RESUMO
OBJECTIVES: Near-infrared spectroscopy (NIRS) has been increasingly accepted as a noninvasive marker of regional tissue oxygenation despite concerns of imprecision and wide limits of agreement (LOA) with invasive oximetry. New generation absolute monitors may have improved accuracy compared with trend monitors. We sought to compare the concordance with invasive venous oximetry of a new generation absolute NIRS-oximeter (FORESIGHT ELITE; CASMED, Branford, CT) with a modern widely used trend monitor (INVOS 5100C; Medtronic, Minneapolis, MN). DESIGN: Prospective single-center study. SETTING: Tertiary pediatric heart center. PATIENTS: Children undergoing elective cardiac catheterization under general anesthesia. Time-paired venous oximetry samples (jugular and renal) were compared with NIRS-derived oximetry by two monitors using regression and Bland-Altman analysis. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We enrolled 36 children (19 female, 10 cyanotic) with median age 4.1 years (25-75%, 2.5-7.8 yr) and weight 16.7 kg (12.3-29.1 kg). The absolute difference between NIRS-derived and invasive jugular oximetry was less than 10% in 67% of occasions for both monitors. Correlation was fair (Spearman rs = 0.40; p = 0.001) for the FORESIGHT ELITE and poor ( rs = 0.06; p = 0.71) for the INVOS 5100C. Bias and LOA were +6.7% (+22%, -9%) versus +1.3% (LOA = +24%, -21%), respectively. The absolute difference between NIRS-derived and invasive renal oximetry was less than 10% in 80% of occasions with moderate correlation ( rs = 0.57; p < 0.001) for the FORESIGHT ELITE and in 61% of occasions with moderate correlation ( rs = 0.58; p < 0.001) for the INVOS 5100C; bias and LOA were +3.6% (+19%, -12%) and -1.4 % (+27%, -30%), respectively. NIRS correlation with renal venous oximetry was worse for cyanotic versus noncyanotic patients ( p = 0.02). CONCLUSIONS: Concordance and LOA of NIRS-derived oximetry with invasive venous oximetry in the cerebral and renal vascular beds was suboptimal for clinical decision-making. Cyanosis adversely affected NIRS performance in the renal site.
Assuntos
Oximetria , Espectroscopia de Luz Próxima ao Infravermelho , Criança , Humanos , Feminino , Pré-Escolar , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Estudos Prospectivos , Oximetria/métodos , Oxigênio , Cateterismo Cardíaco , CianoseRESUMO
The Fontan procedure (FP) is typically a semi-elective surgery performed between 2 and 5 years of age to complete staged single ventricle palliation. Optimal timing for the FP, particularly in relation to seasonal infectious burden, remains unclear. We queried the Pediatric Health Information System (PHIS) database for all admissions for viral respiratory infections (VRI) from January 2006 to September 2015 and separately for all admissions with a primary procedure code of FP. The PHIS query generated 2,767,142 admissions for VRI and 6349 admissions for the FP from 45 children's hospitals. Of all FP, 2124 (33.5%) were performed from October through March. The median length of stay after Fontan procedure was 9 days (IQR 7-15). Median length of stay after FP was correlated with VRI burden (correlation coefficient = 0.3, p = 0.03). April through August (weeks 18 through 35) had the lowest VRI admission burden and FP length of stay was significantly shorter during this time (13.6 ± 14.8 days vs 14.9 ± 20.3 days, p = 0.03). The FP is frequently performed during the viral respiratory season. This timing is associated with an increased post-operative length of stay after the FP. For elective FP, ideal timing that avoids the viral respiratory season and minimizes post-operative LOS is April through August.
Assuntos
Técnica de Fontan , Pneumonia , Viroses , Criança , Humanos , Estações do Ano , Tempo de Internação , Estudos RetrospectivosRESUMO
OBJECTIVE: To evaluate the acceptability and safety of educational videos utilising visual storytelling to provide information about the cardiac ICU and post-operative care to parents. Videos were designed to educate, further encourage parents to engage in their child's cardiac care, and address common sources of distress. STUDY DESIGN: Two educational videos and survey were sent to 29 families of children previously admitted to the cardiac ICU (April 2020-March 2021). Views regarding information quality, quantity, format, and relevance were assessed, as were parents' emotional responses. Quantitative thresholds for safety and acceptability were set a priori. An inductive approach to content analysis was applied to identify themes in qualitative data. RESULTS: Sixteen parents participated (response rate: 55%). All acceptability and safety thresholds were met; 92% of parents rated the videos as helpful and 85% were "very" or "extremely likely" to recommend them to other families of children with CHD. No participants reported significant distress after viewing the videos. Expressions of parental engagement with their child's care team were common (92%). In qualitative responses, parents perceived the videos as potentially helpful in reducing distress if viewed prior to cardiac ICU admission. CONCLUSION: Visual storytelling to orient parents to the cardiac ICU and address common stressors was found to be safe and acceptable when tested with parents of children previously admitted to the cardiac ICU. Further prospective studies are needed to test intervention effects when videos are viewed before or during cardiac ICU admission, especially for mitigating anxiety and traumatic stress associated with admission.
RESUMO
The growth hormone and insulin-like growth factor (IGF) system is integral to human growth. Genome-wide association studies (GWAS) have identified variants associated with height and located near the genes in this pathway. However, mechanisms underlying these genetic associations are not understood. To investigate the regulation of the genes in this pathway and mechanisms by which regulation could affect growth, we performed GWAS of measured serum protein levels of IGF-I, IGF binding protein-3 (IGFBP-3), pregnancy-associated plasma protein A (PAPP-A2), IGF-II and IGFBP-5 in 838 children (3-18 years) from the Cincinnati Genomic Control Cohort. We identified variants associated with protein levels near IGFBP3 and IGFBP5 genes, which contain multiple signals of association with height and other skeletal growth phenotypes. Surprisingly, variants that associate with protein levels at these two loci do not colocalize with height associations, confirmed through conditional analysis. Rather, the IGFBP3 signal (associated with total IGFBP-3 and IGF-II levels) colocalizes with an association with sitting height ratio (SHR); the IGFBP5 signal (associated with IGFBP-5 levels) colocalizes with birth weight. Indeed, height-associated single nucleotide polymorphisms near genes encoding other proteins in this pathway are not associated with serum levels, possibly excluding PAPP-A2. Mendelian randomization supports a stronger causal relationship of measured serum levels with SHR (for IGFBP-3) and birth weight (for IGFBP-5) than with height. In conclusion, we begin to characterize the genetic regulation of serum levels of IGF-related proteins in childhood. Furthermore, our data strongly suggest the existence of growth-regulating mechanisms acting through IGF-related genes in ways that are not reflected in measured serum levels of the corresponding proteins.
Assuntos
Estatura/genética , Hormônio do Crescimento/genética , Proteína 3 de Ligação a Fator de Crescimento Semelhante à Insulina/genética , Proteína 5 de Ligação a Fator de Crescimento Semelhante à Insulina/genética , Fator de Crescimento Insulin-Like I/genética , Adolescente , Peso ao Nascer/genética , Criança , Pré-Escolar , Feminino , Predisposição Genética para Doença/genética , Estudo de Associação Genômica Ampla , Humanos , Proteína 3 de Ligação a Fator de Crescimento Semelhante à Insulina/sangue , Proteína 5 de Ligação a Fator de Crescimento Semelhante à Insulina/sangue , Fator de Crescimento Insulin-Like II/genética , Masculino , Análise da Randomização Mendeliana , Proteína Plasmática A Associada à Gravidez/genética , Postura SentadaRESUMO
OBJECTIVE: To evaluate the impact of the 2017 American Academy of Pediatrics hypertension Clinical Practice Guideline (CPG), compared with the previous guideline ("Fourth Report"), on the frequency of hypertensive blood pressure (BP) measurements in childhood and associations with hypertension in adulthood using data from the International Childhood Cardiovascular Cohort Consortium. STUDY DESIGN: Childhood BPs were categorized in normal, prehypertensive/elevated, and hypertensive (stage 1 and 2) ranges using the Fourth Report and the CPG. Participants were contacted in adulthood to assess self-reported hypertension. The associations between childhood hypertensive range BPs and self-reported adult hypertension were evaluated. RESULTS: Data were available for 34 014 youth (10.4 ± 3.1 years, 50.6% female) with 92 751 BP assessments. Compared with the Fourth Report, the CPG increased hypertensive readings from 7.6% to 13.5% and from 1.3% to 2.5% for stage 1 and 2 hypertensive range, respectively (P < .0001). Of 12 761 adults (48.8 ± 7.9 years, 43% male), 3839 (30.1%) had self-reported hypertension. The sensitivity for predicting adult hypertension among those with hypertensive range BPs at any point in childhood, as defined by the Fourth Report and the CPG, respectively, was 13.4% and 22.4% (specificity 92.3% and 85.9%, P < .001), with no significant impact on positive and negative predictive values. Associations with self-reported adult hypertension were similar and weak (c-statistic range 0.61-0.68) for hypertensive range BPs as defined by the Fourth Report and CPG. CONCLUSIONS: The CPG significantly increased the prevalence of childhood BPs in hypertensive ranges and improved the sensitivity, without an overall strengthened association, of predicting self-reported adult hypertension.
Assuntos
Hipertensão , Pediatria , Academias e Institutos , Adolescente , Adulto , Pressão Sanguínea , Criança , Feminino , Humanos , Hipertensão/epidemiologia , Masculino , Prevalência , Estados Unidos/epidemiologiaRESUMO
OBJECTIVE: Pulse wave analysis estimates arterial wave reflections relating to left ventricular dysfunction and cardiovascular event risk in adults. Forward and backward waves (Pf and Pb) may improve risk stratification for cardiovascular events. Data in youth are lacking. We hypothesized that a significant difference in wave reflections would be identified in young subjects with adverse cardiovascular risk factors. Approach and Results: Vital signs and labs were obtained in 551 patients aged 10 to 24 years who were lean (L=199), obese (O=173), or had type 2 diabetes (T=179). Wave separation was performed. Differences in cardiovascular risk factors and wave reflections were assessed using ANOVA. General linear models were constructed to elucidate independent predictors of wave reflections. O and T subjects had an adverse cardiovascular risk profile versus L. O and T subjects had higher Pf and Pb versus L (P≤0.05). When adjusted for adiposity and other cardiovascular risk factors, reflection magnitude increased from L to O to T with higher T versus L values (P≤0.05) and near-significant O versus L values (P=0.06). Adiposity and blood pressure were major determinants of wave reflections. Pb influenced log left ventricular mass index, log E/e', and log composite carotid intima-media thickness. CONCLUSIONS: Adolescents and young adults with obesity and type 2 diabetes have altered forward and backward wave reflections versus lean controls related to adiposity, BP, and insulin levels. These parameters may help risk stratify patients with adverse cardiovascular risk factors.
Assuntos
Doenças Cardiovasculares/etiologia , Diabetes Mellitus Tipo 2/complicações , Obesidade Infantil/complicações , Análise de Onda de Pulso , Rigidez Vascular , Adiposidade , Adolescente , Fatores Etários , Pressão Sanguínea , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/fisiopatologia , Criança , Estudos Transversais , Diabetes Mellitus Tipo 2/sangue , Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/tratamento farmacológico , Feminino , Fatores de Risco de Doenças Cardíacas , Humanos , Hipoglicemiantes/sangue , Hipoglicemiantes/uso terapêutico , Insulina/sangue , Insulina/uso terapêutico , Masculino , Obesidade Infantil/diagnóstico , Obesidade Infantil/fisiopatologia , Valor Preditivo dos Testes , Medição de Risco , Adulto JovemRESUMO
OBJECTIVES: The objective of this study was to determine the prevalence of ICU delirium in children less than 18 years old that underwent cardiac surgery within the last 30 days. The secondary aim of the study was to identify risk factors associated with ICU delirium in postoperative pediatric cardiac surgical patients. DESIGN: A 1-day, multicenter point-prevalence study of delirium in pediatric postoperative cardiac surgery patients. SETTING: Twenty-seven pediatric cardiac and general critical care units caring for postoperative pediatric cardiac surgery patients in North America. PATIENTS: All children less than 18 years old hospitalized in the cardiac critical care units at 06:00 on a randomly selected, study day. INTERVENTIONS: Eligible children were screened for delirium using the Cornell Assessment of Pediatric Delirium by the study team in collaboration with the bedside nurse. MEASUREMENT AND MAIN RESULTS: Overall, 181 patients were enrolled and 40% (n = 73) screened positive for delirium. There were no statistically significant differences in patient demographic information, severity of defect or surgical procedure, past medical history, or postoperative day between patients screening positive or negative for delirium. Our bivariate analysis found those patients screening positive had a longer duration of mechanical ventilation (12.8 vs 5.1 d; p = 0.02); required more vasoactive support (55% vs 26%; p = 0.0009); and had a higher number of invasive catheters (4 vs 3 catheters; p = 0.001). Delirium-positive patients received more total opioid exposure (1.80 vs 0.36 mg/kg/d of morphine equivalents; p < 0.001), did not have an ambulation or physical therapy schedule (p = 0.02), had not been out of bed in the previous 24 hours (p < 0.0002), and parents were not at the bedside at time of data collection (p = 0.008). In the mixed-effects logistic regression analysis of modifiable risk factors, the following variables were associated with a positive delirium screen: 1) pain score, per point increase (odds ratio, 1.3; 1.06-1.60); 2) total opioid exposure, per mg/kg/d increase (odds ratio, 1.35; 1.06-1.73); 3) SBS less than 0 (odds ratio, 4.01; 1.21-13.27); 4) pain medication or sedative administered in the previous 4 hours (odds ratio, 3.49; 1.32-9.28); 5) no progressive physical therapy or ambulation schedule in their medical record (odds ratio, 4.40; 1.41-13.68); and 6) parents not at bedside at time of data collection (odds ratio, 2.31; 1.01-5.31). CONCLUSIONS: We found delirium to be a common problem after cardiac surgery with several important modifiable risk factors.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Delírio , Adolescente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Delírio/diagnóstico , Delírio/epidemiologia , Delírio/etiologia , Humanos , Unidades de Terapia Intensiva Pediátrica , América do Norte/epidemiologia , Prevalência , Estudos Prospectivos , Fatores de RiscoRESUMO
Vasculopathy has been identified in young individuals with Turner syndrome (TS). No studies in young individuals with TS have investigated whether this vasculopathy progresses over time. The objective of this study is to describe the changes in vasculopathy over time in a cohort of young individuals with TS. Repeat ultrasound and SphygmoCor CPV® (AtCor Medical) measurements of carotid thickness and peripheral arterial stiffness were performed. Vascular measurements were compared at baseline and follow-up. Follow-up measurements were also compared to historical lean (L) and obese (O) age-, race-, and sex-matched non-TS controls. Thirty-five individuals with TS were studied at a mean age of 19.4 years (range, 13.9-27.5). Mean time to follow-up was 7.2 years (range, 7.1-7.8). Carotid intima media thickness increased by 0.03 ± 0.07 mm (p < 0.01) over time, but was less than L and O controls at follow-up. Pulse wave velocity carotid-femoral increased by 0.51 ± 0.86 m/s (p < 0.01) over time, but was similar to L and less than O controls at follow-up. Augmentation index (AIx) remained unchanged (p = 0.09) over time, but was significantly higher at follow-up than both control groups (p < 0.01 for both). There were no identified differences between 45,X and other TS genotypes. We demonstrate evidence of vascular thickening and stiffening over 7 years in a cohort of young individuals with TS, as well as a persistently increased augmentation index compared to L and O non-TS controls. It is unclear whether the increase in vascular structure and function are related to normal aging or if TS is a risk factor. Higher body mass index seems to be a risk factor. Early estrogen replacement and longer exposure to growth hormone therapy need to be further explored as potential protective factors.
Assuntos
Síndrome de Turner/complicações , Doenças Vasculares/etiologia , Doenças Vasculares/fisiopatologia , Adolescente , Adulto , Índice de Massa Corporal , Artérias Carótidas/diagnóstico por imagem , Artérias Carótidas/fisiopatologia , Espessura Intima-Media Carotídea , Estudos de Casos e Controles , Progressão da Doença , Feminino , Humanos , Hipertensão/epidemiologia , Masculino , Obesidade/complicações , Análise de Onda de Pulso , Fatores de Risco , Ultrassonografia/métodos , Doenças Vasculares/diagnóstico por imagem , Rigidez Vascular , Adulto JovemRESUMO
BACKGROUND: Non-invasive imaging markers in patients with repaired tetralogy of Fallot (rTOF) are still being investigated to inform clinical decision making. Atrial function is a prognostic indicator in many acquired and congenital heart diseases. We sought to examine the relationship between cardiac MRI (CMR)-derived indices of left atrial (LA) function, native left ventricular (LV) T1 values, biventricular systolic function, and exercise capacity in rTOF. METHODS: Sixty-six patients with rTOF without prior pulmonary valve replacement who underwent CMR (median age 18.5 years) were identified. Twenty-one adult rTOF patients (age range 19-32 years) were compared with 20 age-matched healthy volunteers (age range 19-34 years). LA reservoir, conduit, and pump global longitudinal strain (GLS) and strain rate (SR) were determined by tissue tracking. Native LV T1 values were measured on rTOF patients. Pearson correlations were performed to determine bivariate associations. RESULTS: Adult rTOF patients had higher pump GLS, pump:conduit, and pump:reservoir GLS ratios, and lower conduit:reservoir GLS ratio, LV ejection fraction (EF), and right ventricular EF compared to controls (p < 0.001 for each comparison). LA conduit:reservoir GLS and pump:reservoir GLS had correlations to native LV T1 (ρ = 0.26, p = 0.03 and ρ = - 0.26, p = 0.03, respectively). LA reservoir SR had positive correlation to RV EF (ρ = 0.27, p = 0.03). There were no statistically significant correlations between LA function and exercise capacity. CONCLUSIONS: LA function is altered in adolescent and young adult patients with rTOF indicating worse diastolic function and relates to increasing native LV T1 values. Future studies are indicated to investigate the progression of adverse atrial-ventricular interactions and poor outcomes in this population.
Assuntos
Função do Átrio Esquerdo , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Ventrículos do Coração/fisiopatologia , Tetralogia de Fallot/cirurgia , Adolescente , Adulto , Estudos de Casos e Controles , Exercício Físico , Teste de Esforço , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Estudos Retrospectivos , Volume Sistólico , Adulto JovemRESUMO
BACKGROUND: Haematopoietic stem cell transplantation is an important and effective treatment strategy for many malignancies, marrow failure syndromes, and immunodeficiencies in children, adolescents, and young adults. Despite advances in supportive care, patients undergoing transplant are at increased risk to develop cardiovascular co-morbidities. METHODS: This study was performed as a feasibility study of a rapid cardiac MRI protocol to substitute for echocardiography in the assessment of left ventricular size and function, pericardial effusion, and right ventricular hypertension. RESULTS: A total of 13 patients were enrolled for the study (age 17.5 ± 7.7 years, 77% male, 77% white). Mean study time was 13.2 ± 5.6 minutes for MRI and 18.8 ± 5.7 minutes for echocardiogram (p = 0.064). Correlation between left ventricular ejection fraction by MRI and echocardiogram was good (ICC 0.76; 95% CI 0.47, 0.92). None of the patients had documented right ventricular hypertension. Patients were given a survey regarding their experiences, with the majority both perceiving that the echocardiogram took longer (7/13) and indicating they would prefer the MRI if given a choice (10/13). CONCLUSION: A rapid cardiac MRI protocol was shown feasible to substitute for echocardiogram in the assessment of key factors prior to or in follow-up after haematopoietic stem cell transplantation.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Função Ventricular Esquerda , Adolescente , Adulto , Criança , Estudos de Viabilidade , Feminino , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Imageamento por Ressonância Magnética , Masculino , Volume Sistólico , Adulto JovemRESUMO
Pediatric cardiomyopathies can be caused by variants in genes encoding the sarcomere and cytoskeleton in cardiomyocytes. Variants are typically inherited in an autosomal dominant manner with variable expressivity. De novo variants have been reported, however their overall frequency is largely unknown. We sought to determine the rate of de novo, pathogenic and likely pathogenic (P/LP) variants in children with a diagnosis of hypertrophic, dilated, or restrictive cardiomyopathy (HCM, DCM, or RCM), and to compare disease outcomes between individuals with and without a de novo variant. A retrospective record review identified 126 individuals with HCM (55%), DCM (37%), or RCM (8%) ≤18 years of age who had genetic testing. Overall, 50 (40%) had positive genetic testing and 18% of P/LP variants occurred de novo. The rate of de novo variation in those with RCM (80%) was higher than in those with HCM (9%) or DCM (20%). There was evidence of germline mosaicism in one family with RCM. Individuals with de novo variants were more likely than those without to have a history of arrhythmia (p = .049), sudden cardiac arrest (p = .024), hospitalization (p = .041), and cardiac transplantation (p = .030). The likelihood of de novo variation and impact on family risk and screening should be integrated into genetic counseling.
Assuntos
Cardiomiopatia Dilatada/genética , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Restritiva/genética , Pediatria , Adolescente , Cardiomiopatia Dilatada/epidemiologia , Cardiomiopatia Dilatada/patologia , Cardiomiopatia Hipertrófica/epidemiologia , Cardiomiopatia Hipertrófica/patologia , Cardiomiopatia Restritiva/epidemiologia , Cardiomiopatia Restritiva/patologia , Criança , Pré-Escolar , Citoesqueleto/genética , Feminino , Testes Genéticos , Variação Genética/genética , Humanos , Lactente , Recém-Nascido , Masculino , Mosaicismo , Mutação , Miócitos Cardíacos/metabolismo , Miócitos Cardíacos/patologia , Linhagem , Sarcômeros/genéticaRESUMO
OBJECTIVE(S): To examine racial differences in the relationship between cardiovascular (CV) risk factors measured since age 10 years and left ventricular mass index (LVMI) in adulthood in the National Heart, Lung, and Blood Institute Growth and Health Study. STUDY DESIGN: Longitudinal investigation with CV risk factors measured throughout childhood and LVMI measured in adulthood. In total, 556 black and white girls were recruited from schools in the greater Cincinnati area. Analyses examined traditional CV risk factors at baseline, follow-up, and over time (ie, area under the curve [AUC]). LVMI was collected with 2-dimensional guided echocardiographic imaging at a mean age of 25.7 ± 1.7 years. RESULTS: Black girls had higher adiposity and insulin and lower heart rate across time (all P < .05). Blacks had higher LVMI compared with whites in adulthood. Major determinants of young adult LVMI, were race, body mass index z score AUC, systolic blood pressure z score AUC, percent body fat by skin fold AUC, heart rate AUC, and an interaction between race and heart rate (model R2 = 0.40, P < .0001). CONCLUSIONS: The major determinants of LVMI in young female adults are race, adiposity, and systolic blood pressure.
Assuntos
Pressão Sanguínea/fisiologia , Ventrículos do Coração/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/etnologia , Obesidade/complicações , Grupos Raciais , Medição de Risco/métodos , Função Ventricular Esquerda/fisiologia , Adolescente , Adulto , Criança , Ecocardiografia/métodos , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico , Hipertrofia Ventricular Esquerda/etiologia , Masculino , Obesidade/etnologia , Fatores de Risco , Estados Unidos/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Adults with obesity and type 2 diabetes mellitus (T2DM) related to obesity are at increased risk of heart failure with preserved ejection fraction (HFpEF). Whether left ventricular (LV) diastolic function abnormalities related to obesity and T2DM start in adolescence and early adulthood is unknown. We non-invasively evaluated the differences seen in LV diastolic and left atrial (LA) function in adolescents and young adults with obesity and T2DM. METHODS: We analyzed echocardiographic measures of LV diastolic function in patients with structurally normal hearts which were divided into 3 groups (normal weight, obese, and T2DM). Spectral and tissue Doppler and 2-D speckle tracking measurements of diastolic function were obtained. Logistic regression was performed to compare the prevalence of abnormalities in diastolic function based on the worst 25th percentile for each measure to determine the prevalence of diastolic and LA function abnormalities in obese and T2DM patients. RESULTS: 331 teenagers and young adults (median age 22.1 years) were analyzed (101 normal weight, 114 obese, 116 T2DM). Obese and T2DM group had lower E/A and higher E/e'. Obese and T2DM patients had significantly lower atrial reservoir, conduit, and booster strain and worse reservoir and conduit strain rate compared to normal patients (p < 0.001 for all measures). All patients had normal LA volumes. On multivariable analysis, conduit strain and reservoir and conduit strain rate were independently associated with having below the 25th percentile e'. Conduit strain rate was independently associated with having below the 25th percentile for mitral E/A ratio on multivariable analysis. CONCLUSIONS: Abnormal indices of LV diastolic function are detected in adolescents and young adults with obesity and T2DM. LA function and strain analysis were able to detect evidence of decreased reservoir, conduit, and booster strain in these patients although LA volume was normal. The use of LA function strain may increase our ability to detect early diastolic function abnormalities in this population.
Assuntos
Função do Átrio Esquerdo , Diabetes Mellitus Tipo 2/epidemiologia , Obesidade Infantil/epidemiologia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda , Adolescente , Fatores Etários , Diabetes Mellitus Tipo 2/diagnóstico , Diástole , Ecocardiografia Doppler , Feminino , Humanos , Masculino , Obesidade Infantil/diagnóstico , Prevalência , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Estados Unidos/epidemiologia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Changes in cholesterol absorption and cholesterol synthesis may promote dyslipidemia and cardiovascular disease in individuals with type 2 diabetes mellitus (T2DM). OBJECTIVE: To assess cholesterol synthesis and absorption in lean individuals, obese individuals, and individuals with T2DM. METHODS: We measured lathosterol and lanosterol (markers of cholesterol synthesis) as well as campesterol and ß-sitosterol (markers of cholesterol absorption) in the serum of 15 to 26 years old individuals with T2DM (n = 95), as well as their lean (n = 98) and obese (n = 92) controls. RESULTS: Individuals with T2DM showed a 51% increase in lathosterol and a 65% increase in lanosterol compared to lean controls. Similarly, obese individuals showed a 31% increase in lathosterol compared to lean controls. Lathosterol and lanosterol were positively correlated with body mass index, fasting insulin and glucose, serum triglycerides, and C-reactive protein, and negatively correlated with HDL-cholesterol. In contrast, campesterol and ß-sitosterol were not altered in individuals with T2DM. Moreover, campesterol and ß-sitosterol were negatively correlated with body mass index, fasting insulin, and C-reactive protein and were positively correlated with HDL-cholesterol. CONCLUSIONS: Adolescents and young adults with T2DM show evidence of increased cholesterol synthesis compared to non-diabetic lean controls. These findings suggest that T2DM may promote cardiovascular disease by increasing cholesterol synthesis, and provide additional rationale for the use of cholesterol synthesis inhibitors in this group.
Assuntos
Colesterol/metabolismo , Diabetes Mellitus Tipo 2/sangue , Adolescente , Adulto , Biomarcadores , Índice de Massa Corporal , Estudos de Casos e Controles , Colesterol/análogos & derivados , Colesterol/sangue , Diabetes Mellitus Tipo 2/complicações , Humanos , Obesidade/sangue , Obesidade/complicações , Fitosteróis/sangue , Sitosteroides/sangue , Adulto JovemRESUMO
OBJECTIVE: To compare length of stay of the initial neonatal hospitalization and mortality across multiple stages of surgical palliation for infants with left-sided obstructive lesions and severely restrictive or intact atrial septum (I/RAS). METHODS: Retrospective cohort study of patients prenatally diagnosed with left-sided obstructive lesions and I/RAS, defined by fetal pulmonary venous Dopplers. RESULTS: We identified 76 fetal patients with 59 live born intending to pursue intervention. Those with I/RAS had longer durations of mechanical ventilation (P = .031) but no difference in intensive care unit or total length of stay. Survival to discharge from neonatal hospitalization was 41.7% in the I/RAS group and 80.7% in the unrestrictive group (P = .001). There was a higher proportion of deaths between stage 1 and stage 2 in the I/RAS group - 5/9 (55.6%) vs 9/50 (18%) in the unrestrictive group (P = .027). Beyond stage 2 palliation there was trend toward a difference in overall mortality (66.7% in I/RAS vs 35.7% in unrestrictive, P = .05) but no statistically significant difference in transplant-free survival (33.3% in I/RAS vs 53.5% in unrestrictive, P = .11). CONCLUSION: The survival disadvantage conferred by prenatally diagnosed severe atrial septal restriction is most pronounced in the neonatal and early infancy period, with no detectable difference in late midterm transplant-free survival in our cohort.
Assuntos
Comunicação Interatrial/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Veias Pulmonares/diagnóstico por imagem , Adulto , Septo Interatrial/diagnóstico por imagem , Septo Interatrial/patologia , Estudos de Coortes , Feminino , Feto/irrigação sanguínea , Feto/diagnóstico por imagem , Idade Gestacional , Humanos , Recém-Nascido , Gravidez , Resultado da Gravidez , Veias Pulmonares/fisiologia , Estudos Retrospectivos , Ultrassonografia Doppler/métodos , Ultrassonografia Pré-Natal/métodos , Adulto JovemRESUMO
Left ventricular outflow tract obstruction (LVOTO) malformations exhibit higher heritability than other cardiac lesions and cardiac screening is encouraged for first-degree relatives. This study sought to determine the uptake of familial cardiac screening in families with an infant with an LVOTO and assess parental knowledge regarding genetics and heritability of LVOTO. A chart review of the period 2010-2015 identified 69 families who received genetic counseling regarding a diagnosis of LVOTO in an infant. Surveys assessing familial cardiac screening and parental knowledge were completed by a parent in 24 families (completion rate of 35%). Forty percent (36/89) of all at-risk first-degree family members completed cardiac screening. The presence of additional congenital malformations in the affected infant was the only significant factor reducing the uptake of familial cardiac screening (p = 0.003). The reported uptake of screening for subsequent at-risk pregnancies was 11/12 (92%) compared to 25/77 (32%) of living at-risk relatives. Survey respondents answered seven knowledge questions with an average score of 5.2 and all correctly identified that LVOTO can run in families. Uptake of familial cardiac screening is occurring in less than half of at-risk individuals, despite parents demonstrating basic knowledge and receiving genetic counseling. Follow-up counseling in the outpatient setting to review familial screening recommendations should be considered to increase uptake and optimize outcomes.
Assuntos
Família , Cardiopatias Congênitas/genética , Pais , Centros de Atenção Terciária , Obstrução do Fluxo Ventricular Externo/genética , Adulto , Ecocardiografia , Feminino , Aconselhamento Genético , Testes Genéticos , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Masculino , Risco , Obstrução do Fluxo Ventricular Externo/fisiopatologiaRESUMO
OBJECTIVE: To evaluate how infant weigh and length growth trajectories associate with body composition at 3 and 7 years because previous studies have noted that rapid infant weight gain increases risk for high body mass index (BMI) in children. STUDY DESIGN: There were 322 children enrolled at 3 years of age with dual x-ray absorptiometry body composition data and pediatrician growth data for 0-2 years of age who were included in analysis. Superimposition by translation and rotation modeling was used to characterize infant weight and length trajectories in terms of size, tempo and velocity measures. Associations of these measures with fat mass, lean mass, percent body fat, bone mineral content, BMI z-score, and overweight prevalence at 3 and 7 years of age were determined. RESULTS: Infant growth trajectories differed by sex, race, and breastfeeding status. Higher overall weight size and weight velocity from 0 to 2 years of age were associated positively with all age 3 body composition and anthropometry outcomes. However, longer length size from 0 to 2 years of age was associated independently with higher bone mineral content and lean mass, but lower percent body fat, BMI z-score, and a lower odds of overweight at 3 years of age. By 7 years of age, later than average infant weight tempo was also associated with lower fat mass, lean mass, and BMI z-score. CONCLUSIONS: Greater average weight size and greater weight velocity in infancy are markers for greater overall body size at 3 and 7 years of age. However, longer average lengths and later weight gain tempo between 0 and 2 years of age may help to establish a leaner body composition by 3 and 7 years of age.
Assuntos
Antropometria/métodos , Composição Corporal , Estatura , Peso Corporal , Absorciometria de Fóton , Peso ao Nascer , Índice de Massa Corporal , Criança , Pré-Escolar , Feminino , Humanos , Recém-Nascido , Masculino , Sobrepeso , Obesidade Infantil/complicações , Pediatria , Aumento de PesoRESUMO
Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden cardiac death (SCD) in young individuals. Implantable cardioverter defibrillators (ICD) are the primary therapy for sudden death prevention; however, are associated with both physical and psychological complications. We sought to determine factors associated with ICD understanding and patient satisfaction. This was a cross-sectional study, using patient/parent answered questionnaires distributed to patients enrolled in the Hypertrophic Cardiomyopathy Association. Patient characteristics and satisfaction data were obtained via questionnaire. Patients were compared based on age at diagnosis and presence of ICD. ICD patients with high satisfaction were compared to those with low satisfaction to determine factors associated with poor satisfaction. A total of 538 responses were obtained (53 ± 16 years); 46% were females. Seventy patients (13%) were diagnosed with HCM < 18 years of age and 356 (66%) had an ICD. Compared to those without an ICD, patients with ICDs were younger at age of diagnosis (P = 0.001) and time of study (P = 0.008). Patients with ICDs were more likely to have presented with syncope and have family history of ICD, SCD, or HCM-related death. Nineteen patients (5%) felt that issues surrounding their ICD outweighed its benefit. Compared to patients with a favorable satisfaction, the only significant difference was the preimplant ICD discussion (P < 0.001) and history of lead replacement (P = 0.01). In conclusion, the majority of HCM patients with ICDs are satisfied with their ICD management and feel the benefits of ICDs outweigh issues associated with ICDs. Additionally, these data highlight the importance of the preimplant patient-physician discussion around the need for ICD prior to implantation.
Assuntos
Cardiomiopatia Hipertrófica/terapia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Conhecimentos, Atitudes e Prática em Saúde , Adolescente , Adulto , Idoso , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Satisfação do Paciente , Inquéritos e QuestionáriosRESUMO
OBJECTIVES: Evaluate the efficacy, outcomes, and complications associated with direct current cardioversion (DCCV) in the treatment of arrhythmias in pediatric and adult congenital heart disease (CHD) populations and identify patient and procedural characteristics associated with adverse events. BACKGROUND: Pediatric and adult patients with CHD are at risk of atrial arrhythmias. DCCV is effective but is associated with potential complications. METHODS: In this single-center retrospective series, patients who underwent DCCV between January 2010 and May 2015 were identified and categorized as pediatric (<18 years) or adult (> 18 years). Records were reviewed for demographic, arrhythmic, and CHD-specific characteristics; acute efficacy; and 3-month arrhythmia recurrence. Complications were categorized as life-threatening (LT) or non-life-threatening (NLT). Univariate followed by multiple variable and logistic regression (LR) analyses were used to identify characteristics associated with complications. RESULTS: We identified 104 patients with 152 discrete DCCV events with median age 17.4 years (0.15-62.2). DCCV efficacy was 89% with 3-month recurrence of 46%. There were 52 complications among 24 patients, median age 17.7 years (0.15-49). Risks associated with NLT complications are as follows: moderate-severe systolic dysfunction (8/152 encounters, P = < 0.01) and more than one shock per DCCV encounter (P = < 0.01). Six of eight encounters with moderate-severe systolic dysfunction were <18 years (P = 0.1). Risks for LT complications included age >18 years and associated NLT complication. Adults had more frequent arrhythmia recurrence within 3 months than children (P = < 0.01). CONCLUSIONS: DCCV is effective for arrhythmias but is associated with frequent recurrence, particularly in adult patients. Complications associated with DCCV may be greater than previously reported. Additional support and precautions should be in place for those at greatest risk.
Assuntos
Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/terapia , Cardioversão Elétrica/métodos , Cardiopatias Congênitas/fisiopatologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
BACKGROUND: Proprotein convertase subtilisin/kexin type 9 (PCSK9) is a key regulator of low-density lipoprotein cholesterol and cardiovascular disease risk, and is an emerging therapeutic target. OBJECTIVE: We compared serum PCSK9 levels in young adults, with and without type 2 diabetes. SUBJECTS AND METHODS: Cross-sectional analysis was conducted in a cohort, aged 15 to 26 years, in Cincinnati, OH, from 2005 to 2010. Serum PCSK9 levels were measured in 94 youth with type 2 diabetes, 93 obese control subjects, and 99 lean control subjects. Correlative analyses were conducted to determine significant covariates of PCSK9 by group and sex, and multivariate linear regression models were used to study the independent determinants of PCSK9. RESULTS: In females, PCSK9 levels were significantly increased in the obese and type 2 diabetes subjects relative to the lean controls (P < .01). Moreover, PCSK9 was positively correlated with multiple metabolic parameters in females: body mass index, systolic blood pressure, fasting glucose, fasting insulin, and C-reactive protein levels (P ≤ .02). In males, PCSK9 levels were decreased overall compared with females (P = .03), and did not differ between the lean, obese, or type 2 diabetes groups. CONCLUSIONS: Obesity and type 2 diabetes were associated with significantly higher levels of PCSK9 in young women, but not in young men. These data suggest that sex could modify the effects of obesity and diabetes on PCSK9 in young adults.