Congenital heart disease (CHD) is the most common congenital anomaly and a major cause of neonatal morbidity and mortality worldwide. A prenatal diagnosis of CHD is a psychologically distressing event that requires sensi...Congenital heart disease (CHD) is the most common congenital anomaly and a major cause of neonatal morbidity and mortality worldwide. A prenatal diagnosis of CHD is a psychologically distressing event that requires sensitive and effective counseling. However, no validated tool exists in Brazilian Portuguese to assess the quality of counseling provided to families following such diagnosis. To conduct the cross-cultural adaptation and content validation of "Kovacevic Questionnaire" for evaluating parental counseling after prenatal CHD diagnosis into Brazilian Portuguese. This cross-sectional study followed the standardized guidelines for cultural adaptation proposed by Beaton et al. The process included translation, synthesis, back-translation, expert review, pre-testing, and psychometric evaluation. Two Expert Committees assessed semantic, idiomatic, cultural, and conceptual equivalence. Content Validity Index (CVI), Prevalence- and Bias-Adjusted Kappa (PABAK), and Content Validity Coefficient (CVC) were calculated. The adapted instrument was pre-tested with ten pregnant women carrying fetuses diagnosed with CHD. The adaptation process resulted in a culturally relevant and linguistically accurate Brazilian version. Expert agreement was high, with most items achieving CVI and PABAK values close to 1.0 and CVC values ≥ 0.80. Minor linguistic refinements were made for clarity and cultural sensitivity. Pre-test participants rated all items as clear and relevant, with CVI and PABAK equal to 1.00. The Brazilian Portuguese version of "Kovacevic Questionnaire" demonstrated high validity and acceptability. It provides clinicians and researchers with a reliable tool to assess and enhance counseling quality for families facing prenatal CHD diagnoses, particularly in diverse and resource-constrained settings such as the Amazon region.
Raghuveer G, Coffee M, Dahdah N
… +23 more, Harahsheh AS, Dionne A, Portman MA, Nowlen TT, Pagano JJ, Gunsaulus M, Wehrmann M, Tierney S, Fabi M, Lee S, Szmuszkovicz J, Lang SM, Thacker D, Elias MD, Burns JC, Yamazaki-Nakashimada MA, C K, Harris, Jain SS, Butris N, Manlhiot C, McCrindle BW, International Kawasaki Disease Registry (IKDR)
We sought to determine if the COVID-19 pandemic was associated with changes in Kawasaki disease (KD) phenotype and cardiac manifestations. Patients hospitalized with acute KD and enrolled into the International KD Regist...We sought to determine if the COVID-19 pandemic was associated with changes in Kawasaki disease (KD) phenotype and cardiac manifestations. Patients hospitalized with acute KD and enrolled into the International KD Registry were categorized into time periods based on admission date: during the pandemic (January 1, 2020 - September 30, 2022, 33 months) and after the pandemic (October 1, 2022 - September 30, 2025, 36 months). Only patients with verified KD diagnoses as per American Heart Association criteria with no evidence of preceding COVID-19 exposure were included. Demographics, clinical features, management, and cardiac manifestations were compared between time periods. From across 45 sites, 726 during pandemic and 813 after pandemic KD patients were included. During pandemic patients were younger (median 2.6 vs. 3.3 years; p < 0.001), more commonly had incomplete KD (15 vs. 9%; p < 0.001), were less likely to have cough (34 vs. 43%; p < 0.001) and sore throat (15 vs. 22%; p < 0.001), and there were no significant differences in immunomodulatory treatments received. Left ventricular ejection fraction was normal and maximal coronary artery Z scores (median, interquartile range 25-75%, 1.38 [0.77, 2.30] vs. 1.41[0.42, 2.37]; p = 0.08) were similar, including coronary artery aneurysm Z score categories. During the pandemic, KD patients were younger, more likely to present as incomplete KD, and less likely to have respiratory symptoms, with no differences in immunomodulatory treatments received. Cardiac manifestations were however similar. These findings suggest that the COVID-19 pandemic had minimal impact on KD phenotype especially cardiac manifestations.
Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging shows myocardial fibrosis and is associated with outcome in repaired Tetralogy of Fallot (rToF). However, patients with rToF have surgical m...Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging shows myocardial fibrosis and is associated with outcome in repaired Tetralogy of Fallot (rToF). However, patients with rToF have surgical material implanted, also appearing bright in LGE images. Therefore, right ventricular myocardial fibrosis could be overestimated or falsely diagnosed, which may affect prognostic value and serial examinations. The aim of this study was to assess right ventricular LGE in patients with rToF and to compare this with placement of surgical material. This retrospective cohort study included patients with rToF operated in Lund between 1981–2022 in whom CMR with LGE had been performed. The LGE images were scored by two blinded observers and matched against patient records for surgical material placement. Ventricular volumes were measured in cine CMR images. Fifty-four patients with rToF (median age 18 [13–22] years) with LGE imaging 17 [12–21] years after repair were analyzed. Increased signal intensity in LGE images was only found at the site of surgical material or surgical incision and in right ventricular insertion points. There was no progression of LGE in patients with serial examinations. Knowing the surgical history is vital to avoid misinterpretation of LGE images since presence of surgical material may lead to overestimation or false diagnosis of right ventricular myocardial fibrosis. Serial LGE examinations could likely be avoided in most patients with rToF.
Ventriculoarterial coupling (VAC), the ratio of arterial elastance (Ea) to LV end-systolic elastance (Ees), measures the relationship between ventricular performance and arterial stiffness. We hypothesized that VAC is ab...Ventriculoarterial coupling (VAC), the ratio of arterial elastance (Ea) to LV end-systolic elastance (Ees), measures the relationship between ventricular performance and arterial stiffness. We hypothesized that VAC is abnormal in muscular dystrophy (MD) patients and worsens with disease progression. MD patients < 21 years were included. Demographics, MD phenotype, medications, medical history, and clinical status were collected. Initial and last follow up echocardiograms were reviewed to determine Ea, Ees, and VAC and compared between MD and age-matched males and between the initial and last follow up studies in the MD patients. Univariable and multivariable regression analyses was performed to determine factors independently associated with VAC. Forty-six MD patients, median age at diagnosis 9.8 years (IQR 7 -12.2), followed for a median of 4.1 years (IQR 3-6) were included for study. 41% patients were wheelchair-bound, 67% were on steroids, and 65% on afterload reducing medications. Ejection Fraction (EF) < 55% was found in 28% of patients at diagnosis and in 44% at the last follow-up. EF and Ees were significantly lower and VAC ratio was significantly higher (worse) in the MD patients compared to controls & showed significant worsening from diagnosis to last follow up. Ambulation status was independently associated with EF and Ees at last follow up. Cardiac performance, as measured by VAC ratio, is impaired in patients with MD compared with a control population and worsens with longitudinal follow-up. Ambulation status is independently associated with this impairment, offering mechanistic insight into the development and progression of cardiac dysfunction.
Electrocardiogram (ECG) values vary significantly across age and sex, particularly during childhood and adolescence. While age- and sex-specific ECG standards exist, they often fail to capture complex multi-dimensional r...Electrocardiogram (ECG) values vary significantly across age and sex, particularly during childhood and adolescence. While age- and sex-specific ECG standards exist, they often fail to capture complex multi-dimensional relationships and have not been applied in machine learning (ML) enhanced ECG analysis. Accuracy of automated ECG analysis in clinical practice improved significantly by applying ML models, however there is a paucity of such studies in the pediatric population. Our aim was to develop age- and sex-classification for children using ECG features with various ML models. We analyzed 29,408 curated resting 12-lead ECGs from healthy subjects aged 0–21 years using 177 digitized ECG variables combined with various ML models including regression and classification analyses and semi-supervised neural networks. Primary outcome variables were age and sex. Model performance was evaluated using F1-score, AUROC, and confusion matrices across repeated train-test splits. Support vector machine (SVM) achieved the highest accuracy in modeling both age and sex. Key predictive features included heart rate, PR interval, QRS duration, and T-wave amplitude. Age-group classification achieved an average true positive rate of 60% with SVM, improving to 94% when allowing one-group misclassification. Sex classification reached F1-scores of 0.91 and AUROC of 0.95 in adolescents and young adults, and moderate accuracy in younger children. Traditional supervised ML models can accurately model physiologic ECG changes related to age and sex, outperforming neural networks, particularly in smaller subgroups. These findings support the feasibility of ML models to capture of age- and sex-related ECG signatures to may aid future research and clinical applications in pediatric cardiology.
Tricuspid regurgitation (TR) is common in single right ventricle (RV) anomalies and affects outcomes during staged surgical palliations. The Pediatric Heart Network's (PHN) Single Ventricle Reconstruction trial followed...Tricuspid regurgitation (TR) is common in single right ventricle (RV) anomalies and affects outcomes during staged surgical palliations. The Pediatric Heart Network's (PHN) Single Ventricle Reconstruction trial followed children with single RV to age 6 years. This study compared serial echocardiographic measures of TR during staged palliations and determine the impact of TR and tricuspid valve (TV) intervention on transplant-free survival in the PHN cohort. Study echocardiograms were performed at 6 pre-determined times: baseline, post-Norwood, pre-stage II, age 14 months, pre-Fontan, and age 6 years. TR was categorized as ≤ mild or > mild. McNemar's test compared TR across time; t-tests compared RV size/function by TR category; and survival analysis assessed the effect of > mild TR on transplant-free survival. TR was assessed in 543 participants prior to Norwood. From baseline to post-Norwood, those with > mild TR increased from 12 to 24% (p < 0.001). Thereafter, the proportion of surviving participants with > mild TR was stable. Participants with > mild TR had increased baseline TV annular area, and increased RV volume but not decreased function. Transplant-free survival at 6 years was lower with > mild TR at any time except pre-Fontan. 78 TV interventions occurred in 66 participants. Those with TV intervention at the Norwood and/or Stage II had a higher rate of death/transplant than those without or those who had a later intervention. At baseline, post Norwood and pre-stage II, > mild TR is associated with decreased transplant-free survival. TV intervention at the Norwood or Stage II operations was not associated with improved survival.Clinical Trial Registration: NCT00115934.
Material hardship is the degree to which families experience housing, food, energy and transportation insecurity. It has been shown to affect various pediatric outcomes. However, it is unclear what role it plays in outco...Material hardship is the degree to which families experience housing, food, energy and transportation insecurity. It has been shown to affect various pediatric outcomes. However, it is unclear what role it plays in outcomes in children with congenital heart disease (CHD). To examine the association between material hardship and mortality and healthcare utilization among infants with CHD starting at prenatal diagnosis. We enrolled pregnant patients at the time of prenatal diagnosis of fetal CHD. We collected level of material hardship and sociodemographic factors using validated surveys at the time of prenatal diagnosis and when the infant was 6 months of age. We assessed outcomes at birth hospitalization and during the first six months of life. The cohort include 58 families with a diverse racial and socioeconomic make-up. There was an overall mortality rate of 8.6% in the first 6 months. At baseline 36.1% of the families had at least one type of material hardship compared to 27.6% at 6 months. Families with income at < 200% of the federal poverty line were 27.6% baseline compared to 20.7% at 6 months. Income < 200% of the federal (financial insecurity) at baseline was associated with increased mortality (p = 0.05). There was an increased length of stay those with housing insecurity (median LOS 28 vs. 16 days, p = 0.032) and in those with any type of material hardship (median LOS 28 vs. 16 days, p = 0.047). Increased levels of material hardship and financial insecurity were associated with increased mortality and length of stay in infants with CHD. Further research is needed to assesses the benefit of interventions designed to identify and address these gaps early since they may reduce morbidity and healthcare utilization. Keywords: Material Hardship, Health Equity, Prenatal Diagnosis.
Percutaneous balloon aortic valvuloplasty (PBAV) remains the initial preferred intervention for congenital aortic stenosis (CAS). Data regarding outcomes of PBAV in infancy are lacking. A retrospective review from 50 chi...Percutaneous balloon aortic valvuloplasty (PBAV) remains the initial preferred intervention for congenital aortic stenosis (CAS). Data regarding outcomes of PBAV in infancy are lacking. A retrospective review from 50 children’s hospitals in the United States participating in Pediatric Health Information System (PHIS) database for neonates (≤ 28 days) and infants (≤ 1 year of age) who underwent PBAV for CAS from 2016 to 2024. Critical AS was defined by prostaglandin E1 (PGE1) use. The primary endpoints were in-hospital mortality and repeat intervention within one year. Immediate procedural success was defined as survival to discharge without post-procedural ECMO, cardiac arrest, and rescue surgery/repeat PBAV during index hospitalization or within 1 month of initial PBAV. We identified 985 patients (525 neonates and 460 infants). Of them, 398 patients (40%) had critical AS. Compared to non-critical AS, the critical AS group had higher postprocedural complications including cardiac arrest, cardiac tamponade, and vascular complications. Procedural success was lower in the critical AS group (85% vs. 96%, p < 0.01), and in-hospital mortality was higher (11.8% vs. 1.7%, p < 0.01). Among the non-critical AS group, procedural success was similar between neonates and infants (96% vs. 95%, p = 0.6). Mortality and complications were comparable. Kaplan-Meier analysis showed freedom from re-intervention of 94.7% at 1 year. A multivariable analysis demonstrated critical AS, genetic syndrome, ECMO, tamponade and cardiac arrest were predictors of mortality. PBAV in infancy demonstrates high immediate procedural success and one year durability in those with non-critical AS. In contrast, patients with critical AS experience substantial mortality, morbidity and resource utilization.
Neonates with Ebstein anomaly or severe tricuspid valve dysplasia represent a high-risk patient cohort. The modified Starnes procedure was historically performed for cases incompatible with biventricular (BV) repair, lea...Neonates with Ebstein anomaly or severe tricuspid valve dysplasia represent a high-risk patient cohort. The modified Starnes procedure was historically performed for cases incompatible with biventricular (BV) repair, leading them towards single-ventricle palliation. However, in recent years, a paradigm shift has emerged in which the Starnes procedure in the acute setting can provide a means for right ventricular (RV) rehabilitation, by providing relief of RV volume overload and time for recovery of RV function, thus serving as a bridge to subsequent one and a half ventricle or BV repair. Herein, we present two cases at our institution of successful transition to BV repair after the Starnes procedure, despite an initially failed index BV repair as a neonate. Both patients presented with severe tricuspid valve dysplasia. The first patient underwent Starnes procedure at 10 days of life and takedown at 4 years of age after bilateral cavopulmonary shunt (BCPS) failure from recurrent bilateral pulmonary artery stenoses. The 2nd patient received Starnes at 4 months of age and successfully underwent takedown at 18mo after being deemed an unsuitable candidate for BCPS. Improvement in RV function was noted throughout the Starnes course. By providing our center's experience in the context of other case reports and series of BV conversion post-Starnes procedure, summarizing clinical and surgical considerations advocated in recent guidelines, and highlighting the topical areas of management that remain to be elucidated, we hope this discussion can lead to appropriate adoption of this new treatment strategy.
To synthesize and critically appraise applications of machine learning (ML) in pediatric cardiac intensive care, focusing on algorithm performance, validation rigor, and readiness for clinical decision-support integratio...To synthesize and critically appraise applications of machine learning (ML) in pediatric cardiac intensive care, focusing on algorithm performance, validation rigor, and readiness for clinical decision-support integration. Scoping review of studies applying ML to congenital heart disease (CHD) or pediatric cardiac intensive care unit (CICU) populations. Setting: PubMed and PubMed Central, 2015-2025. Patients: Neonates, infants, and children admitted to the CICU or undergoing CHD surgery; pediatric ICU cohorts with CICU-relevant outcomes. Twenty-five studies met inclusion criteria, encompassing > 90,000 pediatric encounters. Endpoints included mortality, cardiac arrest, low cardiac output syndrome (LCOS), acute kidney injury (AKI), and postoperative complications. Tree-based ensembles and gradient boosting algorithms (XGBoost, LightGBM, Random Forest) achieved AUROC of 0.83-0.97 for the prediction of target outcomes, outperforming traditional risk scores. Deep-learning models using sequential electronic health records (EHR) or physiologic data reached similar accuracy. Calibration was reported in fewer than one-third of studies; external validation occurred in only four (Lee et al. in NPJ Digit Med, 6(1):215, 2023; Zeng et al. in J Am Med Inform Assoc JAMIA, 30(1):94-102, 2022; Zürn et al. in Interdiscip Cardiovasc Thorac Surg, 37(3):ivad089, 2023; Winter et al. in Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc, 26(8):e997-e1008, 2025). Explainability tools such as SHAP and SurvSHAP(t) improved interpretability. Continuous waveform and NIRS data remain underused. Only one quasi-experimental implementation linked predictive analytics with lower arrest incidence, though without randomization or calibration assessment. In addition, platform-based bedside analytics, most prominently the Etiometry platform, have generated multicenter validation and implementation studies evaluating near real-time physiologic risk indices associated with outcomes such as lactate elevation, acidemia, venous saturation surrogates, extubation failure, and postoperative complications. Pediatric CICU ML models exhibit high discriminative power but limited calibration, validation, and deployment evidence. Translation to safe bedside use will require multicenter waveform-rich repositories, standardized calibration reporting, interpretable model design, and prospective pragmatic trials demonstrating clinical benefit.
Duchenne muscular dystrophy–associated cardiomyopathy (DMDAC) is a leading cause of mortality in patients with Duchenne muscular dystrophy. Conventional reliance on ejection fraction (EF) may underestimate early myocardi...Duchenne muscular dystrophy–associated cardiomyopathy (DMDAC) is a leading cause of mortality in patients with Duchenne muscular dystrophy. Conventional reliance on ejection fraction (EF) may underestimate early myocardial dysfunction. This study utilized the previously published dataset by Ajiboye et al. (Heart Vessels 40(8):696–706, 2025) comprising 30 DMDAC patients who underwent serial cardiac magnetic resonance (CMR) imaging between 2014 and 2023. Noninvasive indices—mean elastance, contractility, and energy efficiency—were derived using an innovative optimization approach by integrating patients specific brachial blood pressure and CMR-based left-ventricular volume changes. Diagnostic performance of the proposed indices relative to EF < 55% was obtained using receiver operating characteristic (ROC) curve to determing the cut-off values. All three indices, derived noninvasively, demonstrated statistically significant delineation for 55% EF, with AUC of 0.92 for mean elastance, 0.96 for contractility, and 1.00 for energy efficiency. Optimal cut-offs were 0.50 mmHg/mL, 1.72 mmHg/mL, and 69.8%, respectively. These findings support newly developed mean elastance, contractility, and energy efficiency as viable noninvasive diagnostic indices for DMDAC. Incorporating these indices alongside EF could improve early detection and risk stratification in clinical decision making.
Patients with dextro-transposition (D-TGA) of the great arteries and Senning or Mustard baffles have risk of sinus node dysfunction as well as ventricular arrhythmias. We demonstrate the implantation of a dual-chamber le...Patients with dextro-transposition (D-TGA) of the great arteries and Senning or Mustard baffles have risk of sinus node dysfunction as well as ventricular arrhythmias. We demonstrate the implantation of a dual-chamber leadless pacemaker in a patient with a Senning baffle and symptomatic bradycardia, in the setting of a primary prevention subcutaneous internal cardioverter defibrillator (SQICD). A retrospective review of a case of dual chamber leadless pacemaker (Aveir DR) implant was performed including atrial baffle and left ventricular septal implant. A 41-year-old female with history of D-TGA and prior defibrillator, post-lead extraction and SQICD placement, after lead malfunction, presented with symptomatic bradycardia. She underwent a dual-chamber leadless pacemaker implant under echocardiographic guidance without complication. At 6-month follow-up, the atrial and ventricular thresholds were 0.5 V@0.2ms and 0.75 V@0.2ms, with impedances of 350 ohms, and 730 ohms, respectively, with an R-wave of 17.3milliVolts. With 99% atrial pacing, and 15% ventricular pacing, the predicted longevity was 13.0 and 18.3 years for atrial and ventricular devices, respectively. No oversensing was noted on the subcutaneous ICD. The patient noted improved activity tolerance and no further presyncope. Successful implantation of a dual-chamber leadless pacemaker can be achieved in the LV septum of a patient with a Senning baffle and previously installed SICD.
Kinesiophobia-an excessive, often debilitating fear of movement or exercise-has emerged as an important moderator of physical activity (PA) and has been linked with quality of life (QoL), anxiety, and depression in adole...Kinesiophobia-an excessive, often debilitating fear of movement or exercise-has emerged as an important moderator of physical activity (PA) and has been linked with quality of life (QoL), anxiety, and depression in adolescents with heart disease (HD). This study explores additional factors that may be related to cardiac-focused kinesiophobia, including self-efficacy, PA engagement, and QoL. Sixty-three adolescents (mean age = 15.5 years; 49% female) with congenital or acquired HD completed the TSK-Heart-A, the Physical Activity Questionnaire for Adolescents (PAQ-A), the Pediatric Quality of Life Inventory (PedsQL™) Generic Core and Cardiac Module, and the Domain-Specific Physical Activity Efficacy Questionnaire (DSPAEQ-A). Data were analyzed using Kendall's Tau correlations and bootstrapped partial correlations controlling for PA. Greater kinesiophobia correlated with lower PA across both PA measures (τ = - 0.248), lower PA self-efficacy across household, leisure-time, and ambulatory domains (τ = - 0.293 to - 0.356), and poorer QoL for both generic and cardiac scales (τ = - 0.403, - 0.400). When controlling for PA, kinesiophobia remained significantly related to lower self-efficacy (r = - 0.314 to - 0.368) and poorer QoL (r = - 0.558, - 0.520). Cardiac-focused kinesiophobia is a salient psychological factor in pediatric HD and is tied to lower self-reported PA, poorer QoL and is independently associated with reduced PA self-efficacy-a modifiable resilience construct. These findings support the development of interventions that target fear-avoidance mechanisms and build self-efficacy to improve everyday activity and psychosocial functioning in adolescent HD.
We retrospectively reviewed 895 patients with Kawasaki disease treated at two Japanese pediatric centers between 2015 and 2024 to assess whether N-terminal pro-brain natriuretic peptide (NT-pro BNP) levels at diagnosis c...We retrospectively reviewed 895 patients with Kawasaki disease treated at two Japanese pediatric centers between 2015 and 2024 to assess whether N-terminal pro-brain natriuretic peptide (NT-pro BNP) levels at diagnosis can predict the need for infliximab (IFX) therapy. Patients were divided into (1) the IFX group (n = 35), including patients who received IFX as third-line therapy due to resistance to first- and second-line treatments, including intravenous immunoglobulin, and (2) the non-IFX group (n = 860), including patients who responded to initial therapies. Clinical and laboratory variables were compared between the groups, and predictors of IFX use were analyzed using multivariate logistic regression and receiver operating characteristic (ROC) curves. Multivariate analysis comparing the IFX and non-IFX groups identified older age, lower hemoglobin levels, and higher NT-pro BNP Z-score at diagnosis as independent predictors of later IFX requirement. Additionally, the incidence of coronary arterial lesions did not differ significantly between the groups. ROC analysis demonstrated NT-pro BNP Z-score as a significant predictor (AUC 0.66, p < 0.001), with a cutoff of 2.2 yielding 71.4% sensitivity, 63.3% specificity, 7.3% positive predictive value, and 98.2% negative predictive value. Elevated serum NT-pro BNP Z-score at diagnosis was associated with later IFX use, whereas a low Z-score reliably identified patients unlikely to require IFX. Early assessment incorporating NT-pro BNP may optimize Kawasaki disease therapy and guide the appropriate timing of IFX administration.
Prenatal prediction of critical coarctation of the aorta remains challenging. Fetal echocardiography in isolated persistent left-sided superior vena cava (IP L-SVC), a benign variant, can mimic suspected coarctation with...Prenatal prediction of critical coarctation of the aorta remains challenging. Fetal echocardiography in isolated persistent left-sided superior vena cava (IP L-SVC), a benign variant, can mimic suspected coarctation with shared right-to-left ventricular size discrepancy. We sought to describe cardiac deformation in fetuses with IP L-SVC and compare to those with suspected coarctation. We hypothesized that cardiac deformation as measured by global longitudinal strain (GLS) could differentiate fetuses with coarctation from IP L-SVC. This is a single-center, retrospective study of fetuses with IP L-SVC and suspected coarctation. Fetal cardiac deformation analysis was performed using a vendor non-specific software. The primary variable was GLS. Secondary variables were left ventricular ejection fraction, left ventricular end-systolic volume, and left ventricular end-diastolic volume (LVEDV). Mean LVEDV z-score was determined. Interrater reliability was performed. Baseline measures of GLS were performed using a cohort of normal fetuses. Ninety fetuses were analyzed (IP L-SVC n=28, suspected coarctation n=30, normal n=32). Seventeen required surgical repair for coarctation. Fetuses with IP L-SVC and suspected coarctation had lower GLS (p <0.001) and smaller left ventricular size compared to normal (p<0.001). Fetuses who required surgical repair for coarctation of the aorta had the lowest LVEDV. Interrater reliability was poor to moderate. Our study is the first to date that examines strain in fetuses with IP L-SVC compared to suspected coarctation of the aorta. Cardiac deformation in fetuses with IP L-SVC was indistinguishable from fetuses with suspected coarctation of the aorta. GLS proved a poor predictor of postnatal surgical repair in isolation.
Kiener AJ, Croft J, Harris KN
… +7 more, Banerjee A, Sanchez AA, Wehrens XHT, Parthiban A, Gao X, Doan TT, Nguyen MB
Pediatr Cardiol
· 2026 Feb · PMID 41670640
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BACKGROUND: Left atrial (LA) volume and function are important for assessing pediatric diastolic dysfunction (DD). Three-dimensional echocardiographic (3DE) LA assessment may overcome limitations and assumptions inherent...BACKGROUND: Left atrial (LA) volume and function are important for assessing pediatric diastolic dysfunction (DD). Three-dimensional echocardiographic (3DE) LA assessment may overcome limitations and assumptions inherent in two-dimensional echocardiographic (2DE). METHODS: We conducted a cross-sectional study of children under 18 years of age with structurally normal hearts, using transthoracic 3DE apical volume datasets. LA volumetric function and phasic deformation analyses were performed using 3DE (GE, EchoPAC 4DLAQ v206, near fully-automated) and 2DE (TomTec, TTA2.51, CPA, manual) was performed by three operators and processing time was collected. Agreement and reproducibility was assessment for each modality. RESULTS: Fifty patients (median age 12.8 years) were included. Processing time for 3DE was shorter than for 2DE (0.6 vs. 2.7 min, p < 0.001). 3DE and 2DE showed good agreement for maximum LA volume (LAV, ICC = 0.79), with 3DE estimates being lower on average by 5.9 mL than 2DE. Agreement for 3DE LA reservoir strain (LAS) was weak (ICC = 0.17), with 3DE values lower by an average of 16.6%. Interobserver agreement for 3DE LAV was excellent (ICC = 0.94, 95% CI 0.90-0.96) and comparable to 2DE (ICC = 0.86, 95% CI 0.63-0.94, p = 0.16). Intra-observer reliability for LA volume was similar between 2DE to 3DE (p = 0.11-0.85). No meaningful differences were found in inter-/intra-observer reliability for LA strain between modalities. CONCLUSION: 3DE provides excellent interobserver reproducibility for LA volume assessment and faster processing times compared to 2DE. 3DE and 2DE show good agreement for LA volume. Agreement for LA strain is weak. Further studies are needed to determine which better predicts clinical outcomes.
Peripheral perfusion index (PPI) represents the ratio of pulsatile to non-pulsatile blood flow in an extremity and may be a valuable supplement to pulse oximetry in identification of critical congenital heart disease (CC...Peripheral perfusion index (PPI) represents the ratio of pulsatile to non-pulsatile blood flow in an extremity and may be a valuable supplement to pulse oximetry in identification of critical congenital heart disease (CCHD). To date, reference ranges of PPI of healthy neonates have not been published in the United States. We performed an observational retrospective study evaluating the PPI of healthy neonates between 24 and 48 h of life born in Minnesota, United States based on electronic reporting of CCHD screening to the Minnesota Department of Health. We evaluated preductal and postductal PPI measured at 24-48 h of age in 11,179 healthy infants born between 35- and 41-weeks gestation. We found that preductal PPI is significantly greater than postductal PPI and that PPI increases with gestational age and with birth weight. There was no difference in PPI by sex when adjusting for birth weight. Smoothed percentile curves of preductal and postductal PPI by gestational age were derived using the GAMLSS R Package with a Sinh-Arcsinh distribution. Our study is the first large, population-based study to establish normative data for PPI in healthy neonates in the United States. Our data overall replicate trends observed in large studies from India and China. Further research is needed to determine feasibility and utility of incorporation of PPI into CCHD screening.
Rhabdomyomas are relatively rare, cardiac tumors that are predominantly seen in infancy. Involvement of the mitral valve is considered exceptionally uncommon. We retrospectively reviewed three cases of mitral valve rhabd...Rhabdomyomas are relatively rare, cardiac tumors that are predominantly seen in infancy. Involvement of the mitral valve is considered exceptionally uncommon. We retrospectively reviewed three cases of mitral valve rhabdomyomas in patients with tuberous sclerosis complex (TSC) at a single institution, focusing on clinical presentation, imaging, and management. All patients presented with low cardiac output and mitral inflow obstruction, resulting in compromised circulatory dynamics. Echocardiography revealed mobile, hyperechoic masses on the mitral leaflets. Despite moments of hemodynamic concerns and fluctuating clinical status, all patients were managed medically with antiarrhythmics, supportive care, and one patient received sirolimus. Tumor regression and symptom improvement were observed with time, indicating a positive long-term prognosis. Neonatal mitral valve rhabdomyomas, though rare, have improved with medical therapy in these cases and surgery was avoided. In select cases, mTOR inhibitors, medical management, and echocardiographic surveillance may offer a safe alternative to surgery.