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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., (Copyright © 2023 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest”., (Copyright © 2021. Published by Elsevier B.V.)

Background: Heart failure (HF) is the leading cause of death in adult patients with congenital heart disease (ACHD). No risk prediction model exists for HF hospitalization (HFH) for ACHD patients. We aimed to develop a clinically relevant one-year risk prediction system to identify ACHD patients at high risk for HFH., Methods: Data source was the Quebec CHD Database. A retrospective cohort including all ACHD patients aged 18-64 (1995-2010) was constructed for assessing the cumulative risk of HFH adjusting for competing risk of death. To identify one-year predictors of incident HFH, multivariable logistic regressions were employed to a nested case-control sample of all ACHD patients aged 18-64 in 2009. The final model was used to create a risk score system based on adjusted odds ratios., Results: The cohort included 29,991 ACHD patients followed for 648,457 person-years. The cumulative HFH risk by age 65 was 12.58%. The case-control sample comprised 26,420 subjects, of whom 189 had HFHs. Significant one-year predictors were age ≥ 50, male sex, CHD lesion severity, recent 12-month HFH history, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes mellitus. The created risk score ranged from 0 to 19. The corresponding HFH risk rose rapidly beyond a score of 8. The risk scoring system demonstrated excellent prediction performance., Conclusions: One eighth of ACHD population experienced HFH before age 65. Age, sex, CHD lesion severity, recent 12-month HFH history, and comorbidities constructed a risk prediction model that successfully identified patients at high risk for HFH., Competing Interests: Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: We sought to describe training activities on self-management support (SMS) for asthma educators and the effects of SMS provided by trained educators on asthma patient outcomes., Methods: We conducted a systematic review of six medical databases and sought for trials assessing SMS provided for adults with asthma by trained educators. Two reviewers independently selected and extracted data on asthma educators' training activities and patient outcomes. We performed meta-analyses for asthma-related quality of life (QoL) and asthma control., Results: We screened 3217 records and included 16 trials. Learning activities and assessments were reported in 8/16 and 4/16 trials, respectively. Compared to usual care, trained asthma educators provided SMS that resulted in clinically important improvements in QoL (pooled mean difference [MD] = 0.52; 95% confidence interval [95%CI]: 0.19 to 0.83) and asthma control (pooled MD= -0.68; 95%CI: -0.99 to -0.38)., Conclusion: Although asthma-specific SMS provided by trained educators had a beneficial effect over the current care, our results highlight the need to better describe training activities for asthma educators., Practice Implications: This systematic review provides key elements of efficient training activities for asthma educators and reaffirms the importance of training educators to provide SMS in order to improve asthma patients' QoL and asthma control., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Equine represents an attractive animal model for musculoskeletal tissue diseases, exhibiting much similarity to the injuries that occur in humans. Cell therapy and tissue bioengineering have been widely used as a therapeutic alternative by regenerative medicine in musculoskeletal diseases. Thus, the aim of this study was to produce an acellular biomaterial of equine skeletal muscle and to evaluate its effectiveness in supporting the in vitro culture of equine induced pluripotency stem cells (iPSCs). Biceps femoris samples were frozen at -20°C for 4 days and incubated in 1% sodium dodecyl sulfate (SDS), 5 mM EDTA + 50 mM Tris and 1% Triton X-100; the effectiveness of the decellularization was evaluated by the absence of remnant nuclei (histological and 4',6-diamidino-2-phenylindole [DAPI] analysis), preservation of extracellular matrix (ECM) proteins (immunofluorescence and immunohistochemistry) and organization of ECM ultrastructure (scanning electron microscopy). Decellularized samples were recellularized with iPSCs at the concentration of 50,000 cells/cm 2 and cultured in vitro for 9 days, and the presence of the cells in the biomaterial was evaluated by histological analysis and presence of nuclei. Decellularized biomaterial showed absence of remnant nuclei and muscle fibers, as well as the preservation of ECM architecture, vascular network and proteins, laminin, fibronectin, elastin, collagen III and IV. After cellularization, iPSC nuclei were present at 9 days after incubation, indicating the decellularized biomaterial-supported iPSC survival. It is concluded that the ECM biomaterial produced from the decellularized equine skeletal muscle has potential for iPSC adhesion, representing a promising biomaterial for regenerative medicine in the therapy of musculoskeletal diseases., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: There is no systematic evidence review of the long-term results of surgical pulmonary valve replacement (PVR) dedicated to adults with repaired tetralogy of Fallot (rTOF) and pulmonary regurgitation., Methods: Our primary objective was to determine whether PVR reduced long-term mortality in adults with rTOF compared with conservative therapy. Secondary objectives were to determine the postoperative incidence rate of death, the changes in functional capacity and in right ventricular (RV) volumes and ejection fraction after PVR, and the postoperative incidence rate of sustained ventricular arrhythmias. A systematic search of multiple databases for studies was conducted without limits., Results: No eligible randomized controlled trial or cohort study compared outcomes of PVR and conservative therapy in adults with rTOF. We selected 10 cohort studies (total 657 patients) reporting secondary outcomes. After PVR, the pooled incidence rate of death was 1% per year (95% confidence interval [CI] 0-1% per year) and the pooled incidence rate of sustained ventricular arrhythmias was 1% per year (95% CI 1%-2% per year). PVR improved symptoms (odds ratio for postoperative New York Heart Association functional class > II 0.08, 95% CI 0.03-0.24). Indexed RV end-diastolic (-61.29 mL/m 2 , -43.64 to -78.94 mL/m 2 ) and end-systolic (-37.20 mL/m 2 , -25.58 to -48.82 mL/m 2 ) volumes decreased after PVR, but RV ejection fraction did not change (0.19%, -2.36% to 2.74%). The effect of PVR on RV volumes remained constant regardless of functional status., Conclusion: Studies comparing PVR and conservative therapy exclusively in adults with rTOF are lacking. After PVR, the incidence rates of death and ventricular tachycardia are both 1 per 100 patient-years. Pooled analyses demonstrated an improved functional status and a reduction in RV volumes., (Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

As life expectancy in patients with congenital heart disease (CHD) has improved, the risk for developing noncardiac morbidities is increasing in adult patients with CHD (ACHD). Among these noncardiac complications, malignancies significantly contribute to the disease burden of ACHD patients. Epidemiologic studies of cancer risk in CHD patients are challenging because they require large numbers of patients, extended follow-up, detailed and validated clinical data, and appropriate reference populations. However, several observational studies suggest that cancer risks are significantly elevated in patients with CHD compared with the general population. CHD and cancer share genetic and environmental risk factors. An association with exposure to low-dose ionizing radiation secondary to medical therapeutic or diagnostic procedures has been reported. Patients with Down syndrome, as well as, to a lesser extent, deletion of 22q11.2 and renin-angiotensin system pathologies, may manifest both CHD and a predisposition to cancer. Such observations suggest that carcinogenesis and CHD may share a common basis in some cases. Finally, specific conditions, such as Fontan circulation and cyanotic CHD, may lead to multisystem consequences and subsequently to cancer. Nonetheless, there is currently no clear consensus regarding appropriate screening for cancer and surveillance modalities in CHD patients. Physicians caring for patients with CHD should be aware of this potential predisposition and meet screening recommendations for the general population fastidiously. An interdisciplinary and global approach is required to bridge the knowledge gap in this field., (Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: The attrition of right ventricle to pulmonary artery (RV-PA) grafts has been attributed in part to the body's immunologic response. We hypothesized that antibodies developed through blood transfusion, directed against the grafts, may result in accelerated degeneration and the need for re-intervention., Methods: This is a population-based study of the province of Quebec. We included children born between January 1, 1987 to December 31, 2006 who were diagnosed with a cono-truncal anomaly and had an RV-PA graft. The patients were followed for transfusion exposure and RV-PA graft re-intervention. Time to re-intervention in those exposed versus non-exposed was analyzed using Cox regression. Analysis was done in two time periods, before and after the calendar year 2000, given the change in blood preparation in the province of Quebec., Results: There were 413 patients who met the inclusion criteria of a cono-truncal disorder. Of the whole study population, 69% received a blood transfusion. Cox regression analysis showed that among patients who had the initial graft performed (n=181) before year 2000, having 2 or more blood transfusion was associated with an nearly tripled risk of a re-intervention comparing to no blood transfusion (hazard ratio of 2.88; 95% confidence interval 1.05-7.91). In patients who had the initial graft performed after year 2000 (n=232), the associated risk increase was 7-fold (hazard ratio of 7.01; 95% confidence interval 3.06-16.02). Kaplan-Meier analyses confirmed the significant difference in the re-intervention free survival probabilities between those who received 2 or more blood product transfusion and those who did not as well: prior to year 2000 (67.9% vs. 88.0% at 5years, p=0.0201) as well as after year 2000 (39.7% vs. 82.8% at 5years, p<0.0001)., Conclusion: In this population-based analysis, repeated blood product transfusion was associated with a significant increased risk of a need for RV-PA graft re-intervention. This data strongly suggest that repeated blood transfusion may adversely impact graft longevity., (Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.)

Background: Significant pulmonary regurgitation, declining right-sided ejection fraction, increased right ventricular (RV) volumes as well as left ventricular (LV) dysfunction have all been identified as predictors of poor outcomes in patients with congenital heart disease (CHD). The prognostic value of the cardiac output (CO) in these patients however has never been studied., Methods: All consecutive ambulatory adult patients with CHD referred for magnetic resonance imaging (MRI) at the Montreal Children's Hospital between June 2007 and May 2009 were included. Right ventricular (RV) and left ventricular (LV) variables including end diastolic and end systolic volumes (EDV, ESV respectively), ejection fractions (EF) and regurgitant volumes were obtained. Cardiac index (CI) was calculated. Patients were followed for cardiac-related hospitalizations and cardiac interventions., Results: Ninety-six patients were included. Median follow up was 3.9 ± 1.4 years. Nineteen percent of patients had a systemic CI<2.4 l/min/m(2). LVEDV, LVEF and RVEF were significantly diminished in the low CI group with a significant increase in RVESV and total regurgitant volume. Best predictors of low CI were LVEF (AUC=0.74), RVEF (AUC=0.71), total RV regurgitant volume (AUC=0.64) and RVESV (AUC=0.563). Low systemic CI was the best predictor of cardiac-related hospitalizations (hazard ratio 3.5, 95% confidence interval 1.5-8.5) and cardiac interventions (hazard ratio 2.2, 95% confidence interval 1.3-4.0), superior to LVEF, RVEF, total regurgitant volume and RVESV parameters., Conclusions: In patients with congenital heart disease, cardiac index is the best predictor of cardiac hospitalizations and cardiac interventions., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined., Methods: Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated., Results: To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults., Conclusions: CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR., (Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

This Canadian Cardiovascular Society position statement aims to provide succinct perspectives on key issues in the management of thoracic aortic disease (TAD). This document is not a comprehensive overview of TAD and important elements of the epidemiology, presentation, diagnosis, and management of acute aortic syndromes are deliberately not discussed; readers are referred to the 2010 guidelines published by the American Heart Association, American College of Cardiology, American Association for Thoracic Surgery, and other stakeholders. Rather, this document is a practical guide for clinicians managing adult patients with TAD. Topics covered include size thresholds for surgical intervention, emerging therapies, imaging modalities, medical and lifestyle management, and genetics of TAD. The primary panel consisted of experts from a variety of disciplines that are essential for comprehensive management of TAD patients. The methodology involved a focused literature review with an emphasis on updates since 2010 and the use of Grading of Recommendations Assessment, Development, and Evaluation methodology to arrive at specific recommendations. The final document then underwent review by a secondary panel. This document aims to provide recommendations for most patients and situations. However, the ultimate judgement regarding the management of any individual patients should be made by their health care team., (Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: The most common congenital anomaly in adults is secundum, which can be closed using a surgical or transcatheter approach. Despite the growing use of transcatheter ASD closure, few studies have examined the cost-effectiveness of this strategy. We sought to compare the long-term cost effectiveness of transcatheter and surgical closure of secundum in adults., Methods: A decision-analytic model was used with all clinical outcome parameter estimates obtained from the province-wide Québec Congenital Heart Disease Database. Costs were obtained from a single academic centre (Canadian dollars). A cost-effectiveness analysis using a discrete event Monte Carlo simulation model from the perspective of a single third party payer and multiple sensitivity analyses were performed. Patients were followed for a maximum of 5 years after ASD closure., Results: Between l998 and 2005, we identified 718 adults (n=335 transcatheter; n=383 surgical) who underwent ASD closure in Quebec. The 5-year cost of surgical closure was $15,304 SD $4581 versus $11,060 SD $5169 for the transcatheter alternative. At 5 years, transcatheter closure was marginally more effective than surgery (4.683 SD 0.379 life-years versus 4.618 SD 0.638 life-years). Probabilistic sensitivity analyses demonstrated that transcatheter ASD closure was a dominant strategy with an 80% probability of cost savings and equal or greater efficacy compared to surgical treatment., Conclusion: Although definitive conclusions are limited given the observational nature of the primary data sources, transcatheter ASD closure appeared to be a cost-effective strategy associated with slightly improved clinical outcomes and reduced costs compared to surgical closure at 5-years follow-up., (Copyright © 2013. Published by Elsevier Ireland Ltd.)

Background: Atrial fibrillation (AF) is largely regarded to be initiated from left atrial (LA) dilatation, with subsequent dilatation of the right atrium (RA) in those who progress to chronic AF. We hypothesized that in adult patients with right-sided congenital heart disease (CHD) and AF, RA dilatation will predominate with subsequent dilatation of the left atrium, as a mirror image., Methods: Adult patients with diagnosis of right-sided, ASD or left-sided CHD who had undergone an echocardiographic study and electrocardiographic recording in 2007 were included. RA and LA area were measured from the apical view. AF was diagnosed from a 12-lead electrocardiogram or Holter recording. A multivariate logistic regression model was used to identify predictors of AF and linear regression models were performed to measure relationship between RA and LA area and AF., Results: A total of 291 patients were included in the study. Multivariate analysis showed that age (p=0.0001), RA (p=0.025) and LA area (p=0.0016) were significantly related to AF. In patients with pure left-sided pathologies, there was progressive and predominant LA dilatation that paralleled the development of AF from none to paroxysmal to chronic AF. In patients with pure right-sided pathologies, there was a mirror image of progressive and predominant RA dilatation with the development of AF., Conclusion: We observed a mirror image atrial dilatation in patients with right sided disease and AF. This may provide novel mechanistic insight as to the origin of AF in these patients and deserves further studying in the form of targeted electrophysiological studies., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: Right ventricular (RV) diastolic dysfunction precedes RV systolic dysfunction. Improvement in noninvasive assessment of RV diastolic function may enable earlier detection of RV dysfunction, especially important in the assessment of patients with congenital heart disease. We investigated a new parameter we call RV end-diastolic wall stress (RVEDWS) in an effort to better characterize RV diastolic function., Methods: We retrospectively studied consecutive adults with right-sided congenital heart disease between January 2005 and November 2006. RVEDWS was calculated with the Laplace law: r × p/λ, where r = basal RV dimension at end-diastole, p = RV end-diastolic pressure obtained from catheterization of the right side of the heart, and λ = thickness of RV free wall at end-diastole in the subcostal view. Calculated RVEDWS was correlated to echocardiographically derived right atrial (RA) measurements by means of the Pearson correlation., Results: Twenty-four patients, aged 41 ± 15 years, were included in the study. Mean RVEDWS was 20 ± 11 g/cm(2) (range, 3-46 g/cm(2)). RVEDWS correlated significantly with RA area and volume (r = 0.71, P < 0.0001; r = 0.69, P < 0.001, respectively). An RVEDWS > 17 g/cm(2) had a sensitivity of 91% and specificity of 85% in predicting significant RA enlargement. RVEDWS was significantly higher in patients with RV volume overload compared with those with pressure or normal loading conditions (28 g/cm(2) vs 17 g/cm(2), P = 0.01)., Conclusions: RVEDWS correlates significantly with RA size and differs considerably between RV volume and pressure overload states. Further work is needed to determine whether this RV diastolic parameter can be predictive of clinical outcomes in patients with RV loading lesions., (Copyright © 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Objectives: The purpose of this study was to assess the comparative effectiveness and long-term safety of transcatheter versus surgical closure of secundum atrial septal defects (ASD) in adults., Background: Transcatheter ASD closure has largely replaced surgery in most industrialized countries, but long-term data comparing the 2 techniques are limited., Methods: We performed a retrospective population-based cohort study of all patients, ages 18 to 75 years, who had surgical or transcatheter ASD closure in Québec, Canada's second-largest province, using provincial administrative databases. Primary outcomes were long-term (5-year) reintervention and all-cause mortality. Secondary outcomes were short-term (1-year) onset of congestive heart failure, stroke, or transient ischemic attack, and markers of health service use., Results: Of the 718 ASD closures performed between 1988 and 2005, 383 were surgical and 335 were transcatheter. The long-term reintervention rate was higher in patients with transcatheter ASD closure (7.9% vs. 0.3% at 5 years, p = 0.0038), but the majority of these reinterventions occurred in the first year. Long-term mortality with the transcatheter technique was not inferior to surgical ASD closure (5.3% vs. 6.3% at 5 years, p = 1.00). Secondary outcomes were similar in the 2 groups., Conclusions: Transcatheter ASD closure is associated with a higher long-term reintervention rate and long-term mortality that is not inferior to surgery. Overall, these data support the current practice of using transcatheter ASD closure in the majority of eligible patients and support the decision to intervene on ASD with significant shunts before symptoms become evident., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: There are more adults than children with congenital heart disease. Of over 96,000 ACHD patients in Canada, approximately 50% require ongoing expert care. In spite of published recommendations, data on the quality of care for ACHD patients are lacking., Methods: Survey methodology targeted all Canadian Adult Congenital Heart (CACH) network affiliated ACHD centers. Clinics were asked to prospectively collect outpatient and procedural volumes for 2007. In 2008, centers were surveyed regarding infrastructure, staffing, patient volumes and waiting times., Results: All 15 CACH network registered centers responded. The total number of patients followed in ACHD clinics was 21,879 (median per clinic=1132 (IQR: 585, 1816)). Of the total 80 adult and pediatric cardiologists affiliated to an ACHD clinic, only 27% had received formal ACHD training. Waiting times for non-urgent consultations were 4 ± 2 months, and 4 ± 3 months for percutaneous and surgical procedures. These were beyond Canadian recommended targets at 11 sites (73%) for non-urgent consultations, at 8 sites (53%) for percutaneous interventions and 13 sites (87%) for surgery., Conclusions: Of a minimum number of 96,000 ACHD patients in Canada, only 21,879 were being regularly followed in 2007. At most sites waiting times for ACHD services were beyond Canadian recommended targets. In spite of universal health care access, published guidelines for ACHD patient structure and process measures of health care quality are not being met., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society consensus conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present introductory section is a summary of the epidemiology and scope of adult CHD in Canada, the structure of the Canadian health care system and adult congenital cardiac health services in Canada. The recommendations for antibiotic prophylaxis and genetic evaluation in this population are included. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, outcomes, diagnostic workups, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up recommendations. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure, and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death.Medical aspects that need to be considered relate to the long-term and multisystemic effects of single-ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the understanding of the late outcomes, genetics, medical therapy and interventional approaches in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present executive summary is a brief overview of the new guidelines and includes the recommendations for interventions. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up requirements. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part III of the guidelines includes recommendations for the care of patients with complete transposition of the great arteries, congenitally corrected transposition of the great arteries, Fontan operations and single ventricles, Eisenmenger's syndrome, and cyanotic heart disease. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with congenital heart disease, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part I of the guidelines includes recommendations for the care of patients with atrial septal defects, ventricular septal defects, atrioventricular septal defects and patent ductus arteriosus. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk, and follow-up requirements. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Introduction: The effects of angiotensin converting enzyme inhibitor on systemic right ventricular size and function are unknown., Methods: Prospective, double blind, randomized, placebo-controlled clinical trial of 1-year therapy with Ramipril in adult patients with DTGA status postatrial baffle procedure. Primary endpoints were change in systemic right ventricular ejection fraction (RVEF) and right ventricular size assessed by magnetic resonance imaging (MRI)., Results: Seventeen patients were enrolled into the study. Mean age at study enrollment was 26.4+/-5.2 years. Mean baseline RVEF was 44+/-6.5%, mean RVEDV was 206.3+/-75.5 ml. Eight patients were randomized to the treatment group and 9 patients were randomized to the placebo group. RVEF did not improve in the Ramipril group from baseline to 1 year (43.8+/-7.1% vs. 40.9+/-13.3%, p=0.52) and remain unchanged in the placebo group (44.3+/-6.3 vs. 46.3+/-9.6%, p=0.42). RVEDV (184.5+/-56.4 ml vs. 179.6+/-66.4 ml, p=0.64) and RVESV (109.5+/-19.4 ml vs. 111.8+/-30.1, p=0.74) remained unchanged in the Ramipril group from baseline to 1 year as well as in the placebo group (228.1+/-89.2 ml vs. 204.5+/-50.4 ml, p=0.42 and 117.5+/-36.9 ml vs. 117.4+/-26.2 ml, p=0.99, respectively)., Conclusion: One-year treatment with Ramipril does not seem to affect right ventricular function or size in adult patients with systemic right ventricles after a Mustard or Senning procedure., Condensed Abstract: 17 Adult patients (mean age of 26.4 +/- 5.2 years) with systemic right ventricles were blindly randomized to 1-year treatment with Ramipril placebo. Systemic right ventricular function (RVEF) and size (RVEDV) were assessed by magnetic resonance imaging at baseline and 1 year. RVEF failed to improve in the Ramipril group (43.8+/-7.1% vs. 40.9+/-13.3%, p=0.52) and remain unchanged in the placebo group (44.3+/-6.3 vs. 46.3+/-9.6%, p=0.42). RVEDV remained unchanged in the study group (184.5+/-56.4 ml vs. 179.6+/-66.4 ml, p=0.64 as well as in the placebo group (228.1 +/- 89.2 ml vs. 204.5 +/- 50.4 ml, p = 0.42). One-year treatment with Ramipril does not improve right ventricular function or attenuate remodeling in adult patients with systemic right ventricles.

A case of sinus venosus atrial septal defect repair of the inferior vena caval type with an unintentional diversion of the inferior vena caval blood to the left atrium is reported. Long-standing, anatomical, right-to-left shunting with cyanosis and hypoxia are associated with systemic and cerebrovascular complications. Cardiac risks depend on the presence or absence of pulmonary hypertension, the associated hematological abnormalities and the degree of anatomical, right-to-left shunting. Cardiac magnetic resonance imaging clarified the etiology of the unexplained cyanosis and delineated the surgical anatomy.

Background: It has long been debated whether patients with atrial septal defect (ASD) Eisenmenger syndrome have idiopathic pulmonary arterial hypertension with an incidental ASD or severe pulmonary hypertension on the basis of their ASD shunt magnitude alone., Hypothesis: It was hypothesized that if ASD Eisenmenger patients had idiopathic pulmonary arterial hypertension with an incidental ASD, a mutation in the bone morphogenetic protein receptor-2 (BMPR2) would be found in some of these patients., Patients and Methods: All adult patients with ASD Eisenmenger syndrome were identified from the databases of two adult congenital cardiac units, and were matched to a control group with similar types of ASDs and no pulmonary hypertension. Gene coding for BMPR2 was examined for mutation using denaturing high-performance liquid chromatography of the entire coding sequence., Results: Eighteen adult patients with ASD Eisenmenger syndrome and 18 control patients were identified. ASD Eisenmenger patients had significantly larger ASDs than the control patients (3.7+/-1.2 cm versus 1.9+/-0.7 cm, P<0.01). A mutation in BMPR2 was not detected in either group., Conclusion: ASD Eisenmenger syndrome may occur without BMPR2 mutation. Whether shunt magnitude alone or in combination with yet another genetic mutation is responsible for the development of pulmonary hypertension in these patients remains to be determined.

The number of patients with congenital cardiac disease reaching adulthood is increasing steadily. Many adults with such disease face both medical and surgical difficulties. Most clinicians know very little about basic cardiac defects, their natural history, complications after surgery, and adequate management of these patients. We aim to provide an overview of the most frequently encountered cardiac lesions and long-term complications and to outline an up-to-date approach to their management. We present a series of hypothetical cases and discuss their management.

Background: Positive effects of physical training in adults with acquired heart disease have been reported. The role of exercise training in adults with congenital heart disease, however, is less well defined., Objectives: To assess the level of physical activity in adult patients with repaired tetralogy of Fallot (TOF), as well as the safety and effect of exercise training in these patients., Patients and Methods: A questionnaire of physical activity was filled upon entering the study by the participants. Eighteen adult patients with repaired TOF were then randomly assigned to participate in a three-month structured exercise program (exercise group, nine patients) or to continue their usual exercise routine (control group, nine patients). Each patient in the exercise group received an individualized exercise program to be performed three times a week, whereas the control group was told to continue living their life as usual. Cardiopulmonary testing was performed in all patients at baseline (before randomization) and at the end of the study., Results: No death or incidents occurred during the study period. All patients but one were physically inactive at the onset of the study. There was a significant increase in peak oxygen consumption in the exercise group by the end of the study (22.1 mL/kg/min [6 metabolic equivalents (METS)] versus 24.3 mL/kg/min [7 METS], P=0.049), whereas it remained unchanged in the control group (21.8 mL/kg/min [6 METS] versus 22.1 mL/kg/min [6 METS], P=0.825)., Conclusion: In clinically stable adult patients with repaired TOF, a moderate level of exercise training improves aerobic capacity. Regular exercise should be encouraged in these sedentary patients.

Most adults with previous repair of tetralogy of Fallot lead unrestricted lives and are asymptomatic. Residual RVOT problems such as significant PR and/or RVOT obstruction however are common and often lead to gradual RV dilation and dysfunction with consequent supraventricular or ventricular arrhythmias. Hemodynamic causes for the tachyarrhythmia should be sought and corrected, and therapy directed towards suppressing the arrhythmia (antiarrhythmics, cryoablation or AICD) should be carried out as well. Recent changes in the surgical approaches to the repair of tetralogy at the time initial repair may well translate into a reduced incidence of late complications.

Systemic right ventricular dysfunction has been closely linked to late mortality and sudden cardiac death in patients with Mustard procedure for dextrotransposition of the great arteries. Two young patients with dextrotransposition of the great arteries late after Mustard procedure who presented with acute transmural myocardial infarction and sudden cardiac death (one patient) without prior exertional angina or causative coronary abnormalities are reported. It is surmised that acute coronary emboli originating from a severely dilated, hypocontractile systemic ventricle were the cause of transmural myocardial infarction. This phenomenon may be an important and as yet unrecognized factor in late morbidity and mortality in such patients.

Objectives: To assess right ventricular function in adults and children with Ebstein's anomaly., Design: Prospective study., Setting: Tertiary referral centre., Subjects: Fifteen patients (8 adults and 7 children) with Ebstein's anomaly and 14 healthy controls., Interventions: Transthoracic echocardiography was performed in all. Right ventricular function was assessed from long axis M-mode recordings of the right atrio-ventricular free wall. Total systolic excursion as well as peak shortening and lenghtening rates of the right ventricle were measured., Results: Children and adult patients with Ebstein's anomaly differed in terms of age at diagnosis, the adult group having been diagnosed later 19.8+/-15.8 vs. 5.9+/-6.2 years, P<0.05. Measures of right ventricular long axis function in children with Ebstein's anomaly showed a significantly reduced systolic excursion 1.4+/-0.4 vs. 2.4+/-0.53 cm, P<0.05 and peak lenghtening rate; early 8.04+/-4.3 vs. 11.8+/-2.4 cm/s and late 6.14+/-3.6 vs. 10.6+/-4.3 cm/s, P=0.05 compared to controls. In contrast, measurements of right ventricular long axis function in adults with Ebstein's anomaly showed no significant difference when compared to the control group. Transtricuspid Doppler flow velocities were not different between patient's groups and corresponding controls., Conclusion: The right ventricle assessed by this simple, non-invasive technique reveals a significantly reduced systolic and diastolic function in children with Ebstein's malformation compared to controls but a 'normal' right ventricular function comparable to controls in adult patients. Significant right ventricular dysfunction in children with Ebstein's anomaly could account for their earlier presentation. Long term follow up of the right ventricular abnormalities is needed in such children to discover more about the natural history of the disease.