RESUMO
BACKGROUND: Disordered lymphatic drainage is common in congenital heart diseases (CHD), but thoracic duct (TD) drainage patterns in heterotaxy have not been described in detail. This study sought to describe terminal TD sidedness in heterotaxy and its associations with other anatomic variables. METHODS: This was a retrospective, single-center study of patients with heterotaxy who underwent cardiovascular magnetic resonance imaging at a single center between July 1, 2019 and May 15, 2023. Patients with (1) asplenia (right isomerism), (2) polysplenia (left isomerism) and (3) pulmonary/abdominal situs inversus (PASI) plus CHD were included. Terminal TD sidedness was described as left-sided, right-sided, or bilateral. RESULTS: Of 115 eligible patients, the terminal TD was visualized in 56 (49 %). The terminal TD was left-sided in 25 patients, right-sided in 29, and bilateral in two. On univariate analysis, terminal TD sidedness was associated with atrial situs (p = 0.006), abdominal situs (p = 0.042), type of heterotaxy (p = 0.036), the presence of pulmonary obstruction (p = 0.041), superior vena cava sidedness (p = 0.005), and arch sidedness (p < 0.001). On multivariable analysis, only superior vena cava and aortic arch sidedness were independently associated with terminal TD sidedness. CONCLUSIONS: Terminal TD sidedness is highly variable in patients with heterotaxy. Superior vena cava and arch sidedness are independently associated with terminal TD sidedness. Type of heterotaxy was not independently associated with terminal TD sidedness. This data improves the understanding of anatomic variation in patients with heterotaxy and may be useful for planning for lymphatic interventions.
RESUMO
BACKGROUND: Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes. METHODS: This was a retrospective, single-center study of patients with Fontan circulation who underwent CMR. Frequency matching of age at CMR was used to construct a comparison group of patients with repaired tetralogy of Fallot (rTOF). TD characteristics included maximum diameter and a qualitative assessment of tortuosity. Clinical outcomes included protein-losing enteropathy (PLE), plastic bronchitis, listing for heart transplantation, and death. A composite outcome was defined as presence of any of these events. RESULTS: The study included 189 Fontan patients (median age 16.1 years, IQR 11.0-23.2 years) and 36 rTOF patients (median age 15.7 years, IQR 11.1-23.7 years). The TD diameter was larger (median 2.50 vs. 1.95 mm, p = 0.002) and more often well visualized (65% vs. 22%, p < 0.001) in Fontan patients vs. rTOF patients. TD dimension increased mildly with age in Fontan patients, R = 0.19, p = 0.01. In Fontan patients, the TD diameter was larger in those with PLE vs. without PLE (age-adjusted mean 4.11 vs. 2.72, p = 0.005), and was more tortuous in those with NYHA class ≥ II vs. class I (moderate or greater tortuosity 75% vs. 28.5%, p = 0.02). Larger TD diameter was associated with a lower ventricular ejection fraction that was independent of age (partial correlation = - 0.22, p = 0.02). More tortuous TDs had a higher end-systolic volume (mean 70.0 mL/m2 vs. 57.3 mL/m2, p = 0.03), lower creatinine (mean 0.61 mg/dL vs. 0.70 mg/dL, p = 0.04), and a higher absolute lymphocyte count (mean 1.80 K cells/µL vs. 0.76 K cells/µL, p = 0.003). The composite outcome was present in 6% of Fontan patients and was not associated with TD diameter (p = 0.50) or tortuosity (p = 0.09). CONCLUSIONS: The TD is well visualized in two-thirds of patients with Fontan circulation on 3D-bSSFP images. Larger TD diameter is associated with PLE and increased TD tortuosity is associated with an NYHA class ≥ II.
Assuntos
Técnica de Fontan , Tetralogia de Fallot , Humanos , Adolescente , Ducto Torácico/diagnóstico por imagem , Técnica de Fontan/efeitos adversos , Estudos Retrospectivos , Valor Preditivo dos Testes , Espectroscopia de Ressonância MagnéticaRESUMO
BACKGROUND: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. METHODS: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. RESULTS: Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2-9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (- 0.61%/year vs. - 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). CONCLUSIONS: Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.
Assuntos
Técnica de Fontan , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Técnica de Fontan/efeitos adversos , Estudos Retrospectivos , Estudos Transversais , Valor Preditivo dos Testes , Ventrículos do Coração , Função Ventricular Esquerda , Volume SistólicoRESUMO
BACKGROUND: Due to passive blood flow in palliated single ventricle, central venous pressure increases chronically, ultimately impeding lymphatic drainage. Early visualization and treatment of these malformations is essential to reduce morbidity and mortality. Cardiovascular magnetic resonance (CMR) T2-weighted lymphangiography (T2w) is used for lymphatic assessment, but its low signal-to-noise ratio may result in incomplete visualization of thoracic duct pathway. 3D-balanced steady state free precession (3D-bSSFP) is commonly used to assess congenital cardiac disease anatomy. Here, we aimed to improve diagnostic imaging of thoracic duct pathway using 3D-bSSFP. METHODS: Patients underwent CMR during single ventricle or central lymphatic system assessment using T2w and 3D-bSSFP. T2w parameters included 3D-turbo spin echo (TSE), TE/TR = 600/2500 ms, resolution = 1 × 1 × 1.8 mm, respiratory triggering with bellows. 3D-bSSFP parameters included electrocardiogram triggering and diaphragm navigator, 1.6 mm isotropic resolution, TE/TR = 1.8/3.6 ms. Thoracic duct was identified independently in T2w and 3D-bSSFP images, tracked completely from cisterna chyli to its drainage site, and classified based on severity of lymphatic abnormalities. RESULTS: Forty-eight patients underwent CMR, 46 of whom were included in the study. Forty-five had congenital heart disease with single ventricle physiology. Median age at CMR was 4.3 year (range 0.9-35.1 year, IQR 2.4 year), and median weight was 14.4 kg (range, 7.9-112.9 kg, IQR 5.2 kg). Single ventricle with right dominant ventricle was noted in 31 patients. Thirty-eight patients (84%) were status post bidirectional Glenn and 7 (16%) were status post Fontan anastomosis. Thoracic duct visualization was achieved in 45 patients by T2w and 3D-bSSFP. Complete tracking to drainage site was attained in 11 patients (24%) by T2w vs 25 (54%) by 3D-bSSFP and in 28 (61%) by both. Classification of lymphatics was performed in 31 patients. CONCLUSION: Thoracic duct pathway can be visualized by 3D-bSSFP combined with T2w lymphangiography. Cardiac triggering and respiratory navigation likely help retain lymphatic signal in the retrocardiac area by 3D-bSSFP. Visualizing lymphatic system leaks is challenging on 3D-bSSFP images alone, but 3D-bSSFP offers good visualization of duct anatomy and landmark structures to help plan interventions. Together, these sequences can define abnormal lymphatic pathway following single ventricle palliative surgery, thus guiding lymphatic interventional procedures.
Assuntos
Cardiopatias Congênitas/diagnóstico por imagem , Imageamento Tridimensional , Linfografia , Imageamento por Ressonância Magnética , Ducto Torácico/diagnóstico por imagem , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Interpretação de Imagem Assistida por Computador , Lactente , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Ducto Torácico/fisiopatologia , Adulto JovemRESUMO
Ventricular contouring of cardiac magnetic resonance imaging is the gold standard for volumetric analysis for repaired tetralogy of Fallot (rTOF), but can be time-consuming and subject to variability. A convolutional neural network (CNN) ventricular contouring algorithm was developed to generate contours for mostly structural normal hearts. We aimed to improve this algorithm for use in rTOF and propose a more comprehensive method of evaluating algorithm performance. We evaluated the performance of a ventricular contouring CNN, that was trained on mostly structurally normal hearts, on rTOF patients. We then created an updated CNN by adding rTOF training cases and evaluated the new algorithm's performance generating contours for both the left and right ventricles (LV and RV) on new testing data. Algorithm performance was evaluated with spatial metrics (Dice Similarity Coefficient (DSC), Hausdorff distance, and average Hausdorff distance) and volumetric comparisons (e.g., differences in RV volumes). The original Mostly Structurally Normal (MSN) algorithm was better at contouring the LV than the RV in patients with rTOF. After retraining the algorithm, the new MSN + rTOF algorithm showed improvements for LV epicardial and RV endocardial contours on testing data to which it was naïve (N = 30; e.g., DSC 0.883 vs. 0.905 for LV epicardium at end diastole, p < 0.0001) and improvements in RV end-diastolic volumetrics (median %error 8.1 vs 11.4, p = 0.0022). Even with a small number of cases, CNN-based contouring for rTOF can be improved. This work should be extended to other forms of congenital heart disease with more extreme structural abnormalities. Aspects of this work have already been implemented in clinical practice, representing rapid clinical translation. The combined use of both spatial and volumetric comparisons yielded insights into algorithm errors.
Assuntos
Algoritmos , Ventrículos do Coração/diagnóstico por imagem , Redes Neurais de Computação , Tetralogia de Fallot/diagnóstico por imagem , Adulto , Estudos de Casos e Controles , Feminino , Ventrículos do Coração/anatomia & histologia , Humanos , Imageamento por Ressonância Magnética/métodos , MasculinoRESUMO
OBJECTIVE: To examine state-wide population trends in preterm delivery of children with critical congenital heart disease (CHD) over an 18-year period. We hypothesized that, coincident with early advancements in prenatal diagnosis, preterm delivery initially increased compared with the general population, and more recently has decreased. STUDY DESIGN: Data from the Texas Public Use Data File 1999-2016 was used to evaluate annual percent preterm delivery (<37 weeks) in critical CHD (diagnoses requiring intervention at <1 year of age). We first evaluated for pattern change over time using joinpoint segmented regression. Trends in preterm delivery were then compared with all Texas livebirths. We then compared trends examining sociodemographic covariates including race/ethnicity, sex, and neighborhood poverty levels. RESULTS: Of 7146 births with critical CHD, 1339 (18.7%) were delivered preterm. The rate of preterm birth increased from 1999 to 2004 (a mean increase of 1.69% per year) then decreased between 2005 and 2016 (a mean decrease of -0.41% per year). This represented a faster increase and then a similar decrease to that noted in the general population. Although the greatest proportion of preterm births occurred in newborns of Hispanic ethnicity and non-Hispanic black race, newborns with higher neighborhood poverty level had the most rapidly increasing rate of preterm delivery in the first era, and only a plateau rather than decrease in the latter era. CONCLUSIONS: Rates of preterm birth for newborns with critical CHD in Texas first were increasing rapidly, then have been decreasing since 2005.
Assuntos
Cardiopatias Congênitas/epidemiologia , Nascimento Prematuro/epidemiologia , Estado Terminal , Feminino , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos , Texas/epidemiologia , Fatores de TempoRESUMO
Transposition of the great arteries (TGA) with a posterior aorta is an uncommon but historically important variant of TGA. In this arrangement, the aorta is posterior to the pulmonary valve, maintains fibrous continuity with the mitral valve, and arises from the right ventricle. We present a case of fetal echocardiography demonstrating TGA with a posterior aorta. Postnatal transthoracic echocardiography and intra-operative assessment confirm the anatomy. To the best of our knowledge, this is the first published case of this anatomic lesion by fetal echocardiography.
Assuntos
Valva Aórtica/anormalidades , Ecocardiografia/métodos , Valva Pulmonar/anormalidades , Transposição dos Grandes Vasos/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodos , Valva Aórtica/diagnóstico por imagem , Feminino , Coração Fetal/diagnóstico por imagem , Coração Fetal/cirurgia , Humanos , Recém-Nascido , Gravidez , Valva Pulmonar/diagnóstico por imagem , Transposição dos Grandes Vasos/embriologia , Transposição dos Grandes Vasos/cirurgiaAssuntos
Infarto Encefálico/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Neoplasias Cardíacas/diagnóstico por imagem , Mixoma/diagnóstico por imagem , Doenças do Sistema Nervoso/etiologia , Convulsões/etiologia , Encéfalo/patologia , Infarto Encefálico/etiologia , Criança , Diagnóstico Diferencial , Ecocardiografia , Eletroencefalografia , Paralisia Facial/etiologia , Feminino , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/cirurgia , Humanos , Embolia Intracraniana/diagnóstico por imagem , Embolia Intracraniana/etiologia , Imageamento por Ressonância Magnética , Mixoma/complicações , Mixoma/cirurgiaRESUMO
BACKGROUND: In patients who have difficulty holding their breath, a free breathing (FB) respiratory-triggered (RT) bSSFP cine technique may be used. However, this technique may have inferior image quality and a longer scan time than breath-hold (BH) bSSFP cine acquisitions. This study examined the effect of an audiovisual breathing guidance (BG) system on RT bSSFP cine image quality, scan time, and ventricular measurements. METHODS: This study evaluated a BG system that provides audiovisual instructions and feedback on the timing of inspiration and expiration to the patient during image acquisition using input from the respiratory bellows to guide them toward a regular breathing pattern with extended end-expiration. In this single-center prospective study in patients undergoing a clinical cardiac magnetic resonance examination, a ventricular short-axis stack of bSSFP cine images was acquired using 3 techniques in each patient: 1) FB and RT (FBRT), 2) BG system and RT (BGRT), and 3) BH. The 3 acquisitions were compared for image quality metrics (endocardial edge definition, motion artifact, and blood-to-myocardial contrast) scored on a Likert scale, scan time, and ventricular volumes and mass. RESULTS: Thirty-two patients (19 females; median age 21 years, IQR 18-32) completed the study protocol. For scan time, BGRT was faster than FBRT (163 s vs. 345 s, p < 0.001). Endocardial edge definition, motion artifact, and blood-to-myocardial contrast were all better for BGRT than FBRT (p < 0.001). Left ventricular (LV) end-systolic volume (ESV) was smaller (3%, p = 0.02) and LV ejection fraction (EF) was larger (0.5%, p = 0.003) with BGRT than with FBRT. There was no significant difference in LV end-diastolic volume (EDV), LV mass, right ventricular (RV) EDV, RV ESV, and RV EF. Scan times were shorter for BGRT compared to BH. Endocardial edge definition and blood-to-myocardial contrast were better for BH than BGRT. Compared to BH, the LV EDV, LV ESV, RV EDV, and RV ESV were mildly smaller (all differences <7%) for BGRT. CONCLUSIONS: The addition of a BG system to RT bSSFP cine acquisitions decreased the scan time and improved image quality. Further exploration of this BG approach is warranted in more diverse populations and with other free breathing sequences.
Assuntos
Imagem Cinética por Ressonância Magnética , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Feminino , Masculino , Adulto , Estudos Prospectivos , Respiração , Pessoa de Meia-Idade , Técnicas de Imagem de Sincronização Respiratória/métodos , Coração/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Suspensão da Respiração , Artefatos , Reprodutibilidade dos Testes , Recursos Audiovisuais , Adulto JovemRESUMO
BACKGROUND: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. OBJECTIVE: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. METHODS: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. RESULTS: For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. CONCLUSION: Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding.
Assuntos
Miocárdio , Marca-Passo Artificial , Humanos , Feminino , Adulto Jovem , Adulto , Masculino , Estudos Retrospectivos , Miocárdio/patologia , Coração , Marca-Passo Artificial/efeitos adversos , FibroseRESUMO
BACKGROUND: American Heart Association (AHA) guidelines for management of Kawasaki disease (KD) rely on coronary artery (CA) z-scores from echocardiograms. Compared with echocardiography, cardiac CT (CCT) offers better visualization of distal segments and evaluation for thrombosis and stenosis. Despite increasing use of CCT in KD, CA z-scores for CCT are not available and measurement concordance between imaging modalities is a critical knowledge gap. METHODS: We retrospectively reviewed KD patients with CA aneurysms who had concurrent echocardiography and CCT between 2016 and 2020. Patients were included if they had history of CA z-scores of ≥3 on echocardiography during their clinical course. Agreement between CCT and echocardiography was assessed using Bland-Altman analysis. RESULTS: Paired CCT and echocardiography studies were available in 18 patients (21 studies). The largest CA aneurysms were large/giant (z-score ≥10) in 14 studies, medium (z-score ≥5, <10) in 3 studies, and small (z score ≥2.5, <5) in 2 studies. Intra- and inter-observer reliability for CCT measurements were high for all CA segments (ICC 99.7% and 98.6%). For the LMCA, proximal LAD and proximal and distal RCA there was high correlation between echocardiogram and CCT absolute measurements with wider variation between modalities for the distal LAD and circumflex. Overall, CCT measurements tended to be smaller than echocardiogram measurements, and led to a lower AHA z-score risk classification in 24% of studies. CONCLUSION: CCT and echocardiography have high agreement for absolute measurements of proximal CA segments, but more measurement discrepancy exists for distal CA segments with bias toward lower dimensions on CCT.
Assuntos
Aneurisma Coronário , Doença da Artéria Coronariana , Síndrome de Linfonodos Mucocutâneos , Aneurisma Coronário/diagnóstico por imagem , Aneurisma Coronário/etiologia , Vasos Coronários/diagnóstico por imagem , Ecocardiografia , Humanos , Lactente , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
Importance: The BNT162b2 (Pfizer-BioNTech) messenger RNA COVID-19 vaccine was authorized on May 10, 2021, for emergency use in children aged 12 years and older. Initial reports showed that the vaccine was well tolerated without serious adverse events; however, cases of myocarditis have been reported since approval. Objective: To review results of comprehensive cardiac imaging in children with myocarditis after COVID-19 vaccine. Design, Setting, and Participants: This study was a case series of children younger than 19 years hospitalized with myocarditis within 30 days of BNT162b2 messenger RNA COVID-19 vaccine. The setting was a single-center pediatric referral facility, and admissions occurred between May 1 and July 15, 2021. Main Outcomes and Measures: All patients underwent cardiac evaluation including an electrocardiogram, echocardiogram, and cardiac magnetic resonance imaging. Results: Fifteen patients (14 male patients [93%]; median age, 15 years [range, 12-18 years]) were hospitalized for management of myocarditis after receiving the BNT162b2 (Pfizer) vaccine. Symptoms started 1 to 6 days after receipt of the vaccine and included chest pain in 15 patients (100%), fever in 10 patients (67%), myalgia in 8 patients (53%), and headache in 6 patients (40%). Troponin levels were elevated in all patients at admission (median, 0.25 ng/mL [range, 0.08-3.15 ng/mL]) and peaked 0.1 to 2.3 days after admission. By echocardiographic examination, decreased left ventricular (LV) ejection fraction (EF) was present in 3 patients (20%), and abnormal global longitudinal or circumferential strain was present in 5 patients (33%). No patient had a pericardial effusion. Cardiac magnetic resonance imaging findings were consistent with myocarditis in 13 patients (87%) including late gadolinium enhancement in 12 patients (80%), regional hyperintensity on T2-weighted imaging in 2 patients (13%), elevated extracellular volume fraction in 3 patients (20%), and elevated LV global native T1 in 2 patients (20%). No patient required intensive care unit admission, and median hospital length of stay was 2 days (range 1-5). At follow-up 1 to 13 days after hospital discharge, 11 patients (73%) had resolution of symptoms. One patient (7%) had persistent borderline low LV systolic function on echocardiogram (EF 54%). Troponin levels remained mildly elevated in 3 patients (20%). One patient (7%) had nonsustained ventricular tachycardia on ambulatory monitor. Conclusions and Relevance: In this small case series study, myocarditis was diagnosed in children after COVID-19 vaccination, most commonly in boys after the second dose. In this case series, in short-term follow-up, patients were mildly affected. The long-term risks associated with postvaccination myocarditis remain unknown. Larger studies with longer follow-up are needed to inform recommendations for COVID-19 vaccination in this population.
Assuntos
Vacina BNT162/efeitos adversos , COVID-19/prevenção & controle , Hospitalização/estatística & dados numéricos , Miocardite/etiologia , Adolescente , COVID-19/diagnóstico , COVID-19/epidemiologia , COVID-19/imunologia , Vacinas contra COVID-19/administração & dosagem , Técnicas de Imagem Cardíaca/métodos , Criança , Ecocardiografia/métodos , Eletrocardiografia/métodos , Feminino , Seguimentos , Coração/diagnóstico por imagem , Coração/fisiopatologia , Humanos , Tempo de Internação/estatística & dados numéricos , Imageamento por Ressonância Magnética/métodos , Masculino , Miocardite/diagnóstico , SARS-CoV-2/genética , SARS-CoV-2/imunologia , Volume Sistólico/fisiologia , Troponina/sangue , Função Ventricular Esquerda/fisiologiaRESUMO
Fontan-associated protein-losing enteropathy is difficult to treat and associated with poor prognosis. Cardiac rehabilitation and exercise are thought to have beneficial effects for patients with Fontan circulation. We report the case of a young adult patient palliated to Fontan circulation, with a decade-long history of symptoms related to protein-losing enteropathy. At age 23 years, he appreciated an improvement in symptoms and laboratory values after cardiac rehabilitation and prescriptive exercise.
Assuntos
Reabilitação Cardíaca/métodos , Terapia por Exercício/métodos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Enteropatias Perdedoras de Proteínas/reabilitação , Técnica de Fontan , Hospitalização , Humanos , Masculino , Complicações Pós-Operatórias/etiologia , Enteropatias Perdedoras de Proteínas/etiologia , Stents , Taquicardia Paroxística/tratamento farmacológico , Taquicardia Paroxística/etiologia , Adulto JovemRESUMO
BACKGROUND: Anatomic lesions are a common cause of decompensation during the interstage period after Norwood stage 1 palliation (S1P). This study describes the risk factors for and outcomes after unplanned surgical and catheter-based interstage cardiac interventions. METHODS: Participants in the National Pediatric Cardiology Quality Improvement Collaborative registry discharged from the hospital after S1P between 2008 and 2016 were studied. Variables at S1P, interstage, and at stage 2 palliation (S2P) hospitalizations were examined. Multivariable logistic regression was used to compare those who had an unplanned interstage intervention to those who did not. RESULTS: Of 1994 participants from 60 programs, 343 (17.1%) had at least 1 unplanned interstage intervention. Aortic valve dilation before S1P, longer S1P cardiopulmonary bypass time, pulmonary artery stent placement between S1P and discharge, aortic arch obstruction on the S1P discharge echocardiogram, and lower weight at S1P discharge were independently associated with receiving an unplanned interstage intervention. Interstage mortality between groups was similar at 6%, as was interstage duration. Participants undergoing unplanned interstage interventions were more likely to undergo heart transplant before S2P or deemed to be unsuitable for S2P (7.3% vs 2.7%, P < .001). CONCLUSIONS: Unplanned interstage interventions after S1P did not increase interstage mortality, but participants with an unplanned intervention were less likely to progress to S2P. Residual anatomic lesions are risk factors for unplanned interstage interventions. For those with progressive ventricular dysfunction in the presence of arch obstruction by echocardiogram, aortic arch reintervention is warranted.
Assuntos
Procedimentos de Norwood , Cuidados Paliativos , Complicações Pós-Operatórias/cirurgia , Coração Univentricular/cirurgia , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos , Feminino , Humanos , Recém-Nascido , Masculino , Procedimentos de Norwood/métodos , Fatores de Risco , Resultado do TratamentoRESUMO
Cell-based therapy for neuropathic pain could provide analgesics to local pain modulatory regions in a sustained, renewable fashion. In order to provide enhanced analgesic efficacy, transplantable cells may be engineered to produce complementary or increased levels of analgesic peptides. In addition, genetic labeling of modified cells is desirable for identification and tracking, but it should be retained intracellularly as desired analgesic peptides are secreted. Usually constructs encode proteins destined for either extra- or intracellular compartments, as these pathways do not cross. However, interactions between intracellular destinations provide a window of opportunity to overcome this limitation. In this report, we have explored this approach using a potential supplementary analgesic peptide, [Ser1]-histogranin (SHG), the stable synthetic derivative of a naturally occurring peptide with N-methyl D-aspartate (NMDA) antagonistic properties. A synthetic SHG gene was combined with (i) nerve growth factor-beta (NGF-beta) amino-terminal signal peptide to enable secretion, and (ii) a fluorescent cellular label (mRFP) with intervening cathepsin L cleavage site, and subcloned into a lentiviral vector. In addition, an endoplasmic retention signal, KDEL, was added to enable retrieval of mRFP. Using immunocytochemistry and confocal microscopic profile analysis, cells transduced by such lentiviruses were shown to synthesize a single SHG-mRFP polypeptide that was processed, targeted to expected subcellular destinations in several cell types. Dot blot and Western analysis revealed stable transduction and long-term secretion of SHG from PC12 cells in vitro. Transplantation of such cells provided modest analgesia in a rodent pain model consistent with low levels of SHG peptide in the cerebrospinal fluid (CSF). These results suggest that it is possible to deliver proteins with different final destinations from a single construct, such as pharmacologically active peptide for secretion and intracellular label for identifying transplantable cells.
Assuntos
Analgésicos , Dor/tratamento farmacológico , Peptídeos , Analgésicos/metabolismo , Analgésicos/uso terapêutico , Animais , Linhagem Celular , Transplante de Células , Humanos , Oligopeptídeos , Peptídeos/genética , Peptídeos/metabolismo , Peptídeos/uso terapêutico , Sinais Direcionadores de Proteínas , Proteínas/química , Proteínas/genética , Proteínas/metabolismo , Ratos , Proteínas Recombinantes de Fusão/genética , Proteínas Recombinantes de Fusão/metabolismo , Coloração e RotulagemRESUMO
Unilateral lesioning of the spinal dorsal horn with the excitotoxin quisqualic acid (QUIS) leads to robust degeneration of dorsal horn grey matter, and robust pain-related symptoms, such as cutaneous hypersensitivity, persist long after injury. A possible mechanism that underlies the pain-related symptoms is the disruption of dorsal horn inhibitory neuron function, leading to decreased inhibition of nociceptive neurons. Five percent formalin was injected into the hind paw of rats with either a QUIS lesion or sham lesion. Both QUIS- and sham-lesioned rats displayed bi-phasic hind paw flinches following formalin injection, but a prolonged response was observed in QUIS-lesioned rats. The expression of the immediate-early gene product Fos in the dorsal horn ipsilateral to formalin injection was similar between QUIS- and sham-lesioned rats. In QUIS-lesioned rats, however, there was a marked absence of dorsal horn neurons, particularly GABAergic neurons, compared to sham-lesioned rats. The prolonged nociceptive response observed with a unilateral QUIS lesion may be due to generalized changes in dorsal horn neuron function including a loss of inhibitory neuron function.
Assuntos
Fixadores/efeitos adversos , Formaldeído/efeitos adversos , Membro Posterior/efeitos dos fármacos , Nociceptores/fisiologia , Limiar da Dor/efeitos dos fármacos , Traumatismos da Medula Espinal/fisiopatologia , Animais , Comportamento Animal , Modelos Animais de Doenças , Lateralidade Funcional , Regulação da Expressão Gênica/efeitos dos fármacos , Membro Posterior/inervação , Masculino , Proteínas Oncogênicas v-fos/metabolismo , Medição da Dor/métodos , Fosfopiruvato Hidratase/metabolismo , Ratos , Ratos Sprague-Dawley , Tempo de Reação/efeitos dos fármacos , Fatores de Tempo , Ácido gama-Aminobutírico/metabolismoRESUMO
There is a growing interest in the use of percutaneously delivered ventricular assist devices (PVAD) in the pediatric patient population. A 16 year old female and 18 year old male both status post heart transplantation presented with evidence of graft rejection and acute severe global systolic and diastolic heart failure necessitating hemodynamic catheterization and inotropic support. Both underwent percutaneous Impella CP LVAD (Abiomed, Danvers, MA) placement with close monitoring of right ventricular function. Although pulmonary artery wedge pressure (PAWP) improved, persistently elevated central venous pressures (CVP) and ongoing renal and liver injury prompted insertion of Impella RP RVAD. Explantation of bilateral devices was achieved within 4 days in the 18 year old patient. He would go on to require total artificial heart support before successful retransplantation. Despite bilateral PVAD support and antirejection medications, our 16 year old patient went on to develop multisystem organ failure and ultimately passed away after withdrawal of life-sustaining therapies. We report the first description of biventricular percutaneous Impella VAD for the treatment of acute decompensated heart failure in a pediatric institution. This innovative VAD option for older children/adolescents represents a less-invasive advancement in mechanical support technology with potential applicability across the pediatric population.
Assuntos
Coração Auxiliar , Adolescente , Feminino , Coração/fisiopatologia , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Humanos , MasculinoRESUMO
PURPOSE: A proliferation of stem/progenitor cells is observed after brain injury. We examined the regional and temporal profile of mitotically active cells to determine whether traumatic brain injury (TBI) would increase neurogenesis in selective brain regions. METHODS: Male Sprague-Dawley rats received injections (IP) of 5-bromo-deoxyuridine (BrdU), a compound used to detect mitotic cells, before and after fluid-percussion brain injury. At 3 hr, 1, 2, 3, 7, and 14 days after moderate fluid percussion, brains were processed for immunocytochemical and confocal analysis. Sections were double-labeled for markers selective for neurons (NeuN), astrocytes (GFAP), olidgodendrocytes (CNPase and MBP) and macrophage/microglia (ED1). RESULTS: At 3 hr post-trauma, the majority of BrdU labeled cells were associated with the subventricular zone of the traumatized hemisphere. At later time points, a significant increase in BrdU positive cells was observed throughout the traumatized cerebral cortex, hippocampus, white matter structures, and some contralateral regions. BrdU labeled cells were observed as late as 14 days post-injury. Double-label studies with confocal microscopy demonstrated that cell phenotypes including astrocytes, macrophage/microglia, oligodendrocytes, and neurons were BrdU positive with the majority of cells appearing glial in nature. Evidence for neurogenesis was seen in the granular cell layer of the hippocampus. CONCLUSION: These findings indicate that TBI stimulates widespread cellular proliferation for days after injury and results in focal neurogenesis in the dentate gyrus of the hippocampus. These cellular responses to injury may participate in brain repair and functional recovery.