In Vivo Evaluation of Bioprinted Cardiac Patches Composed of Cardiac-Specific Extracellular Matrix and Progenitor Cells in a Model of Pediatric Heart Failure

2021 ◽  
Author(s):  
Donald Bejleri ◽  
Matthew Robeson ◽  
Milton Brown ◽  
Jervaughn Hunter ◽  
Joshua Maxwell ◽  
...  
Keyword(s):  
Heart Failure ◽  
Extracellular Matrix ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
Heart Defects ◽  
In Vivo Evaluation ◽  
Surgical Methods ◽  
Pediatric Heart Failure ◽  
The Right

Pediatric patients with congenital heart defects (CHD) often present with heart failure from increased load on the right ventricle (RV) due to both surgical methods to treat CHD and the...

Late Dissection of a Contegra Conduit: A Rare Complication

2019 ◽  
Vol 10 (2) ◽  
pp. 239-241
Author(s):  
Christian Ortega-Loubon ◽  
Joaquín Fernández-Doblas ◽  
Joaquín Pérez-Andreu ◽  
Manuel Fernández-Molina ◽  
María Sol Siliato ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Congenital Heart ◽  
Heart Defects ◽  
Rare Complication ◽  
Right Heart Failure ◽  
Right Heart ◽  
Surgical Palliation ◽  
The Right

Surgical palliation of many types of congenital heart defects requires the use of a conduit between the right ventricle and the pulmonary artery. Dissections of these conduits are very infrequent. We report a case of a ten-year-old girl who developed acute right heart failure related to a Contegra conduit dissection. She underwent a new conduit replacement on an emergency basis.

Abstract 416: Pluripotent Stem Cell Signaling in Pediatric Heart Failure Patients

2016 ◽  
Vol 119 (suppl_1) ◽  
Author(s):  
Kathleen C Woulfe ◽  
Phillip Tatman ◽  
Anis Karimpour-Fard ◽  
Xuan Jiang ◽  
Karin Nunley ◽  
...  
Keyword(s):  
Heart Failure ◽  
Stem Cell ◽  
Cell Signaling ◽  
Dilated Cardiomyopathy ◽  
Pediatric Patients ◽  
Disease Process ◽  
Idiopathic Dilated Cardiomyopathy ◽  
Cytokine Signaling ◽  
Common Indication ◽  
Pediatric Heart Failure

Pediatric heart failure is a heterogeneous disease process with congenital heart disease being the most common indication for heart transplantation in infants, and dilated cardiomyopathy (DCM) the most common indication in older children. Due to limitations of studying pediatric populations, there is no specific therapy for children with DCM. Importantly, the application of proven adult therapies to pediatric patients with DCM has resulted in no substantial improvement in survival over the past three decades. Therefore, there is a clear need for new approaches to better understand this disease process. In this study, we investigated global cardiac transcriptome and circulating serum proteome changes in pediatric patients with idiopathic dilated cardiomyopathy (IDC) and non-failing controls. Changes were identified in cytokine signaling, signal transduction, and transcription in pediatric IDC patients when compared to non-failing controls. Interestingly, these changes closely resemble the induction of stem cell pluripotency and signaling in resting stem cell populations. Importantly, the transcriptome changes are age-specific and were not observed in adult IDC patients. In addition, the protein profile in serum from pediatric IDC patients identified several factors related to inflammation, and treatment of cardiomyocyte-derived induced pluripotent stem cells (iPSC) with pediatric IDC serum recapitulated pathologic changes in gene expression, including expression of genes altered in the failing pediatric heart. These findings suggest that pathologic changes in cardiomyocytes may be triggered by circulating factors present in the serum resulting in pluripotent and stem cell signaling. These results provide several novel targets for future research and therapeutic intervention.

Abstract 418: Caveolar Disruption of L-type Calcium Channel and Ryanodine Receptor Facilitates Atrial Ectopy and Arrhythmogenesis in Heart Failure Mice

2020 ◽  
Vol 127 (Suppl_1) ◽  
Author(s):  
Di Lang ◽  
Lucas ratajczyk ◽  
Leonid Tyan ◽  
Daniel Turner ◽  
Francisco Alvarado ◽  
...  
Keyword(s):  
Heart Failure ◽  
Optical Mapping ◽  
Ryanodine Receptors ◽  
Tissue Level ◽  
Confocal Imaging ◽  
Right Atrial ◽  
Isolated Atria ◽  
The Right

Atrial fibrillation (AF) often occurs during heart failure (HF). Ectopic foci that trigger AF, are linked to discrete atrial regions that experience the highest remodeling and clinically used for AF ablation; however, mechanisms of their arrhythmogenic propensity remain elusive. We employed in vivo ECG telemetry, in vitro optical mapping and confocal imaging of Ca 2+ transients (CaT) from myocytes isolated from the right atrial appendage (RAA) and inter-caval region (ICR) of wild type (WT, n=10), caveolin-3 knockout (KO, n=6) and 8-weeks post-myocardial infarction HF (n=8) mice. HF and KO mice showed an increased susceptibility to pacing-induced AF and enhanced ectopy originated exclusively from ICR. Optical mapping in isolated atria showed prolongation of CaT rise up time (CaT-RT) in HF ICR, which suggested a remodeled coupling between L-type Ca 2+ channels (LTCCs) and ryanodine receptors (RyRs) in this specific region. In WT mice, RAA consists of structured myocytes with a prominent transverse-axial tubular system (TATS) while ICR myocytes don’t have TATS. In RAA, CaT-RT depends on LTCCs in TATS triggering RyR, while in ICR, all the LTCCs are localized in surface caveolae where they can activate subsarcolemmal RyRs and lead to a slow diffusion of Ca 2+ inside the cell interior. Downregulation of caveolae was observed specifically in HF ICR. To mimic this, we used cav3-KO mice. Triggered activities were observed in myocytes isolated from HF and KO ICR, which presumably underlie the ectopic activities in tissue level. These myocytes presented significantly unsynchronized sarcoplasmic reticulum (SR) Ca 2+ releases (synchronization index: 10.8±0.9 in WT vs 38.3±4.1 in HF vs 21.5±2.1 in KO, p <0.01 for HF and KO vs WT respectively) especially at the subsarcolemmal space that prolongs CaT-RT (62.2±4.1 ms in WT vs 122.5±12.8 ms in KO, p <0.01). In addition, failing ICR myocytes showed a higher occurrence and size of spontaneous Ca 2+ sparks which were linked to CaMKII activity and associated phosphorylation of RyR. Our findings demonstrate that in HF, caveolar disruption creates “hot spots” for arrhythmogenic ectopic activity emanated from discrete vulnerable regions of the right atrium which are associated with desynchronized SR Ca 2+ release and elevated fibrosis.

Cardiac embryogenesis

ESC CardioMed ◽  
2018 ◽  
pp. 33-36
Author(s):  
Robert G. Kelly
Keyword(s):  
Progenitor Cells ◽  
Progenitor Cell ◽  
Congenital Heart ◽  
Heart Defects ◽  
Embryonic Heart ◽  
Heart Tube ◽  
Second Heart Field ◽  
Heart Field ◽  
Cell Niche ◽  
The Right

The embryonic heart forms in anterior lateral splanchnic mesoderm and is derived from Mesp1-expressing progenitor cells. During embryonic folding, the earliest differentiating progenitor cells form the linear heart tube in the ventral midline. The heart tube extends in length and loops to the right as new myocardium is progressively added at the venous and arterial poles from multipotent second heart field cardiovascular progenitor cells in contiguous pharyngeal mesoderm. While the linear heart tube gives rise to the left ventricle, the right ventricle, outflow tract, and a large part of atrial myocardium are derived from the second heart field. Progressive myocardial differentiation is controlled by intercellular signals within the progenitor cell niche. The embryonic heart is the template for septation and growth of the four-chambered definitive heart and defects in progenitor cell deployment result in a spectrum of common forms of congenital heart defects.

Transition from compensatory hypertrophy to dilated, failing left ventricles in Dahl salt-sensitive rats

1994 ◽  
Vol 267 (6) ◽  
pp. H2471-H2482 ◽  
Author(s):  
M. Inoko ◽  
Y. Kihara ◽  
I. Morii ◽  
H. Fujiwara ◽  
S. Sasayama
Keyword(s):  
Heart Failure ◽  
Systolic Pressure ◽  
Myocardial Necrosis ◽  
Compensatory Hypertrophy ◽  
High Salt ◽  
High Salt Diet ◽  
Tension Development ◽  
Salt Diet ◽  
The Right

To establish an experimental model for studying a specific transitional stage for compensatory hypertrophy to heart failure, we studied the pathophysiology of the left ventricle (LV) in Dahl salt-sensitive (DS) rats fed a high-salt diet. DS rats fed an 8% NaCl diet after the age of 6 wk developed concentric LV hypertrophy at 11 wk, followed by marked LV dilatation at 15-20 wk. During the latter stage, the DS rats showed labored respiration with LV global hypokinesis. All the DS rats died within 1 wk by massive pulmonary congestion. The dissected left ventricles revealed chamber dilatation and a marked increase in mass without myocardial necrosis. In contrast, corresponding Dahl salt-resistant (DR) rats fed the same diet showed neither mortality nor any of these pathological changes. The in vivo LV end-systolic pressure-volume relationship shifted to the right with a less steep slope in the failing DS rats compared with that in age-matched DR rats. Isometric contractions of LV papillary muscles isolated from these DS rats showed reduced tension development in the failing stage, but normal tension development in the hypertrophied stage. In conclusion, the DS rat fed a high-salt diet is a useful model showing rapidly developing congestive heart failure, in which the transition from compensatory hypertrophy to decompensatory dilatation of LV is easily and consistently manifested.

scholarly journals In-vitro and in-vivo evaluation of a novel bioprosthetic pulmonary valve for use in congenital heart surgery

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Jonas Rasmussen ◽  
Søren Nielsen Skov ◽  
Ditte Bruus Nielsen ◽  
Ida Lindhardt Jensen ◽  
Marcell Juan Tjørnild ◽  
...  
Keyword(s):  
Congenital Heart ◽  
Heart Surgery ◽  
Pulmonary Valve ◽  
Congenital Heart Surgery ◽  
In Vivo Evaluation

scholarly journals Where the congenital heart disease meets the pulmonary arterial hypertension, FLNA matters: a case report and literature review

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Xiaoxian Deng ◽  
Shanshan Li ◽  
Qiu Qiu ◽  
Bowen Jin ◽  
Menghuan Yan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
De Novo ◽  
Wide Spectrum ◽  
Heart Defects ◽  
Pulmonary Arterial

Abstract Background Pediatric patients with genetic disorders have a higher incidence of pulmonary arterial hypertension (PAH) regardless of their heart defects. Filamin A (FLNA) mutation is recently recognized to be associated with pediatric pulmonary disorders, however, the clinical courses of PAH related to the mutation were reported in limited cases. Here, we presented a case and pooled data for better understanding of the correlation between FLNA mutation and pediatric PAH. Case presentation The patient was a 8-month-old female with repeated episodes of pneumonia. Physical examination revealed cleft lip, cleft palate and developmental retardation. Imaging examination showed a small atrial septal defect (ASD), central pulmonary artery enlargement, left upper lobe of lung atelectasis, and pulmonary infiltration. Genetic test showed she carried a de novo pathogenic variant of FLNA gene (c.5417-1G > A, p.-). Oral medications didn’t slow the progression of PAH in the patient, and she died two years later. Conclusions FLNA mutation causes rare but progressive PAH in addition to a wide spectrum of congenital heart disease and other comorbidities in pediatric patients. We highly recommend genetic testing for pediatric patients when suspected with PAH. Given the high mortality in this group, lung transplantation may offer a better outcome.

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