Common atrial reservoir strain during the interstage period is a predictor of poor outcomes prior to Fontan completion in hypoplastic left heart syndrome.

BACKGROUND: The atrium augments ventricular function, but the significance of atrial function in hypoplastic left heart syndrome (HLHS) has not been well evaluated. OBJECTIVE: We investigated the association of atrial reservoir strain (common atrial strain [CAS]) to death or need for transplantation in patients with HLHS. METHODS: In this retrospective single-center study, echocardiograms from three timepoints (pre-stage 1 palliation [S1P], 4-8 weeks post-S1P, and pre-Glenn) were analyzed in infants with classic HLHS. Patients were separated based on transplant-free survival to Fontan (survivors) versus death or heart transplant prior to Fontan (composite outcome). Echocardiographic parameters evaluated included CAS, right ventricle (RV) global longitudinal strain (RVGLS), RV fractional area change (FAC), and tricuspid annular plane systolic excursion (TAPSE). An equal variance t-test, regression, and receiver operating characteristic (ROC) analyses were performed. RESULTS: A total of 45 HLHS patients (25 survivors, 20 patients meeting endpoint) were included in this study. There were no significant differences in any of the functional parameters during the pre-stage 1 or post-stage 1 timepoints. Pre-Glenn CAS and RVGLS were significantly worse in those meeting composite endpoint compared to survivors. CAS was significantly correlated to RVGLS during the pre-S1P and pre-Glenn timepoints. A pre-Glenn CAS < 19.5 had an area under the curve of  .78 and a 75% sensitivity and 83% specificity for death or need for transplantation. CONCLUSION: Pre-Glenn CAS is significantly lower in patients with mortality or need for the transplantation prior to Fontan completion and may carry prognostic significance in patients with HLHS.

Regional variability of cardiovascular magnetic resonance access and utilization in the United States.

BACKGROUND: Clinical guidelines and scientific data increasingly support the appropriate use of cardiovascular magnetic resonance (CMR) . The extent of CMR adoption across the United States (US) remains unclear. This observational analysis aims to capture CMR practice patterns in the US. METHODS: Commissioned reports from the Society for Cardiovascular Magnetic Resonance (SCMR), pre-existing survey data from CMR centers, and socioeconomic and coronary heart disease data from the Centers for Disease Control and Prevention were used. The location of imaging centers performing CMR was based on 2018 Medicare claims. Secondary analysis was performed on center-specific survey data from 2017-2019, which were collected by members of the SCMR US Advocacy Subcommittee for quality improvement purposes. The correlation between the number of imaging centers billing for CMR services per million persons, socioeconomic determinants, and coronary heart disease epidemiology was determined. RESULTS: A total of 591 imaging centers billed the Center for Medicare & Medicaid Services for CMR services in 2018 and 112 (of 155) unique CMR centers responded to the survey. In 2018, CMR services were available in almost all 50 states. Minnesota was the state with the highest number of CMR centers per million Medicare beneficiaries (52.6 centers per million), and Maine had the lowest (4.4 per million). The total density of CMR centers was 16 per million for US Medicare beneficiaries. Sixty-eight percent (83 of 112) of survey responders were cardiologists, and 28% (31/112) were radiologists. In 72% (71/112) of centers, academic health care systems performed 81%-100% of CMR exams. The number of high-volume centers (>500 scans per year) increased by seven between 2017 and 2019. In 2019, 53% (59/112) of centers were considered high-volume centers and had an average of 19 years of experience. Centers performing <50 scans had on average 3.5 years of experience. Approximate patient wait time for a CMR exam was 2 weeks to 1 month. CONCLUSION: Despite increasing volume and availability in almost all 50 states, CMR access remains geographically variable. Advocacy efforts to improve access and innovations that reduce imaging time and exam complexity have the potential to increase the adoption of CMR technology.

Rate of Change in Cardiac Magnetic Resonance Imaging Measures Is Associated With Death in Duchenne Muscular Dystrophy.

BACKGROUND: Cardiovascular disease is the leading cause of death among patients with Duchenne muscular dystrophy (DMD). Identifying patients at risk of early death could allow for increased monitoring and more intensive therapy. Measures that associate with death could serve as surrogate outcomes in clinical trials. METHODS AND RESULTS: Duchenne muscular dystrophy subjects prospectively enrolled in observational studies were included. Models using generalized least squares were used to assess the difference of cardiac magnetic resonance measurements between deceased and alive subjects. A total of 63 participants underwent multiple cardiac magnetic resonance imaging and were included in the analyses. Twelve subjects (19.1%) died over a median follow-up of 5 years (interquartile range, 3.1-7.0). Rate of decline in left ventricular ejection fraction was faster in deceased than alive subjects (P<0.0001). Rate of increase in indexed left ventricular end-diastolic (P=0.0132) and systolic (P<0.0001) volumes were higher in deceased subjects. Faster worsening in midcircumferential strain was seen in deceased subjects (P=0.049) while no difference in global circumferential strain was seen. The rate of increase in late gadolinium enhancement, base T1, and mid T1 did not differ between groups. CONCLUSIONS: Duchenne muscular dystrophy death is associated with the rate of change in left ventricular ejection fraction, midcircumferential strain, and ventricular volumes. Aggressive medical therapy to decrease the rate of progression may improve the mortality rate in this population. A decrease in the rate of progression may serve as a valid surrogate outcome for therapeutic trials.

Prognostic Value of RV Function Analysis During the Interstage Period in Patients with Hypoplastic Left Heart Syndrome.

Cardiac dysfunction is associated with mortality in children with hypoplastic left heart syndrome (HLHS). We evaluated the ability of qualitative and quantitative RV functional parameters to predict outcomes in HLHS patients. In this retrospective, single-center study, echocardiograms from 3 timepoints (pre-stage 1 palliation, 4-8 weeks post-stage 1 palliation, and pre-Glenn) were analyzed in infants with HLHS. Patients were stratified into two groups based on outcome of transplant-free survival post-Glenn (survivors) versus mortality or transplantation prior to Fontan (non-survivors). Images were retrospectively reviewed to obtain RV global longitudinal strain (RVGLS), RV-free wall strain (RVFWS), fractional area change (FAC), tricuspid annular systolic plane excursion (TAPSE), tissue motion annular displacement of the tricuspid valve (TMAD-TV) and qualitative systolic function assessment during the predetermined timepoints. An equal variance t-test and chi-square were used to determine significant differences and ROC curve analysis was performed to derive optimal cutoff values to predict mortality/transplant. A total of 47 patients met inclusion criteria, of which, 21 patients met composite endpoint. There were no significant differences in any RV functional parameter during the pre- or post-stage 1 palliation timepoints. The absolute values of RVFWS, RVGLS, and TMAD-TV were significantly greater in survivors than non-survivors during the pre-Glenn timepoint. A pre-Glenn RVGLS > -15.6 (AUC 0.79), RVFWS > -18.6 (AUC 0.75), and TMAD-TV < 12.6% (AUC 0.82) were sensitive and specific for predicting death or need for transplantation prior to Fontan completion. RVGLS, RVFWS, and TMAD-TV may help identify higher-risk HLHS patients during the interstage period.

Cardiac magnetic resonance imaging in detection of progressive graft dysfunction in pediatric heart transplantation.

BACKGROUND: Chronic graft failure (CGF) in pediatric heart transplant (PHT) is multifactorial and may present with findings of fibrosis and microvessel disease (MVD) on endomyocardial biopsy (EMB). There is no optimal CGF surveillance method. We evaluated associations between cardiac magnetic resonance imaging (CMR) and historical/EMB correlates of CGF to assess CMR's utility as a surveillance method. METHODS: Retrospective analysis of PHT undergoing comprehensive CMR between September 2015 and January 2022 was performed. EMB within 6 months was graded for fibrosis (scale 0-5) and MVD (number of capillaries with stenotic wall thickening per field of view). Correlation analysis and logistic regression were performed. RESULTS: Forty-seven PHT with median age at CMR of 15.7 years (11.6, 19.3) and time from transplant of 6.4 years (4.1, 11.0) were studied. Cardiac allograft vasculopathy (CAV) was present in 11/44 (22.0%) and historical rejection in 14/41 (34.2%). CAV was associated with higher global T2 (49.0 vs. 47.0 ms; p = 0.038) and peak T2 (57.0 vs. 53.0 ms; p = 0.013) on CMR. Historical rejection was associated with higher global T2 (49.0 vs. 47.0 ms; p = 0.007) and peak T2 (57.0 vs. 53.0 ms; p = 0.03) as well as global extracellular volume (31.0 vs. 26.3%; p = 0.03). Higher fibrosis score on EMB correlated with smaller indexed left ventricular mass (rho = -0.34; p = 0.019) and greater degree of MVD with lower indexed left ventricular end-diastolic volume (rho = -0.35; p = 0.017). CONCLUSION: Adverse ventricular remodeling and abnormal myocardial characteristics on CMR are present in PHT with CAV, historical rejection, as well as greater fibrosis and MVD on EMB. CMR has the potential use for screening of CGF.

Late-gadolinium enhancement is common in older pediatric heart transplant recipients and is associated with lower ejection fraction.

BACKGROUND: Chronic graft failure and cumulative rejection history in pediatric heart transplant recipients (PHTR) are associated with myocardial fibrosis on endomyocardial biopsy (EMB). Cardiovascular magnetic resonance imaging (CMR) is a validated, non-invasive method to detect myocardial fibrosis via the presence of late gadolinium enhancement (LGE). In adult heart transplant recipients, LGE is associated with increased risk of future adverse clinical events including hospitalization and death. We describe the prevalence, pattern, and extent of LGE on CMR in a cohort of PHTR and its associations with recipient and graft characteristics. METHODS: This was a retrospective study of consecutive PHTR who underwent CMR over a 6-year period at a single center. Two independent reviewers assessed the presence and distribution of left ventricular (LV) LGE using the American Heart Association (AHA) 17-segment model. LGE quantification was performed on studies with visible fibrosis (LGE+). Patient demographics, clinical history, and CMR-derived volumetry and ejection fractions were obtained. RESULTS: Eighty-one CMR studies were performed on 59 unique PHTR. Mean age at CMR was 14.8 ± 6.2 years; mean time since transplant was 7.3 ± 5.0 years. The CMR indication was routine surveillance (without a clinical concern based on laboratory parameters, echocardiography, or cardiac catheterization) in 63% (51/81) of studies. LGE was present in 36% (29/81) of PHTR. In these LGE + studies, patterns included inferoseptal in 76% of LGE + studies (22/29), lateral wall in 41% (12/29), and diffuse, involving > 4 AHA segments, in 21% (6/29). The mean LV LGE burden as a percentage of myocardial mass was 18.0 ± 9.0%. When reviewing only the initial CMR per PHTR (n = 59), LGE + patients were older (16.7 ± 2.9 vs. 12.8 ± 4.6 years, p = 0.001), with greater time since transplant (8.3 ± 5.4 vs. 5.7 ± 3.9 years, p = 0.041). These patients demonstrated higher LV end-systolic volume index (LVESVI) (34.7 ± 11.7 vs. 28.7 ± 6.1 ml/m2, p = 0.011) and decreased LV ejection fraction (LVEF) (56.2 ± 8.1 vs. 60.6 ± 5.3%, p = 0.015). There were no significant differences in history of moderate/severe rejection (p = 0.196) or cardiac allograft vasculopathy (CAV) (p = 0.709). CONCLUSIONS: LV LGE was present in approximately one third of PHTR, more commonly in older patients with longer time since transplantation. Grafts with LGE have lower LVEF. CMR-derived LGE may aid in surveillance of chronic graft failure in PHTR.

Echocardiogram-Guided Balloon Valvuloplasty of the Aortic Valve in Neonates and Infants Reduces Contrast Exposure with Maintained Efficacy and Less Aortic Regurgitation.

Balloon aortic valvuloplasty (BAV) is performed in children with significant aortic stenosis (AS). Traditionally, contrast angiography measures the annulus and assesses aortic regurgitation (AR) after each dilation. Echocardiographic guidance is hypothesized to reduce contrast and radiation exposure, without compromising efficacy or safety. Patients < 10 kg undergoing BAV from 2013 to 2022 were retrospectively investigated. Agreement between echocardiographic and angiographic annulus measurements was assessed. Echocardiogram-guided (eBAV) and traditional angiogram-guided (tBAV) outcomes were compared controlling for weight, critical AS, and other congenital heart disease (CHD). Twelve eBAV and 19 tBAV procedures were performed. The median age was 33 days, median weight was 4.3 kg, 7 patients (23%) had critical AS, and 9 patients (29%) had other CHD. Annulus measurements by intraprocedural echocardiography and angiography displayed excellent correlation (ICC 0.95, p < 0.001). eBAV patients received less contrast (0.5 vs 3.5 ml/kg, p < 0.01). Five recent eBAV procedures were performed contrast free. Radiation exposure was not statistically different between the eBAV and tBAV groups (155 vs 313 µGy·M2, p = 0.12). One eBAV patient (8%) and 3 tBAV patients (16%, p = 0.62) experienced serious adverse events. Technical success (gradient < 35 mmHg and increase in AR by ≤ 1 grade) occurred in 11 eBAV patients (92%) and 16 tBAV patients (84%, p = 0.22). AR increased in 2 eBAV patients (17%) and 8 tBAV patients (44%, p = 0.02). eBAV was associated with similar efficacy and significantly lower contrast exposure and risk of aortic regurgitation. There was strong agreement between aortic valve annulus measurements by intraprocedural echocardiography and angiography, ultimately permitting contrast free BAV.

Multisystem Inflammatory Syndrome in Children (MIS-C): Reduced Exercise Duration and Capacity at Six Month Follow-Up.

Myocarditis is common in Multisystem Inflammatory Syndrome in Children (MIS-C), and the mechanism may differ from idiopathic/viral myocarditis as MIS-C involves a hyper-inflammatory state weeks after COVID-19. We sought to evaluate exercise stress testing (EST) in these patients as EST may help guide return-to-play recommendations. Retrospective cohort study evaluating ESTs (standard Bruce treadmill protocol) from MIS-C patients from 2020 to 2022, compared to myocarditis patients and age, sex, and weight matched controls from 2005 to 2019. ESTs included 22 MIS-C patients (mean age 11.9 years) with 14 cardiopulmonary and 8 cardiovascular tests, 33 myocarditis (15.5 years), and 44 controls (12.0 years). Percent-predicted peak VO2 was abnormal (< 80% predicted) in 11/14 (79%) MIS-C patients, 13/33 (39%) myocarditis, and 17/44 (39%) controls (p = 0.04). Exercise duration was shorter in MIS-C than myocarditis or control cohorts (p = 0.01). Isolated atrial or ventricular ectopy was seen in 8/22 (36%) MIS-C, 9/33 (27%) myocarditis, and 5/44 (11%) controls (p = 0.049). No arrhythmias/complex ectopy or evidence of ischemia were noted, though non-specific ST/T wave abnormalities occurred in 4/22 (18%) MIS-C, 5/33 (15%) myocarditis, and 3/44 (7%) controls. Exercise duration and percent-predicted peak VO2 were significantly reduced in MIS-C at mean 6-month follow-up compared to pre-COVID era idiopathic/viral myocarditis and control cohorts. This may be secondary to deconditioning during the pandemic and/or chronic cardiopulmonary or autonomic effects of COVID/MIS-C. Although there were no exercise-induced arrhythmias in our MIS-C patients, larger cohort studies are warranted. EST in MIS-C follow-up may help evaluate safety and timing of return to play and potentially mitigate further deconditioning.

Fetal Echocardiographic Variables Associated with Pre-Surgical Mortality in Truncus Arteriosus: A Pilot Study.

Truncus arteriosus (TA) is a rare congenital heart defect that can be prenatally detected by fetal echocardiography. However, prognostication and prenatal counseling focus primarily on surgical outcomes due to limited fetal and neonatal pre-surgical mortality data. We aimed to describe the incidence and identify predictors of pre-surgical mortality in prenatally detected TA. This two-center, retrospective cohort study included fetuses diagnosed with TA between 01/2010 and 04/2020. The primary outcome was pre-surgical mortality, defined by fetal or neonatal pre-surgical death or primary listing for transplantation prior to discharge. Univariable regression modeling, Chi-square tests, and t tests assessed for associations between prenatal clinical, demographic, and fetal echocardiographic (fetal-echo) variables and pre-surgical mortality. Of 23 pregnancies with prenatal diagnosis of TA, 4 (17%) were terminated. Of the remaining 19, pre-surgical mortality occurred in 4 (26%), including 2 (11%) fetal deaths and 2 (11%) neonatal pre-surgical deaths. No transplantation listings. Of liveborn fetuses (n = 17), 15 (88%) underwent a neonatal surgery, and 1 (6%) required ECMO. As compared to the survivors, the pre-surgical mortality group had a higher likelihood of having left ventricular dysfunction (0% vs. 40%; p = 0.01), right ventricular dysfunction (0% vs. 60%; p = 0.002), cardiovascular profile score < 7 (0% vs. 40%; p = 0.01), skin edema (0% vs. 40%; p = 0.01), and abnormal umbilical venous (UV) Doppler (0% vs. 60%; p = 0.002). The presence of truncal valve regurgitation or stenosis neared significance. In this cohort with prenatally diagnosed TA, there is significant pre-surgical mortality, including fetal death and neonatal pre-surgical death. Termination rate is also high. Fetal-echo variables associated with pre-surgical mortality in this cohort include ventricular dysfunction, low CVP, skin edema, and abnormal UV Doppler. Knowledge about prenatal risk factors for pre-surgical mortality may guide parental counseling and postnatal planning in prenatally diagnosed TA.

Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload.

BACKGROUND: Beta thalassemia major (Beta-TM) is an inherited condition which presents at around two years of life. Patients with Beta-;TM may develop cardiac iron toxicity secondary to transfusion dependence. Cardiovascular magnetic resonance (CMR) T2*, a technique designed to quantify myocardial iron deposition, is a driving component of disease management. A decreased T2* value represents increasing cardiac iron overload. The clinical manifestation is a decline in ejection fraction (EF). However, there may be early subclinical changes in cardiac function that are not detected by changes in EF. CMR-derived strain assesses myocardial dysfunction prior to decline in EF. Our primary aim was to assess the correlation between CMR strain and T2* in the Beta-TM population. METHODS: Circumferential and longitudinal strain was analyzed. Pearson's correlation was calculated for T2* values and strain in the Beta-TM population. RESULTS: We identified 49 patients and 18 controls. Patients with severe disease (low T2*) were found to have decreased global circumferential strain (GCS) in comparison to other T2* groups. A correlation was identified between GCS and T2* (r = 0.5; p < 0.01). CONCLUSION: CMR-derived strain can be a clinically useful tool to predict early myocardial dysfunction in Beta-TM.

Right Ventricular Remodeling Assessed by MRI in Duchenne Muscular Dystrophy.

BACKGROUND: In Duchenne muscular dystrophy (DMD), the right ventricle (RV) tends to be relatively well preserved, but characterization remains difficult due to its complex architecture. Tissue phase mapping (TPM) is a phase contrast cine MRI technique that allows for multidirectional assessment of myocardial velocities. PURPOSE: To use TPM to elucidate relationships between myocardial structure, function, and clinical variables in DMD. STUDY TYPE: Retrospective. SUBJECTS: A total of 20 patients with muscular dystrophy (median age: 16 years); 18 age-matched normal controls (median age: 15 years). FIELD STRENGTH/SEQUENCE: Three-directional velocity encoded cine gradient echo sequence (TPM) at 1.5 T, balanced steady-state free procession (bSSFP), T1 mapping with extracellular volume (ECV), and late gadolinium enhancement (LGE). ASSESSMENT: TPM in basal, mid, and apical short-axis planes was performed as part of a standard MRI study with collection of clinical data. Radial, circumferential, and longitudinal velocities (Vr, Vφ, and Vz, respectively) and corresponding time to peak (TTP) velocities were quantified from TPM and used to calculate RV twist as well as intraventricular and interventricular dyssynchrony. The correlations between TPM velocities, myocardial structure/function, and clinical variables were assessed. STATISTICAL TEST: Unpaired t-test, Wilcoxon rank-sum test, Bland-Altman analyses were used for comparisons between DMD patients and controls and between DMD subgroups. Pearson's test was used for correlations (r). Significance level: P < 0.05. RESULTS: Compared to controls, DMD patients had preserved RV ejection fraction (RVEF 53% ± 8%) but significantly increased interventricular dyssynchrony (Vφ: 0.49 ± 0.21 vs. 0.72 ± 0.17). Within the DMD cohort, RV dyssynchrony significantly increased with lower LV ejection fraction (intraventricular Vr and Vz: r = -0.49; interventricular Vz: r = 0.48). In addition, RV intraventricular dyssynchrony significantly increased with older age (Vz: r = 0.67). DATA CONCLUSION: RV remodeling in DMD occurs in the context of preserved RVEF. Within DMD, this abnormal RV deformation is associated with older age and decreased LVEF. EVIDENCE LEVEL: 4. TECHNICAL EFFICACY: Stage 2.

Increased fibrosis and microvessel disease in allograft endomyocardial biopsies of children with chronic graft failure due to cardiac allograft vasculopathy.

INTRODUCTION: Chronic graft failure (CGF) is the leading cause of mortality in pediatric heart transplant (PHT) patients and has multifactorial pathogenesis including cardiac allograft vasculopathy (CAV). CGF can present with microvessel disease (MVD) and myocardial fibrosis on endomyocardial biopsies (EMB). We investigated if CGF due to moderate- severe (M-S) CAV has histopathologic MVD and fibrosis prior to or at the time of CAV diagnosis. METHOD: This retrospective case-control study included PHT with CGF secondary to M-S CAV. Control patients had no CAV or CGF. EMBs from CAV (3 sets: at 1-year post-transplant 1yrCAV, pre-CAV, and at the time of CAV diagnosis) and non-CAV cohorts were reviewed to grade the fibrosis and quantify MVD. Histopathologic changes were correlated and compared between CAV/non-CAV groups. RESULTS: Each group had 8 patients. The median age at transplantation and time since transplant were similar between the two groups (P=.71 and P=.91, respectively). Fibrosis grade was 3.0 for CAV cohort compared to 1.0 for control (P= .003) and MVD score was 2.1 in CAV and 0.5 in non-CAV patients (P=.003). Similar degrees of fibrosis and MVD were present even before any evidence of CAV (1yrCAV fibrosis grade 2.5, pre-CAV fibrosis grade 2; 1yrCAV vs CAV P=.75, pre-CAV vs CAV P=.63; 1yrCAV MVD score 2, pre-CAV MVD score 2; 1yrCAV vs CAV P=1, pre-CAV vs CAV P=.91). The degree of MVD correlated with fibrosis (r=0.63, P<.0001) for all EMBs. CONCLUSION: Simultaneous myocardial fibrosis and MVD are noted in CGF secondary to M-S CAV, changes that occur before angiographic CAV. EMBs can reveal significant changes in patients with subsequent development of CAV and may be used to modify the follow-up and treatment for these high-risk patients.

Simulation Based Mastery Learning of Transesophageal Echocardiography.

Transesophageal echocardiography (TEE) education is part of pediatric cardiology fellow training. Simulation-based mastery learning (SBML) is an efficient and valuable education experience. The aim of this project was to equip trainees with the basic knowledge and skill required to perform a pediatric TEE. The secondary aim was to assess the utility of using SBML for pediatric TEE training. The target group is trainees from pediatric cardiology and cardiac anesthesia who participated in a TEE bootcamp. A baseline knowledge pretest was obtained. The knowledge session consisted of preparation via reading material, viewing recorded lectures and completing an iterative multiple-choice examination, which was repeated until a minimum passing score of 90% was achieved. The skills session involved a review of TEE probe manipulation and image acquisition, followed by rapid cycle deliberate practice using simulation to acquire TEE skills at 3 levels, advancing in complexity from level 1 to level 3. Eight individuals (7 pediatric cardiology fellows at varying training levels and one anesthesia attending) participated in the TEE bootcamp. All reached a minimum knowledge post test score of at least 90% before the skills session. All subjects reached mastery in TEE probe manipulation. All reached mastery in image acquisition for the skill level that they attempted (level 1-8/8, level 2-8/8, level 3-4/4, with 4 participants not attempting level 3). A TEE bootcamp using SBML is a powerful medical education strategy. SBML is a rigorous approach that can be used to achieve high and uniform TEE learning outcomes among trainees of different training levels and backgrounds.

Clinical Predictors of Subacute Myocardial Dysfunction in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19.

Multisystem Inflammatory Syndrome in Children (MIS-C) often involves a post-viral myocarditis and associated left ventricular dysfunction. We aimed to assess myocardial function by strain echocardiography after hospital discharge and to identify risk factors for subacute myocardial dysfunction. We conducted a retrospective single-center study of MIS-C patients admitted between 03/2020 and 03/2021. Global longitudinal strain (GLS), 4-chamber longitudinal strain (4C-LS), mid-ventricular circumferential strain (CS), and left atrial strain (LAS) were measured on echocardiograms performed 3-10 weeks after discharge and compared with controls. Among 60 MIS-C patients, hypotension (65%), ICU admission (57%), and vasopressor support (45%) were common, with no mortality. LVEF was abnormal (< 55%) in 29% during hospitalization but only 4% at follow-up. Follow-up strain abnormalities were prevalent (GLS abnormal in 13%, 4C-LS in 18%, CS in 16%, LAS in 5%). Hypotension, ICU admission, ICU and hospital length of stay, and any LVEF < 55% during hospitalization were factors associated with lower strain at follow-up. Higher peak C-reactive protein (CRP) was associated with hypotension, ICU admission, total ICU days, and with lower follow-up GLS (r = - 0.55; p = 0.01) and CS (r = 0.41; p = 0.02). Peak CRP < 18 mg/dL had negative predictive values of 100% and 88% for normal follow-up GLS and CS, respectively. A subset of MIS-C patients demonstrate subclinical systolic and diastolic function abnormalities at subacute follow-up. Peak CRP during hospitalization may be a useful marker for outpatient cardiac risk stratification. MIS-C patients with hypotension, ICU admission, any LVEF < 55% during hospitalization, or a peak CRP > 18 mg/dL may warrant closer monitoring than those without these risk factors.

Postnatal Outcomes in Infants With a History of Fetal Supraventricular Tachycardia.

BACKGROUND: Fetal supraventricular tachycardia (SVT) is rare and proposed predictors of postnatal outcomes in fetal SVT have not been validated. Valid predictors can guide postnatal management. OBJECTIVES: The authors correlated fetal characteristics to the incidence of postnatal SVT and compared SVT outcomes in infants with and without a history of fetal SVT. METHODS: Mother-fetus dyads with fetal SVT and a structurally normal heart were described and compared with a second cohort of infants with a postnatal diagnosis of SVT. RESULTS: SVT was observed in 78 fetuses and 76 survived to delivery. Maternally administered transplacental antiarrhythmics were used in 49 mother-fetus dyads. Rhythm control was achieved in 37 of 49 (76%). Among fetuses with intermittent SVT, there was no ventricular dysfunction or hydrops. Postnatal SVT occurred in one-half of infants (37 of 76), and 94% presented within the first 2 days of life. The following fetal characteristics were associated with postnatal SVT on univariable analysis: sustained SVT (87% vs 56%), ventricular dysfunction (41% vs 15%), lack of conversion to sinus rhythm (49% vs 10%), and earlier gestational age at delivery (37.6 weeks vs 38.9 weeks; P ≤ 0.01 for each comparison). Compared with infants with a postnatal diagnosis of SVT, infants with a fetal diagnosis presented earlier (median age 0 days vs 17 days; P < 0.01) and had a lower incidence ventricular dysfunction at presentation (5% vs 42%; P < 0.01). CONCLUSIONS: One-half of infants with fetal SVT had postnatal SVT, nearly all within 2 days of life. These data and predictors of postnatal SVT may influence parental counseling and postnatal clinical decision-making.

Safety and Efficacy of Regadenoson for Pediatric Stress Perfusion Cardiac MRI with Quantification of Myocardial Blood Flow.

Myocardial stress perfusion magnetic resonance imaging is a non-invasive tool to assess for myocardial ischemia and viability. Pediatric myocardial stress perfusion MRI can be challenging due to multiple intravenous lines, sedation, inadequate breath holding, fast heart rates, and complex anatomy. We performed a retrospective analysis in 39 children to evaluate safety and efficacy of regadenoson, a coronary vasodilator administered via a single intravenous line (6−10 mcg/kg), with respiratory motion correction (MOCO) and semi-quantitative blood flow analysis. Stress response data and adverse events were recorded, and image quality compared between native and MOCO reconstructions, assessing for perfusion deficits. Semi-quantitative analysis compared myocardial perfusion reserve index (MPRI) between patients who had a focal perfusion defect, patients who had undergone an orthotopic heart transplant, and non-transplant patients with no focal defects. Stress perfusion was completed in 38/39 patients (median age 15 years with a 41 ± 27% rise in heart rate (p < 0.005). Fifteen out of thirty-eight had transient minor side effects with no major adverse events. MOCO image quality was better than non-MOCO (4.63 vs. 4.01 at rest, p < 0.005: 4.41 vs. 3.84 at stress, p < 0.005). Reversible perfusion defects were seen in 4/38 patients with lower segmental mean MPRI in the area of the perfusion defect, nearing statistical significance when compared to non-transplant patients with no defects (0.78 ± 0.22 vs. 0.99 ± 0.36, p = 0.07). The global MPRI of the 16 patients who had undergone orthotopic heart transplant was significantly lower than the non-transplant patients (0.75 ± 0.22 vs. 0.92 ± 0.23, p = 0.03). Regadenoson is a safe and effective coronary vasodilator for pediatric stress perfusion MRI with MOCO producing better image quality and allowing for semi-quantitative assessment of perfusion deficits that correlate with qualitative assessment.