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1.
J Cardiovasc Magn Reson ; 26(1): 101041, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38527706

RESUMEN

Cardiovascular magnetic resonance (CMR) has become the reference standard for quantitative and qualitative assessment of ventricular function, blood flow, and myocardial tissue characterization. There is a preponderance of large CMR studies and registries in adults; However, similarly powered studies are lacking for the pediatric and congenital heart disease (PCHD) population. To date, most CMR studies in children are limited to small single or multicenter studies, thereby limiting the conclusions that can be drawn. Within the PCHD CMR community, a collaborative effort has been successfully employed to recognize knowledge gaps with the aim to embolden the development and initiation of high-quality, large-scale multicenter research. In this publication, we highlight the underlying challenges and provide a practical guide toward the development of larger, multicenter initiatives focusing on PCHD populations, which can serve as a model for future multicenter efforts.


Asunto(s)
Cardiopatías Congénitas , Estudios Multicéntricos como Asunto , Valor Predictivo de las Pruebas , Humanos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/fisiopatología , Niño , Macrodatos , Imagen por Resonancia Magnética , Proyectos de Investigación , Factores de Edad , Adolescente , Preescolar
2.
J Cardiovasc Magn Reson ; 25(1): 65, 2023 Nov 16.
Artículo en Inglés | MEDLINE | ID: mdl-37968709

RESUMEN

The early career professionals in the field of Cardiovascular Magnetic Resonance (CMR) face unique challenges and hurdles while establishing their careers in the field. The Society for Cardiovascular Magnetic Resonance (SCMR) has expanded the role of the early career section within the society to foster the careers of future CMR leaders. This paper aims to describe the obstacles and available opportunities for the early career CMR professionals worldwide. Societal opportunities and actions targeted at the professional advancement of the early career CMR imagers are needed to ensure continuous growth of CMR as an imaging modality globally.


Asunto(s)
Enfermedades Cardiovasculares , Humanos , Enfermedades Cardiovasculares/diagnóstico por imagen , Valor Predictivo de las Pruebas , Imagen por Resonancia Magnética , Espectroscopía de Resonancia Magnética
3.
Pediatr Radiol ; 53(3): 394-403, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36255453

RESUMEN

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a severe life-threatening manifestation of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection that often presents with acute cardiac dysfunction and cardiogenic shock. While recovery from acute illness is excellent, the long-term myocardial impact is unknown. OBJECTIVE: To compare cardiac MRI findings in children 6-9 months after their hospitalization with MIS-C against MRI findings in healthy controls to assess for residual myocardial disease. MATERIALS AND METHODS: We prospectively performed cardiac MRI on 13 children 6-9 months following their hospitalization with MIS-C: eight of these children had a history of left ventricle ejection fraction (LVEF) < 50%, persistent symptoms, or electrocardiogram (ECG) abnormalities and underwent clinical MRI; five of these children without cardiac abnormalities during their hospitalization underwent research MRIs. We compared their native T1 and T2 mapping values with those of 20 normal controls. RESULTS: Cardiac MRI was performed at 13.6 years of age (interquartile range [IQR] 11.9-16.4 years) and 8.2 months (IQR 6.8-9.6 months) following hospitalization. Twelve children displayed normal ejection fraction: left ventricle (LV) 57.2%, IQR 56.1-58.4; right ventricle (RV) 53.1%, IQR 52.0-55.7. One had low-normal LVEF (52%). They had normal extracellular volume (ECV) and normal T2 and native T1 times compared to controls. There was no qualitative evidence of edema. One child had late gadolinium enhancement (LGE) with normal ejection fraction, no edema, and normal T1 and T2 times. When stratifying children who had MIS-C according to history of LVEF <55% on echocardiography, there was no difference in MRI values. CONCLUSION: Although many children with MIS-C present acutely with cardiac dysfunction, residual myocardial damage 6-9 months afterward appears minimal. Long-term implications warrant further study.


Asunto(s)
COVID-19 , Cardiomiopatías , Niño , Humanos , Lactante , Estudios Prospectivos , Medios de Contraste , Imagen por Resonancia Cinemagnética/métodos , SARS-CoV-2 , Gadolinio , Imagen por Resonancia Magnética , Miocardio , Función Ventricular Izquierda , Volumen Sistólico , Hospitalización , Valor Predictivo de las Pruebas
4.
Pediatr Cardiol ; 44(8): 1800-1807, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37199756

RESUMEN

A fetal cardiology consultation involves using two-dimensional drawings to explain the cardiac anatomy which can result in inherent variation in how the congenital heart disease (CHD) is conveyed. In this pilot study, we incorporated three-dimensional printed (3DP) models into fetal counseling to demonstrate feasibility and evaluate the impact on parental knowledge, understanding, and anxiety. Parents with a prenatal diagnosis of a muscular ventricular septal defect (VSD) and/or coarctation of aorta were enrolled. Providers were randomized into a Model or Drawing Group and crossed after six months. Parents completed a survey after the consultation which evaluated knowledge of the CHD lesion, expectant surgical management, self-rated understanding, attitude towards the visualization tool, and anxiety. Twenty-nine patients enrolled over a 12 month period. Twelve consultations were done for coarctation of aorta, 13 for VSD, and four for coarctation with a VSD. Both Model and Drawing groups scored similarly in self-reported understanding and confidence, helpfulness of and improvement in communication with the visualization tool. The Model group had higher scores on questions related to the CHD anatomy and surgical intervention [5 [4-5] versus 4 [3.5-5]], p = 0.23 although this didn't reach statistical significance. For the majority (83%) of consultations, the cardiologist agreed that the 3D model improved communication. In this pilot study, we demonstrate the use of 3DP cardiac models during prenatal CHD counseling is feasible and produces results related to parental understanding and knowledge that are equal to and possibly better than the current standard of care.


Asunto(s)
Coartación Aórtica , Cardiopatías Congénitas , Femenino , Humanos , Embarazo , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/cirugía , Comunicación , Consejo , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Modelos Anatómicos , Proyectos Piloto , Impresión Tridimensional
5.
Pediatr Emerg Care ; 39(7): 476-481, 2023 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-37383008

RESUMEN

OBJECTIVES: The case definition for multisystem inflammatory syndrome in children (MIS-C) is broad and encompasses symptoms and signs commonly seen in children with fever. Our aim was to identify clinical predictors that, independently or in combination, identify febrile children presenting to the emergency department (ED) as low risk for MIS-C. METHODS: We conducted a retrospective single-center study of otherwise healthy children 2 months to 20 years of age presenting to the ED with fever and who had a laboratory evaluation for MIS-C between April 15, 2020, and October 31, 2020. We excluded children with a diagnosis of Kawasaki disease. Our outcome was an MIS-C diagnosis defined by the Centers for Disease Control and Prevention criteria. We conducted multivariable logistic regression analyses to identify variables independently associated with MIS-C. RESULTS: Thirty-three patients with and 128 patients without MIS-C were analyzed. Of those with MIS-C, 16 of 33 (48.5%) had hypotension for age, signs of hypoperfusion, or required ionotropic support. Four variables were independently associated with the presence of MIS-C; known or suspected SARS CoV-2 exposure (adjusted odds ratio [aOR], 4.0; 95% confidence interval [CI], 1.4-11.9) and the following 3 symptoms and signs: abdominal pain on history (aOR, 4.8; 95% CI, 1.7-15.0), conjunctival injection (aOR, 15.2; 95% CI, 5.4-48.1), and rash involving the palms or soles (aOR, 12.2; 95% CI, 2.4-69.4). Children were at low risk of MIS-C if none of the 3 symptoms or signs were present (sensitivity 87.9% [95% CI, 71.8-96.6]; specificity 62.5% [53.5-70.9], negative predictive value 95.2% [88.3-98.7]). Of the 4 MIS-C patients without any of these 3 factors, 2 were ill-appearing in the ED and the other 2 had no cardiovascular involvement during their clinical course. CONCLUSIONS: A combination of 3 clinical symptoms and signs had moderate to high sensitivity and high negative predictive value for identifying febrile children at low risk of MIS-C. If validated, these factors could aid clinicians in determining the need to obtain or forego an MIS-C laboratory evaluation during SARS-CoV-2 prevalent periods in febrile children.


Asunto(s)
COVID-19 , Enfermedades del Tejido Conjuntivo , Estados Unidos , Humanos , COVID-19/complicaciones , COVID-19/diagnóstico , COVID-19/epidemiología , SARS-CoV-2 , Estudios Retrospectivos , Fiebre/etiología
6.
Heart Fail Clin ; 18(2): 325-333, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-35341544

RESUMEN

Advanced imaging is often used to supplement clinical information in guiding management for patients with heart failure. 3 dimensional (3D) imaging datasets allow for a better understanding of the relevant cardiac spatial anatomic relationships. 3D printing technology takes this one step further and allows for the creation of patient-specific physical cardiac models. In this review, we summarize some of the recent innovative applications of this technique to patients with heart failure from different etiologies, to provide more patient-directed care.


Asunto(s)
Insuficiencia Cardíaca , Impresión Tridimensional , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/terapia , Humanos
7.
Medicina (Kaunas) ; 58(2)2022 Jan 21.
Artículo en Inglés | MEDLINE | ID: mdl-35208490

RESUMEN

Background and Objectives: Barefoot peak plantar pressures (PPPs) are elevated in diabetes patients with neuropathic foot ulcer (DFU) history; however, there is limited reported evidence for a causative link between high barefoot PPP and DFU risk. We aimed to determine, using a simple mat-based methodology, the site-specific, barefoot PPP critical threshold that will identify a plantar site with a previous DFU. Materials and Methods: In a cross-sectional study, barefoot, site-specific PPPs were measured with normal gait for patients with DFU history (n = 21) and healthy controls (n = 12), using a validated carbon footprint system. For each participant, PPP was recorded at twelve distinct plantar sites (1st-5th toes, 1st-5th metatarsal heads (MTHs), midfoot and heel), per right and left foot, resulting in the analysis of n = 504 distinct plantar sites in the diabetes group, and n = 288 sites in the control group. Receiver operator characteristic curve analysis determined the optimal critical threshold for sites with DFU history. Results: Median PPPs for the groups were: diabetes sites with DFU history (n = 32) = 5.0 (3.25-7.5) kg/cm2, diabetes sites without DFU history (n = 472) = 3.25 (2.0-5.0) kg/cm2, control sites (n = 288) = 2.0 (2.0-3.25) kg/cm2; (p < 0.0001). Diabetes sites with elevated PPP (>6 kg/cm2) were six times more likely to have had DFU than diabetes sites with PPP ≤ 6 kg/cm2 (OR = 6.4 (2.8-14.6, 95% CI), p < 0.0001). PPP > 4.1 kg/cm2 was determined as the optimal critical threshold for identifying DFU at a specific plantar site, with sensitivity/specificity = 100%/79% at midfoot; 80%/65% at 5th metatarsal head; 73%/62% at combined midfoot/metatarsal head areas. Conclusions: We have demonstrated, for the first time, a strong, site-specific relationship between elevated barefoot PPP and previous DFU. We have determined a critical, highly-sensitive, barefoot PPP threshold value of >4.1 kg/cm2, which may be easily used to identify sites of previous DFU occurrence and, therefore, increased risk of re-ulceration. This site-specific approach may have implications for how high PPPs should be investigated in future trials.


Asunto(s)
Diabetes Mellitus , Pie Diabético , Estudios Transversales , Pie Diabético/epidemiología , Pie , Humanos , Presión , Dedos del Pie
8.
Prog Pediatr Cardiol ; 60: 101265, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32837147

RESUMEN

The current pandemic has driven the medical community to adapt quickly to unprecedented challenges. Among these challenges is the need to minimize staff exposure to COVID-19 during neonatal cardiac procedures. In this report, we describe measures we have taken to protect health care workers while ensuring successful outcomes. These measures include wearing appropriate personal protective equipment, physical distancing, designating separate delivery and transport teams, and limiting the number of providers in direct contact with any patient who is infected or whose infection status is unknown. LEARNING OBJECTIVES: 1.To understand specific challenges caused by the COVID-19 pandemic for patients with congenital heart disease needing urgent neonatal intervention.2.To recognize measures that can be taken to minimize health care workers' exposures to the virus during high-risk neonatal cardiac procedures.3.To review the management of neonates with d-transposition of the great arteries and inadequate mixing.

9.
Pediatr Cardiol ; 40(6): 1258-1265, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31240370

RESUMEN

The most common modes of medical education for congenital heart disease (CHD) rely heavily on 2-dimensional imaging. Three-dimensional (3D) printing technology allows for the creation of physical cardiac models that can be used for teaching trainees. 3D printed cardiac models were created for the following lesions: pulmonic stenosis, atrial septal defect, tetralogy of Fallot, d-transposition of the great arteries, coarctation of the aorta, and hypoplastic left heart syndrome. Medical students participated in a workshop consisting of different teaching stations. At the 3D printed station, students completed a pre- and post-intervention survey assessing their knowledge of each cardiac lesion on a Likert scale. Students were asked to rank the educational benefit of each modality. Linear regression was utilized to assess the correlation of the mean increase in knowledge with increasing complexity of CHD based on the Aristotle Basic Complexity Level. 45 medical students attended the CHD workshop. Students' knowledge significantly improved for every lesion (p < 0.001). A strong positive correlation was found between mean increase in knowledge and increasing complexity of CHD (R2 = 0.73, p < 0.05). The 3D printed models, pathology specimens and spoken explanation were found to be the most helpful modalities. Students "strongly agreed" the 3D printed models made them more confident in explaining congenital cardiac anatomy to others (mean = 4.23, ± 0.69), and that they recommend the use of 3D models for future educational sessions (mean = 4.40, ± 0.69). 3D printed cardiac models should be included in medical student education particularly for lesions that require a complex understanding of spatial relationships.


Asunto(s)
Educación Médica/métodos , Cardiopatías Congénitas/patología , Modelos Anatómicos , Impresión Tridimensional , Adulto , Femenino , Cardiopatías Congénitas/diagnóstico por imagen , Humanos , Masculino , Autoinforme , Estudiantes de Medicina/estadística & datos numéricos , Adulto Joven
10.
J Cardiothorac Vasc Anesth ; 32(4): 1937-1945, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29277300

RESUMEN

Two-dimensional visualization of complex congenital heart disease has limitations in that there is variation in the interpretation by different individuals. Three-dimensional printing technology has been in use for decades but is currently becoming more commonly used in the medical field. Congenital heart disease serves as an ideal pathology to employ this technology because of the variation of anatomy between patients. In this review, the authors aim to discuss basics of applicability of three-dimensional printing, the process involved in creating a model, as well as challenges with establishing utility and quality.


Asunto(s)
Cardiopatías/diagnóstico por imagen , Imagenología Tridimensional/tendencias , Invenciones/tendencias , Modelos Anatómicos , Cuidados Preoperatorios/tendencias , Impresión Tridimensional/tendencias , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías/cirugía , Humanos , Imagenología Tridimensional/métodos , Cuidados Preoperatorios/métodos
11.
J Dairy Sci ; 99(1): 758-70, 2016 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-26601585

RESUMEN

Recent studies demonstrating a higher incidence of metabolic disorders after calving have challenged the management practice of increasing dietary energy density during the last ~3 wk prepartum. Despite our knowledge at the whole-animal level, the tissue-level mechanisms that are altered in response to feeding management prepartum remain unclear. Our hypothesis was that prepartum body condition score (BCS), in combination with feeding management, plays a central role in the peripartum changes associated with energy balance and inflammatory state. Twenty-eight mid-lactation grazing dairy cows of mixed age and breed were randomly allocated to 1 of 4 treatment groups in a 2 × 2 factorial arrangement: 2 prepartum BCS categories (4.0 and 5.0, based on a 10-point scale; BCS4, BCS5) obtained via differential feeding management during late-lactation, and 2 levels of energy intake during the 3 wk preceding calving (75 and 125% of estimated requirements). Subcutaneous adipose tissue was harvested via biopsy at -1, 1, and 4 wk relative to parturition. Quantitative polymerase chain reaction was used to measure mRNA and microRNA (miRNA) expression of targets related to fatty acid metabolism (lipogenesis, lipolysis), adipokine synthesis, and inflammation. Both prepartum BCS and feeding management had a significant effect on mRNA and miRNA expression throughout the peripartum period. Overfed BCS5 cows had the greatest prepartum expression of fatty acid synthase (FASN) and an overall greater expression of leptin (LEP); BCS5 was also associated with greater overall adiponectin (ADIPOQ) and peroxisome proliferator-activated receptor gamma (PPARG), whereas overfeeding upregulated expression of proadipogenic miRNA. Higher postpartum expression of chemokine ligand 5 (CCL5) and the cytokines interleukin 6 (IL6) and tumor necrosis factor (TNF) was detected in overfed BCS5 cows. Feed-restricted BCS4 cows had the highest overall interleukin 1 (IL1B) expression. Prepartum feed restriction resulted in greater chemokine ligand 2 (CCL2) expression. Overall, changes in mRNA expression were consistent with the expression pattern of inflammation-related miRNA. These data shed light on molecular mechanisms underlying the effect of prepartum BCS and feeding management on metabolic and inflammatory status of adipose tissue during the peripartum period. Data support the use of a controlled feed restriction prepartum in optimally conditioned cows, as well as the use of a higher level of dietary energy in under-conditioned cows.


Asunto(s)
Tejido Adiposo/metabolismo , Bovinos/fisiología , Inflamación/metabolismo , Transcriptoma , Adiponectina/genética , Adiponectina/metabolismo , Alimentación Animal , Animales , Cruzamiento , Quimiocina CCL2/genética , Quimiocina CCL2/metabolismo , Quimiocina CCL5/genética , Quimiocina CCL5/metabolismo , Dieta/veterinaria , Ingestión de Energía , Metabolismo Energético , Ácido Graso Sintasas/genética , Ácido Graso Sintasas/metabolismo , Femenino , Interleucina-1beta/genética , Interleucina-1beta/metabolismo , Interleucina-6/genética , Interleucina-6/metabolismo , Leptina/genética , Leptina/metabolismo , Metabolismo de los Lípidos , Lipogénesis , Lipólisis , MicroARNs/genética , MicroARNs/metabolismo , Estado Nutricional , Hipernutrición/metabolismo , Hipernutrición/veterinaria , PPAR gamma/genética , PPAR gamma/metabolismo , Periodo Periparto/fisiología , ARN Mensajero/genética , ARN Mensajero/metabolismo , Grasa Subcutánea/metabolismo , Factor de Necrosis Tumoral alfa/genética , Factor de Necrosis Tumoral alfa/metabolismo , Regulación hacia Arriba
12.
Pediatr Cardiol ; 37(6): 1028-36, 2016 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-27041098

RESUMEN

The method of cardiac magnetic resonance (CMR) three-dimensional (3D) image acquisition and post-processing which should be used to create optimal virtual models for 3D printing has not been studied systematically. Patients (n = 19) who had undergone CMR including both 3D balanced steady-state free precession (bSSFP) imaging and contrast-enhanced magnetic resonance angiography (MRA) were retrospectively identified. Post-processing for the creation of virtual 3D models involved using both myocardial (MS) and blood pool (BP) segmentation, resulting in four groups: Group 1-bSSFP/MS, Group 2-bSSFP/BP, Group 3-MRA/MS and Group 4-MRA/BP. The models created were assessed by two raters for overall quality (1-poor; 2-good; 3-excellent) and ability to identify predefined vessels (1-5: superior vena cava, inferior vena cava, main pulmonary artery, ascending aorta and at least one pulmonary vein). A total of 76 virtual models were created from 19 patient CMR datasets. The mean overall quality scores for Raters 1/2 were 1.63 ± 0.50/1.26 ± 0.45 for Group 1, 2.12 ± 0.50/2.26 ± 0.73 for Group 2, 1.74 ± 0.56/1.53 ± 0.61 for Group 3 and 2.26 ± 0.65/2.68 ± 0.48 for Group 4. The numbers of identified vessels for Raters 1/2 were 4.11 ± 1.32/4.05 ± 1.31 for Group 1, 4.90 ± 0.46/4.95 ± 0.23 for Group 2, 4.32 ± 1.00/4.47 ± 0.84 for Group 3 and 4.74 ± 0.56/4.63 ± 0.49 for Group 4. Models created using BP segmentation (Groups 2 and 4) received significantly higher ratings than those created using MS for both overall quality and number of vessels visualized (p < 0.05), regardless of the acquisition technique. There were no significant differences between Groups 1 and 3. The ratings for Raters 1 and 2 had good correlation for overall quality (ICC = 0.63) and excellent correlation for the total number of vessels visualized (ICC = 0.77). The intra-rater reliability was good for Rater A (ICC = 0.65). Three models were successfully printed on desktop 3D printers with good quality and accurate representation of the virtual 3D models. We recommend using BP segmentation with either MRA or bSSFP source datasets to create virtual 3D models for 3D printing. Desktop 3D printers can offer good quality printed models with accurate representation of anatomic detail.


Asunto(s)
Corazón , Humanos , Imagenología Tridimensional , Angiografía por Resonancia Magnética , Impresión Tridimensional , Reproducibilidad de los Resultados
13.
Pediatr Cardiol ; 37(1): 90-8, 2016 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-26254102

RESUMEN

Our goal was to construct three-dimensional (3D) virtual models to allow simultaneous visualization of the ventricles, ventricular septal defect (VSD) and great arteries in patients with complex intracardiac anatomy to aid in surgical planning. We also sought to correlate measurements from the source cardiac magnetic resonance (CMR) image dataset and the 3D model. Complicated ventriculo-arterial relationships in patients with complex conotruncal malformations make preoperative assessment of possible repair pathways difficult. Patients were chosen with double outlet right ventricle for the complexity of intracardiac anatomy and potential for better delineation of anatomic spatial relationships. Virtual 3D models were generated from CMR 3D datasets. Measurements were made on the source CMR as well as the 3D model for the following structures: aortic diameter in orthogonal planes, VSD diameter in orthogonal planes and long axis of right ventricle. A total of six patients were identified for inclusion. The path from the ventricles to each respective outflow tract and the location of the VSD with respect to each great vessel was visualized clearly in all patients. Measurements on the virtual model showed excellent correlation with the source CMR when all measurements were included by Pearson coefficient, r = 0.99 as well as for each individual structure. Construction of virtual 3D models in patients with complex conotruncal defects from 3D CMR datasets allows for simultaneous visualization of anatomic relationships relevant for surgical repair. The availability of these models may allow for a more informed preoperative evaluation in these patients.


Asunto(s)
Ventrículo Derecho con Doble Salida/diagnóstico , Imagenología Tridimensional/métodos , Imagen por Resonancia Magnética/métodos , Adolescente , Adulto , Niño , Preescolar , Simulación por Computador , Ventrículo Derecho con Doble Salida/complicaciones , Corazón , Humanos , Estudios Retrospectivos
14.
Pediatr Cardiol ; 35(6): 922-7, 2014 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-24509636

RESUMEN

After heart transplantation (HT) in infants and young children, environmental and intrinsic factors may lead to changes in the geometry and compliance of the donor heart. Serial demographic, clinical, hemodynamic, and echocardiographic data were obtained from HT recipients younger than 4 years of age. Echocardiographic chamber measurement z-scores were compared using recipient body surface area from the time of HT to 1 week, 3 months, and last follow-up visit. Left ventricular end-diastolic volume (LVEDV) z-scores were correlated with pulmonary capillary wedge pressure (PCWP) at each time point. Heart transplantation was performed for 13 children between March 2009 and December 2012, 9 of whom (69%) were boys. The median age at HT was 8 months (range, 4-43 months), and the mean follow-up period was 13 ± 7 months. Left ventricular end-diastolic dimension z-scores decreased significantly (p = 0.03) between HT and 1 week, then increased from 1 week to 3 and 12 months. (-1.32 ± 1.7, -0.71 ± 1.8, 0.41 ± 2.1, 0.79 ± 2.3, respectively). A positive relationship (R(2) = 0.48) between the LVEDV z-score and PCPW was present at the last follow-up visit. For infants and young children, the allograft demonstrates appropriate growth by 1 year after HT. Left ventricular compliance improves over time.


Asunto(s)
Insuficiencia Cardíaca/cirugía , Trasplante de Corazón , Ventrículos Cardíacos , Trasplantes , Remodelación Ventricular/fisiología , Cateterismo Cardíaco/métodos , Preescolar , Ecocardiografía/métodos , Femenino , Supervivencia de Injerto , Insuficiencia Cardíaca/fisiopatología , Trasplante de Corazón/efectos adversos , Trasplante de Corazón/métodos , Ventrículos Cardíacos/crecimiento & desarrollo , Ventrículos Cardíacos/inmunología , Ventrículos Cardíacos/fisiopatología , Humanos , Lactante , Masculino , Periodo Posoperatorio , Presión Esfenoidal Pulmonar , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Volumen Sistólico , Trasplantes/crecimiento & desarrollo , Trasplantes/inmunología , Trasplantes/fisiopatología , Resultado del Tratamiento , Estados Unidos
15.
Front Oncol ; 14: 1326754, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38690164

RESUMEN

Acute myeloid leukemia (AML) is a heterogeneous hematological malignancy characterized by disrupted blood cell production and function. Recent investigations have highlighted the potential of targeting glutamine metabolism as a promising therapeutic approach for AML. Asparaginases, enzymes that deplete circulating glutamine and asparagine, are approved for the treatment of acute lymphoblastic leukemia, but are also under investigation in AML, with promising results. We previously reported an elevation in plasma serine levels following treatment with Erwinia-derived asparaginase (also called crisantaspase). This led us to hypothesize that AML cells initiate the de novo serine biosynthesis pathway in response to crisantaspase treatment and that inhibiting this pathway in combination with crisantaspase would enhance AML cell death. Here we report that in AML cell lines, treatment with the clinically available crisantaspase, Rylaze, upregulates the serine biosynthesis enzymes phosphoglycerate dehydrogenase (PHGDH) and phosphoserine aminotransferase (PSAT1) through activation of the Amino Acid Response (AAR) pathway, a cellular stress response mechanism that regulates amino acid metabolism and protein synthesis under conditions of nutrient limitation. Inhibition of serine biosynthesis through CRISPR-Cas9-mediated knockout of PHGDH resulted in a ~250-fold reduction in the half-maximal inhibitory concentration (IC50) for Rylaze, indicating heightened sensitivity to crisantaspase therapy. Treatment of AML cells with a combination of Rylaze and a small molecule inhibitor of PHGDH (BI4916) revealed synergistic anti-proliferative effects in both cell lines and primary AML patient samples. Rylaze-BI4916 treatment in AML cell lines led to the inhibition of cap-dependent mRNA translation and protein synthesis, as well as a marked decrease in intracellular glutathione levels, a critical cellular antioxidant. Collectively, our results highlight the clinical potential of targeting serine biosynthesis in combination with crisantaspase as a novel therapeutic strategy for AML.

16.
J Cardiovasc Comput Tomogr ; 18(3): 267-273, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38360501

RESUMEN

BACKGROUND: The use of cardiac CT (CCT) has increased dramatically in recent years among patients with pediatric and congenital heart disease (CHD), but little is known about trends and practice pattern variation in CCT utilization for this population among centers. METHODS: A 21-item survey was created to assess CCT utilization in the pediatric/CHD population in calendar years 2011 and 2021. The survey was sent to all non-invasive cardiac imaging directors of pediatric cardiology centers in North America in September 2022. RESULTS: Forty-one centers completed the survey. In 2021, 98% of centers performed CCT in pediatric and CHD patients (vs. 73% in 2011), and 61% of centers performed >100 CCTs annually (vs. 5% in 2011). While 62% of centers in 2021 utilized dual-source technology for high-pitch helical acquisition, 15% of centers reported primarily performing CCT on a 64-slice scanner. Anesthesia utilization, use of medications for heart rate control, and type of subspecialty training for physicians interpreting CCT varied widely among centers. 50% of centers reported barriers to CCT performance, with the most commonly cited concerns being radiation exposure, the need for anesthesia, and limited CT scan staffing or machine access. 37% (11/30) of centers with a pediatric cardiology fellowship program offer no clinical or didactic CCT training for categorical fellows. CONCLUSION: While CCT usage in the CHD/pediatric population has risen significantly in the past decade, there is broad center variability in CCT acquisition techniques, staffing, workflow, and utilization. Potential areas for improvement include expanding CT scanner access and staffing, formal CCT education for pediatric cardiology fellows, and increasing utilization of existing technological advances.


Asunto(s)
Encuestas de Atención de la Salud , Cardiopatías Congénitas , Pautas de la Práctica en Medicina , Valor Predictivo de las Pruebas , Humanos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/terapia , Pautas de la Práctica en Medicina/tendencias , América del Norte , Niño , Factores de Edad , Preescolar , Lactante , Tomografía Computarizada por Rayos X/tendencias , Adolescente , Recién Nacido , Factores de Tiempo , Masculino , Femenino , Exposición a la Radiación , Angiografía Coronaria/tendencias , Angiografía Coronaria/estadística & datos numéricos
17.
Ann Thorac Surg ; 116(6): 1124-1141, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37855783

RESUMEN

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.


Asunto(s)
Anomalías de los Vasos Coronarios , Vasos Coronarios , Humanos , Adulto , Vasos Coronarios/cirugía , Anomalías de los Vasos Coronarios/complicaciones , Anomalías de los Vasos Coronarios/diagnóstico , Anomalías de los Vasos Coronarios/cirugía , Aorta
18.
J Am Coll Cardiol ; 82(21): 2034-2053, 2023 Nov 21.
Artículo en Inglés | MEDLINE | ID: mdl-37855757

RESUMEN

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.


Asunto(s)
Anomalías de los Vasos Coronarios , Vasos Coronarios , Adulto , Humanos , Vasos Coronarios/cirugía , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Anomalías de los Vasos Coronarios/terapia , Estudios Retrospectivos , Aorta
19.
J Cardiovasc Comput Tomogr ; 17(2): 86-95, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36934047

RESUMEN

This review aims to summarize key articles published in the Journal of Cardiovascular Computed Tomography (JCCT) in 2022, focusing on those that had the most scientific and educational impact. The JCCT continues to expand; the number of submissions, published manuscripts, cited articles, article downloads, social media presence, and impact factor continues to grow. The articles selected by the Editorial Board of the JCCT in this review highlight the role of cardiovascular computed tomography (CCT) to detect subclinical atherosclerosis, assess the functional relevance of stenoses, and plan invasive coronary and valve procedures. A section is dedicated to CCT in infants and other patients with congenital heart disease, in women, and to the importance of training in CT. In addition, we highlight key consensus documents and guidelines published in JCCT last year. The Journal values the tremendous work by authors, reviewers, and editors to accomplish these contributions.


Asunto(s)
Estenosis de la Válvula Aórtica , Sistema Cardiovascular , Reemplazo de la Válvula Aórtica Transcatéter , Femenino , Humanos , Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/cirugía , Angiografía por Tomografía Computarizada , Constricción Patológica , Corazón , Valor Predictivo de las Pruebas , Tomografía Computarizada por Rayos X/métodos , Reemplazo de la Válvula Aórtica Transcatéter/métodos
20.
Front Pediatr ; 10: 820229, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35433557

RESUMEN

The acute manifestations and short-term outcomes of multisystem inflammatory syndrome (MIS-C) have been extensively described; however, our understanding of the longitudinal outcomes associated with this condition continue to evolve. Here we review the existing literature on outcomes of MIS-C up to 1 year following diagnosis and summarize current published expert recommendations for management and follow up of these patients.

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