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1.
Ann Agric Environ Med ; 27(3): 384-387, 2020 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-32955219

RESUMO

INTRODUCTION AND OBJECTIVE: Single ventricle anomaly is one of the complex congenital heart defects. A dependable non-invasive method of evaluation of Fontan circulation haemodynamics for early diagnosing unstable patients is hardly available in routine clinical practice. The aim of the study is non-invasive evaluation of the haemodynamic parameters in patients after Fontan operation. MATERIAL AND METHODS: The study involved 11 participants (age 24.4±4.3 years) with functionally univentricular hearts after Fontan operation. Evaluation of haemodynamic parameters was performed in supine and sitting positions using the impedance cardiography method. RESULTS: In comparative analysis, heart rate (70.1 vs.78.3 1/min; p=0.001), diastolic blood pressure (73.9 vs. 76.7 mm Hg; p=0.026), mean arterial blood pressure (84.5 vs. 88.0 mm Hg; p=0.013), systemic vascular resistance (1284.8 vs. 1334.9 dyn*s*cm-5; p=0.024), systemic vascular resistance index (2178.7 vs. 2272.8 dyn*s*cm-5*m2 ; p=0.018), pre-ejection period (124.2 vs. 136.2 ms; p=0.009), systolic time ratio (0.43 vs. 0.53; p=0.0001), and Zo (26.2 vs. 28.7 Ω; p<0.00001), were significantly higher in the sitting position. Stroke volume (75.4 vs. 68.5 ml; p=0.013), stroke index (42.7 vs. 39.0 ml*m-2; p=0.014), thoracic fluid content (38.5 vs. 35.4 1*kΩ-1; p=<0.00001), thoracic fluid content index (22.8 vs. 21.0 1*kΩ-1*m-2; p=<0.00001), and leftventricular ejection time 291.1 vs. 260.1 ms; p <0.00001, were significantly higher in the supine position. CONCLUSIONS: In patients after Fontan procedure, impedance cardiography can be a useful tool the assessment of shortterm haemodynamic changes provoked by postural changes. Its clinical value in patients with congenital heart defects should be further investigated.


Assuntos
Pressão Sanguínea , Técnica de Fontan/efeitos adversos , Frequência Cardíaca , Resistência Vascular , Adulto , Cardiografia de Impedância , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Humanos , Polônia , Adulto Jovem
2.
J Cardiovasc Magn Reson ; 22(1): 68, 2020 09 17.
Artigo em Inglês | MEDLINE | ID: mdl-32938483

RESUMO

BACKGROUND: Cardiovascular magnetic resonance (CMR) phase contrast (PC) flow measurements suffer from phase offset errors. Background subtraction based on stationary phantom measurements can most reliably be used to overcome this inaccuracy. Stationary tissue correction is an alternative and does not require additional phantom scanning. The aim of this study was 1) to compare measurements with and without stationary tissue correction to phantom corrected measurements on different GE Healthcare CMR scanners using different software packages and 2) to evaluate the clinical implications of these methods. METHODS: CMR PC imaging of both the aortic and pulmonary artery flow was performed in patients on three different 1.5 T CMR scanners (GE Healthcare) using identical scan parameters. Uncorrected, first, second and third order stationary tissue corrected flow measurement were compared to phantom corrected flow measurements, our reference method, using Medis QFlow, Circle cvi42 and MASS software. The optimal (optimized) stationary tissue order was determined per scanner and software program. Velocity offsets, net flow, clinically significant difference (deviation > 10% net flow), and regurgitation severity were assessed. RESULTS: Data from 175 patients (28 (17-38) years) were included, of which 84% had congenital heart disease. First, second and third order and optimized stationary tissue correction did not improve the velocity offsets and net flow measurements. Uncorrected measurements resulted in the least clinically significant differences in net flow compared to phantom corrected data. Optimized stationary tissue correction per scanner and software program resulted in net flow differences (> 10%) in 19% (MASS) and 30% (Circle cvi42) of all measurements compared to 18% (MASS) and 23% (Circle cvi42) with no correction. Compared to phantom correction, regurgitation reclassification was the least common using uncorrected data. One CMR scanner performed worse and significant net flow differences of > 10% were present both with and without stationary tissue correction in more than 30% of all measurements. CONCLUSION: Phase offset errors had a significant impact on net flow quantification, regurgitation assessment and varied greatly between CMR scanners. Background phase correction using stationary tissue correction worsened accuracy compared to no correction on three GE Healthcare CMR scanners. Therefore, careful assessment of phase offset errors at each individual scanner is essential to determine whether routine use of phantom correction is necessary. TRIAL REGISTRATION: Observational Study.


Assuntos
Aorta/diagnóstico por imagem , Insuficiência da Valva Aórtica/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Hemodinâmica , Interpretação de Imagem Assistida por Computador , Imagem Cinética por Ressonância Magnética/instrumentação , Artéria Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Adolescente , Adulto , Aorta/fisiopatologia , Insuficiência da Valva Aórtica/fisiopatologia , Velocidade do Fluxo Sanguíneo , Criança , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Imagens de Fantasmas , Valor Preditivo dos Testes , Artéria Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/fisiopatologia , Fluxo Sanguíneo Regional , Reprodutibilidade dos Testes , Estudos Retrospectivos , Índice de Gravidade de Doença , Adulto Jovem
3.
J Cardiovasc Magn Reson ; 22(1): 56, 2020 08 03.
Artigo em Inglês | MEDLINE | ID: mdl-32753047

RESUMO

BACKGROUND: Three-dimensional, whole heart, balanced steady state free precession (WH-bSSFP) sequences provide delineation of intra-cardiac and vascular anatomy. However, they have long acquisition times. Here, we propose significant speed-ups using a deep-learning single volume super-resolution reconstruction, to recover high-resolution features from rapidly acquired low-resolution WH-bSSFP images. METHODS: A 3D residual U-Net was trained using synthetic data, created from a library of 500 high-resolution WH-bSSFP images by simulating 50% slice resolution and 50% phase resolution. The trained network was validated with 25 synthetic test data sets. Additionally, prospective low-resolution data and high-resolution data were acquired in 40 patients. In the prospective data, vessel diameters, quantitative and qualitative image quality, and diagnostic scoring was compared between the low-resolution, super-resolution and reference high-resolution WH-bSSFP data. RESULTS: The synthetic test data showed a significant increase in image quality of the low-resolution images after super-resolution reconstruction. Prospectively acquired low-resolution data was acquired ~× 3 faster than the prospective high-resolution data (173 s vs 488 s). Super-resolution reconstruction of the low-resolution data took < 1 s per volume. Qualitative image scores showed super-resolved images had better edge sharpness, fewer residual artefacts and less image distortion than low-resolution images, with similar scores to high-resolution data. Quantitative image scores showed super-resolved images had significantly better edge sharpness than low-resolution or high-resolution images, with significantly better signal-to-noise ratio than high-resolution data. Vessel diameters measurements showed over-estimation in the low-resolution measurements, compared to the high-resolution data. No significant differences and no bias was found in the super-resolution measurements in any of the great vessels. However, a small but significant for the underestimation was found in the proximal left coronary artery diameter measurement from super-resolution data. Diagnostic scoring showed that although super-resolution did not improve accuracy of diagnosis, it did improve diagnostic confidence compared to low-resolution imaging. CONCLUSION: This paper demonstrates the potential of using a residual U-Net for super-resolution reconstruction of rapidly acquired low-resolution whole heart bSSFP data within a clinical setting. We were able to train the network using synthetic training data from retrospective high-resolution whole heart data. The resulting network can be applied very quickly, making these techniques particularly appealing within busy clinical workflow. Thus, we believe that this technique may help speed up whole heart CMR in clinical practice.


Assuntos
Aprendizado Profundo , Coração/diagnóstico por imagem , Interpretação de Imagem Assistida por Computador , Imagem por Ressonância Magnética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Coração/fisiopatologia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Fatores de Tempo , Fluxo de Trabalho , Adulto Jovem
4.
J Cardiovasc Magn Reson ; 22(1): 49, 2020 06 29.
Artigo em Inglês | MEDLINE | ID: mdl-32600420

RESUMO

BACKGROUND: The right ventricle (RV) often fails when functioning as the systemic ventricle, but the cause is not understood. We tested the hypothesis that myofiber organization is abnormal in the failing systemic right ventricle. METHODS: We used diffusion-weighted cardiovascular magnetic resonance imaging to examine 3 failing hearts explanted from young patients with a systemic RV and one structurally normal heart with postnatally acquired RV hypertrophy for comparison. Diffusion compartment imaging was computed to separate the free diffusive component representing free water from an anisotropic component characterizing the orientation and diffusion characteristics of myofibers. The orientation of each anisotropic compartment was displayed in glyph format and used for qualitative description of myofibers and for construction of tractograms. The helix angle was calculated across the ventricular walls in 5 locations and displayed graphically. Scalar parameters (fractional anisotropy and mean diffusivity) were compared among specimens. RESULTS: The hypertrophied systemic RV has an inner layer, comprising about 2/3 of the wall, composed of hypertrophied trabeculae and an epicardial layer of circumferential myofibers. Myofibers within smaller trabeculae are aligned and organized with parallel fibers while larger, composite bundles show marked disarray, largely between component trabeculae. We observed a narrow range of helix angles in the outer, compact part of the wall consistent with aligned, approximately circumferential fibers. However, there was marked variation of helix angle in the inner, trabecular part of the wall consistent with marked variation in fiber orientation. The apical whorl was disrupted or incomplete and we observed myocardial whorls or vortices at other locations. Fractional anisotropy was lower in abnormal hearts while mean diffusivity was more variable, being higher in 2 but lower in 1 heart, compared to the structurally normal heart. CONCLUSIONS: Myofiber organization is abnormal in the failing systemic RV and might be an important substrate for heart failure and arrhythmia. It is unclear if myofiber disorganization is due to hemodynamic factors, developmental problems, or both.


Assuntos
Imagem de Difusão por Ressonância Magnética , Cardiopatias Congênitas/diagnóstico por imagem , Insuficiência Cardíaca/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Miocárdio/patologia , Miofibrilas/patologia , Disfunção Ventricular Direita/diagnóstico por imagem , Função Ventricular Direita , Adolescente , Pré-Escolar , Feminino , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/patologia , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Ventrículos do Coração/patologia , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/cirurgia , Humanos , Masculino , Valor Preditivo dos Testes , Disfunção Ventricular Direita/patologia , Disfunção Ventricular Direita/fisiopatologia , Disfunção Ventricular Direita/cirurgia , Adulto Jovem
6.
J Am Soc Echocardiogr ; 33(6): 658-665, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32503702
7.
Heart ; 106(17): 1302-1309, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32522822

RESUMO

Adults with congenital heart disease (ACHD) may be at high risk in the case of COVID-19. Due to the heterogeneity of ACHD and secondary complications, risk profiles are, however, not uniform. This document aims to give an overview of relevant data and outline our pragmatic approach to disease prevention and management. Based on anatomy and additional physiological factors including symptoms, exercise capacity, heart failure, pulmonary hypertension and cyanosis, we propose a pragmatic approach to categorising patients into low-risk, intermediate-risk and high-risk groups. We regard especially patients with complex cyanotic conditions, those with palliated univentricular hearts, heart failure, severe valvular disease or pulmonary hypertension as high-risk patients. To avoid infection, we recommend self-isolation and exemption from work for these cohorts. Infected ACHD patients with low or moderate risk and without signs of deterioration may be remotely followed and cared for at home while in self isolation. High-risk patients or those with signs of respiratory or cardiovascular impairment require admission ideally at a tertiary ACHD centre. Especially patients with complex, cyanotic disease, heart failure and arrhythmias require particular attention. Treatment in patients with cyanotic heart disease should be guided by the relative degree of desaturation compared with baseline and lactate levels rather than absolute oxygen saturation levels. Patients with right heart dilatation or dysfunction are potentially at increased risk of right heart failure as mechanical ventilation and acute respiratory distress syndrome can lead to increase in pulmonary arterial pressures.


Assuntos
Controle de Doenças Transmissíveis/métodos , Infecções por Coronavirus , Cardiopatias Congênitas , Pandemias , Administração dos Cuidados ao Paciente/métodos , Pneumonia Viral , Adulto , Betacoronavirus , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Humanos , Pandemias/prevenção & controle , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Medição de Risco
8.
Clin Imaging ; 66: 121-126, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32480266

RESUMO

BACKGROUND AND PURPOSE: Subendocardial fibrosis is recognized finding in left ventricular noncompaction (LVNC); however, the evidence regarding the patterns and the frequency of late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMRI) is controversial. The present study sought to assess the frequency and patterns of LGE in LVNC. MATERIALS AND METHODS: Patients with a diagnosis of LVNC based on the echocardiographic CMRI criteria were enrolled in this retrospective study. The myocardial noncompacted-to-compacted ratio (NC/C) was perpendicularly measured on short-axis cine images. Two observers jointly assessed the presence of LGE on short-axis LGE images. The long-axis four-chamber and long-axis two-chamber images were used to confirm the presence of LGE if needed. RESULTS: A total of 42 patients, 20 females (47.7%) and 22 were males (52.3%), were included in the study. The median age of the patients was 32.4 years (range 18-63). LGE was identified in 2 out of 42 patients (4.7%) with LVNC. LGE was identified in the interventricular septum involving the subendocardial layer and noncompacted lateral myocardial wall involving the trabeculae at mid-ventricular and basal levels. CONCLUSION: LGE is uncommon in patients with LVNC. We highlight that the diagnosis of LVNC in patients with atypical LGE patterns, such as epicardial or transmural enhancement, should be reappraised and the other cardiac diseases should be discarded before establishing the final diagnosis.


Assuntos
Gadolínio , Imagem Cinética por Ressonância Magnética/métodos , Adolescente , Adulto , Meios de Contraste , Ecocardiografia , Feminino , Fibrose , Coração , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Aumento da Imagem , Imagem por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Estudos Retrospectivos , Adulto Jovem
9.
Am Heart J ; 224: 192-200, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32428726

RESUMO

BACKGROUND: Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity. METHODS: This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies. RESULTS: Fontan 1 (F1) included 546 participants (12 ±â€¯3.4 years); Fontan 2 (F2), 427 (19 ±â€¯3.4 years); and Fontan 3 (F3), 362 (21 ±â€¯3.5 years), with ~60% male at each time point. Height z-score was -0.67 ±â€¯-1.27, -0.60 ±â€¯1.34, and- 0.43 ±â€¯1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ±â€¯0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ±â€¯1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = -1.25 ±â€¯0.33; P ≤ .001). CONCLUSIONS: Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity.


Assuntos
Antropometria/métodos , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/fisiopatologia , Qualidade de Vida , Adolescente , Índice de Massa Corporal , Canadá/epidemiologia , Criança , Exercício Físico , Teste de Esforço , Feminino , Seguimentos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Período Pós-Operatório , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologia , Adulto Jovem
10.
J Cardiovasc Magn Reson ; 22(1): 33, 2020 05 14.
Artigo em Inglês | MEDLINE | ID: mdl-32404159

RESUMO

BACKGROUND: 3D-time resolved flow (4DF) cardiovascular magnetic resonance (CMR) with retrospective analysis of atrioventricular valve regurgitation (AVVR) allows for internal validation by multiple direct and indirect methods. Limited data exist on direct measurement of AVVR by 4DF CMR in pediatric congenital heart disease (CHD). We aimed to validate direct measurement of the AVVR jet as accurate and reliable compared to the volumetric method (clinical standard by 2D CMR) and as a superior method of internal validation than the annular inflow method. METHODS: We identified 44 consecutive patients with diverse CHD referred for evaluation of AVVR by CMR. 1.5 T or 3 T scanners, intravenous contrast, and a combination of parallel imaging and compressed sensing were used. Four methods of measuring AVVR volume (RVol) were used: volumetric method (VOL; the clinical standard) = stroke volume by 2D balanced steady-state free precession - semilunar valve forward flow (SLFF); annular inflow method (AIM) = atrioventricular valve forward flow [AVFF] - semilunar valve net flow (SLNF); and direct measurement (JET). AVFF was measured using static and retrospective valve tracking planes. SLFF, SLNF, AVFF, and JET were measured by 4DF phase contrast. Regurgitant fraction was calculated as [RVol/(RVol+SLNF)]× 100. Statistical methods included Spearman, Wilcoxon rank sum test/Student paired t-test, Bland Altman analysis, and intra-class coefficient (ICC), where appropriate. RESULTS: Regurgitant fraction by JET strongly correlated with the indirect methods (VOL and AIM) (ρ = 0.73-0.80, p < 0.001) and was similar to VOL with a median difference (interquartile range) of - 1.5% (- 8.3-7.2%; p = 0.624). VOL had weaker correlations with AIM and JET (ρ = 0.69-0.73, p < 0.001). AIM underestimated RF by 3.6-6.9% compared to VOL and JET, p < 0.03. Intra- and inter- observer reliability were excellent for all methods (ICC 0.94-0.99). The mean (±standard deviation) inter-observer difference for VOL was 2.4% (±5.1%), p < 0.05. CONCLUSIONS: In a diverse cohort of pediatric CHD, measurement of AVVR using JET is accurate and reliable to VOL and is a superior method of internal validation compared to AIM. This study supports use of 4DF CMR for measurement of AVVR, obviating need for expert prospective prescription during image acquisition by 2D CMR.


Assuntos
Cardiopatias Congênitas/diagnóstico por imagem , Hemodinâmica , Interpretação de Imagem Assistida por Computador , Imagem por Ressonância Magnética , Insuficiência da Valva Mitral/diagnóstico por imagem , Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/diagnóstico por imagem , Adolescente , Fatores Etários , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Valva Mitral/fisiopatologia , Insuficiência da Valva Mitral/fisiopatologia , Variações Dependentes do Observador , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/fisiopatologia , Adulto Jovem
11.
Arch Cardiovasc Dis ; 113(8-9): 492-502, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32461091

RESUMO

The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD. In light of the unique issues, challenges and considerations involved in managing arrhythmias in this growing, ageing and heterogeneous patient population and in children, it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The introduction of catheter ablation techniques has greatly improved the treatment of cardiac arrhythmias. However, catheter ablation in adults or children with CHD and in children without CHD is more technically demanding, potentially causing various complications, and thus requires a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding required technical competence and equipment are lacking in this situation, the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Affiliate Group of Paediatric and Adult Congenital Cardiology have decided to produce a common position paper compiled from expert opinions from cardiac electrophysiology and paediatric cardiology. The paper details the features of an interventional cardiac electrophysiology centre that are required for ablation procedures in adults with CHD and in children, the importance of being able to diagnose, monitor and manage complications associated with ablations in these patients and the supplemental hospital-based resources required, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Lastly, the need for quality evaluations and French registries of ablations in these populations is discussed. The purpose of this consensus statement is therefore to define optimal conditions for the delivery of invasive care regarding ablation of arrhythmias in adults with CHD and in children, and to provide expert and - when possible - evidence-based recommendations on best practice for catheter-based ablation procedures in these specific populations.


Assuntos
Arritmias Cardíacas/cirurgia , Procedimentos Cirúrgicos Cardíacos , Cardiologistas/normas , Serviço Hospitalar de Cardiologia/normas , Ablação por Cateter/normas , Competência Clínica/normas , Criocirurgia/normas , Cardiopatias Congênitas/cirurgia , Adolescente , Adulto , Fatores Etários , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Ablação por Cateter/efeitos adversos , Ablação por Cateter/mortalidade , Criança , Pré-Escolar , Consenso , Criocirurgia/efeitos adversos , Criocirurgia/mortalidade , Técnicas Eletrofisiológicas Cardíacas/normas , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Frequência Cardíaca , Humanos , Lactente , Recém-Nascido , Fatores de Risco , Sobreviventes , Resultado do Tratamento , Adulto Jovem
12.
Anesth Analg ; 131(2): 403-409, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32459667
14.
PLoS One ; 15(4): e0232606, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32353083

RESUMO

INTRODUCTION: The examination of the fetal heart in mid-pregnancy is by ultrasound examination. The quality of the examination is highly dependent on the skill of the sonographer, fetal position and maternal body mass index. An additional tool that is less dependent on human experience and interpretation is desirable. The fetal electrocardiogram (ECG) could fulfill this purpose. We aimed to show the feasibility of recording a standardized fetal ECG in mid-pregnancy and explored its possibility to detect congenital heart disease (CHD). MATERIALS AND METHODS: Women older than 18 years of age with an uneventful pregnancy, carrying a healthy singleton fetus with a gestational age between 18 and 24 weeks were included. A fetal ECG was performed via electrodes on the maternal abdomen. After removal of interferences, a vectorcardiogram was constructed. Based on the ultrasound assessment of the fetal orientation, the vectorcardiogram was rotated to standardize for fetal orientation and converted into a 12-lead ECG. Median ECG waveforms for each lead were calculated. RESULTS: 328 fetal ECGs were recorded. 281 were available for analysis. The calculated median ECG waveform showed the electrical heart axis oriented to the right and inferiorly i.e. a negative QRS deflection in lead I and a positive deflection in lead aVF. The two CHD cases show ECG abnormalities when compared to the mean ECG of the healthy cohort. DISCUSSION: We have presented a method for estimating a standardized 12-lead fetal ECG. In mid-pregnancy, the median electrical heart axis is right inferiorly oriented in healthy fetuses. Future research should focus on fetuses with congenital heart disease.


Assuntos
Eletrocardiografia/normas , Coração Fetal/fisiologia , Cardiopatias Congênitas/diagnóstico , Diagnóstico Pré-Natal/métodos , Adulto , Estudos de Casos e Controles , Estudos Transversais , Eletrocardiografia/instrumentação , Estudos de Viabilidade , Feminino , Idade Gestacional , Cardiopatias Congênitas/fisiopatologia , Frequência Cardíaca Fetal/fisiologia , Humanos , Lactente , Países Baixos , Projetos Piloto , Gravidez , Segundo Trimestre da Gravidez , Valores de Referência
15.
Int J Cardiovasc Imaging ; 36(8): 1567-1573, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32335820

RESUMO

Beginning with the discovery of X-rays to the development of three-dimensional (3D) imaging, improvements in acquisition, post-processing, and visualization have provided clinicians with detailed information for increasingly accurate medical diagnosis and clinical management. This paper highlights advances in imaging technologies for congenital heart disease (CHD), medical adoption, and future developments required to improve pre-procedural and intra-procedural guidance.


Assuntos
Ecocardiografia Tridimensional , Cardiopatias Congênitas/diagnóstico por imagem , Coração/diagnóstico por imagem , Imageamento Tridimensional , Impressão Tridimensional , Tomografia Computadorizada por Raios X , Inteligência Artificial , Coração/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Humanos , Imagem por Ressonância Magnética , Modelos Anatômicos , Modelos Cardiovasculares , Valor Preditivo dos Testes , Prognóstico , Interpretação de Imagem Radiográfica Assistida por Computador
16.
Pediatr Cardiol ; 41(4): 654-668, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32342149

RESUMO

Diastolic dysfunction has correlated with adverse outcomes in various forms of unrepaired and repaired or palliated congenital heart disease (CHD). The non-invasive assessment of diastolic function in pediatric and adult patients with CHD remains challenging. Atrial size has a pivotal role in the evaluation of diastolic function; however, a growing body of evidence supports the additional role of atrial function as a more sensitive parameter of ventricular diastolic dysfunction. While the importance of atrial function is becoming clearer in adult acquired heart disease, it remains ambiguous in those with CHD. In this review we set the stage with the current understanding of diastolic function assessment in CHD, followed by insight into atrial form and function including its non-invasive assessment, and conclude with the current knowledge of atrial function in CHD. A general pattern of decrease in reservoir and conduit function with compensatory increase followed by decompensatory decrease in contractile function seems to be the common pathway of atrial dysfunction in most forms of CHD.


Assuntos
Função Atrial , Cardiopatias Congênitas/fisiopatologia , Diástole , Ecocardiografia Doppler , Feminino , Átrios do Coração/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino
17.
BMC Med Genet ; 21(1): 78, 2020 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-32293321

RESUMO

BACKGROUND: The protein Kruppel-like factor 13 (KLF13) is a member of the KLF family and has been identified as a cardiac transcription factor that is involved in heart development. However, the relationship between KLF13 variants and CHDs in humans remains largely unknown. The present study aimed to screen the KLF13 variants in CHD patients and genetically analyze the functions of these variants. METHODS: KLF13 variants were sequenced in a cohort of 309 CHD patients and population-matched healthy controls (n = 200) using targeted sequencing. To investigate the effect of variants on the functional properties of the KLF13 protein, the expression and subcellular localization of the protein, as well as the transcriptional activities of downstream genes and physical interactions with other transcription factors, were assessed. RESULTS: Two heterozygous variants, c.487C > T (P163S) and c.467G > A (S156N), were identified in two out of 309 CHD patients with tricuspid valve atresia and transposition of the great arteries, respectively. No variants were found among healthy controls. The variant c.467G > A (S156N) had increased protein expression and enhanced functionality compared with the wild type, without affecting the subcellular localization. The other variant, c.487C > T (P163S), did not show any abnormalities in protein expression or subcellular localization; however, it inhibited the transcriptional activities of downstream target genes and physically interacted with TBX5, another cardiac transcription factor. CONCLUSION: Our results show that the S156N and P163S variants may affect the transcriptional function of KLF13 and physical interaction with TBX5. These results identified KLF13 as a potential genetic risk factor for congenital heart disease.


Assuntos
Proteínas de Ciclo Celular/genética , Cardiopatias Congênitas/genética , Fatores de Transcrição Kruppel-Like/genética , Proteínas Repressoras/genética , Proteínas com Domínio T/genética , Atresia Tricúspide/genética , Criança , Pré-Escolar , Feminino , Regulação da Expressão Gênica/genética , Coração/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Heterozigoto , Humanos , Lactente , Recém-Nascido , Masculino , Polimorfismo de Nucleotídeo Único/genética , Transposição dos Grandes Vasos/metabolismo , Transposição dos Grandes Vasos/fisiopatologia , Atresia Tricúspide/fisiopatologia
19.
Pan Afr Med J ; 35: 6, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32128022

RESUMO

Women with complex functionally univentricular hearts rarely survive into adulthood without corrective or palliative surgery. Reports of pregnancy outcome in this group of patients in resource-poor settings are sparse. We report a case of unrepaired pulmonary atresia ventricular septal defect (VSD) with major aorto-pulmonary collateral arteries (MAPCA) who survived into adulthood and was able to complete a successful pregnancy in a resource-poor country.


Assuntos
Cardiopatias Congênitas/fisiopatologia , Comunicação Interventricular/fisiopatologia , Complicações Cardiovasculares na Gravidez/fisiopatologia , Atresia Pulmonar/fisiopatologia , Adulto , Países em Desenvolvimento , Feminino , Humanos , Gravidez , Resultado da Gravidez
20.
Georgian Med News ; (298): 42-46, 2020 Jan.
Artigo em Russo | MEDLINE | ID: mdl-32141846

RESUMO

The main initiator of the development of PAH is the development of endothelial dysfunction of the pulmonary vessels. The cause of this dysfunction is associated with functional disorders of the serotonin system. This research is devoted to study the role of the serotonin system in the development mechanism of PAH in children with CHD. We examined 30 young children (from 1 month to 2 years) with congenital heart defects (CHD), which were divided into 3 groups: group I - 12 children with CHD complicated by pulmonary arterial hypertension (PAH); Group II - 9 children with CHD, not complicated by PAH; Group III - 9 relatively healthy children (control). The serotonin content of platelets, serum and serotonin transporter (SERT) in platelets was determined. To determine serotonin and its carrier, a quantitative enzyme-linked immunosorbent assay (Elisa kit) was used. The study was performed using the Serotonin ELISA diagnostic kit, IBL Hamburg at the automatic ELISA of the Evolis robotic station (BioRad). The results of our studies showed a pronounced (p <0.001) increase in SERT concentration in platelets in the group of children with CHD complicated by PAH compared with the group of children with CHD and the control group. The amount of serotonin in serum tended to increase in this group compared with the control groups and CHD, and the amount of serotonin in platelets tended to decrease. It is likely that an increase in the serotonin transporter in platelets against the background of an increase in serotonin in the blood plasma and a decrease in serotonin in the platelets indicates the inadequacy of SERT activity as a serotonin carrier. However, by activating the proliferation of smooth muscle cells in the pulmonary artery, SERT leads to the development of PAH in children with congenital heart defects. It is necessary to continue research in this direction, which will reveal the molecular mechanisms by which SERT regulates PASMC proliferation and clarify the role of serotonin and its transporter in the development of PAH in children with CHD.


Assuntos
Plaquetas/metabolismo , Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/etiologia , Artéria Pulmonar/metabolismo , Proteínas da Membrana Plasmática de Transporte de Serotonina/sangue , Serotonina/sangue , Biomarcadores/sangue , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/fisiopatologia , Humanos , Hipertensão Pulmonar/fisiopatologia , Hipertensão Arterial Pulmonar , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar
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