Clinical Outcomes and Echocardiographic Predictors of Reintervention After Interrupted Aortic Arch Repair.

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm2/BSAm2) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge.

HPV education and vaccination uptake in college students: A DNP project.

INTRODUCTION: The purpose of this DNP project was to establish baseline human papillomavirus (HPV) vaccination rates, improve baseline knowledge of HPV and HPV vaccines, reduce barriers to HPV vaccination, and increase intention of HPV vaccine uptake among college students. METHODS: A precaution adoption process model-based survey, in-person education session, and posteducation survey were administered to college students in a Midwestern university to measure perceptions of HPV and HPV vaccination as well as to identify barriers to HPV vaccination. RESULTS: From the preeducation survey to the posteducation survey, correct answers to questions increased, and barriers and misconceptions about HPV and the HPV vaccine decreased, indicating successful education. HPV vaccination intent, defined as indication of "likely" or "very likely" to receive the vaccine on the surveys, rose from 28.0% to 90.7% among participating college students. CONCLUSION: This study showed that implementing engaging, in-person HPV prevention education is an effective method for decreasing barriers related to vaccination and increasing vaccination intention.

Synthetically glycosylated antigens for the antigen-specific suppression of established immune responses.

Inducing antigen-specific tolerance during an established immune response typically requires non-specific immunosuppressive signalling molecules. Hence, standard treatments for autoimmunity trigger global immunosuppression. Here we show that established antigen-specific responses in effector T cells and memory T cells can be suppressed by a polymer glycosylated with N-acetylgalactosamine (pGal) and conjugated to the antigen via a self-immolative linker that allows for the dissociation of the antigen on endocytosis and its presentation in the immunoregulatory environment. We show that pGal-antigen therapy induces antigen-specific tolerance in a mouse model of experimental autoimmune encephalomyelitis (with programmed cell-death-1 and the co-inhibitory ligand CD276 driving the tolerogenic responses), as well as the suppression of antigen-specific responses to vaccination against a DNA-based simian immunodeficiency virus in non-human primates. Our findings show that pGal-antigen therapy invokes mechanisms of immune tolerance to resolve antigen-specific inflammatory T-cell responses and suggest that the therapy may be applicable across autoimmune diseases.

Congenitally Corrected Transposition of the Great Arteries: Fetal Diagnosis, Associations, and Postnatal Outcome: A Fetal Heart Society Research Collaborative Study.

Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21-27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow-up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live-born. Of 158 with postnatal data (median follow-up 3.7 years), 10 (6.6%) had death/transplant before 1 year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1 year included ≥ mild tricuspid regurgitation, pulmonary atresia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). Conclusions Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.

3D-Printed Cardiac Models for Fetal Counseling: A Pilot Study and Novel Approach to Improve Communication.

A fetal cardiology consultation involves using two-dimensional drawings to explain the cardiac anatomy which can result in inherent variation in how the congenital heart disease (CHD) is conveyed. In this pilot study, we incorporated three-dimensional printed (3DP) models into fetal counseling to demonstrate feasibility and evaluate the impact on parental knowledge, understanding, and anxiety. Parents with a prenatal diagnosis of a muscular ventricular septal defect (VSD) and/or coarctation of aorta were enrolled. Providers were randomized into a Model or Drawing Group and crossed after six months. Parents completed a survey after the consultation which evaluated knowledge of the CHD lesion, expectant surgical management, self-rated understanding, attitude towards the visualization tool, and anxiety. Twenty-nine patients enrolled over a 12 month period. Twelve consultations were done for coarctation of aorta, 13 for VSD, and four for coarctation with a VSD. Both Model and Drawing groups scored similarly in self-reported understanding and confidence, helpfulness of and improvement in communication with the visualization tool. The Model group had higher scores on questions related to the CHD anatomy and surgical intervention [5 [4-5] versus 4 [3.5-5]], p = 0.23 although this didn't reach statistical significance. For the majority (83%) of consultations, the cardiologist agreed that the 3D model improved communication. In this pilot study, we demonstrate the use of 3DP cardiac models during prenatal CHD counseling is feasible and produces results related to parental understanding and knowledge that are equal to and possibly better than the current standard of care.

Ventricular function and tissue characterization by cardiac magnetic resonance imaging following hospitalization for multisystem inflammatory syndrome in children: a prospective study.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a severe life-threatening manifestation of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection that often presents with acute cardiac dysfunction and cardiogenic shock. While recovery from acute illness is excellent, the long-term myocardial impact is unknown. OBJECTIVE: To compare cardiac MRI findings in children 6-9 months after their hospitalization with MIS-C against MRI findings in healthy controls to assess for residual myocardial disease. MATERIALS AND METHODS: We prospectively performed cardiac MRI on 13 children 6-9 months following their hospitalization with MIS-C: eight of these children had a history of left ventricle ejection fraction (LVEF) < 50%, persistent symptoms, or electrocardiogram (ECG) abnormalities and underwent clinical MRI; five of these children without cardiac abnormalities during their hospitalization underwent research MRIs. We compared their native T1 and T2 mapping values with those of 20 normal controls. RESULTS: Cardiac MRI was performed at 13.6 years of age (interquartile range [IQR] 11.9-16.4 years) and 8.2 months (IQR 6.8-9.6 months) following hospitalization. Twelve children displayed normal ejection fraction: left ventricle (LV) 57.2%, IQR 56.1-58.4; right ventricle (RV) 53.1%, IQR 52.0-55.7. One had low-normal LVEF (52%). They had normal extracellular volume (ECV) and normal T2 and native T1 times compared to controls. There was no qualitative evidence of edema. One child had late gadolinium enhancement (LGE) with normal ejection fraction, no edema, and normal T1 and T2 times. When stratifying children who had MIS-C according to history of LVEF <55% on echocardiography, there was no difference in MRI values. CONCLUSION: Although many children with MIS-C present acutely with cardiac dysfunction, residual myocardial damage 6-9 months afterward appears minimal. Long-term implications warrant further study.

Pediatric Normal Values and Z Score Equations for Left and Right Ventricular Strain by Two-Dimensional Speckle-Tracking Echocardiography Derived from a Large Cohort of Healthy Children.

BACKGROUND: Strain values vary with age in children and are both vendor and platform specific. Philips QLAB 10.8 and TomTec AutoSTRAIN are two widely used strain analysis platforms, and both incorporate recent European Association of Cardiovascular Imaging/American Society of Echocardiography/Industry Task Force to Standardize Deformation Imaging guidelines. The aims of this study were to establish normal strain values and Z scores for both platforms using a large data set of healthy children and to compare values among these two platforms and a previous version, QLAB 10.5, which predated the task force guidelines. METHODS: Echocardiograms from 1,032 subjects <21 years old with structurally and functionally normal hearts were included. Images were obtained on the Philips EPIQ platform. Left ventricular (LV) and right ventricular (RV) strain was analyzed using QLAB 10.8 and AutoSTRAIN, and measurement reliability was assessed. Z score equations were derived as a function of age for QLAB 10.8 (LV longitudinal and circumferential strain) and AutoSTRAIN (LV and RV longitudinal strain). A subset (n = 309) was analyzed using QLAB 10.5. Strain values were compared among the three platforms. RESULTS: For both of the newer platforms, strain varied with age, with magnitude reaching a maximum at 4 to 5 years. For LV longitudinal strain, the largest differences in value were observed in the youngest patients when using QLAB 10.5; the other two platforms were similar. LV circumferential strain measurements (QLAB 10.5 vs QLAB 10.8) were different for all ages, as were measurements of RV longitudinal strain (QLAB 10.8 vs AutoSTRAIN). Reliability was greater for AutoSTRAIN than for QLAB 10.8 and greater for LV than for RV strain. CONCLUSIONS: Normal RV and LV strain values and Z scores were generated from a large cohort of children for two commonly used platforms in pediatric echocardiography laboratories. Following the incorporation of task force guidelines, the greatest improvement in standardization was seen in infants. Small differences persist between modern platforms; however, these results support the cautious consideration of comparing interplatform measurements.

Surgical Management of Complex Aortic Valve Disease in Young Adults: Repair, Replacement, and Future Alternatives.

The ideal aortic valve substitute in young adults remains unknown. Prosthetic valves are associated with a suboptimal survival and carry a significant risk of valve-related complications in young patients, mainly reinterventions with tissue valves and, thromboembolic events and major bleeding with mechanical prostheses. The Ross procedure is the only substitute that restores a survival curve similar to that of a matched general population, and permits a normal life without functional limitations. Though the risk of reintervention is the Achilles' heel of this procedure, it is very low in patients with aortic stenosis and can be mitigated in patients with aortic regurgitation by tailored surgical techniques. Finally, the Ozaki procedure and the transcatheter aortic valve implantation are seen by many as future alternatives but lack evidence and long-term follow-up in this specific patient population.

Activation of Nkx2.5 transcriptional program is required for adult myocardial repair.

The cardiac developmental network has been associated with myocardial regenerative potential. However, the embryonic signals triggered following injury have yet to be fully elucidated. Nkx2.5 is a key causative transcription factor associated with human congenital heart disease and one of the earliest markers of cardiac progenitors, thus it serves as a promising candidate. Here, we show that cardiac-specific RNA-sequencing studies reveal a disrupted embryonic transcriptional profile in the adult Nkx2.5 loss-of-function myocardium. nkx2.5-/- fish exhibit an impaired ability to recover following ventricular apex amputation with diminished dedifferentiation and proliferation. Complex network analyses illuminate that Nkx2.5 is required to provoke proteolytic pathways necessary for sarcomere disassembly and to mount a proliferative response for cardiomyocyte renewal. Moreover, Nkx2.5 targets embedded in these distinct gene regulatory modules coordinate appropriate, multi-faceted injury responses. Altogether, our findings support a previously unrecognized, Nkx2.5-dependent regenerative circuit that invokes myocardial cell cycle re-entry, proteolysis, and mitochondrial metabolism to ensure effective regeneration in the teleost heart.

Ventricular Function and Tissue Characterization By Cardiac MRI in Children Following Hospitalization for Multisystem Inflammatory Syndrome in Children (MIS-C): A Prospective Study.

Background Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe life-threatening manifestation of SARS-CoV-2 infection. Acute cardiac dysfunction and resultant cardiogenic shock are common in children with MIS-C. While most children recover rapidly from acute illness, the long-term impact on the myocardium and cardiac function is unknown. Methods In this prospective study, cardiac MRI (CMR) was performed on patients <21 years of age with a history of MIS-C, 6-9 months following hospitalization. Per institutional protocol, patients with any history of LVEF<50%, persistent cardiorespiratory symptoms, or ECG abnormalities underwent clinical CMR. Research CMRs were offered to all others >10 years old. Native T1 and T2 mapping values were compared with 20 children with normal CMR examinations. Results We performed CMRs on 13 subjects at a median age of 13.6 years (interquartile range [IQR] 11.9-16.0) and a median time from hospitalization of 8.2 months (IQR 6.8-9.6). Twelve subjects displayed normal ventricular function with a median left ventricle ejection fraction (LVEF) of 57.2% (IQR 56.1-58.4) and median right ventricular (RV) EF of 53.1% (IQR 52.0-55.7). One subject had low normal EF (52%). There was normal T2 and native T1 as compared to normal controls. There was qualitatively no evidence of edema by T2 weighted imaging. One subject had late gadolinium enhancement (LGE) at the inferior insertion point and mid-ventricular inferolateral region, with normal EF, no evidence of edema or perfusion defects, and normal T1 and T2 times. When stratifying by a history of abnormal LVEF (LVEF <55%) on echocardiography, there was no difference in or parametric mapping values, though LVEF and LVEDV approached significance (p=0.06 and 0.05, respectively). Conclusions Although many children with MIS-C present acutely with cardiac dysfunction, myocardial recovery is overall excellent with minimal to no evidence of residual cardiac dysfunction or myocardial involvement. LVEF by CMR at 6-9 months among children with history of echocardiographic LV dysfunction is slightly lower, though does not meet statistical significance and is still within normal range. The long-term functional implications of this finding and the cardiac implications of MIS-C more broadly are unclear and warrant further study.

Fetal echocardiographic diagnosis of a triad with common embryological origins: Cervical aortic arch, retro-aortic left innominate vein and coarctation of the aorta.

Cervical aortic arch (CAA) and retro-aortic innominate vein (RAIV) are rare entities which can be associated with one another in a structurally normal heart. Less well recognized is the fact, that a third entity may often be present as well, since aortic arch abnormalities are common in a CAA. Hence, these three entities (CAA, RAIV, and an arch anomaly - in this case a post-ductal coarctation) may present as a triad. We present the first prenatal diagnosis of this rare triad which can be explained by common embryological origins which are discussed along with instructive aspects in diagnosis and management.

Normal Left Ventricular Systolic and Diastolic Strain Rate Values in Children Derived from Two-Dimensional Speckle-Tracking Echocardiography.

BACKGROUND: Strain rate (SR) parameters derived from two-dimensional speckle-tracking echocardiography have prognostic value in children with heart disease. Routine use is hindered by a lack of normative data. The aim of this study was to determine reference values and Z scores for left ventricular systolic and diastolic SR in a large cohort of healthy children. METHODS: Echocardiograms from 577 subjects ≤18 years of age (mean age, 9.6 ± 5.6 years; range, 1 day to 18.0 years; 46% female) with structurally and functionally normal hearts were retrospectively included. Left ventricular longitudinal and circumferential systolic and early and late diastolic SR were measured using two-dimensional speckle-tracking echocardiography from the apical four-chamber and short-axis mid-papillary views. Associations with age and body surface area were assessed using Spearman correlation and generalized additive modeling. The relationship between systolic SR and wall stress (afterload) was examined. Analyses were conducted with and without correction for heart rate. Multivariable linear regression modeling was used to identify independent factors associated with the SR parameters. Z score equations were derived from a selected best-fit parametric model. RESULTS: All SR parameters differed significantly by age group. The magnitude of all SR values decreased with increasing age and body surface area. Systolic SR magnitude was inversely related to wall stress in children ≤7 years of age but not did not vary significantly in the older age groups. All relationships were maintained after heart rate correction. SR measurements had very good or excellent agreement. CONCLUSION: Longitudinal and circumferential systolic and diastolic SR parameters are presented from a large cohort of healthy children using two-dimensional speckle-tracking echocardiography from the Philips platform. SR values differ significantly by age and body surface area. These results suggest that the myocardium becomes less sensitive to afterload with maturity. Z score equations based on age are presented, which should promote further clinical and research use.

Impact of pulmonary valve replacement on left ventricular rotational mechanics in repaired tetralogy of Fallot.

BACKGROUND: In repaired tetralogy of Fallot (rTOF), abnormal left ventricular (LV) rotational mechanics are associated with adverse clinical outcomes. We performed a comprehensive analysis of LV rotational mechanics in rTOF patients using cardiac magnetic resonance (CMR) prior to and following surgical pulmonary valve replacement (PVR). METHODS: In this single center retrospective study, we identified rTOF patients who (1) had both a CMR ≤ 1 year before PVR and ≤ 5 years after PVR, (2) had no other intervening procedure between CMRs, (3) had a body surface area > 1.0 m2 at CMR, and (4) had images suitable for feature tracking analysis. These subjects were matched to healthy age- and sex-matched control subjects. CMR feature tracking analysis was performed on a ventricular short-axis stack of balanced steady-state free precession images. Measurements included LV basal and apical rotation, twist, torsion, peak systolic rates of rotation and torsion, and timing of events. Associations with LV torsion were assessed. RESULTS: A total of 60 rTOF patients (23.6 ± 7.9 years, 52% male) and 30 healthy control subjects (20.8 ± 3.1 years, 50% male) were included. Compared with healthy controls, rTOF patients had lower apical and basal rotation, twist, torsion, and systolic rotation rates, and these parameters peaked earlier in systole. The only parameters that were correlated with LV torsion were right ventricular (RV) end-systolic volume (r = - 0.28, p = 0.029) and RV ejection fraction (r = 0.26, p = 0.044). At a median of 1.0 year (IQR 0.5-1.7) following PVR, there was no significant change in LV rotational parameters versus pre-PVR despite reductions in RV volumes, RV mass, pulmonary regurgitation, and RV outflow tract obstruction. CONCLUSION: In this comprehensive study of CMR-derived LV rotational mechanics in rTOF patients, rotation, twist, and torsion were diminished compared to controls and did not improve at a median of 1 year after PVR despite favorable RV remodeling.

Cardiac self-gating using blind source separation for 2D cine cardiovascular magnetic resonance imaging.

PURPOSE: To develop and validate a new cardiac self-gating algorithm using blind source separation for 2D cine steady-state free precession (SSFP) imaging. METHODS: A standard cine SSFP sequence was modified so that the center point of k-space was sampled with each excitation. The center points of k-space were processed by 4 blind source separation methods, and used to detect heartbeats and assign k-space data to appropriate time points in the cardiac cycle. The proposed self-gating technique was prospectively validated in 8 patients against the standard electrocardiogram (ECG)-gating method by comparing the cardiac cycle lengths, image quality metrics, and ventricular volume measurements. RESULTS: There was close agreement between the cardiac cycle length using the ECG- and self-gating methods (bias 0.0 bpm, 95% limits of agreement ±2.1 bpm). The image quality metrics were not significantly different between the ECG- and self-gated images. The ventricular volumes, stroke volumes, and mass measured from self-gated images were all comparable with those from ECG-gated images (all biases <5%). CONCLUSION: The self-gating method yielded comparable cardiac cycle length, image quality, and ventricular measurements compared with standard ECG-gated cine imaging. It may simplify patient preparation, be more robust when there is arrhythmia, and allow cardiac gating at higher field strengths.

Certified Nurse-Midwives in Rural Kansas Hospitals: A Survey of Senior Hospital Administrators.

INTRODUCTION: Little is known about the nurse-midwifery workforce in rural Kansas hospitals, despite Kansas facing a shortage of primary care physicians providing maternity care rurally. This study investigated the current number of hospitals with certified nurse-midwives (CNMs) with privileges to attend births in Kansas hospitals located in frontier, rural, and densely settled rural counties and anticipated trends in the size of the CNM workforce at hospitals over the next 5 years. METHODS: Electronic surveys were distributed to senior hospital administrators at 94 hospitals in rural Kansas from June to July 2019. The survey included both open and closed-ended questions related to scope of CNM privileges, collaborative agreements, and forecasted trends in the CNM workforce in rural Kansas. RESULTS: Fifty-six hospitals completed the survey. Only one hospital reported having CNM-attended births. Twenty-eight of 37 hospital administrators agreed CNMs should have collaborative agreements with physicians. Most respondents did not anticipate the number of CNMs with privileges to increase at their hospitals over the next 5 years. DISCUSSION: Future research should focus on understanding the factors limiting CNM expansion in rural Kansas, because CNMs represent an untapped, additional maternity care workforce for rural Kansas.

Serial Changes in Right Ventricular Systolic Function Among Rejection-Free Children and Young Adults After Heart Transplantation.

BACKGROUND: Evolution of right ventricular (RV) systolic function after pediatric heart transplantation (HT) has not been well described. METHODS: We analyzed echocardiograms performed over the first year after HT among children and young adults who remained rejection-free. Ninety-six patients (median age 7.1 [0.1-24.4] years at HT) were included: 22 infants (≤1 year) and 74 noninfants (>1 year). Two-dimensional tricuspid annular plane systolic excursion (TAPSE), tissue Doppler-derived tricuspid annular systolic velocity (S'), fractional area change (FAC), myocardial performance index (MPI), and two-dimensional speckle-tracking-derived RV global longitudinal (GLS) and free wall strain (FWS) were assessed. RESULTS: All measures of RV function were impaired immediately after HT and significantly improved over the first year: TAPSE z-score (-8.15 ± 1.88 to -3.94 ± 1.65, P < .0001), S' z-score (-4.30 ± 1.36 to -2.28 ± 1.33, P < .0001), FAC (24.37% ± 7.71% to 42.02% ± 7.09%, P < .0001), MPI (0.96 ± 0.47 to 0.41 ± 0.22, P < .0001), GLS (-10.37% ± 3.86% to -21.05% ± 3.41%, P < .0001), and FWS (-11.2% ± 4.08% to -23.66% ± 4.13%, P < .0001). By 1 year post-HT, TAPSE, S', GLS, and FWS, remained abnormal, whereas FAC and MPI nearly normalized. Patients transplanted during infancy demonstrated better recovery of RV systolic function. CONCLUSIONS: Although RV systolic function improved over the first year after HT in children and young adults without rejection, measures that assess longitudinal contractility remained abnormal at 1 year post-HT. These findings contribute to our understanding of RV myocardial contractility after HT in children and young adults and improve our ability to assess function quantitatively in this population.

Three-dimensional transesophageal echocardiography-guided transcatheter closure of multiple mitral paravalvular leaks demonstrating real time avoidance of device-induced valve malfunction.

Transcatheter closure of mitral paravalvular leaks has become an accepted alternative to surgical repair, especially in patients at high risk for reoperation. Device placement using three-dimensional (3D) transesophageal echocardiography (TEE) guidance allows en-face views of the valve and provides detailed information for device sizing and placement. We present a case demonstrating hybrid transapical transcatheter 3D TEE-guided closure of two mitral paravalvular leaks. We demonstrate real time 3D TEE guidance for device sizing and placement, and early real time recognition of mechanical mitral valve leaflet impairment during device deployment.

Early assessment of right ventricular systolic function after pediatric heart transplant.

RV systolic function is important early after HT; however, it has not been critically assessed in children using quantitative measures. The aim of this study was to describe the most validated and commonly used quantitative echocardiographic measures of RV systolic function early after pediatric HT and to assess associations with qualitative function evaluation and clinical factors. RV systolic function was quantified on the first post-HT echocardiogram >24 hours after cardiopulmonary bypass using two-dimensional TAPSE, Tricuspid annular S', FAC, and MPI. In 145 patients (median age 7.6 years), quantitative RV systolic function was markedly abnormal: mean TAPSE z-score -8.43 ± 1.89; S' z-score -4.36 ± 1.22; FAC 24.4 ± 8.34%; and MPI 0.86 ± 0.51. Few patients had normal quantitative function: TAPSE (0%), S' (1.2%), FAC (9.4%), and MPI (28.4%). In contrast, 48.3% were observed as normal by qualitative assessment. Most clinical factors, including diagnosis, pulmonary vascular resistance, posttransplant hemodynamics, inotropic support, and rejection, were not associated with RV function. In this large pediatric HT population, TAPSE, S', FAC, and MPI were strikingly abnormal early post-HT despite reassuring qualitative assessment and no significant association with clinical factors. This suggests that the accepted normal values of these quantitative measures may not apply in the early post-HT period to accurately grade RV systolic function, and there may be utility in adapting a concept of normal reference values after pediatric HT.