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1.
Pediatr Cardiol ; 45(5): 967-975, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38480569

RESUMEN

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm2/BSAm2) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge.


Asunto(s)
Aorta Torácica , Ecocardiografía , Defectos del Tabique Interventricular , Reoperación , Obstrucción del Flujo Ventricular Externo , Humanos , Femenino , Estudios Retrospectivos , Masculino , Aorta Torácica/cirugía , Aorta Torácica/diagnóstico por imagen , Obstrucción del Flujo Ventricular Externo/cirugía , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagen , Defectos del Tabique Interventricular/cirugía , Defectos del Tabique Interventricular/diagnóstico por imagen , Lactante , Complicaciones Posoperatorias , Recién Nacido , Resultado del Tratamiento , Procedimientos Quirúrgicos Cardíacos/métodos
2.
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
3.
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
4.
J Cardiovasc Magn Reson ; 23(1): 61, 2021 05 24.
Artículo en Inglés | MEDLINE | ID: mdl-34024274

RESUMEN

BACKGROUND: In repaired tetralogy of Fallot (rTOF), abnormal left ventricular (LV) rotational mechanics are associated with adverse clinical outcomes. We performed a comprehensive analysis of LV rotational mechanics in rTOF patients using cardiac magnetic resonance (CMR) prior to and following surgical pulmonary valve replacement (PVR). METHODS: In this single center retrospective study, we identified rTOF patients who (1) had both a CMR ≤ 1 year before PVR and ≤ 5 years after PVR, (2) had no other intervening procedure between CMRs, (3) had a body surface area > 1.0 m2 at CMR, and (4) had images suitable for feature tracking analysis. These subjects were matched to healthy age- and sex-matched control subjects. CMR feature tracking analysis was performed on a ventricular short-axis stack of balanced steady-state free precession images. Measurements included LV basal and apical rotation, twist, torsion, peak systolic rates of rotation and torsion, and timing of events. Associations with LV torsion were assessed. RESULTS: A total of 60 rTOF patients (23.6 ± 7.9 years, 52% male) and 30 healthy control subjects (20.8 ± 3.1 years, 50% male) were included. Compared with healthy controls, rTOF patients had lower apical and basal rotation, twist, torsion, and systolic rotation rates, and these parameters peaked earlier in systole. The only parameters that were correlated with LV torsion were right ventricular (RV) end-systolic volume (r = - 0.28, p = 0.029) and RV ejection fraction (r = 0.26, p = 0.044). At a median of 1.0 year (IQR 0.5-1.7) following PVR, there was no significant change in LV rotational parameters versus pre-PVR despite reductions in RV volumes, RV mass, pulmonary regurgitation, and RV outflow tract obstruction. CONCLUSION: In this comprehensive study of CMR-derived LV rotational mechanics in rTOF patients, rotation, twist, and torsion were diminished compared to controls and did not improve at a median of 1 year after PVR despite favorable RV remodeling.


Asunto(s)
Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Tetralogía de Fallot , Femenino , Humanos , Masculino , Valor Predictivo de las Pruebas , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Insuficiencia de la Válvula Pulmonar/diagnóstico por imagen , Insuficiencia de la Válvula Pulmonar/etiología , Insuficiencia de la Válvula Pulmonar/cirugía , Estudios Retrospectivos , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/cirugía
5.
Echocardiography ; 38(9): 1657-1661, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-34510548

RESUMEN

Cervical aortic arch (CAA) and retro-aortic innominate vein (RAIV) are rare entities which can be associated with one another in a structurally normal heart. Less well recognized is the fact, that a third entity may often be present as well, since aortic arch abnormalities are common in a CAA. Hence, these three entities (CAA, RAIV, and an arch anomaly - in this case a post-ductal coarctation) may present as a triad. We present the first prenatal diagnosis of this rare triad which can be explained by common embryological origins which are discussed along with instructive aspects in diagnosis and management.


Asunto(s)
Coartación Aórtica , Cardiopatías Congénitas , Aorta Torácica/diagnóstico por imagen , Coartación Aórtica/diagnóstico por imagen , Venas Braquiocefálicas/diagnóstico por imagen , Ecocardiografía , Femenino , Humanos , Embarazo , Diagnóstico Prenatal
6.
Echocardiography ; 36(7): 1418-1420, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-31162725

RESUMEN

Transcatheter closure of mitral paravalvular leaks has become an accepted alternative to surgical repair, especially in patients at high risk for reoperation. Device placement using three-dimensional (3D) transesophageal echocardiography (TEE) guidance allows en-face views of the valve and provides detailed information for device sizing and placement. We present a case demonstrating hybrid transapical transcatheter 3D TEE-guided closure of two mitral paravalvular leaks. We demonstrate real time 3D TEE guidance for device sizing and placement, and early real time recognition of mechanical mitral valve leaflet impairment during device deployment.


Asunto(s)
Ecocardiografía Tridimensional , Ecocardiografía Transesofágica , Implantación de Prótesis de Válvulas Cardíacas/métodos , Insuficiencia de la Válvula Mitral/diagnóstico por imagen , Insuficiencia de la Válvula Mitral/cirugía , Ultrasonografía Intervencional , Adulto , Cateterismo Cardíaco , Femenino , Fluoroscopía , Prótesis Valvulares Cardíacas , Humanos , Insuficiencia de la Válvula Mitral/fisiopatología , Ajuste de Prótesis
7.
Pediatr Transplant ; 22(8): e13286, 2018 12.
Artículo en Inglés | MEDLINE | ID: mdl-30178513

RESUMEN

RV systolic function is important early after HT; however, it has not been critically assessed in children using quantitative measures. The aim of this study was to describe the most validated and commonly used quantitative echocardiographic measures of RV systolic function early after pediatric HT and to assess associations with qualitative function evaluation and clinical factors. RV systolic function was quantified on the first post-HT echocardiogram >24 hours after cardiopulmonary bypass using two-dimensional TAPSE, Tricuspid annular S', FAC, and MPI. In 145 patients (median age 7.6 years), quantitative RV systolic function was markedly abnormal: mean TAPSE z-score -8.43 ± 1.89; S' z-score -4.36 ± 1.22; FAC 24.4 ± 8.34%; and MPI 0.86 ± 0.51. Few patients had normal quantitative function: TAPSE (0%), S' (1.2%), FAC (9.4%), and MPI (28.4%). In contrast, 48.3% were observed as normal by qualitative assessment. Most clinical factors, including diagnosis, pulmonary vascular resistance, posttransplant hemodynamics, inotropic support, and rejection, were not associated with RV function. In this large pediatric HT population, TAPSE, S', FAC, and MPI were strikingly abnormal early post-HT despite reassuring qualitative assessment and no significant association with clinical factors. This suggests that the accepted normal values of these quantitative measures may not apply in the early post-HT period to accurately grade RV systolic function, and there may be utility in adapting a concept of normal reference values after pediatric HT.


Asunto(s)
Insuficiencia Cardíaca/cirugía , Trasplante de Corazón , Sístole , Disfunción Ventricular Derecha/diagnóstico , Función Ventricular Derecha , Adolescente , Niño , Preescolar , Ecocardiografía , Femenino , Humanos , Lactante , Masculino , Variaciones Dependientes del Observador , Periodo Posoperatorio , Reproducibilidad de los Resultados , Estudios Retrospectivos , Adulto Joven
8.
Pediatr Cardiol ; 39(7): 1404-1412, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-29789916

RESUMEN

Speckle tracking echocardiography (STE)-derived strain indices are believed to detect early cardiac dysfunction in survivors of childhood cancer and have potential to identify patients who may benefit from early heart failure treatment. However, effects of heart failure treatment on STE-derived strain measurements in this population are unknown. The aim of this study was to assess STE-derived strain measurements in survivors of childhood cancer treated with angiotensin converting enzyme inhibition or receptor blockade (ACEi/ARB). Two-dimensional speckle tracking analysis was retrospectively performed on echocardiograms from childhood cancer survivors before and during therapy with ACEi/ARB. Global left ventricular longitudinal and circumferential strain (GLS and GCS) and strain rates (LSR and CSR) were assessed and correlated with conventional echocardiographic measures of function. In 22 childhood cancer survivors (median age: 14.8, range 6.4-21.6 years), mean GLS (- 13.83 ± 0.74% to - 15.94 ± 0.74%, p = 0.002), GCS (- 18.79 ± 1.21% to - 20.74 ± 0.84%, p = 0.027), LSR (- 0.78 ± 0.04 to - 0.88 ± 0.04 s-1, p = 0.022), and CSR (- 1.08 ± 0.07 to - 1.21 ± 0.06 s-1, p = 0.027) improved on therapy. Improvement in GLS was maintained for greater than 1 year on ACEi/ARB (p = 0.02). Measures of strain and strain rate correlated with standard echocardiographic measures of function and were reproducible. These findings support the use of ACEi/ARB to treat post-chemotherapy-related cardiovascular changes in childhood cancer survivors, provide proof-of-concept that STE-derived strain and strain rate may be used to reliably monitor cardiac function during therapy, and support continued investigation into the clinical benefit of strain measurements in this population.


Asunto(s)
Antagonistas de Receptores de Angiotensina/uso terapéutico , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Antraciclinas/efectos adversos , Ecocardiografía/métodos , Función Ventricular Izquierda/efectos de los fármacos , Adolescente , Adulto , Antraciclinas/uso terapéutico , Antineoplásicos/efectos adversos , Supervivientes de Cáncer/estadística & datos numéricos , Niño , Estudios de Cohortes , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/efectos de los fármacos , Ventrículos Cardíacos/fisiopatología , Humanos , Masculino , Neoplasias/tratamiento farmacológico , Reproducibilidad de los Resultados , Estudios Retrospectivos , Función Ventricular Izquierda/fisiología , Adulto Joven
9.
Cardiol Young ; 28(5): 773-775, 2018 May.
Artículo en Inglés | MEDLINE | ID: mdl-29502551

RESUMEN

If coronary artery sequelae are the only suggestive signs of previous Kawasaki disease, the diagnosis may easily be missed. We describe a rare case of a child with severe occlusive coronary disease likely owing to missed Kawasaki disease. This diagnosis was not initially considered given the age and absence of suggestive history. Careful echocardiographic assessment and low-radiation coronary CT angiogram resulted in successful diagnosis and treatment.


Asunto(s)
Cardiomiopatía Dilatada/diagnóstico , Oclusión Coronaria/etiología , Vasos Coronarios/diagnóstico por imagen , Errores Diagnósticos , Síndrome Mucocutáneo Linfonodular/diagnóstico , Niño , Oclusión Coronaria/diagnóstico , Diagnóstico Diferencial , Ecocardiografía Doppler en Color , Electrocardiografía , Humanos , Masculino , Síndrome Mucocutáneo Linfonodular/complicaciones
10.
Am J Physiol Regul Integr Comp Physiol ; 313(3): R265-R271, 2017 Sep 01.
Artículo en Inglés | MEDLINE | ID: mdl-28615160

RESUMEN

Heart rate variability (HRV) has become an important clinical marker of cardiovascular health and a research measure for the study of the cardiac conduction system and its autonomic controls. While the zebrafish (Danio rerio) is an ideal vertebrate model for understanding heart development, HRV has only recently been investigated in this system. We have previously demonstrated that nkx2.5 and nkx2.7, two homologues of Nkx2-5 expressed in zebrafish cardiomyocytes, play vital roles in maintaining cardiac chamber-specific characteristics. Given observed defects in ventricular and atrial chamber identities in nkx2.5-/- embryos coupled with conduction system abnormalities in murine models of Nkx2.5 insufficiency, we postulated that reduced HRV would serve as a marker of poor cardiac health in nkx2.5 mutants and in other zebrafish models of human congenital heart disease. Using live video image acquisition, we derived beat-to-beat intervals to compare HRV in wild-type and nkx2.5-/- embryos. Our data illustrate that the nkx2.5 loss-of-function model exhibits increased heart rate and decreased HRV when compared with wild type during embryogenesis. These findings validate HRV analysis as a useful quantitative tool for assessment of cardiac health in zebrafish and underscore the importance of nkx2.5 in maintaining normal heart rate and HRV during early conduction system development.


Asunto(s)
Desarrollo Embrionario/fisiología , Frecuencia Cardíaca/fisiología , Proteína Homeótica Nkx-2.5/metabolismo , Proteínas de Pez Cebra/metabolismo , Pez Cebra/embriología , Pez Cebra/fisiología , Animales
11.
J Pediatr Hematol Oncol ; 39(7): 500-505, 2017 10.
Artículo en Inglés | MEDLINE | ID: mdl-28859033

RESUMEN

BACKGROUND: Cardiac abnormalities have been described in echocardiograms of children with sickle cell disease (SCD). However, longitudinal studies investigating progression of echocardiographic abnormalities across the pediatric age spectrum in SCD are lacking. METHODS: A retrospective longitudinal analysis of 829 echocardiograms from pediatric patients with SCD at steady-state was performed. Left heart parameters included left ventricular end-systolic, end-diastolic diameters, fractional shortening, and mass. Right ventricular pressure was estimated by tricuspid regurgitation gradient. Tricuspid regurgitation gradient ≥25 mm Hg, a z-score ≥2 for LV parameters and ≤-2 for left ventricular fractional shortening were considered abnormal. RESULTS: Kaplan-Meier analysis revealed that echocardiographic abnormalities were detected by 5 years of age, and the cumulative incidence progressively increased throughout childhood. Age, male gender, HbSS and Sß thalassemia genotype, white blood cell count, platelet count, total bilirubin, admissions for pain crises and acute chest syndrome were positively, whereas hemoglobin was negatively associated with cardiac abnormalities. CONCLUSION: Cardiac abnormalities began early in childhood and progressively increased with age. Our study highlights the high cumulative incidence of cardiac abnormalities in children with SCD, which could represent a marker of disease severity.


Asunto(s)
Anemia de Células Falciformes/complicaciones , Cardiopatías/patología , Niño , Progresión de la Enfermedad , Ecocardiografía , Femenino , Cardiopatías/diagnóstico por imagen , Cardiopatías/etiología , Humanos , Estimación de Kaplan-Meier , Estudios Longitudinales , Masculino , Estudios Retrospectivos , Índice de Severidad de la Enfermedad
12.
Cardiol Young ; : 1-4, 2017 May 29.
Artículo en Inglés | MEDLINE | ID: mdl-28552080

RESUMEN

The mitral-aortic intervalvular fibrosa is an area of fibrous continuity between the mitral and aortic valves. We present the first case of a congenital pseudoaneurysm in this region, detected prenatally as an isolated cardiac defect, which was followed-up conservatively postnatally. The diagnosis was confirmed by echocardiogram demonstrating blood flow into the pouch during systole and into the left ventricular outflow tract during diastole. The infant has been followed-up with serial echocardiograms demonstrating stable size and appearance of the lesion, without signs of obstruction, making close continued observation a reasonable approach.

13.
J Am Soc Echocardiogr ; 36(3): 310-323, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36414123

RESUMEN

BACKGROUND: Strain values vary with age in children and are both vendor and platform specific. Philips QLAB 10.8 and TomTec AutoSTRAIN are two widely used strain analysis platforms, and both incorporate recent European Association of Cardiovascular Imaging/American Society of Echocardiography/Industry Task Force to Standardize Deformation Imaging guidelines. The aims of this study were to establish normal strain values and Z scores for both platforms using a large data set of healthy children and to compare values among these two platforms and a previous version, QLAB 10.5, which predated the task force guidelines. METHODS: Echocardiograms from 1,032 subjects <21 years old with structurally and functionally normal hearts were included. Images were obtained on the Philips EPIQ platform. Left ventricular (LV) and right ventricular (RV) strain was analyzed using QLAB 10.8 and AutoSTRAIN, and measurement reliability was assessed. Z score equations were derived as a function of age for QLAB 10.8 (LV longitudinal and circumferential strain) and AutoSTRAIN (LV and RV longitudinal strain). A subset (n = 309) was analyzed using QLAB 10.5. Strain values were compared among the three platforms. RESULTS: For both of the newer platforms, strain varied with age, with magnitude reaching a maximum at 4 to 5 years. For LV longitudinal strain, the largest differences in value were observed in the youngest patients when using QLAB 10.5; the other two platforms were similar. LV circumferential strain measurements (QLAB 10.5 vs QLAB 10.8) were different for all ages, as were measurements of RV longitudinal strain (QLAB 10.8 vs AutoSTRAIN). Reliability was greater for AutoSTRAIN than for QLAB 10.8 and greater for LV than for RV strain. CONCLUSIONS: Normal RV and LV strain values and Z scores were generated from a large cohort of children for two commonly used platforms in pediatric echocardiography laboratories. Following the incorporation of task force guidelines, the greatest improvement in standardization was seen in infants. Small differences persist between modern platforms; however, these results support the cautious consideration of comparing interplatform measurements.


Asunto(s)
Ecocardiografía , Ventrículos Cardíacos , Lactante , Niño , Humanos , Preescolar , Adulto Joven , Adulto , Valores de Referencia , Reproducibilidad de los Resultados , Estudios Prospectivos , Ecocardiografía/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Función Ventricular Izquierda
14.
JACC Adv ; 2(4): 100360, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38938250

RESUMEN

Background: Abnormal left ventricular (LV) rotational mechanics in biventricular hearts are associated with adverse outcomes; however, these are less well characterized for hearts with functionally single ventricles. Objectives: The purpose of this study was to characterize ventricular rotational mechanics in the Fontan circulation and their relationship to outcomes. Methods: Single-center, retrospective analysis of magnetic resonance examinations for 329 Fontan patients (15 [IQR: 10-21] years) and 42 controls. The ventricular cine short-axis stack was analyzed to derive torsion metrics. Torsion calculated as the difference between apical and basal rotation normalized to ventricular length. Results: Fontan patients had higher indexed ventricular end-diastolic volume (97 mL/body surface area1.3 vs 72 mL/body surface area1.3), lower ejection fraction (53% vs 60%), and lower proportion of basal clockwise rotation (62% vs 93%), apical counterclockwise rotation (77% vs 95%), and positive torsion (82% vs 100%); P < 0.001 for all. A composite outcome of death or heart transplant-listing occurred in 31 (9%) patients at a median follow-up of 3.9 years. Torsion metrics were associated with the outcome; although, on multivariate analysis only right ventricular (RV) morphology and indexed ventricular end-diastolic volume were independently associated. LVs with negative torsion, and RVs regardless of torsional pattern, had worse outcomes compared to LVs with positive torsion (P = 0.020). Conclusions: Single ventricles in a Fontan circulation exhibit abnormal torsional mechanics, which are more pronounced for RV morphology. Abnormal torsion is associated with death or need for heart transplantation. Fontan patients with LV morphology and preserved torsion exhibit the highest transplant-free survival and torsion may offer incremental prognostic data in this group of patients.

15.
J Am Heart Assoc ; 12(11): e029706, 2023 06 06.
Artículo en Inglés | MEDLINE | ID: mdl-37259984

RESUMEN

Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21-27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow-up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live-born. Of 158 with postnatal data (median follow-up 3.7 years), 10 (6.6%) had death/transplant before 1 year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1 year included ≥ mild tricuspid regurgitation, pulmonary atresia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). Conclusions Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.


Asunto(s)
Bloqueo Atrioventricular , Cardiopatías Congénitas , Atresia Pulmonar , Transposición de los Grandes Vasos , Insuficiencia de la Válvula Tricúspide , Femenino , Humanos , Embarazo , Lactante , Transposición Congénitamente Corregida de las Grandes Arterias , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/cirugía , Transposición de los Grandes Vasos/complicaciones , Insuficiencia de la Válvula Tricúspide/complicaciones , Bloqueo Atrioventricular/complicaciones , Estudios Retrospectivos , Estudios de Seguimiento , Diagnóstico Prenatal , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/complicaciones , Corazón Fetal/diagnóstico por imagen , Corazón Fetal/patología , Arritmias Cardíacas/complicaciones , Muerte Fetal
16.
FASEB J ; 25(8): 2731-9, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21566209

RESUMEN

We developed a tissue-engineered vascular graft composed of biodegradable scaffold seeded with autologous bone marrow-derived mononuclear cells (BMMCs) that is currently in clinical trial and developed analogous mouse models to study mechanisms of neovessel formation. We previously reported that seeded human BMMCs were rapidly lost after implantation into immunodeficient mice as host macrophages invaded the graft. As a consequence, the resulting neovessel was entirely of host cell origin. Here, we investigate the source of neotissue cells in syngeneic BMMC-seeded grafts, implanted into immunocompetent mouse recipients. We again find that seeded BMMCs are lost, declining to 0.02% at 14 d, concomitant with host macrophage invasion. In addition, we demonstrate using sex-mismatched chimeric hosts that bone marrow is not a significant source of endothelial or smooth muscle cells that comprise the neovessel. Furthermore, using composite grafts formed from seeded scaffold anastomosed to sex-mismatched natural vessel segments, we demonstrate that the adjacent vessel wall is the principal source of these endothelial and smooth muscle cells, forming 93% of proximal neotissue. These findings have important implications regarding fundamental mechanisms underlying neotissue formation; in this setting, the tissue-engineered construct functions by mobilizing the body's innate healing capabilities to "regenerate" neotissue from preexisting committed tissue cells.


Asunto(s)
Prótesis Vascular , Vasos Sanguíneos/fisiología , Regeneración Tisular Dirigida/métodos , Animales , Trasplante de Médula Ósea , Supervivencia Celular , Femenino , Humanos , Leucocitos Mononucleares/trasplante , Masculino , Ratones , Ratones Endogámicos C57BL , Ratones Transgénicos , Andamios del Tejido , Trasplante Isogénico
17.
FASEB J ; 25(12): 4150-61, 2011 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-21846838

RESUMEN

A major limitation of tissue engineering research is the lack of noninvasive monitoring techniques for observations of dynamic changes in single tissue-engineered constructs. We use cellular magnetic resonance imaging (MRI) to track the fate of cells seeded onto functional tissue-engineered vascular grafts (TEVGs) through serial imaging. After in vitro optimization, murine macrophages were labeled with ultrasmall superparamagnetic iron oxide (USPIO) nanoparticles and seeded onto scaffolds that were surgically implanted as inferior vena cava interposition grafts in SCID/bg mice. Serial MRI showed the transverse relaxation times (T(2)) were significantly lower immediately following implantation of USPIO-labeled scaffolds (T(2) = 44 ± 6.8 vs. 71 ± 10.2 ms) but increased rapidly at 2 h to values identical to control implants seeded with unlabeled macrophages (T(2) = 63 ± 12 vs. 63 ± 14 ms). This strongly indicates the rapid loss of seeded cells from the scaffolds, a finding verified using Prussian blue staining for iron containing macrophages on explanted TEVGs. Our results support a novel paradigm where seeded cells are rapidly lost from implanted scaffolds instead of developing into cells of the neovessel, as traditionally thought. Our findings confirm and validate this paradigm shift while demonstrating the first successful application of noninvasive MRI for serial study of cellular-level processes in tissue engineering.


Asunto(s)
Prótesis Vascular , Macrófagos/citología , Ingeniería de Tejidos , Animales , Línea Celular , Supervivencia Celular , Macrófagos/metabolismo , Imagen por Resonancia Magnética , Nanopartículas de Magnetita , Ratones , Ratones SCID , Andamios del Tejido , Vena Cava Inferior/citología , Vena Cava Inferior/cirugía
18.
Nat Commun ; 13(1): 2970, 2022 05 27.
Artículo en Inglés | MEDLINE | ID: mdl-35624100

RESUMEN

The cardiac developmental network has been associated with myocardial regenerative potential. However, the embryonic signals triggered following injury have yet to be fully elucidated. Nkx2.5 is a key causative transcription factor associated with human congenital heart disease and one of the earliest markers of cardiac progenitors, thus it serves as a promising candidate. Here, we show that cardiac-specific RNA-sequencing studies reveal a disrupted embryonic transcriptional profile in the adult Nkx2.5 loss-of-function myocardium. nkx2.5-/- fish exhibit an impaired ability to recover following ventricular apex amputation with diminished dedifferentiation and proliferation. Complex network analyses illuminate that Nkx2.5 is required to provoke proteolytic pathways necessary for sarcomere disassembly and to mount a proliferative response for cardiomyocyte renewal. Moreover, Nkx2.5 targets embedded in these distinct gene regulatory modules coordinate appropriate, multi-faceted injury responses. Altogether, our findings support a previously unrecognized, Nkx2.5-dependent regenerative circuit that invokes myocardial cell cycle re-entry, proteolysis, and mitochondrial metabolism to ensure effective regeneration in the teleost heart.


Asunto(s)
Miocardio , Miocitos Cardíacos , Animales , Ventrículos Cardíacos/metabolismo , Proteína Homeótica Nkx-2.5/genética , Proteína Homeótica Nkx-2.5/metabolismo , Miocardio/metabolismo , Miocitos Cardíacos/metabolismo , Factores de Transcripción/metabolismo
19.
Res Sq ; 2022 Jan 24.
Artículo en Inglés | MEDLINE | ID: mdl-35118462

RESUMEN

Background Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe life-threatening manifestation of SARS-CoV-2 infection. Acute cardiac dysfunction and resultant cardiogenic shock are common in children with MIS-C. While most children recover rapidly from acute illness, the long-term impact on the myocardium and cardiac function is unknown. Methods In this prospective study, cardiac MRI (CMR) was performed on patients <21 years of age with a history of MIS-C, 6-9 months following hospitalization. Per institutional protocol, patients with any history of LVEF<50%, persistent cardiorespiratory symptoms, or ECG abnormalities underwent clinical CMR. Research CMRs were offered to all others >10 years old. Native T1 and T2 mapping values were compared with 20 children with normal CMR examinations. Results We performed CMRs on 13 subjects at a median age of 13.6 years (interquartile range [IQR] 11.9-16.0) and a median time from hospitalization of 8.2 months (IQR 6.8-9.6). Twelve subjects displayed normal ventricular function with a median left ventricle ejection fraction (LVEF) of 57.2% (IQR 56.1-58.4) and median right ventricular (RV) EF of 53.1% (IQR 52.0-55.7). One subject had low normal EF (52%). There was normal T2 and native T1 as compared to normal controls. There was qualitatively no evidence of edema by T2 weighted imaging. One subject had late gadolinium enhancement (LGE) at the inferior insertion point and mid-ventricular inferolateral region, with normal EF, no evidence of edema or perfusion defects, and normal T1 and T2 times. When stratifying by a history of abnormal LVEF (LVEF <55%) on echocardiography, there was no difference in or parametric mapping values, though LVEF and LVEDV approached significance (p=0.06 and 0.05, respectively). Conclusions Although many children with MIS-C present acutely with cardiac dysfunction, myocardial recovery is overall excellent with minimal to no evidence of residual cardiac dysfunction or myocardial involvement. LVEF by CMR at 6-9 months among children with history of echocardiographic LV dysfunction is slightly lower, though does not meet statistical significance and is still within normal range. The long-term functional implications of this finding and the cardiac implications of MIS-C more broadly are unclear and warrant further study.

20.
Magn Reson Imaging ; 81: 42-52, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-33905835

RESUMEN

PURPOSE: To develop and validate a new cardiac self-gating algorithm using blind source separation for 2D cine steady-state free precession (SSFP) imaging. METHODS: A standard cine SSFP sequence was modified so that the center point of k-space was sampled with each excitation. The center points of k-space were processed by 4 blind source separation methods, and used to detect heartbeats and assign k-space data to appropriate time points in the cardiac cycle. The proposed self-gating technique was prospectively validated in 8 patients against the standard electrocardiogram (ECG)-gating method by comparing the cardiac cycle lengths, image quality metrics, and ventricular volume measurements. RESULTS: There was close agreement between the cardiac cycle length using the ECG- and self-gating methods (bias 0.0 bpm, 95% limits of agreement ±2.1 bpm). The image quality metrics were not significantly different between the ECG- and self-gated images. The ventricular volumes, stroke volumes, and mass measured from self-gated images were all comparable with those from ECG-gated images (all biases <5%). CONCLUSION: The self-gating method yielded comparable cardiac cycle length, image quality, and ventricular measurements compared with standard ECG-gated cine imaging. It may simplify patient preparation, be more robust when there is arrhythmia, and allow cardiac gating at higher field strengths.


Asunto(s)
Técnicas de Imagen Sincronizada Cardíacas , Interpretación de Imagen Asistida por Computador , Algoritmos , Corazón/diagnóstico por imagen , Humanos , Imagen por Resonancia Cinemagnética
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