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Many factors affect patient outcome after congenital heart surgery, including the complexity of the heart disease, pre-operative status, patient specific factors (prematurity, nutritional status and/or presence of comorbid conditions or genetic syndromes), and post-operative residual lesions. The Residual Lesion Score is a novel tool for assessing whether specific residual cardiac lesions after surgery have a measurable impact on outcome. The goal is to understand which residual lesions can be tolerated and which should be addressed prior to leaving the operating room. The Residual Lesion Score study is a large multicentre prospective study designed to evaluate the association of Residual Lesion Score to outcomes in infants undergoing surgery for CHD. This Pediatric Heart Network and National Heart, Lung, and Blood Institute-funded study prospectively enrolled 1,149 infants undergoing 5 different congenital cardiac surgical repairs at 17 surgical centres. Given the contribution of echocardiographic measurements in assigning the Residual Lesion Score, the Residual Lesion Score study made use of a centralised core lab in addition to site review of all data. The data collection plan was designed with the added goal of collecting image quality information in a way that would permit us to improve our understanding of the reproducibility, variability, and feasibility of the echocardiographic measurements being made. There were significant challenges along the way, including the coordination, de-identification, storage, and interpretation of very large quantities of imaging data. This necessitated the development of new infrastructure and technology, as well as use of novel statistical methods. The study was successfully completed, but the size and complexity of the population being studied and the data being extracted required more technologic and human resources than expected which impacted the length and cost of conducting the study. This paper outlines the process of designing and executing this complex protocol, some of the barriers to implementation and lessons to be considered in the design of future studies.
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Ecocardiografia , Coração , Lactente , Humanos , Criança , Estudos Prospectivos , Reprodutibilidade dos Testes , Coleta de DadosRESUMO
BACKGROUND: Three-dimensional (3D) speckle tracking echocardiography (STE) can overcome some of the inherent limitations of two-dimensional (2D) STE; however, clinical experience is lacking. We aimed to assess and compare the feasibility, agreement, and reproducibility of left ventricular (LV) global longitudinal (GLS), and regional strain by 3D vs 2D STE in normal children. METHODS: Healthy pediatric subjects (n = 105, age mean = 11.2 ± 5.5 years) were prospectively enrolled. Three-dimensional and 2D LV GLS, as well as regional strain in 16 myocardial segments were quantified. Bland Altman analysis, intra- class correlation coefficients (ICC), percent error and linear regression were used for agreement and correlation between the two techniques. Analysis and acquisition times were compared. Inter- and intra-observer reproducibility was assessed in 20 studies. RESULTS: There was good to excellent agreement for 2D and 3D global longitudinal strain (ICC =0.82) and modest agreement for regional strain (ICC range 0.43-0.71). Both methods had high feasibility (88.6% for 2D vs 85.7% for 3D, p = 0.21), although 3D STE required significantly shorter acquisition and analysis time than 2D STE (acquisition time 1 ± 1.2 mins vs 2.4 ± 1 mins; p = 0.03, analysis time = 3.3 ± 1 mins vs 8.2 ± 2.5 mins; p = 0.001, respectively). Inter and intra-observer reproducibility was excellent for GLS by the two techniques (ICC = 0.78-0.93) but moderate to poor for regional strain (ICC = 0.21-0.64). CONCLUSION: Three-dimensional global LV strain is as feasible and reproducible as 2D strain, with good agreement yet significantly more efficient acquisition and analysis. Regional strain is less concordant and 2D and 3D values should not be used interchangeably. 3D LV GLS may represent a viable alternative in evaluation of LV deformation in pediatric subjects.
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Ecocardiografia Tridimensional , Disfunção Ventricular Esquerda , Adolescente , Criança , Pré-Escolar , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Reprodutibilidade dos Testes , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular EsquerdaRESUMO
BACKGROUND: Three-dimensional echocardiography (3DE) evaluation of left ventricular (LV) volume and function in pediatrics compares favorably with cardiac magnetic resonance imaging. The aim of this study was to establish from a multicenter, normal pediatric z-score values of 3DE left ventricular volumes and function. METHODS: Six hundred and ninety-eight healthy children (ages 0-18 years) were recruited from five centers. LV 3DE was acquired from the 4-chamber view. A vendor-independent software analyzed end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), and ejection fraction (EF) using semi-automated quantification. Body surface area (BSA)-based z-scores were generated. Intraobserver and interobserver variability were calculated using intraclass correlation (ICC) and repeatability coefficient (RC). RESULTS: Z-scores were generated for ESV, EDV, and SV. The ICC for intraobserver variability for EDV, ESV, and SV was 0.99, 0.99, and 0.99, respectively. The ICC for interobserver variability for EDV, ESV, and SV was 0.98, 0.94, and 0.98, respectively. The RC for intraobserver and interobserver variability for LV EF was 4.39% (95% CI: 3.01, 5.59) and interobserver was 7.08% (95%CI: 5.51, 8.42). CONCLUSIONS: We report pediatric z-scores for normal LV volumes using the semi-automated method from five centers, enhancing its generalizability. 3DE evaluation of LV volumes and EF in pediatric patients is highly reproducible.
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Ecocardiografia Tridimensional , Pediatria , Adolescente , Criança , Pré-Escolar , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Reprodutibilidade dos Testes , Volume Sistólico , Função Ventricular EsquerdaRESUMO
Children and adolescents with cardiac disease (CCD) have significant morbidity and lower quality of life. However, there are no broadly applicable tools similar to the frailty score as described in the elderly, to define functional phenotype in terms of physical capability and psychosocial wellbeing in CCD. The purpose of this study is to investigate the domains of the frailty in CCD. We prospectively recruited CCD (8-17.5 years old, 70% single ventricle, 27% heart failure, 12% pulmonary hypertension; NYHA classes I, II and III) and age and gender matched healthy controls (total n = 56; CCD n = 34, controls n = 22; age 12.6 ± 2.6 years; 39.3% female). We measured the five domains of frailty: slowness, weakness, exhaustion, body composition and physical activity using developmentally appropriate methods. Age and gender-based population norms were used to obtain Z scores and percentiles for each measurement. Two-tailed t-tests were used to compare the two groups. The CCD group performed significantly worse in all five domains of frailty compared to healthy controls. Slowness: 6-min walk test with Z score -3.9 ± 1.3 vs -1.4 ± 1.3, p < 0.001; weakness: handgrip strength percentile 18.9 ± 20.9 vs 57.9 ± 26.0, p < 0.001; exhaustion: multidimensional fatigue scale percentile 63.7 ± 13.5 vs 83.3 ± 14.4, p < 0.001; body composition: height percentile 43.4 ± 29.5 vs 71.4 ± 25.2, p < 0.001, weight percentile 46.0 ± 36.0 vs 70.9 ± 24.3, p = 0.006, BMI percentile 48.4 ± 35.5 vs 66.9 ± 24.2, p = 0.04, triceps skinfold thickness 41.0 ± 24.0 vs 54.4 ± 22.1, p = 0.04; physical activity: pediatric activity questionnaire score 2 ± 0.6 vs 2.7 ± 0.6, p < 0.001. The domains of frailty can be quantified in children using developmentally appropriate methods. CCD differ significantly from controls in all five domains, supporting the concept of quantifying the domains of frailty. Larger longitudinal studies are needed to study frailty in CCD and examine if it predicts adverse health outcomes.Clinical Trial Registration: The ClinicalTrials.gov identification number is NCT02999438. https://clinicaltrials.gov/ct2/show/NCT02999438.
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Fragilidade/diagnóstico , Cardiopatias Congênitas/complicações , Adolescente , Estudos de Casos e Controles , Criança , Feminino , Fragilidade/etiologia , Fragilidade/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Fenótipo , Desempenho Físico Funcional , Estudos Prospectivos , Qualidade de VidaRESUMO
PURPOSE: To quantify the differences in daily physical activity (PA) patterns, intensity-specific volumes, and PA bouts in youth with and without heart disease (HD). METHODS: Seven-day PA was measured on children/adolescents with HD (n = 34; median age 12.4 y; 61.8% male; 70.6% single ventricle, 17.7% heart failure, and 11.8% pulmonary hypertension) and controls without HD (n = 22; median age 12.3 y; 59.1% male). Mean counts per minute were classified as sedentary, light, and moderate to vigorous PA (MVPA), and bouts of MVPA were calculated. PA was calculated separately for each hour of wear time from 8:00 to 22:00. Multilevel linear mixed modeling compared the outcomes, stratifying by group, time of day, and day part (presented as median percentage of valid wear time [interquartile range]). RESULTS: Compared with the controls, the HD group had more light PA (33.9% [15%] vs 29.6% [9.5%]), less MVPA (1.7% [2.5%] vs 3.2% [3.3%]), and more sporadic bouts (97.4% [5.7%] vs 89.9% [9.2%]), but fewer short (2.0% [3.9%] vs 7.1% [5.7%]) and medium-to-long bouts (0.0% [1.9%] vs 1.6% [4.6%]) of MVPA. The HD group was less active in the late afternoon, between 15:00 and 17:00 (P < .03). There were no differences between groups in sedentary time. CONCLUSION: Children/adolescents with HD exhibit differences in intensity-specific volumes, PA bouts, and daily PA patterns compared with controls.
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Exercício Físico , Cardiopatias , Acelerometria , Adolescente , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Comportamento SedentárioRESUMO
Technical Performance Score (TPS) is based largely on the presence and magnitude of residual lesions on postoperative echocardiograms; this score correlates with outcomes following repair of congenital heart defects. We evaluated reader variability for echocardiographic components of TPS for complete repair of tetralogy of Fallot (TOF) and arterial switch operation (ASO) in two centers and measured its effect on TPS. Postoperative echocardiograms were evaluated in 67 children (39 TOF and 28 ASO). Two readers (one per center) interpreted each echocardiogram. Reader variability in image quality assessments and measurements was compared using weighted kappa (κ), percent agreement, and intra-class correlation. TPS class (1 optimal-no residua, 2 adequate-minor residua, 3 inadequate-major residua) was assigned for each echocardiographic review by an independent investigator. The effect of reader interpretation variability on TPS classification was measured. There was strong agreement for TPS between the two readers (κ = 0.88). The readers were concordant for TPS classes for 57 children (85%) and discordant for classes 2 (minor residua) versus 3 (major residua) in six (9%). Coronary arteries and branch pulmonary arteries were frequently suboptimally visualized. Although inter-reader agreement for TPS was strong, inter-reader variation in echocardiographic interpretations had a small, but important effect on TPS for TOF and ASO, particularly for the distinction between minor and major residua. Further studies of generalizability and reproducibility of TPS and refinement of scoring modules may be needed before it can be used as a tool to assess pediatric cardiac surgical performance and outcomes.
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Procedimentos Cirúrgicos Cardíacos/métodos , Ecocardiografia/métodos , Cardiopatias Congênitas/cirurgia , Humanos , Projetos Piloto , Indicadores de Qualidade em Assistência à Saúde , Reprodutibilidade dos TestesRESUMO
Echocardiography is the primary modality for imaging the aorta for the diagnosis and serial evaluation of pathological conditions. In this article, we review the methodology for optimal echocardiographic imaging of the various segments of the aorta and discuss abnormalities of the aorta including stenosis, dilation including aortopathy and sinus of Valsalva aneurysms, and fistulous communications involving the ascending aorta including aortoventricular tunnel and ruptured sinus of Valsalva aneurysm. We review novel echocardiographic measurements of aortic functional properties of the aorta such as elasticity and stiffness, and review the literature on the potential additive value of such measurements for structural assessment alone. Finally, we discuss the limitations of echocardiography in the precise and optimal imaging of the aorta.
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Aneurisma Aórtico/diagnóstico por imagem , Ruptura Aórtica/diagnóstico por imagem , Estenose Aórtica Supravalvular/diagnóstico por imagem , Ecocardiografia , Seio Aórtico/diagnóstico por imagem , Dilatação Patológica , Humanos , Rigidez VascularRESUMO
Heart failure in children can result from a wide range of aetiologies and can manifest in systolic and/or diastolic dysfunction. Echocardiography is the primary test for the diagnosis and follow-up of children with heart failure. In this article, we critically review standard echocardiographic measurements that have been shown to have prognostic importance in children with various types of heart failure. Each of the common forms of cardiomyopathy that is encountered in childhood--dilated, hypertrophic, restrictive, left ventricular non-compaction, and arrhythmogenic right ventricular cardiomyopathy--is discussed separately. Special attention is paid to the failing right ventricle, both in the systemic and in the sub-pulmonary position, to the failing univentricular heart, and to the assessment of diastolic function in children.
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Cardiomiopatias/complicações , Cardiomiopatias/diagnóstico , Ecocardiografia/métodos , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Pediatria , Cardiomiopatias/classificação , Diástole/fisiologia , Humanos , Prognóstico , Sístole/fisiologia , Disfunção Ventricular Direita/diagnóstico por imagemRESUMO
Over the past decade, new echocardiographic techniques such as three-dimensional echocardiography and the imaging of myocardial deformation (strain) have been developed, and are increasingly used in clinical practice. In this article, we describe the rationale and methodology, review available guidelines for practice, and discuss the advantages and limitations of each of these modalities. When available, we have also summarised the scientific evidence for the clinical application of these techniques to detect heart failure in children.
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Ecocardiografia/instrumentação , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Pediatria , Ecocardiografia Tridimensional/métodos , Humanos , MiocárdioRESUMO
Recent years have witnessed exponential growth in cardiac imaging technologies, allowing better visualization of complex cardiac anatomy and improved assessment of physiology. These advances have become increasingly important as more complex surgical and catheter-based procedures are evolving to address the needs of a growing congenital heart disease population. This state-of-the-art review presents advances in echocardiography, cardiac magnetic resonance, cardiac computed tomography, invasive angiography, 3-dimensional modeling, and digital twin technology. The paper also highlights the integration of artificial intelligence with imaging technology. While some techniques are in their infancy and need further refinement, others have found their way into clinical workflow at well-resourced centers. Studies to evaluate the clinical value and cost-effectiveness of these techniques are needed. For techniques that enhance the value of care for congenital heart disease patients, resources will need to be allocated for education and training to promote widespread implementation.
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Inteligência Artificial , Cardiopatias Congênitas , Humanos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Ecocardiografia , Técnicas de Imagem Cardíaca/métodos , Imageamento por Ressonância Magnética/métodosRESUMO
BACKGROUND: Data on mitral annular disjunction (MAD) in children with Marfan syndrome (MFS) are sparse. OBJECTIVES: To investigate the diagnostic yield of MAD by echocardiography and cardiac magnetic resonance imaging (CMR), its prevalence and progression during childhood. METHODS: We included patients <21 years old with MFS, defined by 2010 Ghent criteria and a pathogenic FBN1 variant or ectopia lentis. Two readers measured systolic separation between the mitral valve (MV) posterior hinge point and left ventricular (LV) myocardium on initial and subsequent imaging. MAD was defined as MV-LV separation ≥2â mm, MV prolapse (MVP) as atrial displacement ≥2â mm. Kappa coefficients evaluated echocardiogram-CMR agreement. Bland-Altman and intraclass correlation coefficients (ICC) assessed interrater and intermodality reliability. Univariable mixed-effects linear regression was used to evaluate longitudinal changes of MAD. RESULTS: MAD was detected in 60% (110/185) eligible patients. MVP was present in 48% (53/110) of MAD and MAD in 90% (53/59) of MVP. MAD detection by CMR and echocardiography had 96% overall agreement (Kappa = 0.89, p < 0.001) and a 0.32-mm estimate bias (95%CI 0.00, 0.65). ICC by echocardiography, CMR, and between modalities were 0.97 (95%CI 0.93, 0.98), 0.92 (95%CI 0.79, 0.97), and 0.91 (95%CI 0.85, 0.94), respectively. MAD was associated with aortic root dilation (p < 0.001). MAD was found in children of all ages, increased +0.18â mm/year (95%CI +0.14, + 0.22) during a median duration of 5.5 years (IQR 3.1, 7.5 years). MAD indexed by height yielded a constant value +0.0002â mm/m/year (95%CI -0.0002, + 0.0005â mm/m/year). CONCLUSIONS: MAD was common in pediatric MFS and was associated with aortic root dilation. MAD detection by echocardiography and CMR was highly reliable, suggesting that routine assessment in MFS is feasible. MAD was present in neonates and progressed over time but remained constant when indexing by height. Further studies are needed to evaluate MAD as a biomarker for clinical outcomes in pediatric MFS.
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BACKGROUND: The complexity of congenital heart disease has been primarily stratified on the basis of surgical technical difficulty, specific diagnoses, and associated outcomes. We report on the refinement and validation of a pediatric echocardiography complexity (PEC) score. METHODS AND RESULTS: The American College of Cardiology Quality Network assembled a panel from 12 centers to refine a previously published PEC score developed in a single institution. The panel refined complexity categories and included study modifiers to account for complexity related to performance of the echocardiogram. Each center submitted data using the PEC scoring tool on 15 consecutive inpatient and outpatient echocardiograms. Univariate and multivariate analyses were performed to assess for independent predictors of longer study duration. Among the 174 echocardiograms analyzed, 68.9% had underlying congenital heart disease; 44.8% were outpatient; 34.5% were performed in an intensive care setting; 61.5% were follow-up; 46.6% were initial or preoperative; and 9.8% were sedated. All studies had an assigned PEC score. In univariate analysis, longer study duration was associated with several patient and study variables (age <2 years, PEC 4 or 5, initial study, preoperative study, junior or trainee scanner, and need for additional imaging). In multivariable analysis, a higher PEC score of 4 or 5 was independently associated with longer study duration after controlling for study variables and center variation. CONCLUSIONS: The PEC scoring tool is feasible and applicable in a variety of clinical settings and can be used for correlation with diagnostic errors, allocation of resources, and assessment of physician and sonographer effort in performing, interpreting, and training in pediatric echocardiography.
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Cardiopatias Congênitas , Melhoria de Qualidade , Criança , Humanos , Pré-Escolar , Consenso , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Erros de Diagnóstico , EcocardiografiaRESUMO
In the pediatric and congenital heart disease (CHD) population, tricuspid valve (TV) disorders are complex due to the variable TV morphology, its sophisticated interaction with the right ventricle as well as associated congenital and acquired lesions. While surgery is the standard of care for TV dysfunction in this patient population, transcatheter treatment for bioprosthetic TV dysfunction has been performed successfully. Detailed and accurate anatomic assessment of the abnormal TV is essential in the preoperative/preprocedural planning. Three-dimensional transthoracic and 3D transesophageal echocardiography (3DTEE) provides added value to 2-dimensional imaging in the characterization of the TV to guide therapy and 3DTEE serves as an excellent tool for intraoperative assessment and procedural guidance of transcatheter treatment. Notwithstanding advances in imaging and therapy, the timing and indication for intervention for TV disorders in this population are not well defined. In this manuscript, we aim to review the available literature, provide our institutional experience with 3DTEE, and briefly discuss the perceived challenges and future directions in the assessment, surgical planning, and procedural guidance of (1) congenital TV malformations, (2) acquired TV dysfunction from transvenous pacing leads, or following cardiac surgeries, and (3) bioprosthetic TV dysfunction.
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BACKGROUND: To promote the rational use of cardiovascular imaging in patients with congenital heart disease, the American College of Cardiology developed Appropriate Use Criteria (AUC), but its clinical application and pre-release benchmarks have not been evaluated. We aimed to evaluate the appropriateness of indications for cardiovascular magnetic resonance (CMR) and cardiovascular computed tomography (CCT) in patients with conotruncal defects and to identify factors associated with maybe or rarely appropriate (M/R) indications. METHODS: Twelve centers each contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. To incorporate patient characteristics and center-level effects, a hierarchical generalized linear mixed model was used. RESULTS: Of the 1753 studies (80% CMR, and 20% CCT), 16% were rated M/R. Center M/R ranged from 4 to 39%. Infants accounted for 8.4% of studies. In multivariable analyses, patient- and study-level factors associated with M/R rating included: age <1 year (OR 1.90 [1.15-3.13]), truncus arteriosus (vs. tetralogy of Fallot, OR 2.55 [1.5-4.35]), and CCT (vs. CMR, OR 2.67 [1.87-3.83]). None of the provider- or center-level factors reached statistical significance in the multivariable model. CONCLUSIONS: Most CMRs and CCTs ordered for the follow-up care of patients with conotruncal defects were rated appropriate. However, there was significant center-level variation in appropriateness ratings. Younger age, CCT, and truncus arteriosus were independently associated with higher odds of M/R rating. These findings could inform future quality improvement initiatives and further exploration of factors resulting in center-level variation.
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Cardiopatias Congênitas , Lactente , Humanos , Valor Preditivo dos Testes , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Tomografia Computadorizada por Raios X , Imageamento por Ressonância MagnéticaRESUMO
Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition in which a neonate is born with an underdeveloped left ventricle and associated structures. Without palliative interventions, HLHS is fatal. Treatment typically includes medical management at the time of birth to maintain patency of the ductus arteriosus, followed by three palliative procedures: most commonly the Norwood procedure, bidirectional cavopulmonary shunt, and Fontan procedures. With recent advances in surgical management of HLHS patients, high survival rates are now obtained at tertiary treatment centers, though adverse neurodevelopmental outcomes remain a clinical challenge. While surgical management remains the standard of care for HLHS patients, innovative treatment strategies continue to be developing. Important for the development of new strategies for HLHS patients is an understanding of the genetic basis of this condition. Another investigational strategy being developed for HLHS patients is the injection of stem cells within the myocardium of the right ventricle. Recent innovations in tissue engineering and regenerative medicine promise to provide important tools to both understand the underlying basis of HLHS as well as provide new therapeutic strategies. In this review article, we provide an overview of HLHS, starting with a historical description and progressing through a discussion of the genetics, surgical management, post-surgical outcomes, stem cell therapy, hemodynamics and tissue engineering approaches.
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BACKGROUND: Three-dimensional echocardiography (3DE) evaluation of right ventricular (RV) volumes and ejection fraction (EF) is increasingly used for clinical serial assessments and management in children. This study aims to generate sex-specific reference values and z-score equations for RV volumetric parameters, independent of age and body size indices, derived from multiple populations across North America. METHODS: We prospectively recruited 455 healthy children (ages 0 to 18 years) from 5 centres. 3DE of the RV were acquired using various vendors with analyses performed offline using vendor-independent software. 3DE datasets with all walls of the RV endocardium visible were included. We reported data on RV EF, and generated z scores for end-systolic volumes (ESV), end-diastolic volumes (EDV) and stroke volume (SV). Differences between the sexes were explored. RESULTS: Of 455 3DE datasets, 312 (68%) met imaging criteria for analysis. Median age was 10.1 years (interquartile ratio [IQR]: 5.6, 14.0) with 17% being younger than 3 years of age. The mean and standard deviation for RV EDV, ESV, and SV for male and female patients were reported. We provided a downloadable z-score calculator with height and weight as independent variables to facilitate clinical utility. Although statistically significant differences between male and female RVEF was present (female 52.9 ± 3.9% vs male 51.6 ± 3.5%, P = 0.006), after adjusting for age, height, and weight, the magnitude of difference was clinically insignificant. CONCLUSIONS: Sex-specific reference values for pediatric RV volumes and EF, and z-score equations were derived from children 3DE datasets across 5 centres in North America.
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Ecocardiografia Tridimensional , Adolescente , Criança , Pré-Escolar , Ecocardiografia Tridimensional/métodos , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Masculino , Valores de Referência , Reprodutibilidade dos Testes , Volume Sistólico , Função Ventricular EsquerdaRESUMO
BACKGROUND: The FUEL trial (Fontan Udenafil Exercise Longitudinal) demonstrated statistical improvements in exercise capacity following 6 months of treatment with udenafil (87.5 mg po BID). The effect of udenafil on echocardiographic measures of single ventricle function in this cohort has not been studied. METHODS: The 400 enrolled participants were randomized 1:1 to udenafil or placebo. Protocol echocardiograms were obtained at baseline and 26 weeks after initiation of udenafil/placebo. Linear regression compared change from baseline indices of single ventricle systolic, diastolic and global function, atrioventricular valve regurgitation, and mean Fontan fenestration gradient in the udenafil cohort versus placebo, controlling for ventricular morphology (left ventricle versus right ventricle/other) and baseline value. RESULTS: The udenafil participants (n=191) had significantly improved between baseline and 26 weeks visits compared to placebo participants (n=195) in myocardial performance index (P=0.03, adjusted mean difference [SE] of changes between groups -0.03[0.01]), atrioventricular valve inflow peak E (P=0.009, 3.95 [1.50]), and A velocities (P=0.034, 3.46 [1.62]), and annular Doppler tissue imaging-derived peak e' velocity (P=0.008, 0.60[0.23]). There were no significant differences in change in single ventricle size, systolic function, atrioventricular valve regurgitation severity, or mean fenestration gradient. Participants with a dominant left ventricle had significantly more favorable baseline values of indices of single ventricle size and function (lower volumes and areas, E/e' ratio, systolic:diastolic time and atrioventricular valve regurgitation, and higher annular s' and e' velocity). CONCLUSIONS: FUEL participants who received udenafil demonstrated a statistically significant improvement in some global and diastolic echo indices. Although small, the changes in diastolic function suggest improvement in pulmonary venous return and/or augmented ventricular compliance, which may help explain improved exercise performance in that cohort. REGISTRATION: URL: https://clinicaltrials.gov; Unique Identifier: NCT02741115.
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Ecocardiografia , Sulfonamidas , Humanos , Sulfonamidas/uso terapêutico , Pirimidinas/uso terapêutico , Diástole , Função Ventricular EsquerdaRESUMO
OBJECTIVES: Echocardiography performed under sedation allows for better coronary artery visualization in children with Kawasaki disease. We sought to describe hospital-level variability in the percentage of sedated echocardiography (SE) among children with nonrefractory Kawasaki disease (NRKD) and then test its association with repeat echocardiography, length of stay (LOS), and costs. METHODS: We identified children in the Pediatric Health Information System <36 months of age hospitalized with NRKD from March 2010 to February 2017. Hospital-level percentage of SE was the primary outcome. Secondary outcomes were repeat echocardiography during the same hospitalization, LOS, and costs. We used χ2 and Wilcoxon rank tests to compare differences in repeat echocardiography between SE and nonsedated echocardiography. Pearson correlation was used to test associations between SE and repeat echocardiography, LOS, and costs. RESULTS: There were 2887 NRKD hospitalizations from 40 children's hospitals. Initial SE varied from 0.0% to 87.0% (median 5.9%; interquartile range 1.4%-21.1%). Of initial echocardiographies, 22.4% were sedated and 10.1% of all hospitalizations had a repeat echocardiography. Use of SE at the hospital level was associated with a lower likelihood for repeat echocardiography (r = -0.32; 95% confidence interval -0.58 to -0.01; P = .042). Absolute risk reduction was 3.5% and the number needed to sedate was 29. Initial SE was not associated with LOS or hospital-level costs but was associated with increased patient-level costs. CONCLUSIONS: Significant variation exists in the use of SE for children <36 months of age with NRKD at children's hospitals. Our results suggest that determination of SE should not be driven by concern for repeat echocardiography or LOS considerations.
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Síndrome de Linfonodos Mucocutâneos , Criança , Ecocardiografia , Custos Hospitalares , Hospitais Pediátricos , Humanos , Lactente , Tempo de Internação , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Estudos RetrospectivosRESUMO
Left juxtaposition of the right atrial appendage (RAA) is a rare congenital cardiac malformation, where both atrial appendages are located leftward of the great arteries. Despite well-described echocardiographic markers, this diagnosis remains challenging to establish, especially for the novice imager. We describe the apical view sign in three patients with juxtaposed RAA. We noted that the normal left atrial appendage was visualized from the apical four-chamber or equivalent coronal view at the level of the atrioventricular valves.
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Apêndice Atrial/anormalidades , Cardiopatias Congênitas/diagnóstico , Apêndice Atrial/diagnóstico por imagem , Criança , Ecocardiografia , Ecocardiografia Doppler em Cores , Átrios do Coração/anormalidades , Átrios do Coração/diagnóstico por imagem , Humanos , Recém-NascidoRESUMO
OBJECTIVES: The aim of this study was to demonstrate the safety and functionality of the Alterra Adaptive Prestent and SAPIEN 3 transcatheter heart valve (THV) in patients with dysfunctional, dilated right ventricular outflow tract (RVOT) greater or equal to moderate pulmonary regurgitation (PR). BACKGROUND: Significant variations in the size and morphology of the RVOT affect the placement of transcatheter pulmonary valves. The Alterra Prestent internally reduces and reconfigures the RVOT, providing a stable landing zone for the 29-mm SAPIEN 3 THV. METHODS: Eligible patients had moderate or greater PR, weighed >20 kg, and had RVOT diameter 27 to 38 mm and length >35 mm. The primary endpoint was device success, a 5-item composite: 1 Alterra Prestent deployed in the desired location, 1 SAPIEN 3 THV implanted in the desired location within the Prestent, right ventricular-to-pulmonary artery peak-to-peak gradient <35 mm Hg after THV implantation, less than moderate PR at discharge, and no explantation 24 h post-implantation. The secondary composite endpoint was freedom from THV dysfunction (RVOT/pulmonary valve (PV) reintervention, greater or equal to moderate total PR, mean RVOT/PV gradient ≥ 35 mm Hg at 30 days and 6 months. Descriptive statistics are reported. RESULTS: Enrolled patients (N = 15) had a median age and weight of 20 years and 61.7 kg, respectively; 93.3% were in New York Heart Association functional class I or II. Device success was 100%. No staged procedures were necessary. No THV dysfunction was reported to 6 months. No serious safety signals were reported. CONCLUSIONS: This early feasibility study demonstrated the safety and functionality of the Alterra Adaptive Prestent in patients with congenital RVOT dysfunction and moderate or greater PR. Durability and long-term outcome data are needed.