Background Adults with a systemic right ventricle (sRV) due to congenitally corrected transposition of the great arteries (ccTGA) or atrial switch repair for d-transposition of the great arteries (d-TGA) experience substantial physiologic and psychosocial morbidity. Relationships among exercise capacity, sRV function, and mental health remain incompletely characterized. Objectives To characterize relationships among anatomic subtype, exercise capacity, sRV function, and mental health in adults with sRV physiology. Methods We performed a retrospective cohort study of adults with ccTGA or d-TGA (Mustard/Senning) followed at a tertiary Adult Congenital Heart Disease program from 2000 to 2025. Clinical, imaging, cardiopulmonary exercise testing, and patient-reported data were obtained from electronic health records. Mental health diagnoses were identified from clinical documentation. Functional status was assessed using NYHA class and the Kansas City Cardiomyopathy Questionnaire (KCCQ-12). Results Among 137 adults (ccTGA, n = 51; d-TGA, n = 86), percent-predicted peak VO2 was lower in d-TGA than ccTGA (60% vs 74%, p < 0.001), as was sRV systolic function (41 +/- 11% vs 47 +/- 10%, p < 0.01). Anxiety or depression was more common in d-TGA (46% vs 25%, p < 0.05). Across the cohort, anxiety or depression was associated with lower exercise capacity, worse NYHA functional class, and lower KCCQ scores. Conclusions Adults with d-TGA following atrial switch have lower exercise capacity, reduced sRV systolic function, and greater mental health burden than adults with ccTGA. These findings support integrated assessment of physiologic performance, functional status, and mental health in adults with sRV physiology. ### Competing Interest Statement The authors have declared no competing interest. ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: The Stanford University institutional review board approved this study. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes All data produced in the present work are contained in the manuscript Adult Congenital Heart Association, https://ror.org/031689s30

"Cases of SCMR" is a case series on the Society for Cardiovascular Magnetic Resonance (SCMR) website (https://www.scmr.org) for the purpose of education. The cases reflect the clinical presentation, and the use of cardiovascular magnetic resonance in the diagnosis and management of cardiovascular disease. The 2024 digital collection of cases are presented in this article.

Due to the presence of complex flow states and significant jet eccentricity in patients with congenital heart disease (CHD), accurate quantification of aortic regurgitation (AR) using standard echocardiographic or conventional cardiac magnetic resonance (CMR) imaging measures remains challenging. Four-dimensional flow (4DF) CMR permits transvalvular flow quantification under non-laminar flow states, although has not been well validated for AR quantification in CHD. In 186 patients with moderate or complex CHD, we evaluated the agreement between different methods of AR quantification by 4DF CMR when compared to volumetry. Regurgitant flow volumes were measured (1) conventionally on time-resolved, velocity-encoded 4DF sequences at the aortic annulus, sinotubular junction (STJ), and ascending aorta (AAo), and via (2) direct regurgitant jet quantification 5mm proximal to the vena contracta. Moderate overall agreement in AR quantification was observed between study methods (ρ=0.58-0.73). Compared with conventional flow quantification at the annulus, STJ, and AAo, direct regurgitant jet measurements showed improved correlation with volumetry (ρ=0.76), especially in patients with significant aortic dilation (r=0.95-0.97). In this latter group, regurgitant flow quantification at all other aortic levels resulted in AR severity classifications that were nearly a full grade lower (mean aortic regurgitant fraction difference: 7-12% ± 10-12%; p<0.001). 4DF CMR permits AR quantification in complex CHD with comparable accuracy to volumetry. Under non-laminar or complex flow states, as observed with significant aortic dilation, direct regurgitant jet measurements may be preferable to regurgitant flow quantification at all other aortic levels.

BACKGROUND:Due to the presence of complex flow states and significant jet eccentricity in patients with congenital heart disease (CHD), accurate quantification of aortic regurgitation (AR) using standard echocardiographic or conventional cardiovascular magnetic resonance (CMR) imaging measures remains challenging. Four-dimensional flow (4DF) CMR permits transvalvular flow quantification under non-laminar flow states, although it has not been well validated for AR quantification in CHD. METHODS:In 186 patients with moderate or complex CHD, we evaluated the agreement between different methods of AR quantification by 4DF CMR when compared to volumetry. Regurgitant flow volumes were measured (1) conventionally on time-resolved, velocity-encoded 4DF sequences at the aortic annulus, sinotubular junction (STJ), and ascending aorta (AAo), and via (2) direct regurgitant jet quantification 5 mm proximal to the vena contracta. RESULTS:Moderate overall agreement in AR quantification was observed between study methods (ρ=0.58-0.73). Compared with conventional flow quantification at the annulus, STJ, and AAo, direct regurgitant jet measurements showed improved correlation with volumetry (ρ=0.76), especially in patients with significant aortic dilation (r=0.95-0.97). In this latter group, regurgitant flow quantification at all other aortic levels resulted in AR severity classifications that were nearly a full grade lower (mean aortic regurgitant fraction difference: 7-12% ± 10-12%; p<0.001). CONCLUSION:4DF CMR permits AR quantification in complex CHD with comparable accuracy to volumetry. Under non-laminar or complex flow states, as observed with significant aortic dilation, direct regurgitant jet measurements may be preferable to regurgitant flow quantification at all other aortic levels.

BACKGROUND:Adults with Fontan failure (FF) have variable presentations and are often diagnosed late. Reliable predictors of FF are unknown. Diastolic dysfunction may be associated with adverse outcomes late after Fontan palliation. METHODS:Fontan patients were compared to healthy controls. FF was defined as death, transplant, diagnosis of protein-losing enteropathy, peak VO2 <50% predicted, or new diuretic requirement. All phases in the short-axis plane were contoured to calculate filling and ejection curves. The following variables were measured by cardiovascular magnetic resonance (CMR): peak filling rate (PFR), peak ejection rate (PER), PFR and PER indexed to end diastolic volume (EDV), time to PFR (tPFR), and time to PER (tPER). RESULTS:Compared to healthy controls (N=96), the Fontan group (N=98) had worse diastolic function as evidenced by decreased PFR and PFR/EDV and increased tPFR. Patients with FF (N=39) had similar ventricular systolic function and volumetrics to the Fontan subjects without failure (NF; N=59). PFR/EDV was significantly reduced, and indexed common ventricular mass was significantly higher among FF patients with the most severe adverse outcomes of death or heart transplantation. The prevalence of late gadolinium enhancement was higher in the FF cohort than the NF cohort. CONCLUSION:CMR can identify diastolic dysfunction in the Fontan population. Patients with Fontan circulation who died or had a combined outcome of death or transplant had worse diastolic function by CMR compared to Fontan patients without death or transplant.

Background: Most studies of left ventricular wall stress in patients with aortic stenosis (AS) focus on the end systolic (LVESWS) component with suggestion of an inverse association with adverse outcomes. LV end diastolic wall stress (LVEDWS) is less characterized and LVEDWS to LVESWS ratio, which may provide a combined measure of diastolic and systolic dysfunction and remodeling, has not been previously reported in patients with severe AS undergoing transcatheter aortic valve replacement (TAVR). Hypothesis: Higher LVEDWS to LVESWS ratio assessed by echocardiography prior to TAVR associates with greater risk of post-TAVR death. Methods: From a multicenter registry of patients with severe AS undergoing TAVR, we examined 637 participants (median age 84 years, 43% female, 95% white) who underwent pre-TAVR echocardiogram from which LVEDWS and LVESWS could be calculated from chamber dimensions, wall thicknesses, aortic valve gradient, blood pressure, and E/e’. Multivariable-adjusted Cox regression was used to examine associations between LVEDWS, LVESWS, their ratio, and the risk of post-TAVR mortality. Results: Median LVEDWS, LVESWS, and LVEDWS/LVESWS ratio were 22.1 (IQR: 16.2, 29.7) kdynes/cm2, 64.7 (IQR: 50.0, 90.7) kdynes/cm2, and 0.32 (IQR: 0.24, 0.43), respectively. Correlates of higher LVEDWS/LVESWS included: female gender, non-white race, hypertension medication use, higher natriuretic peptide and LV ejection fraction, and lower systolic and diastolic BP and hemoglobin. Over a median follow-up of 3 years post-TAVR, 262 (41%) individuals died. Higher LVEDWS tended towards greater risk of death (aHR: 1.013; 95% CI 0.999 – 1.027, p=0.071), while higher LVESWS associated with lower mortality risk (aHR: 0.993; 95% CI 0.988-0.998, p=0.008). Higher LVEDWS/LVESWS ratio associated with greater risk of death (aHR: 1.130, 95% CI 1.043-1.224, p=0.003, per 0.1 unit) (Figure), independent of age, comorbidities, LVEF, mass index, natriuretic peptide, and troponin. Conclusion: Among patients with severe aortic stenosis, higher LVEDWS to LVESWS ratio prior to TAVR associated with greater risk of post-TAVR mortality.

BACKGROUND:Multisystem inflammatory syndrome in children (MIS-C) is a rare but serious complication of severe acute respiratory syndrome coronavirus 2 infection. Features of MIS-C overlap with those of Kawasaki disease (KD). OBJECTIVE:The study objective was to develop a prediction model to assist with this diagnostic dilemma. METHODS:Data from a retrospective cohort of children hospitalized with KD before the coronavirus disease 2019 pandemic were compared to a prospective cohort of children hospitalized with MIS-C. A bootstrapped backwards selection process was used to develop a logistic regression model predicting the probability of MIS-C diagnosis. A nomogram was created for application to individual patients. RESULTS:Compared to children with incomplete and complete KD (N = 602), children with MIS-C (N = 105) were older and had longer hospitalizations; more frequent intensive care unit admissions and vasopressor use; lower white blood cell count, lymphocyte count, erythrocyte sedimentation rate, platelet count, sodium, and alanine aminotransferase; and higher hemoglobin and C-reactive protein (CRP) at admission. Left ventricular dysfunction was more frequent in patients with MIS-C, whereas coronary abnormalities were more common in those with KD. The final prediction model included age, sodium, platelet count, alanine aminotransferase, reduction in left ventricular ejection fraction, and CRP. The model exhibited good discrimination with AUC 0.96 (95% confidence interval: [0.94-0.98]) and was well calibrated (optimism-corrected intercept of -0.020 and slope of 0.99). CONCLUSIONS:A diagnostic prediction model utilizing admission information provides excellent discrimination between MIS-C and KD. This model may be useful for diagnosis of MIS-C but requires external validation.

Cardiovascular disease is the leading cause of morbidity and mortality in patients with autoimmune rheumatic diseases. Much of this may be attributed to systemic inflammation resulting in coronary atherosclerosis and myocarditis. Cardiac magnetic resonance imaging is the gold standard for the evaluation of cardiac structure and function, including tissue characterization, which allows for detection of myocardial edema, inflammation, and fibrosis. Advances in parametric mapping and coronary flow reserve measurement techniques have the potential to change the diagnosis, risk stratification, and management of patients with autoimmune rheumatic diseases. We provide an overview of the current evidence and suggest potential future roles for the use of comprehensive cardiac magnetic resonance in patients with autoimmune rheumatic diseases in the field of cardio-rheumatology.

Background:Anomalous aortic origin of a coronary artery from the opposite sinus is a rare congenital abnormality that may be encountered during primary percutaneous coronary intervention (PCI) for ST-elevation myocardial infarction (STEMI).Case summary:A 65-year-old man presented with chest pain and signs of heart failure. Electrocardiogram demonstrated atrial fibrillation with ST elevation in the high lateral leads, and he was taken emergently to the cardiac catheterization laboratory for primary PCI. Coronary angiography identified the culprit to be an occluded anomalous left main coronary artery (LMCA) arising from the right coronary cusp, and primary PCI was successfully performed in the LMCA and the left anterior descending artery (LAD). Computed tomography angiography confirmed a benign retroaortic course of the anomalous LMCA with no additional high-risk features, as well as a new left atrial appendage thrombus. He subsequently developed deep venous thrombosis, acute pulmonary embolism, and acute kidney injury secondary to renal artery embolism with associated infarction. Workup for patent foramen ovale and thrombophilia were negative, and he was discharged in a stable condition. At 2-month follow-up, he was asymptomatic with no evidence of myocardial ischaemia on stress cardiac magnetic resonance imaging.Discussion:We present the first reported case of an occluded anomalous LMCA arising from the right coronary sinus in a patient presenting with STEMI. Rapid recognition of this congenital anomaly and selection of an appropriate guide catheter were keys to achieving timely reperfusion and a good outcome in this case.

Post-acute sequelae of Coronavirus (PASC), or Long COVID, has emerged as a critical health concern. The clinical manifestations of PASC have been described, but studies have not quantified the cardiopulmonary effects. The goal of this study was to quantify PASC cardiopulmonary changes among endurance athletes. Endurance athletes were recruited via social media; 45 met inclusion criteria, 32 had PASC and 13 were asymptomatic at 3 months (control). Comprehensive interviews were conducted to assess: cardiopulmonary symptoms at 3 months; quantitative and qualitative changes in cardiovascular endurance; exercise hours per week at baseline and 3 months; and Modified Oslo, Dyspnea, and EQ-5D-5L scales. All collected data was based on self-reported symptoms. Wilcoxon rank sum compared PASC with control to distinguish the effects of PASC vs effects of COVID infection/lockdown. PASC subjects were more likely to be female (Table). The most common 3-month symptoms in PASC were fatigue and shortness of breath. Based on self-reported data, subjects endorsed a median decrease of 27% in cardiopulmonary endurance levels compared with 0% in controls (p = 0.0019). PASC subjects exercised less hours and had worse self-reported health as compared with controls. PASC subjects also had significantly worse Modified Oslo, Dyspnea, and EQ-5D-5L scores. Of the 32 PASC patients, 10 (31%) reported a complete inability to engage in any cardiovascular endurance exercise at 3 months. PASC leads to a significant, quantifiable decrease in cardiopulmonary health and endurance.

OBJECTIVE:To evaluate the association between vitamin D status and CV disease after COVID-19 in college athletes. DESIGN:Retrospective cohort study. SETTING:National College Athletic Association Division-I college athletes from a single academic institution. PATIENTS:A total of 157 athletes (60 female; median age: 20 years) from 9 sports with a positive SARS-CoV-2 test, cardiac magnetic resonance imaging (CMR), and vitamin D level. INDEPENDENT VARIABLES:Serum 25-hydroxyvitamin D level (primary); age, sex (regression models). MAIN OUTCOMES MEASURES:Differences in age, sex, race, ethnicity, myocarditis, pericarditis, and CMR metrics by vitamin D status were analyzed. Regression models were used to assess the relationship between vitamin D status and CMR metrics accounting for age and sex. RESULTS:Low vitamin D (LVD) was found in 33 (21.0%) of athletes, particularly Black males ( P < 0.001). Athletes with LVD had higher biventricular and lower mid-ventricular extracellular volumes, but these differences were not significant when corrected for age and sex. Athletes with LVD had higher left ventricle (LV) mass ( P < 0.001) and LV mass index ( P = 0.001) independent of age and sex. Differences in global circumferential strain were noted but are likely clinically insignificant. Vitamin D status did not associate with myocarditis and pericarditis ( P = 0.544). CONCLUSIONS:LVD is common in athletes, particularly in Black males. Although athletes with LVD had higher LV mass, cardiac function and tissue characterization did not differ by vitamin D status. Future studies are needed to determine if the differences in LV mass and LV mass index by vitamin D status are clinically significant. This study suggests that vitamin D status does not impact the development of myocarditis or pericarditis after COVID-19 infection.

Hepatitis C Virus positive (HCV+) organ donors offer a viable strategy for expansion of the heart donor pool without an increased risk of recipient mortality. Cardiac magnetic resonance imaging (CMR) is an effective tool for graft surveillance in heart transplant (HT) recipients. However, there are no data comparing CMR findings in HT recipients based on donor HCV status. The aim of this propensity score matched case-control study was to evaluate baseline CMR characteristics of HCV+ HT recipients and HCV- HT recipients, as well as compare cardiac allograft structure, function, performance, and tissue characterization between groups. CMR normative values did not differ between groups in matched analysis. There were no significant differences in CMR-derived biventricular function, strain, LGE, or myocardial tissue characteristics by parametric mapping. This study suggests CMR can be a valuable tool for surveillance in HCV+ HT patients, and abnormalities on imaging should not be attributed to HCV infection.