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.

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.

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.

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.

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

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

The Adult With Repaired Coarctation: Need for Lifelong Surveillance.

Because surgical repair for coarctation of the aorta has been performed since 1945, growing numbers of patients with repaired coarctation are reaching adulthood. Primary transcatheter intervention for coarctation emerged as an alternative to surgery after 1983, and it provides comparable relief of the aortic gradient with few complications at a cost of an increased need for reintervention and a higher risk of aneurysm after repair. Although short-term outcomes are good after coarctation repair, alterations of vascular form and function persist. Mortality is increased after coarctation repair compared with that in the general population, which is related to several predictable complications. Hypertension mediates much of the late morbidity with increased rates of stroke, coronary artery disease, and heart failure after coarctation repair. Prevalence of hypertension in patients with coarctation increases over time, with a majority of patients being affected by middle age. Other late complications include recoarctation, which can usually be addressed with percutaneous balloon dilation and stenting with covered stents. Aneurysms at the coarctation repair site and the ascending aorta require surveillance with imaging and timely treatment. Intracranial aneurysms occur 5 times more commonly in patients with coarctation than in the general population. Finally, bicuspid aortic valve disease, which is present in at least half of these patients, requires surveillance and ultimately becomes the most common reason for reoperation. Awareness, identification, and appropriate treatment of long-term complications after coarctation repair are paramount to reducing long-term morbidity and mortality.

Psychosocial Maturity, Autonomy, and Transition Readiness among Young Adults with Congenital Heart Disease or a Heart Transplant.

OBJECTIVE: The population of young adults with congenital heart disease (CHD) or a heart transplant (HTx) is growing rapidly. These survivors require lifelong cardiology care and must assume self-management responsibilities with respect to their health. Accordingly, we sought to assess psychosocial maturity and validity of the Transition Readiness Assessment Questionnaire (TRAQ) in this population. DESIGN: The study was designed as a cross-sectional observational study. SETTING: The study was set at tertiary-care pediatric and adult cardiology clinics in Edmonton and Toronto, Canada. PATIENTS: The patients were 18- to 25-year-olds with moderate or complex CHD or a HTx in childhood. OUTCOME MEASURES: Participants completed validated instruments including the TRAQ, Erickson's Psychosocial Stage Inventory (EPSI), and Kenny's Parental Attachment Questionnaire (PAQ). RESULTS: We enrolled 188 participants (41% women), 109 (58%) having moderate CHD, 64 (34%) complex CHD, and 15 (8%) with a HTx. Mean age for those followed in a pediatric clinic was 21.3 ± 2.3 years, compared with 21.8 ± 2.3 years for those attending an adult clinic (P = 0.20). All questionnaire scores were similar among participants with moderate CHD vs. complex CHD vs. an HTx, and among participants followed in a pediatric clinic vs. an adult clinic. EPSI and PAQ scores were similar to those of healthy populations, though perception of maternal overprotection was common. TRAQ scores increased with age. A response of "not needed for my care" to 5 or more of the 29 TRAQ items was provided by 110 (75%) participants. CONCLUSIONS: Age, but not disease severity should be a factor when considering the transition needs of young adults with heart disease. The TRAQ has important limitations in the adult CHD/HTx population and a cardiac-specific measure of transition readiness is needed.

Rationale and design of the Canadian Outcomes Registry Late After Tetralogy of Fallot Repair: the CORRELATE study.

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.

Healthcare transition for youth with heart disease: a clinical trial.

OBJECTIVES: Adolescents with heart disease have complex health needs and require lifelong cardiology follow-up. Interventions to facilitate paediatric to adult healthcare transition are recommended, although outcomes are unknown. We sought to determine the impact of a transition intervention on improving knowledge and self-management skills among this population. METHODS: We conducted a clinical trial of 15-17 year olds with moderate or complex congenital heart disease (CHD) or cardiomyopathy. Participants were systematically allocated to either usual care (controls) or a 1 h nurse-led one-on-one teaching session about their heart. Allocation was determined by week of attendance in the cardiology clinic. The primary outcome was change in Transition Readiness Assessment Questionnaire (TRAQ) score at 6 months, possible scores ranging from 1 (low) to 5 (optimal). Cardiac knowledge (MyHeart score, range 0-100) was a secondary outcome. Analysis was intention to treat. RESULTS: Of 58 participants (48% female), 52 had CHD and 6 had cardiomyopathy. 27 were allocated to the intervention group; 3 declined the intervention and received usual care. When comparing the intervention group with the usual care group at 6 months postintervention, the mean self-management TRAQ score was 3.59 (±0.83) vs. 3.16 (±1.05), respectively (p=0.048, adjusted for baseline score); the mean self-advocacy TRAQ score was 4.38 (±0.56) vs. 4.01 (±0.95) (p=0.18) and the mean MyHeart score was 75% (±15) vs. 61% (±25) (p=0.019). CONCLUSIONS: A 1 h nurse-led transition intervention resulted in a significant improvement in self-management and cardiac knowledge scores. An educational intervention should be routine for youth with congenital or acquired heart disease. TRIAL REGISTRATION NUMBER: NCT01286480.

Management of adults with congenital heart disease using videoconferencing across Western Canada: a 3-year experience.

BACKGROUND: Management of adults with congenital heart disease (ACHD) requires quaternary centres with cardiologists and cardiac surgeons who have expertise in the diagnosis and management of this patient population. We report on the feasibility of the management of ACHD patients using videoconferencing and streaming through 1 regional referral centre covering 4 western Canadian provinces, roughly 30% of Canada's land and population. METHODS: Videoconferencing sessions from January 2008 to December 2010 were systematically reviewed. Case presentations were classified as successful or unsuccessful. All patients were followed to assess whether the recommendations were acted upon. The hosting institution used the Alberta Health Services internet protocol network, while other connections used integrated service digital network. The videoconference equipment at the different sites includes Polycom HDX 9000 (Polycom, San Jose, CA), Tandberg Edge 95 (Tandberg, San Jose, CA), and Tandberg 990 (Tandberg). RESULTS: From January 2008 to December 2010 there were 26 sessions, 213 case presentations, and 177 patients discussed with an average 8.2 case presentations per session. Thirty-two case presentations were deferred, 10 of which were because of transmission errors and the remainder were because of unavailability of staff or images. Of the 177 recommendations, 124 procedures (91 surgical, 29 percutaneous, and 4 electrophysiological) were booked directly at the regional referral centre. Only 6 recommendations were not carried out (4 because of premature deaths, and 2 because of patient logistic issues). CONCLUSIONS: The results of this study illustrate that telehealth is a feasible medium for arriving at consensus recommendation in the management of ACHD patients living in a geographically diverse area.

A prominent Eustachian valve dividing the right atrium imaged by three-dimensional transesophageal echocardiography.

A 63-year-old man underwent transesophageal echocardiography (TEE) to rule out left atrial thrombi prior to cardioversion. Initial two-dimensional TEE with color flow Doppler imaging was suggestive of an atrial septal defect. However, three-dimensional TEE imaging revealed that the unusually large elongated Eustachian valve extended toward the superior vena cava and mimicked the interatrial septum, while the true septum was located more posteriorly than the Eustachian valve. Three-dimensional TEE imaging was crucial to understanding the anatomical relationship between the Eustachian valve and the interatrial septum and hence proved helpful in characterizing this unusual anatomical variant.

Cardiac outcomes in adults with supravalvar aortic stenosis.

AIMS: Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis. METHODS AND RESULTS: This is a multicentre retrospective study of cardiac outcomes in adults (≥18 years) with supravalvar aortic stenosis. We examined: (i) adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and (ii) the need for cardiac surgery in adulthood. One hundred and thirteen adults (median age at first visit 19 years; 55% with Williams-Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams-Beuren syndrome had more severe supravalvar aortic stenosis and more often associated left ventricular outflow tract obstructions (P < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams-Beuren syndrome. Eighty-five per cent of adults (96/113) had serial follow-up information (median follow-up 6.0 years). Of these patients, 13% (12/96) had an adverse cardiac event and 13% (12/96) had cardiac operations (7 valve repair or replacements, 4 supravalvar aortic stenosis repairs, 1 other). Cardiac surgery was more common in adults without Williams-Beuren syndrome (P = 0.007). Progression of supravalvar aortic stenosis during adulthood was rare. CONCLUSION: Adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperations, while progression of supravalvar aortic stenosis in adulthood is rare. Valve surgery is the most common indication for cardiac surgery in adulthood.

Atrial septal defect closure in the elderly is associated with excellent quality of life, functional improvement, and ventricular remodelling.

BACKGROUND: While atrial septal defect (ASD) closure is known to improve morbidity and mortality in children and adults, data are only beginning to emerge about its role in elderly cohorts. The goals of this study were to compare outcomes after device or surgical closure of ASDs in the elderly, and to quantitatively assess quality of life. METHODS: Patients>60 years old who underwent ASD repair were studied. Functional status, arrhythmia burden, biventricular size and function were compared before and after ASD closure. Quality of life after ASD closure was assessed with the RAND SF-36 instrument. RESULTS: Sixty-seven patients, mean age of 68 years (range 60-86 years), were followed for 3.3 years. Nineteen percent underwent surgical closure and 81% underwent device closure. Major complication rates were 23% and 7% respectively with no procedure- related deaths. After surgical and device closure, quality of life was comparable to age-matched healthy controls, right ventricular end-diastolic dimension decreased by 10 mm (P<0.001), left ventricular end-diastolic dimension increased by 4 mm (P=0.001), biventricular function improved (right ventricular, P<0.001; left ventricular, P=0.007) and New York Heart Association class improved (P<0.001). Prevalence of atrial arrhythmias however, was unchanged. Beneficial effects were similar for patients treated surgically or with device closure. CONCLUSIONS: Given the favorable structural, functional, and quality of life outcomes after ASD closure in the elderly, advanced age alone should not be a contraindication to recommending surgical or device closure of an ASD.