RESUMO
Cardiovascular magnetic resonance (CMR) has become the reference standard for quantitative and qualitative assessment of ventricular function, blood flow, and myocardial tissue characterization. There is a preponderance of large CMR studies and registries in adults; However, similarly powered studies are lacking for the pediatric and congenital heart disease (PCHD) population. To date, most CMR studies in children are limited to small single or multicenter studies, thereby limiting the conclusions that can be drawn. Within the PCHD CMR community, a collaborative effort has been successfully employed to recognize knowledge gaps with the aim to embolden the development and initiation of high-quality, large-scale multicenter research. In this publication, we highlight the underlying challenges and provide a practical guide toward the development of larger, multicenter initiatives focusing on PCHD populations, which can serve as a model for future multicenter efforts.
Assuntos
Cardiopatias Congênitas , Estudos Multicêntricos como Assunto , Valor Preditivo dos Testes , Humanos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Criança , Big Data , Imageamento por Ressonância Magnética , Projetos de Pesquisa , Fatores Etários , Adolescente , Pré-EscolarRESUMO
Pediatric ECG standards have been defined without echocardiographic confirmation of normal anatomy. The Pediatric Heart Network Normal Echocardiogram Z-score Project provides a racially diverse group of healthy children with normal echocardiograms. We hypothesized that ECG and echocardiographic measures of left ventricular (LV) dimensions are sufficiently correlated in healthy children to imply a clinically meaningful relationship. This was a secondary analysis of a previously described cohort including 2170 digital ECGs. The relationship between 6 ECG measures associated with LV size were analyzed with LV Mass (LVMass-z) and left ventricular end-diastolic volume (LVEDV-z) along with 11 additional parameters. Pearson or Spearman correlations were calculated for the 78 ECG-echocardiographic pairs with regression analyses assessing the variance in ECG measures explained by variation in LV dimensions and demographic variables. ECG/echocardiographic measurement correlations were significant and concordant in 41/78 (53%), though many were significant and discordant (13/78). Of the 6 ECG parameters, 5 correlated in the clinically predicted direction for LV Mass-z and LVEDV-z. Even when statistically significant, correlations were weak (0.05-0.24). R2 was higher for demographic variables than for echocardiographic measures or body surface area in all pairs, but remained weak (R2 ≤ 0.17). In a large cohort of healthy children, there was a positive association between echocardiographic measures of LV size and ECG measures of LVH. These correlations were weak and dependent on factors other than echocardiographic or patient derived variables. Thus, our data support deemphasizing the use of solitary, traditional measurement-based ECG markers traditionally thought to be characteristic of LVH as standalone indications for further cardiac evaluation of LVH in children and adolescents.
Assuntos
Ecocardiografia , Eletrocardiografia , Ventrículos do Coração , Humanos , Criança , Feminino , Masculino , Ventrículos do Coração/diagnóstico por imagem , Ecocardiografia/métodos , Pré-Escolar , Adolescente , Valores de Referência , Lactente , Volume Sistólico/fisiologia , Tamanho do ÓrgãoRESUMO
Cardiovascular magnetic resonance (CMR) has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of CMR in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking. This guidelines document outlines the use of CMR in this patient population for a significant number of heart lesions in this age group and although admittedly, is not an exhaustive treatment, it does deal with an expansive list of many common clinical issues encountered in daily practice.
Assuntos
Cardiologia , Cardiopatias , Radiologia , American Heart Association , Criança , Ecocardiografia , Cardiopatias/diagnóstico por imagem , Humanos , Espectroscopia de Ressonância Magnética , América do Norte , Valor Preditivo dos Testes , Estados UnidosRESUMO
Children with Friedreich's ataxia (FA) are at risk of perioperative morbidity and mortality from severe unpredictable heart failure. There is currently no clear way of identifying patients at highest risk. We used myocardial perfusion reserve (MPR), an MRI technique used to assess the maximal myocardial blood flow above baseline, to help determine potential surgical risk in FA subjects. In total, seven children with genetically confirmed FA, ages 8-17 years, underwent MPR stress testing using regadenoson. Six of the seven demonstrated impaired endocardial perfusion during coronary hyperemia. The same six were also found to have evidence of ongoing myocardial damage as illustrated by cardiac troponin I leak (range 0.04-0.17 ng/mL, normal < 0.03 ng/mL). None of the patients had a reduced ejection fraction (range 59-74%) or elevated insulin level (range 2.46-14.23 mCU/mL). This retrospective study shows that children with FA develop MPR defects early in the disease process. It also suggests MPR may be a sensitive tool to evaluate underlying cardiac compromise and could be of use in directing surgical management decisions in children with FA.
Assuntos
Ataxia de Friedreich , Adolescente , Criança , Circulação Coronária , Humanos , Miocárdio , Perfusão , Estudos RetrospectivosRESUMO
Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18 years of age enrolled in the Pediatric Heart Network Echo Z-Score Project. The echocardiographic study parameters included proximal aortic diameters at 3 locations, LV end-diastolic volume, and LV mass. Using the statistical methodology described in the original project, Z-scores based on height and BSA were determined for the study parameters and tested for any clinically significant relationships with age, sex, race, ethnicity, and body mass index (BMI). Normalization models based on height versus BSA were compared among underweight, normal weight, and overweight (but not obese) children in the study population. Z-scores based on height and BSA were calculated for the 5 study parameters and revealed no clinically significant relationships with age, sex, race, and ethnicity. Normalization based on height resulted in lower Z-scores in the underweight group compared to the overweight group, whereas normalization based on BSA resulted in higher Z-scores in the underweight group compared to the overweight group. In other words, increasing BMI had an opposite effect on height-based Z-scores compared to BSA-based Z-scores. Allometric normalization based on height and BSA for aortic and LV sizes is feasible. However, height-based normalization results in higher cardiovascular Z-scores in heavier children, and BSA-based normalization results in higher cardiovascular Z-scores in lighter children. Further studies are needed to assess the performance of these approaches in obese children with or without cardiac disease.
Assuntos
Estatura , Superfície Corporal , Doenças Cardiovasculares/diagnóstico , Coração/anatomia & histologia , Adolescente , Doenças Cardiovasculares/diagnóstico por imagem , Criança , Pré-Escolar , Bases de Dados Factuais , Ecocardiografia , Feminino , Coração/diagnóstico por imagem , Humanos , Masculino , Obesidade Infantil/epidemiologia , Pediatria , Valores de ReferênciaRESUMO
BACKGROUND: Cardiovascular magnetic resonance (CMR) is increasingly used to diagnose myocarditis in adults but its use in children is not well-established. We sought to describe the presentation, CMR protocol and findings, and outcomes in a multicenter cohort of children with myocarditis. METHODS: Thirteen hospitals retrospectively identified patients meeting the following inclusion criteria: 1) diagnosis of myocarditis by the managing physicians, 2) age <21 years, 3) CMR examination within 30 days of presentation, and 4) no congenital heart disease. Clinical data and test results, including CMR findings, were abstracted from the medical record. RESULTS: For the 143 patients meeting inclusion criteria, the median age was 16.0 years (range, 0.1-20.3) and 139 (97 %) were hospitalized at the time of CMR. The median time from presentation to CMR was 2 days (0-28). The median left ventricular ejection fraction at CMR was 56 % (10-74), with 29 (20 %) below 45 %. The median right ventricular ejection fraction was 54 % (15-72), with 11 (8 %) below 40 %. There was significant variability among centers in the types of tissue characterization techniques employed (p < 0.001). Overall, late gadolinium enhancement (LGE) was used in 100 % of studies, followed by T2-weighted imaging (T2W) in 69 %, first-pass contrast perfusion (FPP) in 48 %, and early gadolinium enhancement (EGE) in 28 %. Abnormalities were most common with LGE (81 %), followed by T2W (74 %), EGE (55 %), and FPP (8 %). The CMR study was interpreted as positive for myocarditis in 117 patients (82 %), negative in 18 (13 %), and equivocal in 7 (5 %), yielding a sensitivity of 82 %. At a median follow-up of 7.1 months (0-87), all patients were alive and 5 had undergone cardiac transplantation. CMR parameters at presentation associated with persistent left ventricular dysfunction were larger left ventricular end-diastolic volume and lower left and right ventricular ejection fraction but not abnormal LGE. CONCLUSIONS: Despite significant practice variation in imaging protocol among centers, CMR had a high sensitivity for the diagnosis of myocarditis in pediatric patients. Abnormalities were most often seen with LGE followed by T2W, EGE, and FPP. These findings should be useful in designing future prospective studies.
Assuntos
Imageamento por Ressonância Magnética , Miocardite/diagnóstico , Miocárdio/patologia , Volume Sistólico , Função Ventricular Esquerda , Função Ventricular Direita , Adolescente , Fatores Etários , Criança , Pré-Escolar , Meios de Contraste , Transplante de Coração , Hospitalização , Humanos , Lactente , Miocardite/patologia , Miocardite/fisiopatologia , Miocardite/cirurgia , Variações Dependentes do Observador , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Tempo , Adulto JovemRESUMO
Patients may develop hemodynamic abnormalities after right ventricular outflow tract (RVOT) repair. Re-intervention timing remains a dilemma. This study evaluates exercise capacity and RV function before and after intervention using age-related comparisons. Twenty-six patients with severe pulmonary regurgitation (PR) after initial repair scheduled for pulmonary valve replacement (PVR) were enrolled. Metabolic treadmill testing (EST) and MRI were obtained before and after surgery. EST results were compared with matched controls. Preoperative exercise time and peak oxygen consumption (VO2 max) were significantly diminished compared with controls but were not significantly different postoperatively. The patients were then split into age-related cohorts. When comparing pre-PVR and post-PVR exercise time and VO2 max among themselves, neither cohort showed significant differences. However, patients younger than 25 years had better postoperative results, an age-related difference not seen in the controls. Preoperative MRI showed significantly dilated RV, PR, and low normal function. After PVR, the right to left ventricular end-diastolic volume ratio (RVEDV:LVEDV) and pulmonary artery regurgitant fraction (RF) significantly decreased. There was no change in ventricular ejection fractions (EF). Severe PR, decreased RVEF, and RV dilation can significantly diminish exercise capacity. PVR improves RVEDV:LVEDV and RF, but not EF. Younger patients had better exercise capacity that was maintained postoperatively. This age-related difference was not seen in the controls, indicating that earlier intervention may preserve exercise capacity. Serial ESTs in patients with severe PR following RVOT repair may identify deteriorating exercise capacity as an early indicator for the need for PVR.
Assuntos
Exercício Físico/fisiologia , Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Função Ventricular Direita/fisiologia , Adolescente , Fatores Etários , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Pré-Escolar , Teste de Esforço/métodos , Feminino , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Consumo de Oxigênio/fisiologia , Período Pós-Operatório , Período Pré-Operatório , Valva Pulmonar/patologia , Insuficiência da Valva Pulmonar/patologia , Tetralogia de Fallot/patologia , Resultado do TratamentoRESUMO
Cardiovascular magnetic resonance has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of cardiovascular magnetic resonance in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking. This guidelines document outlines the use of cardiovascular magnetic resonance in this patient population for a significant number of heart lesions in this age group and although admittedly, is not an exhaustive treatment, it does deal with an expansive list of many common clinical issues encountered in daily practice.
Assuntos
Cardiologia , Cardiopatias , Radiologia , American Heart Association , Criança , Ecocardiografia , Cardiopatias/diagnóstico por imagem , Humanos , Espectroscopia de Ressonância Magnética , América do Norte , Sociedades Médicas , Estados UnidosRESUMO
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.
Assuntos
Ecocardiografia , Sulfonamidas , Humanos , Sulfonamidas/uso terapêutico , Pirimidinas/uso terapêutico , Diástole , Função Ventricular EsquerdaRESUMO
The Fontan procedure refers to any operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. It is performed to treat several complex congenital heart abnormalities including tricuspid atresia, pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, and double-inlet ventricle. The original Fontan procedure included direct anastomosis of the right atrium to the main pulmonary artery; however, multiple modifications have been employed. Creation of Fontan circulation is palliative in nature, with good results in patients with ideal hemodynamics and substantial morbidity and mortality in those with poor hemodynamics. Complications of Fontan circulation include exercise intolerance, ventricular failure, right atrium dilatation and arrhythmia, systemic and hepatic venous hypertension, portal hypertension, coagulopathy, pulmonary arteriovenous malformation, venovenous shunts, and lymphatic dysfunction (eg, ascites, edema, effusion, protein-losing enteropathy, and plastic bronchitis). Magnetic resonance imaging is best for postoperative evaluation of patients who underwent the Fontan procedure, and cardiac transplantation remains the only definitive treatment for those with failing Fontan circulation.
Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias/etiologia , Cardiopatias/patologia , Imageamento por Ressonância Magnética/métodos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , HumanosRESUMO
OBJECTIVE: Our objectives are to review the surgical procedures that have been performed for the treatment of transposition of the great vessels. Using MRI, we illustrate the normal postoperative findings and many of the long-term complications for each of the surgical procedures. CONCLUSION: MRI is an extremely useful imaging method for evaluation of normal and abnormal findings after surgical repair of transposition of the aorta and pulmonary artery.
Assuntos
Imageamento por Ressonância Magnética/métodos , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/cirurgia , Humanos , Imageamento Tridimensional , Complicações Pós-Operatórias/diagnósticoRESUMO
Conotruncal cardiac defects make up a significant portion of congenital heart disease. For proper diagnosis and subsequent care of patients with these defects, different and sometimes multiple imaging modalities are needed at various stages of care. This article reviews the characteristics of some of the most common conotruncal defects and the imaging options available along with the advantages and disadvantages of each. Intricate knowledge of the capabilities of each modality will aid the practitioner in making optimal clinical decisions.
Assuntos
Cardiopatias Congênitas/diagnóstico , Obstrução do Fluxo Ventricular Externo/genética , Cateterismo Cardíaco , Ecocardiografia , Ecocardiografia Transesofagiana , Predisposição Genética para Doença , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Imagem Cinética por Ressonância Magnética , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/genética , Tomografia Computadorizada por Raios X , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/genética , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/embriologia , Obstrução do Fluxo Ventricular Externo/diagnóstico , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagemRESUMO
BACKGROUND: The reliability of left ventricular (LV) systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of LV size and systolic function in children with normal cardiac anatomy and qualitatively normal function. METHODS: The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race. A core lab used two-dimensional echocardiograms to measure LV dimensions from which a separate data coordinating center calculated LV volumes and systolic functional indices. To evaluate interobserver variability, two independent expert pediatric echocardiographic observers remeasured LV dimensions on a subset of studies, while blinded to calculated volumes and functional indices. RESULTS: Of 3,215 subjects with measurable images, 552 (17%) had a calculated LV shortening fraction (SF) < 25% and/or LV ejection fraction (EF) < 50%; the subjects were significantly younger and smaller than those with normal values. When the core lab and independent observer measurements were compared, individual LV size parameter intraclass correlation coefficients were high (0.81-0.99), indicating high reproducibility. The intraclass correlation coefficients were lower for SF (0.24) and EF (0.56). Comparing reviewers, 40/56 (71%) of those with an abnormal SF and 36/104 (35%) of those with a normal SF based on core lab measurements were calculated as abnormal from at least one independent observer. In contrast, an abnormal EF was less commonly calculated from the independent observers' repeat measures; only 9/47 (19%) of those with an abnormal EF and 8/113 (7%) of those with a normal EF based on core lab measurements were calculated as abnormal by at least one independent observer. CONCLUSIONS: Although blinded measurements of LV size show good reproducibility in healthy children, subsequently calculated LV functional indices reveal significant variability despite qualitatively normal systolic function. This suggests that, in clinical practice, abnormal SF/EF values may result in repeat measures of LV size to match the subjective assessment of function. Abnormal LV functional indices were more prevalent in younger, smaller children.
Assuntos
Ecocardiografia , Função Ventricular Esquerda , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Valores de Referência , Reprodutibilidade dos Testes , Sístole , WisconsinRESUMO
INTRODUCTION: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors. METHODS: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL). Mixed effects regression modeling adjusting for clustering by center was used to evaluate factors associated with abnormal anthropometry and delayed puberty and associations with QoL. RESULTS: Of the 299 subjects, 42% were female. The median enrollment age was 13.9 years, and the median age at Fontan was 3 years. Fontan survivors had a higher prevalence of short stature relative to normative data (20% vs 5%, P < .0001) and an increased prevalence of abnormal BMI (16% vs 10%, P < .0001) (low [43%] and high [57%]). Fontan subjects, both males (58%) and females (58%), had a delay of 1.5-2 years in ≥1 Tanner stage parameter compared to normal population. There was no association between delayed puberty and QoL. Abnormal anthropometry was associated with lower overall (62.3 ± 17.3 vs 72.5 ± 16.6; P < .001) and physical appearance scores (72.2 ± 27.4 vs 79.8 ± 21.5; P < .01). Lower exercise capacity was associated with abnormal anthropometry and >2 surgeries before Fontan was associated with delayed puberty. Lower family income (<$25 000) and hypoplastic left heart syndrome were associated with lower QoL. CONCLUSION: Compared to the normal population, Fontan survivors have high prevalence of short stature, abnormal BMI and delayed puberty. Abnormal anthropometry, but not delayed puberty, was associated with lower overall QoL and perceived physical appearance scores. Routine screening for abnormal anthropometry, especially in HLHS and in lower socioeconomic status families, should be considered to allow interventions, which might ameliorate the negative psychosocial impact.
Assuntos
Antropometria , Técnica de Fontan/psicologia , Nível de Saúde , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Puberdade Tardia/psicologia , Qualidade de Vida , Sobreviventes/estatística & dados numéricos , Adolescente , Criança , Estudos Transversais , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/psicologia , Masculino , Puberdade Tardia/complicações , Puberdade Tardia/fisiopatologia , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Interpretation of pediatric ECGs is limited by lack of accurate sex- and race-specific normal reference values obtained with modern technology for all ages. We sought to obtain contemporary digital ECG measurements in healthy children from North America, to evaluate the effects of sex and race, and to compare our results to commonly used published datasets. METHODS: Digital ECGs (12-lead) were retrospectively collected for children ≤18 years old with normal echocardiograms at 19 centers in the Pediatric Heart Network. Patients were classified into 36 groups: 6 age, 2 sex, and 3 race (white, black, and other/mixed) categories. Standard intervals and amplitudes were measured; mean±SD and 2nd/98th percentiles were determined by age group, sex, and race. For each parameter, multivariable analysis, stratified by age, was conducted using sex and race as predictors. Parameters were compared with 2 large pediatric ECG data sets. RESULTS: Among ECGs from 2400 children, significant differences were found by sex and race categories. The corrected QT interval in lead II was greater for girls compared with boys for age groups ≥3 years (P≤0.03) and for whites compared with blacks for age groups ≥12 years (P<0.05). The R wave amplitude in V6 was greater for boys compared with girls for age groups ≥12 years (P<0.001), for blacks compared with white or other race categories for age groups ≥3 years (P≤0.006), and greater compared with a commonly used public data set for age groups ≥12 years (P<0.0001). CONCLUSIONS: In this large, diverse cohort of healthy children, most ECG intervals and amplitudes varied by sex and race. These differences have important implications for interpreting pediatric ECGs in the modern era when used for diagnosis or screening, including thresholds for left ventricular hypertrophy.
Assuntos
Eletrocardiografia/normas , Frequência Cardíaca , Adolescente , Negro ou Afro-Americano , Fatores Etários , Criança , Pré-Escolar , Feminino , Disparidades nos Níveis de Saúde , Voluntários Saudáveis , Humanos , Lactente , Recém-Nascido , Masculino , América do Norte , Variações Dependentes do Observador , Valor Preditivo dos Testes , Valores de Referência , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores Sexuais , Processamento de Sinais Assistido por Computador , População BrancaRESUMO
BACKGROUND: Published nomograms of pediatric echocardiographic measurements are limited by insufficient sample size to assess the effects of age, sex, race, and ethnicity. Variable methodologies have resulted in a wide range of Z scores for a single measurement. This multicenter study sought to determine Z scores for common measurements adjusted for body surface area (BSA) and stratified by age, sex, race, and ethnicity. METHODS AND RESULTS: Data collected from healthy nonobese children ≤18 years of age at 19 centers with a normal echocardiogram included age, sex, race, ethnicity, height, weight, echocardiographic images, and measurements performed at the Core Laboratory. Z score models involved indexed parameters (X/BSAα) that were normally distributed without residual dependence on BSA. The models were tested for the effects of age, sex, race, and ethnicity. Raw measurements from models with and without these effects were compared, and <5% difference was considered clinically insignificant because interobserver variability for echocardiographic measurements are reported as ≥5% difference. Of the 3566 subjects, 90% had measurable images. Appropriate BSA transformations (BSAα) were selected for each measurement. Multivariable regression revealed statistically significant effects by age, sex, race, and ethnicity for all outcomes, but all effects were clinically insignificant based on comparisons of models with and without the effects, resulting in Z scores independent of age, sex, race, and ethnicity for each measurement. CONCLUSIONS: Echocardiographic Z scores based on BSA were derived from a large, diverse, and healthy North American population. Age, sex, race, and ethnicity have small effects on the Z scores that are statistically significant but not clinically important.
Assuntos
Superfície Corporal , Ecocardiografia/métodos , Etnicidade , Coração/diagnóstico por imagem , Grupos Raciais , Adolescente , Fatores Etários , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Voluntários Saudáveis , Humanos , Masculino , Nomogramas , América do Norte/epidemiologia , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tamanho da Amostra , Fatores SexuaisRESUMO
This study demonstrates, in patients with 22q11.2 deletion, the wide variety of complex aortic arch anomalies that can be accurately defined by cardiac magnetic resonance imaging (MRI) compared with echocardiography. From May 1999 to August 2003, 17 of 68 patients (25%) with 22q11.2 deletion referred for cardiac evaluation required cardiac MRI, after echocardiography, to clarify aortic arch anatomy. Images of cardiac anatomy were obtained using steady-state free precession, half-Fourier acquired single turbo spin-echo dark blood sequences and gadolinium for 3-dimensional reconstruction. All MRI findings were abnormal, and in 16 of 17 cases, echocardiography was unable to define aortic arch anomalies correctly compared with MRI.
Assuntos
Aorta Torácica/anormalidades , Doenças da Aorta/genética , Deleção Cromossômica , Cromossomos Humanos Par 22 , Cardiopatias Congênitas/diagnóstico , Imageamento por Ressonância Magnética/métodos , Adolescente , Adulto , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/patologia , Doenças da Aorta/complicações , Doenças da Aorta/diagnóstico , Criança , Pré-Escolar , Ecocardiografia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/genética , Humanos , Lactente , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
BACKGROUND: When echocardiography is used to follow up cardiac patients on extracorporeal membrane oxygenation (ECMO), ejection fraction as a measure of ventricular function has been used to determine likelihood of survival after decannulation. We hypothesized that systemic atrioventricular (AV) valve regurgitation while on ECMO may be a better predictor of survival. METHODS: From February 1995 to March 2001, 64 patients required ECMO postoperatively. Three were removed from ECMO owing to brain death, so 61 patients formed our study group. Retrospective chart review was performed with systemic AV valve regurgitation and ejection fraction while on ECMO recorded from echocardiography reports. Patients alive 1 month after decannulation were considered survivors. RESULTS: There were 29 survivors (47.5%). Ejection fraction in survivors (mean 41% +/- 0.13%) was not significantly different from that of nonsurvivors (41% +/- 0.18%; p < or = 0.839) but severity of systemic AV valve regurgitation was significantly different. Only 2 survivors (6.8%) had at least moderate AV valve regurgitation compared with 17 of the 32 nonsurvivors (53%). Patients with moderate to severe regurgitation while on ECMO were less likely to survive for 1 month after decannulation, with an odds ratio of 16.63 (95% confidence interval 3.08, 89.70; p = 0.001). Also female sex increased the odds of death by a factor of 5.43 (95% confidence interval 1.45, 20.36; p = 0.012). CONCLUSIONS: Severity of systemic AV valve regurgitation on ECMO is a more reliable predictor than ejection fraction for survival at 1 month after decannulation. Patients with less than moderate AV valve regurgitation have a significantly better chance of survival.
Assuntos
Oxigenação por Membrana Extracorpórea , Insuficiência da Valva Mitral/mortalidade , Insuficiência da Valva Tricúspide/mortalidade , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
The authors present a 25-year-old woman who was admitted to the ICU for treatment of shock, respiratory failure, and acidosis related to congenital lactic acidosis from pyruvate dehydrogenase deficiency. To aid in ongoing management of the metabolic acidosis, the Paratrend blood gas monitoring sensor was inserted through a peripheral venous site to provide a continuous measurement of pH and partial pressure of carbon dioxide (Pco2). With the venous insertion of the Paratrend, a clinically useful correlation with arterial blood gas values was noted. Linear regression analysis of the pH values from the venous blood gas analyses and the Paratrend monitor revealed r2 = 0.71 with p = 0.001 and r2 = 0.78 with a p = 0.0003 for the Pco2 values. Our preliminary experience suggests that venous placement of the Paratrend monitor can be used to provide clinically useful, continuous measurement of pH and Pco2.