Constrictio Cordis: Can a Thin Pericardium Restrict the Heart?
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ABSTRACT:
SUBMITTER: Elming MB
PROVIDER: S-EPMC6058244 | biostudies-literature | 2017 Dec
REPOSITORIES: biostudies-literature
Publications
Constrictio Cordis: Can a Thin Pericardium Restrict the Heart?
Elming Marie Bayer MB Vejlstrup Niels N Thiis Jens Juel JJ Ihlemann Nikolaj N
CASE (Philadelphia, Pa.) 20171120 6
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Project description:Cardiac congenital defects related to inheritance and teratogenesis have been reported in veterinary species and humans worldwide. Among these, ectopia cordis (EC), characterized by an externalized heart through a cleft, is extremely rare in sheep. This report presents the diagnostic features of two cases of complete thoracic EC in newborn lambs. Clinical findings in the lambs, aside from the EC, were unremarkable. Both animals exhibited exteriorized hearts without pericardial coverage, delineated in the thoracic cleft by a fibrous ring of the pericardium and adjacent skin. Histologically, the epicardium was thickened by fibrous tissue in both lambs, with one animal also showing marked edema, hemorrhage, and neutrophilic inflammatory infiltration. The prognosis of EC in the lambs of this study was poor, with fatal outcomes despite attempts at surgical correction.
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Project description:Ectopia cordis is defined as complete or partial displacement of the heart outside the thoracic cavity. It is a rare congenital defect in fusion of the anterior chest wall resulting in extra thoracic location of the heart. Its estimated prevalence is 5.5-7.9 per million live births. The authors had one such case of a 15-h-old full-term male neonate weighing 2.25 kg with an externally visible, beating heart over the chest wall. The neonate had difficulty in respiration with peripheral cyanosis. Patient died of cardiorespiratory arrest before any surgical intervention could be undertaken inspite of best possible resuscitative measures.
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Project description:ObjectiveThe objective of this study was to evaluate the cost effectiveness of tissue engineered bovine tissue pericardium scaffold (CardioCel) for the repair of congenital heart defects in comparison with surgery using xenogeneic, autologous, and synthetic patches over a 40-year time horizon from the perspective of the UK National Health Service.MethodsA six-state Markov state-transition model to model natural history of disease and difference in the interventional effect of surgeries depending on patch type implanted. Patches differed regarding their probability of re-operation due to patch calcification, based on a systematic literature review. Transition probabilities were based on the published literature, other clinical inputs were based on UK registry data, and cost data were based on UK sources and the published literature. Incremental cost-effectiveness ratio (ICER) was determined as incremental costs per quality adjusted life years (QALY) gained. We used a 40-year analytic time-horizon and adopted the payer perspective. Comprehensive sensitivity analyses were performed.ResultsAccording to the model predictions, CardioCel was associated with reduced incidence of re-operation, increased QALY, and costs savings compared to all other patches. Cost savings were greatest compared to synthetic patches. Estimated cost savings associated with CardioCel were greatest within atrioventricular septal defect repair and lowest for ventricular septal defect repair. Based on our model, CardioCel relative risk for re-operations is 0.938, 0.956and 0.902 relative to xenogeneic, autologous, and synthetic patches, respectively.ConclusionCardioCel was estimated to increase health benefits and save cost when used during surgery for congenital heart defects instead of other patches.
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Project description:ObjectivesThe goal of this histological study was to assess the biocompatibility of vascular patches used in the repair of congenital heart defects.MethodsWe examined tissue-engineered bovine (n = 7) and equine (n = 7) patches and autologous human pericardium (n = 7), all explanted due to functional issues or follow-up procedures. Techniques like Movat-Verhoeff, von Kossa and immunohistochemical staining were used to analyse tissue composition, detect calcifications and identify immune cells. A semi-quantitative scoring system was implemented to evaluate the biocompatibility aspects, thrombus formation, extent of pannus, inflammation of pannus, cellular response to patch material, patch degradation, calcification and neoadventitial inflammation.ResultsWe observed distinct material degradation patterns among types of patches. Bovine patches showed collagen disintegration and exudate accumulation, whereas equine patches displayed edematous swelling and material dissolution. Biocompatibility scores were lower in terms of cellular response, degradation and overall score for human autologous pericardial patches compared to tissue-engineered types. The extent of pannus formation was not influenced by the type of patch. Bovine patches had notable calcifications causing tissue hardening, and foreign body giant cells were more frequently seen in equine patches. Plasma cells were frequently detected in the neointimal tissue of engineered patches.ConclusionsOur results confirm the superior biocompatibility of human autologous patches and highlight discernible variations in the changes of patch material and the cellular response to patch material between bovine and equine patches. Our approach implements the semi-quantitative scoring of various aspects of biocompatibility, facilitating a comparative quantitative analysis across all types of patches, despite their inherent differences.
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Project description:The libraries contained in this experiment come from pericardium fibroblast cells (HPcF). They are stranded PE101 Illumina Hi-Seq RAMPAGE libraries from rRNA-depleted Total RNA > 200 nucleotides in size. For data usage terms and conditions, please refer to http://www.genome.gov/27528022 and http://www.genome.gov/Pages/Research/ENCODE/ENCODE_Data_Use_Policy_for_External_Users_03-07-14.pdf
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