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Frontiers in Cardiovascular Medicine 2024Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of...
Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks + 6 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (>85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.
PubMed: 38682104
DOI: 10.3389/fcvm.2024.1351530 -
BMC Pediatrics Apr 2024Plummer-Vinson syndrome (PVS) is characterized by a triad of symptoms consisting of microcytic hypochromic anaemia, oesophageal webs, and dysphagia. PVS is commonly...
BACKGROUND
Plummer-Vinson syndrome (PVS) is characterized by a triad of symptoms consisting of microcytic hypochromic anaemia, oesophageal webs, and dysphagia. PVS is commonly found in women in the fourth and fifth decades of life and is rarely reported in the paediatric population.
CASE PRESENTATION
We report the case of a 1-year-old male South Asian child who presented with dysphagia and anaemia for 4 months and frequent episodes of vomiting after ingesting semisolid and solid food. A complete blood analysis revealed microcytic hypochromic anaemia. An oesophagogram revealed circumferential narrowing of the upper thoracic oesophagus. Based on these findings, our suspicion was that the patient had an oesophageal web and vascular ring. Oesophageal dilation was performed with a Savary-Gilliard dilator; initially, 5 mm and 7 mm probes were used, and final dilation with a 9 mm probe was performed.
CONCLUSION
Although rare in paediatric patients, a high suspicion of this syndrome is necessary in these patients to provide relief to the patient for better growth and development. Iron supplements increase the haemoglobin level but do not subside dysphagia, and oesophageal dilation is needed to open the blocked enteral pathway.
Topics: Humans; Plummer-Vinson Syndrome; Male; Infant; Deglutition Disorders; Dilatation
PubMed: 38678196
DOI: 10.1186/s12887-024-04750-x -
International Journal of Surgery Case... May 2024Left pulmonary artery sling is an uncommon condition observed in infants. The severity of the condition is determined by the compression of the broncho-tracheal tree...
INTRODUCTION AND IMPORTANCE
Left pulmonary artery sling is an uncommon condition observed in infants. The severity of the condition is determined by the compression of the broncho-tracheal tree induced by the ring sling compression. The main goal of the treatment is to adjust the left pulmonary artery and eventually relieving the compression through surgery. The long-term outcome associated with the complexity of the anomalies.
CASE PRESENTATION
A nine-months old patient complained of worsening respiratory distress. The computed tomography scan revealed the potential presence of a left pulmonary artery sling and compression of the trachea, without any abnormalities in the trachea itself. Echocardiography study showed no intracardiac lesion. We successfully did left pulmonary artery transection and re-implantation to main pulmonary artery without cardiopulmonary bypass.
CLINICAL DISCUSSION
Pulmonary artery sling commonly treated with reimplantation of the sling to its origin that usually required cardiopulmonary bypass machine. However, in our case we delivered it without the need of cardiopulmonary bypass. The outcome result turned excellent with echo post-operative showed confluent pulmonary arteries.
CONCLUSION
The optimal approach to treating congenital pulmonary artery sling is through early surgical intervention in symptomatic patients. Following surgical repair devoid of tracheal lesion, the prognosis appears favorable, and routine follow-up is required to determine the long-term effects.
PubMed: 38669803
DOI: 10.1016/j.ijscr.2024.109692 -
BioRxiv : the Preprint Server For... Apr 2024Cerebral amyloid angiopathy (CAA) is a vasculopathy characterized by vascular β-amyloid (Aβ) deposition on cerebral blood vessels. CAA is closely linked to Alzheimer's...
Cerebral amyloid angiopathy (CAA) is a vasculopathy characterized by vascular β-amyloid (Aβ) deposition on cerebral blood vessels. CAA is closely linked to Alzheimer's disease (AD) and intracerebral hemorrhage. CAA is associated with the loss of autoregulation in the brain, vascular rupture, and cognitive decline. To assess morphological and molecular changes associated with the degeneration of penetrating arterioles in CAA, we analyzed post-mortem human brain tissue from 26 patients with mild, moderate, and severe CAA end neurological controls. The tissue was optically cleared for three-dimensional light sheet microscopy, and morphological features were quantified using surface volume rendering. We stained Aβ, vascular smooth muscle (VSM), lysyl oxidase (LOX), and vascular markers to visualize the relationship between degenerative morphological features, including vascular dilation, dolichoectasia (variability in lumenal diameter) and tortuosity, and the volumes of VSM, Aβ, and LOX in arterioles. Atomic force microscopy (AFM) was used to assess arteriolar wall stiffness, and we identified a pattern of morphological features associated with degenerating arterioles in the cortex. The volume of VSM associated with the arteriole was reduced by around 80% in arterioles with severe CAA and around 60% in cases with mild/moderate CAA. This loss of VSM correlated with increased arteriolar diameter and variability of diameter, suggesting VSM loss contributes to arteriolar laxity. These vascular morphological features correlated strongly with Aβ deposits. At sites of microhemorrhage, Aβ was consistently present, although the morphology of the deposits changed from the typical organized ring shape to sharply contoured shards with marked dilation of the vessel. AFM showed that arteriolar walls with CAA were more than 400% stiffer than those without CAA. Finally, we characterized the association of vascular degeneration with LOX, finding strong associations with VSM loss and vascular degeneration. These results show an association between vascular Aβ deposition, microvascular degeneration, and increased vascular stiffness, likely due to the combined effects of replacement of VSM by β-amyloid, cross-linking of extracellular matrices (ECM) by LOX, and possibly fibrosis. This advanced microscopic imaging study clarifies the association between Aβ deposition and vascular fragility. Restoration of physiologic ECM properties in penetrating arteries may yield a novel therapeutic strategy for CAA.
PubMed: 38659767
DOI: 10.1101/2024.03.08.583563 -
Annals of Clinical and Translational... Jun 2024Moyamoya angiopathy (MA) is a rare cerebrovascular disorder characterized by recurrent ischemic/hemorrhagic strokes due to progressive occlusion of the intracranial...
OBJECTIVE
Moyamoya angiopathy (MA) is a rare cerebrovascular disorder characterized by recurrent ischemic/hemorrhagic strokes due to progressive occlusion of the intracranial carotid arteries. The lack of reliable disease severity biomarkers led us to investigate molecular features of a Caucasian cohort of MA patients.
METHODS
The participants consisted of 30 MA patients and 40 controls. We measured cerebrospinal fluid (CSF) levels of angiogenic/inflammatory factors (ELISA). We then applied quantitative real-time PCR on cerebral artery specimens for expression analyses of angiogenic factors. By an immunoassay based on microfluidic technology, we examined the potential correlations between plasma protein expression and MA clinical progression. A RNA interference approach toward Ring Finger Protein 213 (RNF213) and a tube formation assay were applied in cellular model.
RESULTS
We detected a statistically significant (p < 0.000001) up-regulation of Angiopoietin-2 (Ang-2) in CSF and stenotic middle cerebral arteries (RQ >2) of MA patients compared to controls. A high Ang-2 plasma concentration (p = 0.018) was associated with unfavorable outcome in a subset of MA patients. ROC curve analyses indicated Ang-2 as diagnostic CSF biomarker (>3741 pg/mL) and prognostic plasma biomarker (>1162 pg/mL), to distinguish stable-from-progressive MA. Consistently, MA cellular model showed a significant up-regulation (RQ >2) of Ang-2 in RNF213 silenced condition.
INTERPRETATION
Our results pointed out Ang-2 as a reliable biomarker mirroring arterial steno-occlusion and vascular instability of MA in CSF and blood, providing a candidate factor for patient stratification. This pilot study may pave the way to the validation of a biomarker to identify progressive MA patients deserving a specific treatment path.
Topics: Humans; Moyamoya Disease; Angiopoietin-2; Male; Female; Adult; Middle Aged; Prognosis; Biomarkers; Ubiquitin-Protein Ligases; Young Adult; Adenosine Triphosphatases
PubMed: 38655722
DOI: 10.1002/acn3.52076 -
Korean Journal of Anesthesiology Apr 2024Bleeding incidents during percutaneous dilatational tracheostomy are concerning, and most cases occur in patients with unrecognized and unanticipated anatomical...
BACKGROUND
Bleeding incidents during percutaneous dilatational tracheostomy are concerning, and most cases occur in patients with unrecognized and unanticipated anatomical variations in the vascular anatomy. However, the extent of this variation remains unclear. To address this knowledge gap, our study aimed to comprehensively map laryngeal vascular anatomy in a cohort of adult patients.
METHODS
Ultrasound assessments of the soft tissue in the neck were performed, spanning from the thyroid cartilage to the third tracheal ring and extending 2 cm laterally on both sidesperformed. We subdivided this area into 12 zones comprising four medial and eight lateral sections. A pre-planned form was used to document the presence of arteries or veins in each zone. The results are reported as odds ratios, 95% CIs, and corresponding P-values.
RESULTS
Five-hundred patients were enrolled from August 14, 2023, to November 13, 2023, at the University Hospital of Padua. Arteries and veins were identified in all investigated zones (varying from a minimum of 1.0%-46.4%). The presence of invessels progressively increased from the cricothyroid membrane to the third tracheal ring and from the midline to the paramedian laryngeal area.
CONCLUSIONS
Given the prevalence of arteries and veins, particularly in areas where tracheostomies are commonly performed, we strongly advocate for routine ultrasound assessments before such procedures are performed.
PubMed: 38653329
DOI: 10.4097/kja.23900 -
Proceedings of the National Academy of... Apr 2024The complex interplay between malignant cells and the cellular and molecular components of the tumor stroma is a key aspect of cancer growth and development. These...
The complex interplay between malignant cells and the cellular and molecular components of the tumor stroma is a key aspect of cancer growth and development. These tumor-host interactions are often affected by soluble bioactive molecules such as proteoglycans. Decorin, an archetypical small leucine-rich proteoglycan primarily expressed by stromal cells, affects cancer growth in its soluble form by interacting with several receptor tyrosine kinases (RTK). Overall, decorin leads to a context-dependent and protracted cessation of oncogenic RTK activity by attenuating their ability to drive a prosurvival program and to sustain a proangiogenic network. Through an unbiased transcriptomic analysis using deep RNAseq, we identified that decorin down-regulated a cluster of tumor-associated genes involved in lymphatic vessel (LV) development when systemically delivered to mice harboring breast carcinoma allografts. We found that Lyve1 and Podoplanin, two established markers of LVs, were markedly suppressed at both the mRNA and protein levels, and this suppression correlated with a significant reduction in tumor LVs. We further identified that soluble decorin, but not its homologous proteoglycan biglycan, inhibited LV sprouting in an ex vivo 3D model of lymphangiogenesis. Mechanistically, we found that decorin interacted with vascular endothelial growth factor receptor 3 (VEGFR3), the main lymphatic RTK, and its activity was required for the decorin-mediated block of lymphangiogenesis. Finally, we identified that Lyve1 was in part degraded via decorin-evoked autophagy in a nutrient- and energy-independent manner. These findings implicate decorin as a biological factor with antilymphangiogenic activity and provide a potential therapeutic agent for curtailing breast cancer growth and metastasis.
Topics: Decorin; Lymphangiogenesis; Animals; Mice; Humans; Female; Breast Neoplasms; Lymphatic Vessels; Cell Line, Tumor; Disease Progression; Vesicular Transport Proteins; Membrane Glycoproteins; Gene Expression Regulation, Neoplastic
PubMed: 38652741
DOI: 10.1073/pnas.2317760121 -
Cureus Mar 2024Embolization of entrapped intracardiac air represents a significant risk to the patient undergoing open-heart surgery. Entrapment of as little as 0.5 mL of gas in the...
Embolization of entrapped intracardiac air represents a significant risk to the patient undergoing open-heart surgery. Entrapment of as little as 0.5 mL of gas in the heart can cause temporary myocardial dysfunction, cardiac arrhythmias, and systemic emboli. In contrast, larger emboli can disrupt the evaluation of heart function by limiting visualization during echocardiography. We present the case of a 67-year-old male who presented with dizziness, nausea, and chest pain. A left heart catheterization revealed multi-vessel disease. Undergoing general anesthesia, the patient received three-vessel coronary artery bypass grafting, mitral valve repair, ring annuloplasty, and left atrial appendage closure. Upon aortic unclamping, transgastric echocardiography showed significant gas almost wholly obscuring the left heart chambers despite de-airing maneuvers. Successful resolution relied upon higher mean blood pressure and time, demonstrating the importance of intraoperative imaging and interdisciplinary collaboration.
PubMed: 38646393
DOI: 10.7759/cureus.56525 -
Surgical Endoscopy May 2024Virtual reality is a frequently chosen method for learning the basics of robotic surgery. However, it is unclear whether tissue handling is adequately trained in VR... (Randomized Controlled Trial)
Randomized Controlled Trial Comparative Study
BACKGROUND
Virtual reality is a frequently chosen method for learning the basics of robotic surgery. However, it is unclear whether tissue handling is adequately trained in VR training compared to training on a real robotic system.
METHODS
In this randomized controlled trial, participants were split into two groups for "Fundamentals of Robotic Surgery (FRS)" training on either a DaVinci VR simulator (VR group) or a DaVinci robotic system (Robot group). All participants completed four tasks on the DaVinci robotic system before training (Baseline test), after proficiency in three FRS tasks (Midterm test), and after proficiency in all FRS tasks (Final test). Primary endpoints were forces applied across tests.
RESULTS
This trial included 87 robotic novices, of which 43 and 44 participants received FRS training in VR group and Robot group, respectively. The Baseline test showed no significant differences in force application between the groups indicating a sufficient randomization. In the Midterm and Final test, the force application was not different between groups. Both groups displayed sufficient learning curves with significant improvement of force application. However, the Robot group needed significantly less repetitions in the three FRS tasks Ring tower (Robot: 2.48 vs. VR: 5.45; p < 0.001), Knot Tying (Robot: 5.34 vs. VR: 8.13; p = 0.006), and Vessel Energy Dissection (Robot: 2 vs. VR: 2.38; p = 0.001) until reaching proficiency.
CONCLUSION
Robotic tissue handling skills improve significantly and comparably after both VR training and training on a real robotic system, but training on a VR simulator might be less efficient.
Topics: Humans; Robotic Surgical Procedures; Clinical Competence; Female; Male; Virtual Reality; Prospective Studies; Adult; Simulation Training; Learning Curve; Young Adult
PubMed: 38632120
DOI: 10.1007/s00464-024-10842-7 -
Revue Medicale de Liege Apr 2024We report the case of a 36-year-old female whose dysphagia revealed a congenital anomaly of the thoracic aorta: the right aortic arch with mirror image branching. This...
We report the case of a 36-year-old female whose dysphagia revealed a congenital anomaly of the thoracic aorta: the right aortic arch with mirror image branching. This is a rare embryonic developmental anomaly where the aorta wraps around the right bronchus and the supra-aortic trunks emerge from the arch in the opposite order to normal. Most of the patients are asymptomatic unless there is a significant compression of mediastinal structures. Major compression of the esophagus or trachea, aneurysmal disease, dissection of the thoracic aorta, or the presence of a Kommerell diverticulum larger than 2 cm may require a surgical repair. There is no standard treatment and it must be adapted to the clinical presentation and the anatomic configuration of each patient. Our patient did not receive any treatment for her condition.
Topics: Female; Humans; Adult; Aorta, Thoracic; Deglutition Disorders; Mediastinum; Subclavian Artery
PubMed: 38602205
DOI: No ID Found