.2% ± 11.7% vs. 54.7% ± 10.9%, P = 0.39) or GLS (-16.0% ± 5.2% vs. -16.0% ± 3.5%, P = 0.98) associated with AVVR. Larger atrial volumes and longer deceleration time (DT) were observed in the AVVR group. Patients with AVVR and a worse GLS (≥-16%) had higher E velocity, DT, and medial E/E' ratio. The incidence of Fontan failure did not differ from controls (38% vs. 25%, P = 0.45). Patients with worse GLS (≥-16%) demonstrated a marked trend toward a higher incidence of Fontan failure (67% vs. 20%, P = 0.09). Conclusions: In Fontan adults, a short duration of AVVR did not influence EF or GLS but was associated with larger atrial volumes and those with worse GLS demonstrated some differences in diastolic parameters. Larger multicenter studies throughout its disease course are warranted.

Hepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation.

Over time, long-term survival has dramatically increased for patients with complex congenital heart disease who undergo the Fontan operation. With this increased survival, it has become apparent that such a circulation has important consequences for other organ systems, particularly the liver and kidney. The adverse milieu created by chronic venous hypertension, low cardiac output, and an inflammatory state contribute to the pathologic changes observed in the liver and kidneys over the long term in Fontan patients. The clinical importance of these hepatic and renal comorbidities have only recently begun to be recognized in the context of increasing life expectancy in this population. The objectives of this review are to provide an overview of the pathophysiology of the Fontan circulation and how liver and kidney disease evolve in this setting; to summarize the current evidence base as it relates to the diagnostic approach to liver and kidney disease in Fontan patients; and to discuss the therapeutic approaches to Fontan- associated liver and kidney disease. Given that this is a very active area of research in congenital heart disease, we have identified knowledge gaps and priority research areas to improve the care of Fontan patients. These include establishing the optimal diagnostic tests to detect and track liver and kidney disease change over time, determining which treatable risk factors contribute to the development of liver and kidney disease, and evaluating therapies to prevent or slow progression of liver and kidney disease.