Background Identification of individuals at risk for major adverse cardiovascular events is essential for contemporary management of patients with repaired tetralogy of Fallot. We sought to identify clinical and cardiovascular magnetic resonance imaging (CMR) predictors of adverse clinical outcomes in repaired tetralogy of Fallot. Methods Children and adults prospectively enrolled in the CORRELATE (Comprehensive Outcomes Registry Late After Tetralogy of Fallot Repair) registry followed in North American, European, and Asian centers were studied. All patients had at least moderate pulmonary regurgitation and CMR at enrollment. Time‐to‐event analyses were performed from CMR completion to primary outcome, defined as mortality, resuscitated sudden death, sustained ventricular arrhythmia, or heart failure admission. Principal component analysis was used to create distinct CMR scores that collectively captured 80% of the variance among 10 CMR measures (systolic function, biventricular volumes/mass, and biatrial areas). Results In 720 patients (55% male, median age 30.3±14 years, 78% adult) with mean follow‐up 5.7±1.8 years, the primary outcome occurred in 38 patients (5.2%) at a rate of 0.9/100 patient‐years. A well‐calibrated risk scoring system was created for prediction of the primary outcome at 5 years based on 5 predictors: age, diabetes, right ventricular systolic pressure, and 2 CMR principal component scores (predominantly reflecting atrial areas in the first principal component score and ventricular volumes in the second principal component score) (c‐statistic for the composite risk score 0.79 [95% Cl, 0.71–0.88]). Conclusions Clinical and imaging characteristics can contribute to risk prediction in repaired tetralogy of Fallot. Further study will be required to evaluate the utility of a risk scoring system for identification of individuals who may benefit from enhanced surveillance, intensified medical therapy, and/or optimally timed intervention.

BACKGROUND:The timing for intervention in patients with significant chronic aortic regurgitation is based on adult guidelines and criteria which may not apply to children. There is limited data on the use of cardiac MRI parameters to guide surgical decision-making in paediatrics. We examined associations between MRI quantification of aortic regurgitation and left ventricular volumetric function and the need for surgical intervention. METHODS:Forty children and young adults with aortic regurgitation who had undergone cardiac MRI were divided into two groups based on aortic valve surgery (n = 20) or no surgery (n = 20). Ventricular volumetric functional parameters and aortic regurgitant volume and fraction were collected. Differences in MRI parameters between the groups were compared using unpaired t-tests. Receiver operating characteristic analysis identified MRI cut-off values with discriminatory ability towards primary end point of surgery (area under the curve > 0.7). RESULTS:Patients who underwent surgery had significantly larger ventricular volumes and aortic regurgitant fraction than those without surgery. Aortic regurgitant fraction and volume had the highest discriminatory power (0.93 and 0.92, respectively) between the two groups, followed by indexed left ventricular volumes (end-diastolic volume 0.85 and end-systolic volume 0.89). CONCLUSIONS:Current guidelines for surgical intervention in children with chronic aortic regurgitation are limited. Our findings suggest potential MRI-based threshold values that may aid in surgical decision-making and highlight the need future research for aortic valve surgery in children with chronic aortic regurgitation.

BACKGROUND:Aortopathy in Turner syndrome is associated with aortic dilation, and the risk of dissection is increased when the aortic size index is ≥ 2-2.5 cm/m2. We evaluated the aortic biophysical properties in paediatric Turner syndrome using cardiac MRI to determine their relationship to aortic size index. METHODS:Turner syndrome patients underwent cardiac MRI to evaluate ventricular function, aortic dimensions, and biophysical properties (aortic stiffness index, compliance, distensibility, pulse wave velocity, and aortic and left ventricular elastance). Spearman correlation examined correlations between these properties and aortic size index. Data was compared to 10 controls. RESULTS:Of 25 Turner syndrome patients, median age 14.7 years (interquartile range: 11.0-16.8), height z score -2.7 (interquartile range: -2.92 - -1.54), 24% had a bicuspid aortic valve. Turner syndrome had increased diastolic blood pressure (p < 0.001) and decreased left ventricular end-diastolic (p < 0.001) and end-systolic (p = 0.002) volumes compared to controls. Median aortic size index was 1.81 cm/m2 (interquartile range: 1.45-2.1) and 7 had an aortic size index > 2 cm/m2. Aortic and left ventricular elastance were greater in Turner syndrome compared to controls (both p < 0.001). Increased aortic size index correlated with increased aortic elastance (r = 0.5, p = 0.01) and left ventricular elastance (r = 0.59, p = 0.002) but not aortic compliance. Higher ascending aortic areas were associated with increased aortic compliance (r = 0.44, p = 0.03) and left ventricular elastance (r = 0.49, p = 0.01). CONCLUSION:Paediatric Turner syndrome with similar aortic size index to controls showed MRI evidence of abnormal aortic biophysical properties. These findings point to an underlying aortopathy and provide additional parameters that may aid in determining risk factors for aortic dissection.

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. (J Am Coll Cardiol 2023;81:1937-1950) (c) 2023 by the American College of Cardiology Foundation.

Multicenter studies in pediatric cardiovascular magnetic resonance (CMR) improve statistical power and generalizability. However, a structured process for identifying important research topics has not been developed. We aimed to (1) develop a list of high priority knowledge gaps, and (2) pilot the use of a wiki survey to collect a large group of responses. Knowledge gaps were defined as areas that have been either unexplored or under-explored in the research literature. High priority goals were: (1) feasible and answerable from a multicenter research study, and (2) had potential for high impact on the field of pediatric CMR. Seed ideas were contributed by a working group and imported into a pairwise wiki survey format which allows for new ideas to be uploaded and voted upon ( https://allourideas.org ). Knowledge gaps were classified into 2 categories: ‘Clinical CMR Practice’ (16 ideas) and ‘Disease Specific Research’ (22 ideas). Over a 2-month period, 3,658 votes were cast by 96 users, and 2 new ideas were introduced. The 3 highest scoring sub-topics were myocardial disorders (9 ideas), translating new technology & techniques into clinical practice (7 ideas), and normal reference values (5 ideas). The highest priority gaps reflected strengths of CMR (e.g., myocardial tissue characterization; implementation of technologic advances into clinical practice), and deficiencies in pediatrics (e.g., data on normal reference values). The wiki survey format was effective and easy to implement, and could be used for future surveys.

BACKGROUND:Children with a Fontan operation represent a unique form of congenital heart disease (CHD) that requires multiple cardiac surgeries and procedures with an uncertain long-term outcome. Given the rarity of the types of CHD that require this procedure, many children with a Fontan do not know any others like them.METHODS:With the cancelation of medically supervised heart camps due to the COVID-19 pandemic, we have organized several physician-led virtual day camps for children with a Fontan operation to connect with others in their province and across Canada. The aim of this study was to describe the implementation and evaluation of these camps via the use of an anonymous online survey immediately after the event and reminders on days 2 and 4 postevent.RESULTS:Fifty-one children have participated in at least 1 of our camps. Registration data showed that 70% of participants did not know anyone else with a Fontan. Postcamp evaluations showed that 86% to 94% learned something new about their heart and 95% to 100% felt more connected to other children like them.CONCLUSION:We have demonstrated the implementation of a virtual heart camp to expand the support network for children with a Fontan. These experiences may help to promote healthy psychosocial adjustments through inclusion and relatedness.

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.

Background:Fontan-associated liver disease (FALD) is characterized by hepatic congestion and progressive hepatic fibrosis in patients with the Fontan operation. This condition is generally clinically silent until late, necessitating techniques for early detection. Liver T1 mapping has been used to screen for FALD, but without consideration of regional variations in T1 values.Methods:Liver T1 measured with a liver-specific T1 mapping sequence (PROFIT1) in Fontan patients was compared with cohorts of patients with biventricular congenital heart disease (BiV-CHD) and controls with normal cardiac function and anatomy.Results:Liver T1 was significantly elevated in the Fontan cohort (n = 20) compared with patients with BiV-CHD (n = 12) and controls (n = 9) (781, 678, and 675 milliseconds, respectively; P < 0.001), with a consistent pattern of significantly elevated T1 values in the peripheral compared with central liver regions (ΔT1 = 54, 2, and 11 milliseconds; P < 0.001). PROFIT1 also yielded simultaneous T2∗ maps and fat fraction values that were similar in all groups. Fontan liver T1 values were also significantly elevated as compared with BiV-CHD and controls as measured with the cardiac (modified Look-Locker inversion) acquisitions (728, 583, and 583 milliseconds, respectively; P < 0.001) and values correlated with PROFIT1 liver T1 (R = 0.87, P < 0.001).Conclusions:Fontan patients have globally increased liver T1 values and consistent spatial variations, with higher values in the peripheral liver regions as compared with spatially uniform values in BiV-CHD and controls. The spatial patterns may provide insight into the progression of FALD. Liver T1 mapping studies should include uniform spatial coverage to avoid bias based on slice locations in this population.

BACKGROUND:Myocarditis presenting as acute chest pain with elevated troponins without significant cardiac compromise is rare in previously healthy children, often referred to as myopericarditis. Diagnosis is challenging, as conventional echocardiographic measures of systolic function can be normal. The aim of this study was to demonstrate the diagnostic utility of strain imaging in this scenario. METHODS:This was a multicenter, retrospective study including patients presenting with chest pain and elevated troponin from 10 institutions who underwent cardiac magnetic resonance imaging and transthoracic echocardiography within 30 days of each other (group 1). Findings were compared with those among 19 control subjects (group 2). Clinical data and conventional echocardiographic and cardiac magnetic resonance imaging data were collected. Echocardiography-derived strain was measured at the core laboratory. Group 1 was divided into subgroups as myocarditis positive (group 1a) or negative (group 1b) on cardiac magnetic resonance imaging on the basis of established criteria. RESULTS:Group 1 included 108 subjects (88 in group 1a, 20 in group 1b). Although all groups had normal mean fractional shortening and mean left ventricular ejection fraction, group 1 had significantly lower ejection fraction (56.8 ± 7.0%) compared with group 2 (62.3 ± 4.9%; P < .005) and fractional shortening (31.2 ± 4.9%) compared with group 2 (34.1 ± 3.5%; P < .05). Additionally, peak global longitudinal strain (GLS) was markedly abnormal in group 1 (-13.9 ± 3.4%) compared with group 2 (-19.8 ± 2.1%; P < .001). In subgroup analysis, GLS was markedly abnormal in group 1a (-13.2 ± 3.0%) compared with group 1b (-17.3 ± 2.6%; P < .001). Fifty-four subjects underwent follow-up echocardiography (46 in group 1a, eight in group 1b), with mean a follow-up time of 10 ± 11 months. At follow-up, whereas ejection fraction and fractional shortening returned to normal in all patients, abnormalities in strain persisted in group 1, with 22% still having abnormal GLS. Moreover, mean GLS was more abnormal in group 1a (-16.1 ± 2.6%) compared with group 1b (-17.4 ± 1.2%; P < .05). CONCLUSIONS:The present study demonstrates that echocardiographic GLS is significantly worse in subjects with myopericarditis presenting with chest pain and elevated troponins compared with control subjects even when conventional measures of systolic function are largely normal and that these abnormalities persisted over time.

Fontan associated liver disease (FALD) is an increasingly recognized complication of the single ventricle circulation characterized by hepatic venous congestion leading to hepatic fibrosis. Within the Fontan myocardium, fibrotic myocardial remodeling may occur and lead to ventricular dysfunction. Magnetic resonance imaging (MRI) T1 mapping can characterize both myocardial and liver properties. The aim of this study was to compare myocardial and liver T1 between single ventricle patients with and without a Fontan and biventricular controls. A retrospective study of 3 groups of patients: 16 single ventricle patients before Fontan (SVpre 2 newborns, 9 pre-Glenn, 5 pre-Fontan, 31

Education in paediatric cardiology has evolved along with clinical care. The availability and application of new technologies in education, in particular, have had a significant impact. Artificial intelligence; virtual, augmented, and mixed reality learning tools; and gamification of learning have all resulted in new opportunities for today's trainees compared with those of the past. A new training model is also being used. Though currently focused on residency education, competency-based medical education is also being applied to undergraduate education in some Canadian medical schools. Competency-based medical education offers a more transparent relationship between education and physicians' social contract with society. It provides greater accountability for programmes and learners to teach and learn the skills required to function as competent specialists. However, it has not come without challenges. Coincident with the application of this model for learners, there has been increased educational accountability for physicians in practice and for the institutions training them. Despite these changes, some things have remained the same. On the positive side, the importance of good clinical teachers to effective learning remains constant. Unfortunately, the mistreatment of learners within our education system also remains and is perhaps the most important challenge facing medical education in Canada today. Learning to be better teachers and learner advocates is an important goal for all of those involved in educating Canadian medical learners.

Background: The aims of this study were to investigate the dynamic changes in the vena contracta (VC) and proximal isovelocity surface area (PISA) through systole in patients with hypoplastic left heart syndrome and tricuspid regurgitation and to identify the stage of systole (early, mid, or late) in which VC and PISA radius are optimal. Methods: Twenty-eight patients with hypoplastic left heart syndrome were prospectively studied using continuous two-dimensional (2D) and three-dimensional (3D) echocardiography. Two-dimensional VC width, 3D VC area, and PISA radii (2D and 3D) were measured frame by frame throughout systole. The maximal 2D VC width, 3D VC area, and PISA radii in the first, middle, and last thirds of systole were compared, and correlations were explored with 3D tricuspid annular areas, right atrial volumes, and right ventricular volumes. Results: In all, 35 data sets that met inclusion criteria were analyzed. On frame-by-frame analysis, maximal 2D VC width and 3D VC area were found in the first third of systole in 17% and 20% of studies, in the second third in 34% and 31%, and in the final third in 49% and 49%. Similarly, the maximal 2D and 3D PISA radii were found in the first third of systole in 26% and 17% of studies, in the second third in 28% and 34%, and in the final third in 46% and 49%. Conclusions: In hypoplastic left heart syndrome, detailed temporal analysis of tricuspid regurgitation- associated VC and PISA by 2D and 3D echocardiography reveals no reliable pattern predicting when in systole these parameters peak. Frame-by-frame measurement is necessary for identification of maximal VC and PISA radius on 2D and 3D color Doppler echocardiography because the severity of tricuspid regurgitation could be underestimated because of temporal variability in VC and PISA. (J Am Soc Echocardiogr 2021;34:877-86.)

BACKGROUND The Fontan procedure is the final surgical stage for congenital heart disease (CHD) patients with single ventricles. Systemic venous return is routed directly to the lungs while the ventricle pumps arterial blood to the body. One long-term complication is known as Fontan-associated liver disease (FALD). Liver damage is due to a combination of chronic congestion from increased venous pressures and ischemic damage during the multiple surgeries a single ventricle patient undergoes. Fibrotic myocardial remodeling may further increase backup and contribute to liver pathology. Liver biopsy is the gold standard for diagnosis of FALD but is invasive and may not detect heterogenous disease. MRI T1 mapping (longitudinal relaxation time) of the liver provides quantitative assessment of fluid overload and diffuse fibrosis that may overcome these limitations. Our hypothesis is that liver T1 will be elevated in Fontan patients compared to corrected biventricular CHD and normal controls. METHODS AND RESULTS Cross-sectional cohort study of 33 patients within three cohorts: 11 Fontans, 12 biventricular CHD, and 10 controls with structurally normal hearts. Liver T1 was measured with a new water-specific T1 mapping method (PROFIT1 – Proton Density Fat Fraction Imaging with Water-Specific T1) from three axial slices at the widest dimension of the liver (free-breathing acquisition). Whole-liver average values were calculated, excluding blood vessels. Ventricular volumes and ejection fraction (EF) were calculated from the systemic ventricle. Myocardial native T1 mapping (MOLLI) at a mid-ventricular short axis were quantified in both the septum and free wall of the systemic or dominant ventricle. Cohort means were compared with Kruskal Wallis test and Dunn's posthoc test and association was determined with Spearman correlation coefficient. The Fontan cohort had higher liver T1 (795±59 ms) compared to control (668±62 ms; p=0.002) and CHD (672±54 ms; p=0.0007); control and CHD did not differ (Figure 1A). Fontans also had a significantly lower mean EF compared to CHD and controls (p=0.001); there were no significant differences in myocardial T1 between the 3 groups (Table 1). Liver T1 did, however, correlate with septal myocardial T1 within the whole dataset (Figure 1B; r=0.810; p=0.02); liver T1 did not correlate with EF. CONCLUSION T1 mapping with PROFIT1 appears feasible and discriminates Fontan patients from CHD and control. Furthermore, increased liver T1 also was associated with negative cardiac indicators suggesting an association between liver fibrosis and myocardial fibrosis. PROFIT1 is a fast, patient-friendly approach that may provide an early, non-invasive means to detect FALD. The Fontan procedure is the final surgical stage for congenital heart disease (CHD) patients with single ventricles. Systemic venous return is routed directly to the lungs while the ventricle pumps arterial blood to the body. One long-term complication is known as Fontan-associated liver disease (FALD). Liver damage is due to a combination of chronic congestion from increased venous pressures and ischemic damage during the multiple surgeries a single ventricle patient undergoes. Fibrotic myocardial remodeling may further increase backup and contribute to liver pathology. Liver biopsy is the gold standard for diagnosis of FALD but is invasive and may not detect heterogenous disease. MRI T1 mapping (longitudinal relaxation time) of the liver provides quantitative assessment of fluid overload and diffuse fibrosis that may overcome these limitations. Our hypothesis is that liver T1 will be elevated in Fontan patients compared to corrected biventricular CHD and normal controls. Cross-sectional cohort study of 33 patients within three cohorts: 11 Fontans, 12 biventricular CHD, and 10 controls with structurally normal hearts. Liver T1 was measured with a new water-specific T1 mapping method (PROFIT1 – Proton Density Fat Fraction Imaging with Water-Specific T1) from three axial slices at the widest dimension of the liver (free-breathing acquisition). Whole-liver average values were calculated, excluding blood vessels. Ventricular volumes and ejection fraction (EF) were calculated from the systemic ventricle. Myocardial native T1 mapping (MOLLI) at a mid-ventricular short axis were quantified in both the septum and free wall of the systemic or dominant ventricle. Cohort means were compared with Kruskal Wallis test and Dunn's posthoc test and association was determined with Spearman correlation coefficient. The Fontan cohort had higher liver T1 (795±59 ms) compared to control (668±62 ms; p=0.002) and CHD (672±54 ms; p=0.0007); control and CHD did not differ (Figure 1A). Fontans also had a significantly lower mean EF compared to CHD and controls (p=0.001); there were no significant differences in myocardial T1 between the 3 groups (Table 1). Liver T1 did, however, correlate with septal myocardial T1 within the whole dataset (Figure 1B; r=0.810; p=0.02); liver T1 did not correlate with EF. T1 mapping with PROFIT1 appears feasible and discriminates Fontan patients from CHD and control. Furthermore, increased liver T1 also was associated with negative cardiac indicators suggesting an association between liver fibrosis and myocardial fibrosis. PROFIT1 is a fast, patient-friendly approach that may provide an early, non-invasive means to detect FALD.

Introduction: Atypical myocarditis, presenting as isolated chest pain, elevated troponin and preserved cardiac output in childhood is rare. Beyond small volume single center case series, limited data are reported on CMR characteristics at initial presentation and follow up. Methods: This is a pediatric multi-center retrospective study. Patients with chest pain and elevated cardiac troponin who underwent an echo and CMR within 30 days of each other were included. Clinical, echo and CMR parameters were collected at presentation and follow up. CMR global longitudinal strain (GLS) was performed with feature tracking technology (TomTec). Cases were divided into CMR myocarditis positive (Myo+) and CMR myocarditis negative (Myo-) based on Lake Louise criteria. Findings were compared to 19 age matched controls. Results: A total of 108 cases were included (88 Myo+ and 20 Myo-). Majority were male (88%) with average age of 15.6 ± 2.1 years. Median troponin was 13 (0.18-260.1 ng/mL), median BNP was 270.8 (12-3949 pg/ml). Abnormal ECG findings were present in 79% at presentation. While CMR LVEF did not differ between cases (56 ± 9%) and controls (60 ± 4%, p 0.06), GLS was significantly worse in cases (-16.2 ± 2.7%) compared to controls (-19.2 ± 2.1%, p <0.001). Unlike LVEF, GLS was significantly worse in Myo+ (-15.7 ± 2.8%) compared to Myo- cases (-18.0 ± 1.3%, p < 0.001). Fifty-four subjects had follow up data with 35 repeat CMRs. Median follow up was 204 (30-944) days. Late gadolinium enhancement (LGE) persisted in 82% of the Myo+ cases. Of the cases with abnormal GLS at presentation, 77% had persistent LGE. At follow up, 14% of cases were on heart failure medications, 33% had abnormal GLS and 39% had abnormal LVEF (<55%). Neither treatment with steroids or IVIG at presentation nor LGE on initial or follow up CMR were associated with the need for heart failure medications or persistent symptoms at follow up. Conclusions: In atypical myocarditis, CMR GLS can help differentiate between Myo+ and Myo- cases. Abnormal CMR GLS at presentation has higher association with persistence of LGE at follow up, irrespective of type of treatment. Future studies are needed to identify other characteristics that predict persistently abnormal findings important to clinical management and prognosis.