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1.
Pediatr Cardiol ; 42(6): 1442-1448, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33963437

RESUMO

Our institution established a Fontan surveillance plan, which included ambulatory rhythm monitoring (ARM) at 6, 10, 13, 16 and 19 years old, for early detection of Fontan-associated complications. We conducted a retrospective chart review of Fontan patients followed at our institution 2014-2018 to determine the utility of surveillance ARMs. 139 ARMs from 83 patients were included. ARMs with supraventricular tachycardia, sinus node dysfunction, accelerated junctional rhythm, > 1st degree atrioventricular block, and complex ventricular ectopy were classified as positive for arrhythmia. Arrhythmias were occult if detected on surveillance ARM. The ARM indication was surveillance in 78 (56%) and clinically indicated in 61 (44%). 52 (37%) ARMs in 27 (33%) patients had an arrhythmia. There was no difference in the age of patients with and without arrhythmias [median 10.9 (6.5, 17.1 years) vs. 8.8 (7, 13.6 years), p = 0.5]. Clinically indicated ARMs more frequently demonstrated arrhythmias than surveillance ARMs (52% vs. 26%, p < 0.01). Compared to patients without arrhythmias, those with arrhythmias were more likely to be female (48% vs. 23%, p = 0.02), have a single right ventricle (46% vs. 19%, p < 0.01) and longer QRS duration on ECG [100 (91, 116 ms) vs. 94 (84, 104 ms), p = 0.046]. Patients with occult arrhythmias were less likely to have moderate to severe atrioventricular valvar regurgitation (0% vs. 46%; p = 0.04) or ventricular dysfunction (0% vs. 46%; p = 0.04) than those with clinical arrhythmia(s). Arrhythmia findings resulted in change in management for 16/52 (31%) ARMs. The findings suggest the frequent presence of arrhythmias on periodic ARMs in patients following the Fontan procedure regardless of symptomatic status.


Assuntos
Eletrocardiografia Ambulatorial/estatística & dados numéricos , Técnica de Fontan/métodos , Cardiopatias Congênitas/fisiopatologia , Adolescente , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
2.
J Comput Assist Tomogr ; 45(3): 415-420, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33797443

RESUMO

OBJECTIVES: The aim of this study was to determine the difference and correlation in pulmonary artery (PA) size when measured from the electrocardiogram (ECG)-gated computed tomography (CT) and non-ECG-gated CT. METHODS: In the retrospective study, 279 patients who underwent both ECG-gated CT and non-ECG-gated CT were enrolled. Maximum and minimum diameters of main pulmonary artery (MPA), right pulmonary artery (RPA), and ascending aorta (AAO) were measured, whereas mean diameters of MPA and RPA were obtained. The same PA size parameters were also measured on non-ECG-gated CT. RESULTS: There was a significant difference in maximum and minimum PA diameters between ECG-gated CT and non-ECG-gated CT, whereas mean PA diameters showed no statistically difference. The PA parameters showed a strong positive correlation between these 2 examinations. CONCLUSIONS: The PA size was different between ECG-gated CT and non-ECG-gated CT, whereas the PA size parameters on non-ECG-gated CT could be used to predict those with ECG-gated CT, which allow for confident prediction of pulmonary hypertension and guide further surgical intervention.


Assuntos
Técnicas de Imagem de Sincronização Cardíaca/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Eletrocardiografia , Feminino , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Hipertensão Pulmonar/patologia , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/patologia , Artéria Pulmonar/fisiopatologia , Estudos Retrospectivos , Sensibilidade e Especificidade , Adulto Jovem
3.
Pediatr Cardiol ; 42(6): 1356-1364, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33881601

RESUMO

The study aimed to assess the long-term outcomes after fenestration closure in patients at risk for Fontan failure. Of 119 patients who underwent Fontan operation between 1995 and 2004, fenestration was not created in 89 patients (NF group) and created in 30 patients with hypoplastic left heart syndrome, heterotaxy syndrome, high pulmonary arterial pressure, high systemic ventricular end-diastolic pressure, low ventricular ejection fraction, or atrioventricular valve regurgitation. All fenestrations were closed spontaneously or by catheter/surgical interventions, excepting two patients, and therefore, they were excluded. In fenestration group, patients with pre-Fontan mean pulmonary arterial pressure ≥ 15 mmHg or systemic atrioventricular valve regurgitation ≥ moderate were classified as high-risk Fontan candidates (F-HR group, n = 16), and the remaining patients were as standard-risk (F-SR group, n = 12). Protein-losing enteropathy-free survival rates did not differ among the three groups (p = 0.72). Serial follow-up catheter examinations after Fontan operation were completed in 69 patients in NF group and 11 patients in both F-SR and F-HR groups. Cardiac index and pulmonary vascular resistance significantly and similarly decreased over time in all groups, though the F-HR group showed lowest arterial oxygen saturation, lowest cardiac index, and highest pulmonary vascular resistance. The F-HR group also showed much veno-venous collaterals (p = 0.049), low peak oxygen consumption (p = 0.019), and low anaerobic threshold (p = 0.023) as compared to those in the F-SR group. In F-HR group, cyanosis remained after fenestration closure due to transformation from fenestration to veno-venous collaterals, which resulted in elevation of pulmonary vascular resistance, low cardiac index, and deterioration of exercise tolerance.


Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Hemodinâmica/fisiologia , Cateterismo Cardíaco/métodos , Criança , Pré-Escolar , Feminino , Seguimentos , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Fatores de Tempo , Resultado do Tratamento
4.
J Am Coll Cardiol ; 77(13): 1644-1655, 2021 04 06.
Artigo em Inglês | MEDLINE | ID: mdl-33795039

RESUMO

BACKGROUND: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. OBJECTIVES: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. METHODS: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.


Assuntos
COVID-19 , Procedimentos Cirúrgicos Cardíacos , Cianose , Cardiopatias Congênitas , Hipertensão Pulmonar , Adulto , COVID-19/mortalidade , COVID-19/terapia , Teste para COVID-19/métodos , Procedimentos Cirúrgicos Cardíacos/métodos , Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Causalidade , Comorbidade , Cianose/diagnóstico , Cianose/etiologia , Cianose/mortalidade , Feminino , Saúde Global/estatística & dados numéricos , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Hospitalização/estatística & dados numéricos , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Masculino , Mortalidade , Gravidade do Paciente , Fatores de Risco , SARS-CoV-2/isolamento & purificação , Avaliação de Sintomas
5.
Am J Physiol Heart Circ Physiol ; 320(5): H1873-H1886, 2021 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-33739154

RESUMO

Compared with acyanotic congenital heart disease (CHD), cyanotic CHD has an increased risk of lifelong mortality and morbidity. These adverse outcomes may be attributed to delayed cardiomyocyte maturation, since the transition from a hypoxic fetal milieu to oxygen-rich postnatal environment is disrupted. We established a rodent model to replicate hypoxic myocardial conditions spanning perinatal development, and tested the hypothesis that chronic hypoxia impairs cardiac development. Pregnant mice were housed in hypoxia beginning at embryonic day 16. Pups stayed in hypoxia until postnatal day (P)8 when cardiac development is nearly complete. Global gene expression was quantified at P8 and at P30, after recovering in normoxia. Phenotypic testing included electrocardiogram, echocardiogram, and ex vivo electrophysiology study. Hypoxic P8 animals were 47% smaller than controls with preserved heart size. Gene expression was grossly altered by hypoxia at P8 (1,427 genes affected), but normalized after recovery (P30). Electrocardiograms revealed bradycardia and slowed conduction velocity in hypoxic animals at P8, with noticeable resolution after recovery (P30). Notable differences that persisted after recovery (P30) included a 65% prolongation in ventricular effective refractory period, sinus node dysfunction, 23% reduction in ejection fraction, and 16% reduction in fractional shortening in animals exposed to hypoxia. We investigated the impact of chronic hypoxia on the developing heart. Perinatal hypoxia was associated with changes in gene expression and cardiac function. Persistent changes to the electrophysiological substrate and contractile function warrant further investigation and may contribute to adverse outcomes observed in the cyanotic CHD population.NEW & NOTEWORTHY We utilized a new mouse model of chronic perinatal hypoxia to simulate the hypoxic myocardial conditions present in cyanotic congenital heart disease. Hypoxia caused numerous abnormalities in cardiomyocyte gene expression, the electrophysiologic substrate of the heart, and contractile function. Taken together, alterations observed in the neonatal period suggest delayed cardiac development immediately following hypoxia.


Assuntos
Cianose/etiologia , Coração Fetal/crescimento & desenvolvimento , Cardiopatias Congênitas/etiologia , Hipóxia/complicações , Fatores Etários , Animais , Animais Recém-Nascidos , Doença Crônica , Cianose/genética , Cianose/metabolismo , Cianose/fisiopatologia , Modelos Animais de Doenças , Feminino , Coração Fetal/metabolismo , Hipóxia Fetal/complicações , Hipóxia Fetal/genética , Hipóxia Fetal/metabolismo , Hipóxia Fetal/fisiopatologia , Regulação da Expressão Gênica no Desenvolvimento , Idade Gestacional , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/metabolismo , Cardiopatias Congênitas/fisiopatologia , Frequência Cardíaca , Hipóxia/genética , Hipóxia/metabolismo , Hipóxia/fisiopatologia , Camundongos , Contração Miocárdica , Miócitos Cardíacos/metabolismo , Organogênese , Gravidez , Efeitos Tardios da Exposição Pré-Natal
6.
Am J Cardiol ; 149: 119-125, 2021 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-33757789

RESUMO

In the pediatric population, exercise capacity differs between females and males and the gap widens through adolescence. However, specific age- and sex-based changes in adolescents with congenital heart disease and Fontan palliation have not been reported. The purpose of the current study is to identify age- and sex-specific changes in exercise performance at peak and ventilatory anaerobic threshold (AT) for adolescents with Fontan physiology. Retrospective review of the Pediatric Heart Network Fontan cross sectional study (Fontan 1) public use dataset. Comparisons were made for peak and AT exercise parameters for females and males at 2-year age intervals. In addition, normative values were generated by sex and age at 2-year intervals. χ2 test was used for comparison for categorical variables. Changes in exercise parameters between age groups by sex were compared by ANOVA with post-hoc analysis. Exercise testing was performed in 411 patients. AT was reached in 317 subjects (40% female), of whom, 166 (43% female) reached peak exercise. Peak oxygen consumption decreased 32% through adolescence in females and did not have the typical increase through adolescence for males. Oxygen consumption at AT also decreased with age in both sexes. In conclusion, age- and sex-based exercise performance for adolescents with Fontan physiology are predictably low, but there are additional significant decreases through adolescence for this population, especially in females. We have established normative exercise values for several parameters for this population which will better identify at risk patients and allow for earlier intervention.


Assuntos
Limiar Anaeróbio/fisiologia , Tolerância ao Exercício/fisiologia , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Adolescente , Fatores Etários , Criança , Estudos Transversais , Teste de Esforço , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Consumo de Oxigênio/fisiologia , Valores de Referência , Estudos Retrospectivos , Fatores Sexuais
7.
Int Heart J ; 62(2): 448-452, 2021 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-33731520

RESUMO

While the advancement of perioperative management has expanded Fontan candidacy, not all patients have a successful postoperative course. Our case was a right isomerism patient who could not leave the ICU due to high central venous pressure and low output syndrome. Initial observation of the monitor ECG showed his rhythm to be supraventricular, however, an echocardiogram indicated simultaneous contraction of the atrium and ventricle, implying a junctional rhythm. While neither central venous pressure nor blood pressure improved with temporary pacing, better central venous and pulmonary venous blood flow patterns during pacing unraveled its positive impact. The patient successfully left the ICU after permanent pacing implantation. Hemodynamic study revealed a beneficial impact of atrial pacing in securing cardiac output and ventricular preload, lowering central venous pressure, and shortening blood transit time, which is partly attributed to the optimization of the fenestration function in reservation of the preload. Our case emphasizes the significant advantage of atrial pacing in a failing Fontan patient with junctional rhythm by reducing venous congestion and maximizing the benefit of fenestration.


Assuntos
Estimulação Cardíaca Artificial , Técnica de Fontan/métodos , Átrios do Coração/fisiopatologia , Cardiopatias Congênitas/terapia , Ventrículos do Coração/fisiopatologia , Hemodinâmica/fisiologia , Ecocardiografia , Eletrocardiografia , Átrios do Coração/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Masculino
8.
World J Pediatr Congenit Heart Surg ; 12(2): 176-184, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33684010

RESUMO

BACKGROUND: Early warning systems that utilize dense physiologic data and machine learning may aid prediction of decompensation after congenital heart surgery (CHS). The Compensatory Reserve Index (CRI) analyzes changing features of the pulse waveform to predict hemodynamic decompensation in adults, but it has never been studied after CHS. This study sought to understand the feasibility, safety, and potential utility of CRI monitoring after CHS with cardiopulmonary bypass (CPB). METHODS: A single-center prospective pilot cohort of patients undergoing pulmonary valve replacement was studied. Compensatory Reserve Index was continuously measured from preoperative baseline through the first 24 postoperative hours. Average CRI values during selected procedural phases were compared between patients with an intensive care unit (ICU) length of stay (LOS) <3 days versus LOS ≥3 days. RESULTS: Twenty-three patients were enrolled. On average, 17,445 (±3,152) CRI data points were collected and 0.33% (±0.40) of data were missing per patient. There were no adverse events related to monitoring. Five (21.7%) patients had an ICU LOS ≥3 days. Compared to the ICU LOS <3 days group, the ICU LOS ≥3 days group had a greater decrease in CRI from baseline to immediately after CPB (-0.3 ± 0.1 vs -0.1 ± 0.2, P = .003) and were less likely to recover to baseline CRI during the monitoring period (20% vs 83%, P = .017). CONCLUSIONS: Compensatory Reserve Index monitoring after CHS with CPB seems feasible and safe. Early changes in CRI may precede meaningful clinical outcomes, but this requires further study.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Ponte Cardiopulmonar/métodos , Cardiopatias Congênitas/cirurgia , Hemodinâmica/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Tempo de Internação , Masculino , Projetos Piloto , Período Pós-Operatório , Estudos Prospectivos , Adulto Jovem
9.
World J Pediatr Congenit Heart Surg ; 12(2): 213-219, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33684011

RESUMO

BACKGROUND: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. METHODS: Retrospective chart review of adjustable PAB-outline anatomical variants palliated and patient outcomes. RESULTS: Sixteen patients underwent dilatable PAB-median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication-Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). CONCLUSIONS: Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients-allowing for interval growth or even serving as the definitive palliation.


Assuntos
Cardiopatias Congênitas/cirurgia , Cuidados Paliativos/métodos , Artéria Pulmonar/cirurgia , Circulação Pulmonar/fisiologia , Procedimentos Cirúrgicos Vasculares/métodos , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Artéria Pulmonar/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento
10.
PLoS Comput Biol ; 17(2): e1008089, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33591962

RESUMO

Short QT (SQT) syndrome is a genetic cardiac disorder characterized by an abbreviated QT interval of the patient's electrocardiogram. The syndrome is associated with increased risk of arrhythmia and sudden cardiac death and can arise from a number of ion channel mutations. Cardiomyocytes derived from induced pluripotent stem cells generated from SQT patients (SQT hiPSC-CMs) provide promising platforms for testing pharmacological treatments directly in human cardiac cells exhibiting mutations specific for the syndrome. However, a difficulty is posed by the relative immaturity of hiPSC-CMs, with the possibility that drug effects observed in SQT hiPSC-CMs could be very different from the corresponding drug effect in vivo. In this paper, we apply a multistep computational procedure for translating measured drug effects from these cells to human QT response. This process first detects drug effects on individual ion channels based on measurements of SQT hiPSC-CMs and then uses these results to estimate the drug effects on ventricular action potentials and QT intervals of adult SQT patients. We find that the procedure is able to identify IC50 values in line with measured values for the four drugs quinidine, ivabradine, ajmaline and mexiletine. In addition, the predicted effect of quinidine on the adult QT interval is in good agreement with measured effects of quinidine for adult patients. Consequently, the computational procedure appears to be a useful tool for helping predicting adult drug responses from pure in vitro measurements of patient derived cell lines.


Assuntos
Antiarrítmicos/farmacologia , Arritmias Cardíacas/tratamento farmacológico , Arritmias Cardíacas/fisiopatologia , Avaliação Pré-Clínica de Medicamentos/métodos , Sistema de Condução Cardíaco/anormalidades , Cardiopatias Congênitas/tratamento farmacológico , Cardiopatias Congênitas/fisiopatologia , Modelos Cardiovasculares , Miócitos Cardíacos/efeitos dos fármacos , Miócitos Cardíacos/fisiologia , Potenciais de Ação/efeitos dos fármacos , Adulto , Ajmalina/farmacologia , Algoritmos , Arritmias Cardíacas/genética , Linhagem Celular , Biologia Computacional , Avaliação Pré-Clínica de Medicamentos/estatística & dados numéricos , Canal de Potássio ERG1/genética , Eletrocardiografia , Sistema de Condução Cardíaco/fisiopatologia , Cardiopatias Congênitas/genética , Humanos , Técnicas In Vitro , Células-Tronco Pluripotentes Induzidas/efeitos dos fármacos , Células-Tronco Pluripotentes Induzidas/fisiologia , Ivabradina/farmacologia , Mexiletina/farmacologia , Mutação , Quinidina/farmacologia , Pesquisa Médica Translacional
11.
Am J Physiol Heart Circ Physiol ; 320(4): H1687-H1698, 2021 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-33635164

RESUMO

The purpose of this study was to directly assess (patho)physiology of intraventricular hemodynamic interplay between four-dimensional flow cardiovascular magnetic resonance imaging (4D Flow MRI)-derived vorticity with kinetic energy (KE) and viscous energy loss (EL) over the cardiac cycle and their association to ejection fraction (EF) and stroke volume (SV). Fifteen healthy subjects and thirty Fontan patients underwent whole heart 4D Flow MRI. Ventricular vorticity, KE, and EL were computed over systole (vorticity_volavg systole, KEavg systole, and ELavg systole) and diastole (vorticity_volavg diastole, KEavg diastole, and ELavg diastole). The association between vorticity_vol and KE and EL was tested by Spearman correlation. Fontan patients were grouped to normal and impaired EF groups. A significant correlation was found between SV and vorticity in healthy subjects (systolic: ρ = 0.84, P < 0.001; diastolic: ρ = 0.81, P < 0.001) and in Fontan patients (systolic: ρ = 0.61, P < 0.001; diastolic: ρ = 0.54, P = 0.002). Healthy subjects showed positive correlation between vorticity_vol versus KE (systole: ρ = 0.96, P < 0.001; diastole: ρ = 0.90, P < 0.001) and EL (systole: ρ = 0.85, P < 0.001; diastole: ρ = 0.84, P < 0.001). Fontan patients showed significantly elevated vorticity_vol compared with healthy subjects (vorticity_volavg systole: 3.1 [2.3-3.9] vs. 1.7 [1.3-2.4] L/s, P < 0.001; vorticity_volavg diastole: 3.1 [2.0-3.7] vs. 2.1 [1.6-2.8] L/s, P = 0.002). This elevated vorticity in Fontan patients showed strong association with KE (systole: ρ = 0.91, P < 0.001; diastole: ρ = 0.85, P < 0.001) and EL (systole: ρ = 0.82, P < 0.001; diastole: ρ = 0.89, P < 0.001). Fontan patients with normal EF showed significantly higher vorticity_volavg systole and ELavg systole, but significantly decreased KE avg diastole, in the presence of normal SV, compared with healthy subjects. Healthy subjects show strong physiological hemodynamic interplay between vorticity with KE and EL. Fontan patients demonstrate a pathophysiological hemodynamic interplay characterized by correlation of elevated vorticity with KE and EL in the presence of maintained normal stroke volume. Altered vorticity and energetic hemodynamics are found in the presence of normal EF in Fontan patients.NEW & NOTEWORTHY Physiologic intraventricular hemodynamic interplay/coupling is present in the healthy left ventricle between vorticity versus viscous energy loss and kinetic energy from four-dimensional flow cardiovascular magnetic resonance imaging (4D Flow MRI). Conversely, Fontan patients present compensatory pathophysiologic hemodynamic coupling by an increase in intraventricular vorticity that positively correlates to viscous energy loss and kinetic energy levels in the presence of maintained normal stroke volume. Altered vorticity and energetics are found in the presence of normal ejection fraction in Fontan patients.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Hemodinâmica , Imagem Cinética por Ressonância Magnética , Contração Miocárdica , Função Ventricular , Adolescente , Fenômenos Biomecânicos , Estudos de Casos e Controles , Criança , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Imagem de Perfusão do Miocárdio , Países Baixos , Valor Preditivo dos Testes , Estudos Prospectivos , Resultado do Tratamento
12.
Pediatr Cardiol ; 42(4): 774-783, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33523288

RESUMO

Previous research in children and adolescents with congenital heart defects presents contradictory findings concerning their physical activity (PA) level, due to methodological limitations in the PA assessment. The aim of the present cross-sectional study was to compare PA in children and adolescents treated for valvular aortic stenosis with healthy controls using an improved accelerometer method. Seven-day accelerometer data were collected from the hip in a national Swedish sample of 46 patients 6-18 years old treated for valvular aortic stenosis and 44 healthy controls matched for age, gender, geography, and measurement period. Sports participation was self-reported. Accelerometer data were processed with the new improved Frequency Extended Method and with the traditional ActiGraph method for comparison. A high-resolution PA intensity spectrum was investigated as well as traditional crude PA intensity categories. Children treated for aortic stenosis had a pattern of less PA in the highest intensity spectra and had more sedentary time, while the adolescent patients tended to be less physically active in higher intensities overall and with less sedentary time, compared to the controls. These patterns were evident using the Frequency Extended Method with the detailed PA intensity spectrum, but not to the same degree using the ActiGraph method and traditional crude PA intensity categories. Patients reported less sports participation than their controls in both age-groups. Specific differences in PA patterns were revealed using the Frequency Extended Method with the high-resolution PA intensity spectrum in Swedish children and adolescents treated for valvular aortic stenosis.


Assuntos
Estenose da Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/terapia , Exercício Físico , Acelerometria/métodos , Adolescente , Criança , Estudos de Coortes , Estudos Transversais , Feminino , Nível de Saúde , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Humanos , Masculino , Comportamento Sedentário , Autorrelato , Esportes
13.
Spine (Phila Pa 1976) ; 46(14): E791-E796, 2021 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-33394986

RESUMO

STUDY DESIGN: Case series. OBJECTIVE: In this study we focus on this group, and describe the largest cohort to date of single ventricle patients undergoing surgical correction of scoliosis at a single institution. SUMMARY OF BACKGROUND DATA: Outcomes of spinal surgery for patients with congenital heart disease (CHD) have evolved and most affected patients have uncomplicated procedures. The risk of perioperative complications remains highest in patients with single ventricle ("Fontan") physiology. METHODS: We reviewed patients with single ventricle CHD and operative scoliosis repair over a 25-year span. Patients who had undergone a Fontan, bidirectional Glenn, and/or Kawashima procedure before operative scoliosis repair were included. Patients were excluded if they lacked preoperative cardiac care and/or sufficient medical history at our institution. RESULTS: Twenty-three patients were included. The average age at surgery was 13.6 years (range, 5-23). Seventy percent of the cohort was female (16/23). The majority of patients underwent a Fontan before scoliosis surgery (20/23, 87%). Patients without intraoperative tranexamic acid (TXA) had a 67% complication rate (8/12) versus 36% (4/11) in those with intraoperative TXA (P = 0.29). There was a significant difference in estimated blood loss by weight (cc/kg) between non-TXA and TXA patients (P = 0.016). Twelve patients experienced complications (52%), all of which occurred postoperatively. There were no deaths, cerebrovascular events, adverse perioperative cardiac or hemodynamic complications, or wound infections. Using the Clavien-Dindo-Sink classification for postoperative complications, four patients had serious adverse events, including one permanent neurologic deficit. CONCLUSION: Spinal surgery for scoliosis has been performed in selected patients with single ventricle physiology at a single institution without mortality for 25 years. Operative blood loss may be reduced by routine use of TXA. Complications occur most commonly in the postoperative period, and can include pleural effusion.Level of Evidence: 4.


Assuntos
Cardiopatias Congênitas , Ventrículos do Coração , Escoliose , Adolescente , Adulto , Criança , Estudos de Coortes , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Procedimentos Ortopédicos/efeitos adversos , Complicações Pós-Operatórias , Escoliose/complicações , Escoliose/cirurgia , Ácido Tranexâmico/uso terapêutico , Adulto Jovem
14.
Cardiol Young ; 31(3): 344-351, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33407975

RESUMO

The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making.


Assuntos
Antiarrítmicos/uso terapêutico , Anticoagulantes/uso terapêutico , Antivirais/uso terapêutico , Arritmias Cardíacas/tratamento farmacológico , COVID-19/tratamento farmacológico , Cardiopatias Congênitas/terapia , Fatores Imunológicos/uso terapêutico , Monofosfato de Adenosina/análogos & derivados , Monofosfato de Adenosina/uso terapêutico , Alanina/análogos & derivados , Alanina/uso terapêutico , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Síndrome de Brugada/tratamento farmacológico , Síndrome de Brugada/epidemiologia , Síndrome de Brugada/fisiopatologia , COVID-19/epidemiologia , COVID-19/fisiopatologia , Procedimentos Cirúrgicos Cardíacos , Cardiologia , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/fisiopatologia , Transplante de Coração , Humanos , Transmissão Vertical de Doenças Infecciosas , Síndrome do QT Longo/tratamento farmacológico , Síndrome do QT Longo/epidemiologia , Síndrome do QT Longo/fisiopatologia , Miocardite/epidemiologia , Miocardite/fisiopatologia , Miocárdio , Pediatria , Medição de Risco , SARS-CoV-2 , Sociedades Médicas , Síndrome de Resposta Inflamatória Sistêmica/tratamento farmacológico , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Taquicardia Ventricular/tratamento farmacológico , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/fisiopatologia
15.
Arch Biochem Biophys ; 699: 108763, 2021 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-33460581

RESUMO

MicroRNAs (miRNAs) are small non-coding highly conserved RNA molecules that can act as master regulators of gene expression in a sequence-specific manner either by translation repression or mRNA degradation, influencing a wide range of biologic processes that are essential for the maintenance of cellular homeostasis. Chronic pediatric diseases are the leading cause of death worldwide among children and the recent evidence indicates that aberrant miRNA expression significantly contributes to the development of chronic pediatric diseases. This review focuses on the role of miRNAs in five major chronic pediatric diseases including bronchial asthma, congenital heart diseases, cystic fibrosis, type 1 diabetes mellitus, and epilepsy, and their potential use as novel biomarkers for the diagnosis and prognosis of these disorders.


Assuntos
Asma/fisiopatologia , Fibrose Cística/fisiopatologia , Diabetes Mellitus Tipo 1/fisiopatologia , Epilepsia/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , MicroRNAs/fisiologia , Asma/diagnóstico , Asma/metabolismo , Biomarcadores/sangue , Biomarcadores/metabolismo , Doença Crônica , Fibrose Cística/diagnóstico , Fibrose Cística/metabolismo , Diabetes Mellitus Tipo 1/diagnóstico , Diabetes Mellitus Tipo 1/metabolismo , Epilepsia/diagnóstico , Epilepsia/metabolismo , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/metabolismo , Humanos , MicroRNAs/sangue , MicroRNAs/metabolismo , Pediatria , Prognóstico
16.
Am J Cardiol ; 145: 135-142, 2021 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-33460605

RESUMO

In this international study, we (1) compared patient-reported outcomes (PROs) in adults with congenital heart disease (CHD) who had versus had not been hospitalized during the previous 12 month, (2) contrasted PROs in patients who had been hospitalized for cardiac surgery versus nonsurgical reasons, (3) assessed the magnitude of differences between the groups (i.e., effect sizes), and (4) explored differential effect sizes between countries. APPROACH-IS was a cross-sectional, observational study that enrolled 4,028 patients from 15 countries (median age 32 years; 53% females). Self-report questionnaires were administered to measure PROs: health status; anxiety and depression; and quality of life. Overall, 668 patients (17%) had been hospitalized in the previous 12 months. These patients reported poorer outcomes on all PROs, with the exception of anxiety. Patients who underwent cardiac surgery demonstrated a better quality of life compared with those who were hospitalized for nonsurgical reasons. For significant differences, the effect sizes were small, whereas they were negligible in nonsignificant comparisons. Substantial intercountry differences were observed. For various PROs, moderate to large effect sizes were found comparing different countries. In conclusion, adults with CHD who had undergone hospitalization in the previous year had poorer PROs than those who were medically stable. Researchers ought to account for the timing of recruitment when conducting PRO research as hospitalization can impact results.


Assuntos
Ansiedade/psicologia , Depressão/psicologia , Nível de Saúde , Cardiopatias Congênitas/fisiopatologia , Hospitalização , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Adulto , Procedimentos Cirúrgicos Cardíacos , Feminino , Cardiopatias Congênitas/psicologia , Cardiopatias Congênitas/cirurgia , Humanos , Internacionalidade , Masculino , Pessoa de Meia-Idade , Adulto Jovem
17.
Pediatr Cardiol ; 42(3): 560-568, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33481045

RESUMO

The inferior vena cava collapsibility index (IVCCI) is an ultrasound method used to assess central venous pressure (CVP). Our objective was to evaluate the correlation between IVCCI and CVP in children during the early period following surgery for congenital heart disease (CHD). Prospective study performed in a single tertiary care center. Patients ≤ 18 years old, who underwent cardiopulmonary bypass surgery for CHD, were enrolled. Ultrasound images of the inferior vena cava (IVC) were obtained at two time points; the first was within 2 h of arrival to the CICU and the second was 12-18 h from the first measurement. CVP measured by catheter placed during surgery was recorded within minutes of performing ultrasound. Maximum and minimum IVC diameters were measured by 2D images and M mode method. Seventy patients (47.1% males), with median age 7 months (IQR 4-47 months) and weight 6.9 kg (IQR 4.8-13.5 kg), were evaluated. The 2D IVCCI had inverse correlation with CVP in patients breathing spontaneously; r = - 0.76 (p < 0.01) and r = - 0.73 (p < 0.01), during the first and second measurements, respectively. The 2D IVCCI ≤ 0.24 had sensitivity, specificity, and negative predictive value of 94%, 79%, and 88.9% , respectively, to detect CVP ≥ 10 mmHg. No correlation was found between IVCCI and CVP during positive pressure ventilation. There is a significant inverse correlation between 2D IVCCI and CVP in spontaneously breathing children after surgery for CHD. Use of 2D IVCCI for monitoring CVP could reduce the frequency and duration of CVP catheters and their inherent complications.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Pressão Venosa Central , Cardiopatias Congênitas/cirurgia , Veia Cava Inferior/diagnóstico por imagem , Ponte Cardiopulmonar/métodos , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Masculino , Período Perioperatório , Valor Preditivo dos Testes , Estudos Prospectivos , Respiração , Ultrassonografia/métodos , Veia Cava Inferior/fisiopatologia
18.
BMC Cardiovasc Disord ; 21(1): 8, 2021 01 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407125

RESUMO

BACKGROUND: Partial anomalous pulmonary venous return (PAPVR) is a congenital heart defect. Reports of repair and treatment in pediatric cases have been published, but incidence of PAPVR in adults is not common. To our knowledge, there has not been a diagnosis of left-sided PAPVR after a heart transplant an in adult patient. CASE PRESENTATION: A 62-year-old patient with ischemic cardiomyopathy and systolic heart failure underwent orthotopic heart transplantation. The immediate post-operative course was remarkable for an elevated cardiac index and pulmonary artery pressures as well as decreased systemic vascular resistance. The post-operative echocardiogram did not reveal an intra-cardiac shunt. However, computed tomographic angiography (CTA) showed a left superior pulmonary vein draining into the innominate vein. Operative repair of the left superior pulmonary venous connection to the left atrial appendage was completed under cardiopulmonary bypass with beating heart. Her hemodynamics improved immediately, and she had an unremarkable postoperative course. CONCLUSIONS: While uncommon, any patient with a high cardiac output and abnormal hemodynamics after heart transplant should be evaluated for the existence of a shunt. While not a part of all traditional preoperative imaging protocols, a chest CTA should be considered if PAPVR is suspected as it can both diagnose the condition and enable a plot of the corrective course of surgical action.


Assuntos
Cardiopatias Congênitas/fisiopatologia , Insuficiência Cardíaca Sistólica/cirurgia , Transplante de Coração , Hemodinâmica , Circulação Pulmonar , Veias Pulmonares/fisiopatologia , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca Sistólica/diagnóstico por imagem , Insuficiência Cardíaca Sistólica/fisiopatologia , Humanos , Pessoa de Meia-Idade , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Recuperação de Função Fisiológica , Resultado do Tratamento
19.
BMC Cardiovasc Disord ; 21(1): 9, 2021 01 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407150

RESUMO

BACKGROUND: Both systemic-pulmonary shunt and arterial duct stent could be the palliation of duct-dependent pulmonary circulation. We aimed to compare the safety and efficacy of the two approaches. METHODS: The PubMed, EMBASE, and Cochrane Library databases were searched through December 2019 for studies comparing stent implantation and surgical shunt in duct-dependent pulmonary circulation. The baseline characteristics included ventricle physiology and cardiac anomaly. The main outcomes were hospital stay and total mortality. Additional outcomes included procedural complications, intensive care unit (ICU) stay, pulmonary artery growth at follow-up, and other indexes. A random- or fixed-effects model was used to summarize the estimates of the mean difference (MD)/risk ratio (RR) with 95% confidence intervals (CIs). RESULTS: In total, 757 patients with duct-dependent pulmonary circulation from six studies were included. Pooled estimates of hospital stay (MD, - 4.83; 95% CI - 7.92 to - 1.74; p < 0.05), total mortality (RR 0.44; 95% CI 0.28-0.70; p < 0.05), complications (RR 0.49; 95% CI 0.30-0.81; p < 0.05) and ICU stay (MD, - 4.00; 95% CI - 5.96 to - 2.04; p < 0.05) favored the stent group. Significant differences were found in the proportions of patients with a single ventricle (RR 0.82; 95% CI 0.68-0.98; p < 0.05) or a double ventricle (RR 1.23; 95% CI 1.07-1.41; p < 0.05) between the stent and shunt groups. Additionally, pulmonary artery growth showed no significant differences between the two groups. CONCLUSION: Arterial duct stent appears to have not inferior outcomes of procedural complications, mortality, hospital and ICU stay, and pulmonary artery growth in selected patients compared with a surgical shunt. TRIAL REGISTRATION: CRD42019147672.


Assuntos
Procedimento de Blalock-Taussig , Cateterismo Cardíaco/instrumentação , Permeabilidade do Canal Arterial/terapia , Cardiopatias Congênitas/terapia , Hemodinâmica , Artéria Pulmonar/cirurgia , Circulação Pulmonar , Stents , Procedimento de Blalock-Taussig/efeitos adversos , Procedimento de Blalock-Taussig/mortalidade , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/mortalidade , Criança , Pré-Escolar , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/mortalidade , Permeabilidade do Canal Arterial/fisiopatologia , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Masculino , Cuidados Paliativos , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/crescimento & desenvolvimento , Recuperação de Função Fisiológica , Fatores de Tempo , Resultado do Tratamento
20.
BMC Cardiovasc Disord ; 21(1): 17, 2021 01 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407155

RESUMO

BACKGROUND: Short QT syndrome is a rare, inherited channelopathy associated with sudden cardiac arrest (SCA) but the characteristics and prognosis of short QT interval (SQTI) in Korean patients remain unclear. This study aimed to determine the clinical characteristics and outcomes of SQTI in a Korean population. METHODS: Consecutive patients with SQTI from January 1999 to March 2019 in three university hospitals in South Korea were recruited. SQTI was defined as a Bazett's formula-corrected QT interval (QTc) ≤ 340 ms in serial electrocardiograms. Age- and sex-matched patients with a normal QTc and without overt cardiovascular disease were included in a 1:4 ratio. Clinical and ECG features and outcomes were compared between patients with and without SQTI. RESULTS: 34 patients with SQTI [age, 23.5 (21-30.5) years; 31 male] were followed up for 4.8 (2.0-7.8) years. Early repolarization, tall T wave, and U wave were significantly more frequent in patients with SQTI than in those without SQTI. QT dispersion [44.0 (28.0-73.0) vs. 20.0 (12.0-35.0) ms, P < 0.001] was significantly wider and heart rate [52.0 (47.0-58.0) vs. 70.0 (62.3-84.0)/min, P < 0.001] was significantly slower in patients with SQTI than in those without. Atrial fibrillation (AF, 11.8% vs. 2.2%, P = 0.030) and ventricular arrhythmia (VA)/SCA (8.7% vs. 0%, P = 0.007) were significantly more frequent in patients with SQTI than in those without. SQTI was significantly associated with AF [odds ratio, 5.911; 95% confidence interval, 1.257-27.808; P = 0.025] and VA/SCA. CONCLUSIONS: In this subset of Korean population, SQTI was associated with AF and VA/SCA.


Assuntos
Arritmias Cardíacas/fisiopatologia , Fibrilação Atrial/fisiopatologia , Sistema de Condução Cardíaco/anormalidades , Sistema de Condução Cardíaco/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Frequência Cardíaca , Potenciais de Ação , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/mortalidade , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/mortalidade , Morte Súbita Cardíaca/epidemiologia , Eletrocardiografia , Feminino , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/mortalidade , Humanos , Masculino , Prevalência , Prognóstico , República da Coreia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Adulto Jovem
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