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European Heart Journal. Case Reports Feb 2022Moderate or severe tricuspid regurgitation (TR) recurs in up to one-third of patients within 8 years of surgical annuloplasty repair. Reoperation often carries high...
BACKGROUND
Moderate or severe tricuspid regurgitation (TR) recurs in up to one-third of patients within 8 years of surgical annuloplasty repair. Reoperation often carries high risk with poor outcomes. Transcatheter valve-in-ring repair is an emerging alternative treatment. However, residual regurgitation is frequent and may necessitate further procedures.
CASE SUMMARY
A 52-year-old female was diagnosed with severe rheumatic valvular heart disease. The patient underwent mechanical aortic and mitral valve replacement. Additionally, tricuspid repair was performed using a semi-rigid annuloplasty ring (28 mm Edwards Physio Tricuspid). Within 2 years, the patient developed recurrent, isolated severe symptomatic TR, with progressive right ventricular dilatation. The patient was considered prohibitive risk for redo surgery and unsuitable for cardiac transplantation. She underwent percutaneous valve-in-ring transcatheter heart valve (THV) implantation using a 29 mm Sapien S3 (Edwards Lifesciences, CA, USA) valve. Persistent severe residual para-ring TR warranted a further procedure to deploy vascular plugs, significantly reducing the TR to a mild jet with symptomatic improvement.
DISCUSSION
Valve-in-ring THV implantation for failed surgical tricuspid annuloplasty repair is a rare procedure reserved for symptomatic patients at high or prohibitive risk for reoperation. Significant residual TR is a commonly encountered problem with incomplete annuloplasty rings following valve-in-ring procedures and may occur either intra-ring between the THV and the ring or para-ring. Implantation of vascular occlusion devices can be used to successfully treat residual TR at either location with good outcomes at 6-month follow-up. Further work is required to determine the longevity of this treatment.
PubMed: 35233492
DOI: 10.1093/ehjcr/ytac041 -
Annals of Medicine and Surgery (2012) Sep 2021Congenital vascular rings are rare in adults. The management is challenging when vascular rings present with concomitant aortic aneurysm and Kommerall's diverticulum.
INTRODUCTION AND IMPORTANCE
Congenital vascular rings are rare in adults. The management is challenging when vascular rings present with concomitant aortic aneurysm and Kommerall's diverticulum.
CASE PRESENTATION
We present a 68year old patient with a congenital vascular ring in the form of right sided aortic arch, with a retroesophageal left subclavian artery arising from an aneurysmal Kommerell's diverticulum. In addition, the patient had aneurysmal distal aortic arch and proximal descending aorta, causing severe symptomatic tracheal compression. The patient was successfully managed by debranching and reimplantation of head neck vessels to the ascending aorta through a median sternotomy, and concurrent endovascular stenting of the aneurysmal aorta.
CLINICAL DISCUSSION
A combination of complete vascular rings and aortic arch aneurysm poses a potentially higher surgical risk. The use of the less invasive endovascular stenting of the aorta as well as a limited open surgical approach offers a lesser risk, especially in poor pre-operative states.
CONCLUSION
A multidisciplinary team effort is key. Hybrid management of aneurysmal congenital vascular rings in high risk adults offers an attractive management alternative to conventional surgery.
PubMed: 34527233
DOI: 10.1016/j.amsu.2021.102778 -
American Journal of Cardiovascular... 2015Disorders of vascular function contribute importantly to cardiovascular disease which represents a substantial cause of morbidity and mortality worldwide. An emerging... (Review)
Review
Disorders of vascular function contribute importantly to cardiovascular disease which represents a substantial cause of morbidity and mortality worldwide. An emerging paradigm in the study of cardiovascular diseases is that protein ubiquitination and turnover represent key pathological mechanisms. Our understanding of these processes in the vasculature is growing but remains incomplete. Since protein ubiquitination and turnover can represent a terminal event in the life of a given protein, entry into these pathways must be highly regulated. However, at present understanding of these regulatory mechanisms, particularly in the vasculature, is fragmentary. The COP9 (constitutive photomorphogenic mutant 9) signalosome (CSN) is a heteromeric protein complex implicated in the control of protein degradation. The CSN participates critically in the control of Cullin Ring Ligases (CRLs), at least in part via the detachment of a small protein, Nedd8 (deneddylation). CRLs are one of the largest groups of ubiquitin ligases, which represent the most selective control point for protein ubiquitination. Thus, the CSN by virtue of its ability to control the CRLs ubiquitin ligase activity is ideally positioned to effect selective modulation of protein turnover. This review surveys currently available data regarding the potential role of the CSN in control of vascular function. Data potentially linking the CSN to control of regulatory proteins involved in vascular smooth muscle proliferation and to vascular smooth muscle contraction are presented with the intent of providing potentially intriguing possibilities for future investigation.
PubMed: 26064791
DOI: No ID Found -
Journal of Pediatric Surgery Nov 2022Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction...
OBJECTIVES
Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients.
METHODS
We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis.
RESULTS
249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention.
CONCLUSIONS
TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention.
Topics: Airway Obstruction; Child; Humans; Incidence; Retrospective Studies; Trachea; Tracheobronchomalacia; Vascular Ring
PubMed: 35718546
DOI: 10.1016/j.jpedsurg.2022.05.005 -
IUBMB Life May 2019Vascular smooth muscle cell (VSMC) hyperproliferation is the main pathological process in various cardiovascular diseases, such as vascular restenosis. This process may...
Vascular smooth muscle cell (VSMC) hyperproliferation is the main pathological process in various cardiovascular diseases, such as vascular restenosis. This process may be repressed by RING finger protein 10 (RNF10) in metabolic syndrome (MetS) rats. The aim of this study is to evaluate the inhibitory effects and molecular mechanisms of RNF10 on VSMC hyperproliferation. Neointimal hyperplasia in MetS and high-glucose-induced VSMC hyperproliferation were measured after infection with adenoviruses encoding RNF10 (Ad-RNF10), short hairpin RNF10 (Ad-shRNF10), or green fluorescent protein (Ad-GFP). In vivo and in vitro, we found that overexpression of RNF10 significantly affected neointima formation and VSMC proliferation, and displayed further inhibitory activity by promoting mesenchyme homeobox 2 (Meox2) and suppressing activating protein 1 (AP-1). In contrast, Ad-shRNF10 had an opposite effect on neointimal hyperplasia and VSMC hyperproliferation in vivo and in vitro. Our study indicated that RNF10 inhibited the hyperproliferation with the activities of Meox2 and AP-1 proteins. RNF10 may be a next drug target for treating vascular restenosis and other related cardiovascular diseases. © 2018 IUBMB Life, 71(5):632-642, 2019.
Topics: Adenoviridae; Adenoviridae Infections; Angioplasty, Balloon, Coronary; Animals; Carrier Proteins; Cell Movement; Cell Proliferation; Cells, Cultured; Coronary Restenosis; Diet, High-Fat; Hyperplasia; Male; Metabolic Syndrome; Muscle, Smooth, Vascular; Neointima; Nerve Tissue Proteins; Rats; Rats, Sprague-Dawley; Signal Transduction
PubMed: 30597731
DOI: 10.1002/iub.1995 -
Journal of the American College of... Mar 1995This study evaluated our early experience with video-assisted thoracoscopic vascular ring division and compared this approach with division by means of a conventional... (Clinical Trial)
Clinical Trial Comparative Study
OBJECTIVES
This study evaluated our early experience with video-assisted thoracoscopic vascular ring division and compared this approach with division by means of a conventional open thoracotomy.
BACKGROUND
Video-assisted thoracoscopic techniques reduce surgical trauma and have been applied to several adult thoracic procedures; however, pediatric applications have been limited. We developed instruments and techniques for video-assisted thoracoscopic vascular ring division in the pediatric population.
METHODS
We compared patient characteristics, operative results and postoperative hospital courses of all patients undergoing vascular ring division by a video-assisted approach with a historical control group of all patients undergoing division by an open thoracotomy between January 1991 and December 1992.
RESULTS
Eight patients (median age 5 months, range 40 days to 5.5 years; median weight 6.2 kg, range 1.8 to 17.1) underwent video-assisted thoracoscopic vascular ring division. Four had a double aortic arch with an atretic left arch and a left ligamentum, and four had a right aortic arch with aberrant left subclavian artery and a left ligamentum. All eight had successful ring division with symptomatic relief and no mortality. A limited thoracotomy was performed in three patients to divide patent vascular structures, and the hospital period was prolonged in one because of chylothorax. These eight patients were compared with a historical cohort of eight pediatric patients having vascular ring division performed by a conventional thoracotomy. The two groups did not differ in age, weight, intensive care unit or postoperative hospital stay, duration of intubation or thoracostomy tube or hospital charges. Total operating room time was longer for the group undergoing video-assisted operation.
CONCLUSIONS
Early results for video-assisted thoracoscopic vascular ring division are comparable to those of the conventional surgical approach. With further refinement in technique and instrumentation, video-assisted surgical intervention may become a viable alternative to open thoracotomy for management of the symptomatic vascular ring.
Topics: Aorta, Thoracic; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Male; Thoracoscopy; Thoracotomy; Vascular Surgical Procedures; Video Recording
PubMed: 7884102
DOI: 10.1016/0735-1097(94)00461-X -
European Journal of Cardio-thoracic... Feb 2022Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of...
OBJECTIVES
Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division.
METHODS
A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed.
RESULTS
The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up.
CONCLUSIONS
The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.
Topics: Aorta, Thoracic; Aortic Diseases; Child; Heart Defects, Congenital; Humans; Subclavian Artery; Vascular Ring
PubMed: 34632492
DOI: 10.1093/ejcts/ezab432 -
Ultrasound in Obstetrics & Gynecology :... Apr 2016Use of recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA). We aimed to establish the outcome of... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
Use of recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA). We aimed to establish the outcome of fetal RAA without intracardiac abnormalities (ICA) to guide postnatal management.
METHODS
In the retrospective cohort part of our study, outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extracardiac abnormalities (ECA), postnatal ICA and ECA, and symptoms of and surgery for vascular ring. A systematic review and meta-analysis was also performed; results are reported as proportions. Kaplan-Meier analysis of vascular ring cases with surgery as endpoint was performed.
RESULTS
Our cohort included 86 cases; 41 had a vascular ring. Rates of chromosomal abnormalities, 22q11.2 deletion and fetal ECA were 14.1%, 6.4% and 17.4%, respectively. Sixteen studies including our cohort (312 fetuses) were included in the systematic review. Overall rates of chromosomal abnormalities and 22q11.2 deletion were 9.0% (95% CI, 6.0-12.5%) and 6.1% (95% CI, 3.6-9.3%), whilst the respective rates for cases with no ECA were 4.6% (95% CI, 2.3-7.8%) and 5.1% (95% CI, 2.4-8.6%). ECA were seen in 14.6% (95% CI, 10.6-19.0%) prenatally and in 4.0% (95% CI, 1.5-7.6%) after birth. Postnatal ICA were identified in 5.0% (95% CI, 2.7-7.9%). Rate of symptoms of vascular rings (follow-up ≥ 24 months postpartum) was 25.2% (95% CI, 16.6-35.0%), and 17.1% (95% CI, 9.9-25.7%) had surgery. Two-year freedom from surgery was 83.0% (95% CI, 74.3-90.1%).
CONCLUSIONS
Fetal RAA without ICA is more frequently associated with ECA than with chromosomal abnormalities. Most cases, however, are isolated. Vascular-ring symptoms occur in about 25% of cases. Postnatal surveillance is required mainly in the first 2 years after delivery.
Topics: Abnormalities, Multiple; Aorta, Thoracic; Aortic Arch Syndromes; Chromosome Aberrations; Cohort Studies; DiGeorge Syndrome; Female; Fetal Diseases; Fetal Heart; Heart Defects, Congenital; Humans; Kaplan-Meier Estimate; Pregnancy; Prenatal Diagnosis; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 26643657
DOI: 10.1002/uog.15805 -
PloS One 2023To observe the effect of magnesium ion on vascular function in rats after long-term exhaustive exercise. Forty male SD rats were divided into two groups, the control...
To observe the effect of magnesium ion on vascular function in rats after long-term exhaustive exercise. Forty male SD rats were divided into two groups, the control group (CON group, n = 20) and the exhaustive exercise group (EEE group, n = 20). Exhausted rats performed 1W adaptive swimming exercise (6 times/W, 15min/time), and then followed by 3W formal exhaustive exercise intervention. Hematoxylin and eosin (HE) staining was used to detect the morphological changes of rat thoracic aorta. The contents of interleukin-1 β (IL-1β) and tumor necrosis factor-α (TNF-α) in serum of rats were determined by enzyme-linked immunosorbent assay (ELISA), and the contents of malondialdehyde (MDA), reactive oxygen species (ROS), nitric oxide (NO) and endothelin 1 (ET-1) in serum of rats were determined by biochemical kit. Vascular ring test detects vascular function. Compared with the CON group, the smooth muscle layer of the EEE group became thicker, the cell arrangement was disordered, and the integrity of endothelial cells was destroyed; the serum Mg2+ in EEE group was decreased; the serum levels of IL-1β, TNF-α, MDA and ROS in EEE group were significantly higher than those in the CON group (P are all less than 0.05); the serum NO content in EEE group was significantly decreased, and the ratio of NO/ET-1 was significantly decreased. In the exhaustion group, the vasoconstriction response to KCl was increased, and the relaxation response to Ach was weakened, while 4.8mM Mg2+ could significantly improve this phenomenon (P are all less than 0.01). The damage of vascular morphology and function in rats after exhaustion exercise may be related to the significant increase of serum IL-1β, TNF-α, ROS, MDA and ET-1/NO ratio in rats after exhaustion exercise, while Mg2+ can significantly improve the vasomotor function of rats after exhaustion exercise.
Topics: Rats; Male; Animals; Rats, Sprague-Dawley; Magnesium; Reactive Oxygen Species; Tumor Necrosis Factor-alpha; Endothelial Cells
PubMed: 36780490
DOI: 10.1371/journal.pone.0279318 -
JIMD Reports Nov 2020Congenital disorders of glycosylation (CDG) are a group of metabolic disorders well known to be associated with developmental delay and central nervous system anomalies....
BACKGROUND
Congenital disorders of glycosylation (CDG) are a group of metabolic disorders well known to be associated with developmental delay and central nervous system anomalies. The most common CDG is caused by pathogenic variants in the phosphomannomutase 2 gene (), which impairs one of the first steps of N-glycosylation and affects multiple organ systems. Cardiac involvement can include pericardial effusion, cardiomyopathy, and arrhythmia, while an association with cardiovascular congenital anomalies is not well studied.
CASE SUMMARY
We report a 6-year-old individual who initially presented with inverted nipples, developmental delay, and failure to thrive at 3 months of age. At 4 months, due to feeding problems, swallowing exam and echocardiography were performed which revealed a vascular ring anomaly based on a right aortic arch and aberrant left subclavian artery. Subsequent whole exome gene sequencing revealed two pathogenic PMM2-CDG variants (E139K/R141H) and no known pathogenic mutations related to congenital heart defect (CHD).
DISCUSSION
This is the first report of vascular ring anomaly in a patient with PMM2-CDG. We conducted a literature review of PMM2-CDG patients with reported CHD. Of the 14 patients with PMM2-CDG and cardiac malformation, the most common CHD's were tetralogy of Fallot, patent ductus arteriosus, and truncus arteriosus. The potential important link between CDG and CHD is stressed and discussed. Furthermore, the importance of multidisciplinary care for CDG patients including early referral to pediatric cardiologists is highlighted.
PubMed: 33204593
DOI: 10.1002/jmd2.12160