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.

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.

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.

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.

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.

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.

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.

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.

Impact of Socioeconomic Status, Race and Ethnicity, and Geography on Prenatal Detection of Hypoplastic Left Heart Syndrome and Transposition of the Great Arteries.

BACKGROUND: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada. METHODS: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers. RESULTS: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND. CONCLUSIONS: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

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.

Combined Echo and Fluoroscopy-Guided Pulmonary Valvuloplasty in Neonates and Infants: Efficacy and Safety.

Percutaneous balloon pulmonary valvuloplasty (PBPV) is the treatment of choice for isolated pulmonary valve stenosis. While this procedure is highly efficacious and has an excellent safety profile, as currently practiced, patients are obligatorily exposed to the secondary risks of ionizing radiation and contrast media. To mitigate these risks, we developed a protocol which utilized echo guidance for portions of the procedure which typically require fluoroscopy and/or angiography. Ten cases of echo-guided pulmonary valvuloplasty (EG-PBPV) for isolated pulmonary stenosis in children less than a year of age were compared to a historical cohort of nineteen standard cases using fluoroscopy/angiography alone, which demonstrated equivalent procedural outcomes and safety, while achieving a median reduction in radiation (total dose area product) and contrast load of 80% and 84%, respectively. Our early experience demonstrates that EG-PBPV in neonates and infants has results equivalent to standard valvuloplasty but with less radiation and contrast.

Multi-parametric cardiovascular magnetic resonance with regadenoson stress perfusion is safe following pediatric heart transplantation and identifies history of rejection and cardiac allograft vasculopathy.

BACKGROUND: The progressive risk of graft failure in pediatric heart transplantation (PHT) necessitates close surveillance for rejection and coronary allograft vasculopathy (CAV). The current gold standard of surveillance via invasive coronary angiography is costly, imperfect and associated with complications. Our goal was to assess the safety and feasibility of a comprehensive multi-parametric CMR protocol with regadenoson stress perfusion in PHT and evaluate for associations with clinical history of rejection and CAV. METHODS: We performed a retrospective review of 26 PHT recipients who underwent stress CMR with tissue characterization and compared with 18 age-matched healthy controls. CMR protocol included myocardial T2, T1 and extracellular volume (ECV) mapping, late gadolinium enhancement (LGE), qualitative and semi-quantitative stress perfusion (myocardial perfusion reserve index; MPRI) and strain imaging. Clinical, demographics, rejection score and CAV history were recorded and correlated with CMR parameters. RESULTS: Mean age at transplant was 9.3 ± 5.5 years and median duration since transplant was 5.1 years (IQR 7.5 years). One patient had active rejection at the time of CMR, 11/26 (42%) had CAV 1 and 1/26 (4%) had CAV 2. Biventricular volumes were smaller and cardiac output higher in PHT vs. healthy controls. Global T1 (1053 ± 42 ms vs 986 ± 42 ms; p < 0.001) and ECV (26.5 ± 4.0% vs 24.0 ± 2.7%; p = 0.017) were higher in PHT compared to helathy controls. Significant relationships between changes in myocardial tissue structure and function were noted in PHT: increased T2 correlated with reduced LVEF (r = - 0.57, p = 0.005), reduced global circumferential strain (r = - 0.73, p < 0.001) and reduced global longitudinal strain (r = - 0.49, p = 0.03). In addition, significant relationships were noted between higher rejection score and global T1 (r = 0.38, p = 0.05), T2 (r = 0.39, p = 0.058) and ECV (r = 0.68, p < 0.001). The presence of even low-grade CAV was associated with higher global T1, global ECV and maximum segmental T2. No major side effects were noted with stress testing. MPRI was analyzed with good interobserver reliability and was lower in PHT compared to healthy controls (0.69 ± - 0.21 vs 0.94 ± 0.22; p < 0.001). CONCLUSION: In a PHT population with low incidence of rejection or high-grade CAV, CMR demonstrates important differences in myocardial structure, function and perfusion compared to age-matched healthy controls. Regadenoson stress perfusion CMR could be safely and reliably performed. Increasing T2 values were associated with worsening left ventricular function and increasing T1/ECV values were associated with rejection history and low-grade CAV. These findings warrant larger prospective studies to further define the role of CMR in PHT graft surveillance.

Myocardial velocity, intra-, and interventricular dyssynchrony evaluated by tissue phase mapping in pediatric heart transplant recipients.

BACKGROUND: Endomyocardial biopsy (EMB) is the standard method for detecting allograft rejection in pediatric heart transplants (Htx). As EMB is invasive and carries a risk of complications, there is a need for a noninvasive alternative for allograft monitoring. PURPOSE: To quantify left and right ventricular (LV & RV) peak velocities, velocity twist, and intra-/interventricular dyssynchrony using tissue phase mapping (TPM) in pediatric Htx compared with controls, and to explore the relationship between global cardiac function parameters and the number of rejection episodes to these velocities and intra-/interventricular dyssynchrony. STUDY TYPE: Prospective. SUBJECTS: Twenty Htx patients (age: 16.0 ± 3.1 years, 11 males) and 18 age- and sex-matched controls (age: 15.5 ± 4.3 years, nine males). FIELD STRENGTH/SEQUENCE: 5T; 2D balanced cine steady-state free-precession (bSSFP), TPM (2D cine phase contrast with three-directional velocity encoding). ASSESSMENT: LV and RV circumferential, radial, and long-axis velocity-time curves, global and segmental peak velocities were measured using TPM. Short-axis bSSFP images were used to measure global LV and RV function parameters. STATISTICAL TESTS: A normality test (Lilliefors test) was performed on all data. For comparisons, a t-test was used for normally distributed data or a Wilcoxon rank-sum test otherwise. Correlations were determined by a Pearson correlation. RESULTS: Htx patients had significantly reduced LV (P < 0.05-0.001) and RV (P < 0.05-0.001) systolic and diastolic global and segmental long-axis velocities, reduced RV diastolic peak twist (P < 0.01), and presented with higher interventricular dyssynchrony for long-axis and circumferential motions (P < 0.05-0.001). LV diastolic long-axis dyssynchrony (r = 0.48, P = 0.03) and RV diastolic peak twist (r = -0.64, P = 0.004) significantly correlated with the total number of rejection episodes. DATA CONCLUSION: TPM detected differences in biventricular myocardial velocities in pediatric Htx patients compared with controls and indicated a relationship between Htx myocardial velocities and rejection history. LEVEL OF EVIDENCE: 2 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2020;51:1212-1222.

Altered 4-D magnetic resonance imaging flow characteristics in complex congenital aortic arch repair.

BACKGROUND: Interrupted aortic arch (IAA) is a rare but severe congenital abnormality often associated with bicuspid aortic valve (BAV). Complex re-interventions are often needed despite surgical advances, but the impact of aortic hemodynamics in repaired patients is unknown. OBJECTIVE: Investigate effect of IAA repairs on aortic hemodynamics, wall shear stress and flow derangements via 4-D flow MRI. MATERIALS AND METHODS: We retrospectively analyzed age- and gender-matched cohorts (IAA [n=6], BAV alone [n=6], controls [n=6]) undergoing cardiac MRI including 4-D flow. Aortic dimensions were measured from standard MR angiography. We quantified peak systolic velocities, regurgitant fractions and wall shear stress in the ascending aorta (AAo), transverse arch and descending aorta (DAo) from 4-D flow, and we graded helix/vortex flow patterns from 3-D blood flow visualization. RESULTS: Children and young adults with IAA had a wide range of arch dimensions, peak systolic velocities, regurgitant fractions and flow grades. Peak transverse arch systolic velocities were higher in patients with IAA versus controls (P=0.02). Flow derangements in the AAo were found in patients with IAA (median grade=2, 5/6 patients, P=0.04) and BAV (median grade=3, 5/6 patients, P=0.03) versus controls. Flow derangements in the DAo were only seen in patients with IAA (median grade=1, 5/6 patients, P=0.04), and 5/6 people with IAA had helical flow in head and neck vessels. Wall shear stress was increased in people with IAA along the superior transverse arch and proximal DAo versus controls (P=0.02). CONCLUSION: Complex congenital aortic arch repairs can change aortic hemodynamics. Associated cardiac defects can further alter findings. Studies are warranted to investigate clinical implications in larger cohorts.

Diagnosis and Management of Anomalous Left Anterior Descending Coronary Artery from the Pulmonary Artery in an Asymptomatic Infant.

Anomalous origin of the left anterior descending coronary artery from the pulmonary artery is a rare variant of anomalous origin of the left main coronary artery from the pulmonary artery. We report on a seemingly asymptomatic patient with ALADCAPA and a small restrictive muscular ventricular septal defect diagnosed by echocardiogram in the neonatal period. Our patient underwent elective repair at 3.5 months of age after which feeding and growth improved dramatically. Multimodality imaging is helpful to confirm this rare anomaly; however, echocardiographic clues including lack of left coronary branching or an abnormal coronary course should raise suspicion for ALADCAPA. This case provides support for early repair in children with an incidental finding of this anomaly as subclinical ischemia may be under-recognized by available testing but may lead to symptoms later in life.

Catheterization diastolic pressures correlate with diastolic dyssynchrony in patients with single right ventricles.

INTRODUCTION: Diastolic dyssynchrony has correlated with pulmonary capillary wedge pressures in patients with normal cardiac anatomy. No data exist in single right ventricle (sRV) patients. Goal of this study was to determine if diastolic dyssynchrony in sRV patients correlates with ventricular end-diastolic pressures (VEDP). METHODS: Tissue Doppler imaging (TDI) and strain rate (SR) analysis of sRV patients undergoing catheterization were performed. Time interval from onset of QRS to peak TDI e'-wave was obtained. Differences in intervals were calculated: QRS (RV) - QRS (IVS) and QRS (RV) - QRS (LV). Time interval from onset of QRS to peak strain rate early diastolic wave (SRe) was obtained for the 6 segment model sRV. Standard deviation of the 6 SRe time intervals was calculated. Correlation of VEDP with timing intervals was analyzed. RESULTS: Forty sRV patients were evaluated. Age was 2.8 ± 3.5 years. Catheterization VEDP of the sRV was 9.3 ± 3.9 mmHg (median 8 mmHg range 4-24 mmHg). QRS (RV) - QRS (IVS) was 22.3 ± 18.1 msec and QRS (RV) - QRS (LV) was 23.7 ± 19.0 msec. SRe standard deviation of the sRV was 61.6 ± 23.9 msec. There was no significant correlation with VEDP and QRS (RV) - QRS (IVS) (r = 0.1, P = NS) or with QRS (RV) - QRS (LV) (r = 0.2, P = NS). There was a significant correlation of VEDP with the SRe standard deviation value (r = 0.4, P < 0.05). CONCLUSION: Diastolic dyssynchrony correlated with VEDP in patients with sRV physiology. Future studies are needed to determine the significance of these findings.

Comparing echocardiographic assessment of systolic function with catheterization data in patients with single right ventricles.

PURPOSE: Echocardiographic evaluation of systolic function in patients with single right ventricles (SRV) is important but remains challenging. Minimal data exist correlating echocardiographic indices with catheterization data in this population. The goal of this study was to evaluate which echocardiographic measurement correlated best with dP/dt (max) obtained by cardiac catheterization in SRV patients. METHODS: Patients with SRV physiology who underwent simultaneous echocardiography and cardiac catheterization were evaluated. Echocardiographic data included fractional area change % (FAC), displacement, TDI s'wave, myocardial performance index (MPI), global systolic strain, and global SR s wave. Maximum positive rate of ventricular pressure change measured as dP/dt (max) was obtained from the cardiac catheterization report. Correlations of echocardiographic and catheterization variables were examined using the Pearson correlation. RESULTS: Twenty-seven SRV patients were studied. Median age at the time of the catheterization was 11.4 months (range 0 - 132 months). dP/dt (max) values ranged from 337-1860 mmHg/s with a median of 994 mmHg/s. Mean FAC was 27.15 +/- 7.13%, displacement was 7.35 +/- 2.88 mm, TDI s' was 4.98 +/- 1.93 cm/sec, MPI was 0.41 +/- 0.17, global strain was-14.85 +/- 4.32%, and global SR s wave was -1.03 +/- 0.34 sec(-1). There were no significant correlations between dP/dt (max) and any of the echocardiographic measurements of systolic function in SRV patients. CONCLUSION: In patients with SRV physiology, catheterization-derived dP/dt (max) did not correlate with echocardiographic measurements of systolic function. Larger studies are needed to determine which non-invasive parameter best describes systolic function in patients with SRV.

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.