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Pediatric Radiology Dec 2015Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the... (Review)
Review
Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the compressed airway and esophagus are not encircled by patent, opacified vessels, there are useful secondary signs that should be considered and should raise suspicion for the presence of a vascular ring. These signs include a double aortic arch, the four-vessel sign, the distorted subclavian artery sign, a diverticulum of Kommerell, a ductal diverticulum contralateral to the aortic arch, and a descending aorta contralateral to the arch or circumflex aorta. If none of these findings is present, a ring can be excluded with confidence.
Topics: Aorta, Thoracic; Aortic Arch Syndromes; Diagnosis, Differential; Diagnostic Imaging; Humans; Infant, Newborn; Subclavian Artery; Vascular Malformations
PubMed: 26260202
DOI: 10.1007/s00247-015-3424-7 -
Die Pharmazie Jan 2022The relationship between large conductance calcium activated potassium channel (BK) and vascular lesions in type 2 diabetes mellitus (T2DM) was investigated by observing...
The relationship between large conductance calcium activated potassium channel (BK) and vascular lesions in type 2 diabetes mellitus (T2DM) was investigated by observing vascular reactivity of thoracic aorta and mesenteric artery and the current changes of BK in vascular smooth muscle cells (VSMCs). The thoracic aorta and mesenteric artery of T2DM rats were isolated, the whole cell perforated patch clamp experiment and single channel patch clamp experiment of acute enzyme separation of thoracic aorta and mesenteric artery smooth muscle cells were performed to measure the membrane capacitance and the amplitude of macro current. And the vascular ring experiment was performed to observe the change of relaxation percentage. The results showed that the amplitude of BK current in vascular smooth muscle cells of diabetic rats was higher than that of the control group. The channel current had outward rectifying characteristics. BK is related to vascular reactivity and smooth muscle relaxation in T2DM rats. The opening probability of BK in VSMCs of diabetic rats was significantly increased. This study suggests that BK may be a new target for diabetic vascular disease.
Topics: Animals; Aorta, Thoracic; Diabetes Mellitus, Experimental; Diabetes Mellitus, Type 2; Mesenteric Arteries; Muscle, Smooth, Vascular; Myocytes, Smooth Muscle; Rats
PubMed: 35045921
DOI: 10.1691/ph.2022.1740 -
The International Journal of... Oct 2022We analyzed the double arch of a 51-year-old male patient who applied to the outpatient clinic with chest pain and shortness of breath and compared this rare case with...
We analyzed the double arch of a 51-year-old male patient who applied to the outpatient clinic with chest pain and shortness of breath and compared this rare case with the studies in the literature. Double aortic arch (DAA) is defined as a type of vascular ring malformation. The incidence of congenital heart diseases is less than 1%. DAA makes up 46-76% of all rings. We aimed to contribute to cardiac surgery by examining and modeling the diameters in the 2D and 3D images of the patient. For 3D modeling, an open-source software program ITK-SNAP 3.8 was used, which converts 2D images from MRI, CT, and ultrasound to 3D medical image volumes. CT images of the case taken from the SECTRA system of our hospital were uploaded to ITK-SNAP and segmentation was performed. With 3D modeling, a better understanding of the stenosis in the trachea and the double arch was achieved. The ascending aorta diameter was 30 mm. There were atherosclerotic changes in the aorta and its branches. The diameter of the right aortic arch was 22.2 mm, and the diameter of the left aortic arch was 14.5 mm. Trachea diameter was found to be 17 mm/13.2 mm. Esophageal diameter was 9.8 mm. The patient had no specific complaints and no medical or surgical treatment was recommended because his physical examination was normal. We think that a better understanding of such cases in 3D may contribute to cardiovascular surgery.
Topics: Male; Humans; Middle Aged; Predictive Value of Tests; Aorta; Chest Pain; Constriction, Pathologic; Dyspnea
PubMed: 37726466
DOI: 10.1007/s10554-022-02675-z -
Journal of Hypertension Apr 2022CACNA1D gene, which encodes the α1 subunit of the Cav1.3 L-type calcium channel effectively regulates intracellular Ca2+ stability. In recent years, clinical studies...
BACKGROUND
CACNA1D gene, which encodes the α1 subunit of the Cav1.3 L-type calcium channel effectively regulates intracellular Ca2+ stability. In recent years, clinical studies have shown that the CACNA1D polymorphisms were associated with hypertension.
OBJECTIVE
The purpose of this study was to evaluate the effects of CACNA1D exon mutation on blood pressure (BP) in Sprague-Dawley rats.
METHODS
The rats with CACNA1D p.D307G, CACNA1D p.V936I or CACNA1D p.R1516Q were constructed using CRISPR-Cas9 technology. SBP measurements of rats were taken for 32 weeks. Tissue morphology of rats and vasoactive substances in serum was tested. Furthermore, the effects of L-type calcium channel blocker isradipine and endothelin-1 (ET-1) inhibitor BQ-123 on BP of double mutation rats (CACNA1D p.D307G/p.R1516Q) were tested. Then we examined the effects of CACNA1D gene mutation on gene expression in human umbilical vein endothelial cells (HUVECs) and vascular smooth muscle cells (VSMCs).
RESULTS
Elevated SBP and increased circulating ET-1 was observed in CACNA1D p.D307G mutant rats. Morphological assessments showed that the vascular, cardiac and renal remodeling could also be observed in rats with p.D307G mutant. Cav1.3 protein expression and calcineurin phosphatase activity in VSMCs of rats with CACNA1D p.D307G were increased in vitro, and the vascular ring tension test of mesenteric grade 3 arteries in CACNA1D p.D307G rats were increased in vivo. Furthermore, ET-1 expression were increased in isolated primary aortic endothelial cells in p.D307G mutant rats and transfected p.D307G mutant HUVECs. Finally, double heterozygosity rats with CACNA1D p.D307G/p.R1516Q or CACNA1D p.D307G/p.V936I further accelerated the rise of SBP compared with p.D307G mutation rats, and isradipine and BQ-123 reduced BP to the same extent in CACNA1D p.D307G/p.R1516Q rats.
CONCLUSION
CACNA1D gene is key players in the regulation of blood pressure. CACNA1D mutation rat may be a new hypertension animal model.
Topics: Animals; Blood Pressure; Calcium Channels, L-Type; Endothelial Cells; Exons; Humans; Mutation; Rats; Rats, Sprague-Dawley
PubMed: 35142739
DOI: 10.1097/HJH.0000000000003085 -
The Annals of Thoracic Surgery Jul 2019Vascular rings with a Kommerell diverticulum (KD) most commonly occur in patients with a right aortic arch. We report on a less commonly seen subset of vascular ring...
BACKGROUND
Vascular rings with a Kommerell diverticulum (KD) most commonly occur in patients with a right aortic arch. We report on a less commonly seen subset of vascular ring patients-those with a double aortic arch and a KD.
METHODS
Between 2002 and 2017, 66 patients underwent an operation for a double aortic arch. Ten of those patients also had excision of a KD. We performed a retrospective medical record review of these patients to characterize their demographics and outcomes.
RESULTS
All 10 patients (7 male, 3 female) had a double aortic arch that was right dominant and also had a KD. The patients were a mean age of 4.9 ± 4.3 years (range, 6 months to 29 years), and median age was 4 years. All patients had preoperative computed tomographic angiography or magnetic resonance imaging and mean compression of the distal trachea of 63% ± 12% (range, 40% to 80%). The distal left arch was atretic in all patients. All patients underwent division of their left aortic arch, division of the ligamentum, and resection of the KD. The left subclavian artery was transferred to the left carotid artery in 2 patients. The mean size of the diverticulum was 9 × 10 mm. There were no major postoperative complications or readmissions. The postoperative length of stay was 3.1 ± 0.8 days. Five of the patients reported no related persisting symptoms. The remaining 5 patients reported substantial symptomatic relief with only minor respiratory symptoms.
CONCLUSIONS
Vascular ring patients with a double aortic arch can also have a KD. In addition to dividing the smaller aortic arch and the ligamentum, we recommend excision of the KD.
Topics: Adult; Aorta, Thoracic; Aortic Diseases; Carotid Arteries; Child; Child, Preschool; Computed Tomography Angiography; Diverticulum; Female; Humans; Imaging, Three-Dimensional; Infant; Male; Postoperative Complications; Retrospective Studies; Subclavian Artery; Vascular Malformations
PubMed: 30849335
DOI: 10.1016/j.athoracsur.2019.01.062 -
European Heart Journal. Case Reports Jun 2019Double aortic arch (DAA) and pulmonary artery sling (PAS) are vascular ring formations that present in neonates and infants with symptoms of respiratory stenosis.
BACKGROUND
Double aortic arch (DAA) and pulmonary artery sling (PAS) are vascular ring formations that present in neonates and infants with symptoms of respiratory stenosis.
CASE SUMMARY
The patient was a girl with suspected ventricular septal defect (VSD), right aortic arch (AA), left patent ductus arteriosus, and bilateral superior vena cava (SVC) on foetal echography in the first day of life. The girl was delivered at 40 weeks and 4 days of gestation. Ventricular septal defect, DAA, coarctation of the left AA, and bilateral SVC were diagnosed. Contrast-enhanced computed tomography at Day 16 revealed PAS with concurrent anomalous tracheal branching in addition to DAA. The right A2 segmental artery, which supplies the right upper pulmonary artery, showed abnormal branching from the left pulmonary artery (LPA). At 3 months of age, VSD patching, left AA resection distal to the root of the left subclavian artery, arterial ligament dissection, and LPA replacement were performed.
DISCUSSION
Pulmonary artery sling coexists with anomalous branching of the trachea and abnormal branching of the right pulmonary artery (RPA). Our patient had an extremely rare case of DAA concurrent with PAS and presented with anomalous tracheal and RPA branching. We were concerned that increased pulmonary blood flow caused by the VSD would exacerbate tracheal displacement. Radical surgery at 3 months of age resulted in good postoperative progress.
PubMed: 31449592
DOI: 10.1093/ehjcr/ytz036 -
Journal of Pediatric Surgery Sep 2023Vascular rings may cause tracheal and/or oesophageal compression. For many patients, symptoms/signs have been present for a long period before diagnosis. However, in the... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Vascular rings may cause tracheal and/or oesophageal compression. For many patients, symptoms/signs have been present for a long period before diagnosis. However, in the era of prenatal diagnosis, some units advocate universal early surgery. The risks and efficacy of surgery must be known to adequately counsel for the operation. This meta-analysis sought to define the morbidity and mortality associated with surgical correction, and persistent post-operative symptoms.
METHODS
PubMed, Cochrane Library and CINAHL databases were searched for studies that described the outcome of patients undergoing surgery for a double or right aortic arch (DAA or RAA). Non-comparative and random effects model-based meta-analyses were conducted to calculate the pooled rates of mortality, surgical complications, reintervention, and persistent follow-up symptoms.
RESULTS
Nineteen eligible studies were included comprising 18 studies describing outcomes for DAA surgery and 15 for RAA surgery. For DAA surgery, overall mortality rate was 0% [95% confidence interval (CI) 0.0-1.0], post-surgical complication rate 18% [95% CI: 12.0-23.0], prevalence of reintervention 3% [95% CI: 1.0-5.0] and prevalence of symptoms at last follow-up was 33% [95% CI: 17.0-52.0]. For RAA surgery, overall pooled mortality was 0% [95% CI: 0.0-0.0], prevalence of post-surgical complications was 15% [95% CI: 8.0-23.0], reintervention rate was 2% [95% CI: 0.0-4.0], prevalence of symptoms at last follow-up was 40% [95% CI: 26.0-55.0].
CONCLUSIONS
While surgery to correct a vascular ring is safe, the rate of persistent symptoms is high and further strategies must be sought to reduce this burden.
Topics: Pregnancy; Female; Humans; Vascular Ring; Aorta, Thoracic; Prenatal Diagnosis; Trachea; Subclavian Artery
PubMed: 36967253
DOI: 10.1016/j.jpedsurg.2023.02.058 -
The Journal of Thoracic and... Aug 2017
Topics: Aorta, Thoracic; Child; Humans; Muscle, Smooth, Vascular; Thoracotomy; Vascular Ring; Young Adult
PubMed: 28412114
DOI: 10.1016/j.jtcvs.2017.03.024 -
Journal of Pediatric Surgery Oct 2021Kommerell diverticulum (KD) in children with right aortic arch and aberrant left subclavian artery can be treated surgically via left subclavian artery (LSCA)...
BACKGROUND/PURPOSE
Kommerell diverticulum (KD) in children with right aortic arch and aberrant left subclavian artery can be treated surgically via left subclavian artery (LSCA) translocation or aortopexy. These procedures remain controversial. We compared the outcomes of both procedures.
METHODS
This retrospective study included 48 patients who underwent LSCA translocation (n = 26) or aortopexy (n = 22). Lateral thoracotomy and sternotomy were performed in 38 and 10 patients with severe intracardiac malformations, respectively.
RESULTS
The median age of the aortopexy group was 10 months, median follow-up was 14 months, and mean length of hospitalization was 11.41 ± 6.8 days. The median age of the LSCA translocation group was 12 months, median follow-up was 22 months, and mean length of hospitalization was 14.96 ± 7.74 days. Two patients (LSCA translocation group) required secondary intubations and none suffered from recurrent laryngeal nerve injury or complications related to LSCA translocation. Breathing and swallowing symptoms were relieved in all patients. No additional thoracotomies due to respiratory symptoms were required.
CONCLUSIONS
These two procedures relieve the pressure on the trachea and esophagus with similar short-term outcomes. The long-term outcomes are unknown. LSCA translocation may be the best strategy to prevent symptom recurrence and late complications in these patients.
LEVEL OF EVIDENCE
Level III.
Topics: Aorta, Thoracic; Cardiovascular Abnormalities; Child; Diverticulum; Heart Defects, Congenital; Humans; Infant; Neoplasm Recurrence, Local; Retrospective Studies; Subclavian Artery; Vascular Ring
PubMed: 33678405
DOI: 10.1016/j.jpedsurg.2021.02.009 -
The American Journal of Cardiology May 2019The 3-vessel and trachea view is now integrated into obstetrical screening and facilitates prenatal detection of vascular rings. We examined trends in prenatal...
The 3-vessel and trachea view is now integrated into obstetrical screening and facilitates prenatal detection of vascular rings. We examined trends in prenatal detection, associated cardiac and extracardiac anomalies, and surgical management in this population. We reviewed a population-based cohort of pediatric vascular ring patients diagnosed prenatally and postnatally between 2002 and 2017 in Alberta, Canada. Of 106 cases, 28 (26%) had a prenatal diagnosis. Prenatal detection increased over time: 0/29 from 2002 to 2009, 4/28 (14%) from 2009 to 2011, 7/23 (30%) from 2012 to 2014, and 17/26 (65%) from 2015 to 2017 (p <0.01). The prenatal group more commonly had right aortic arch/left ductus/aberrant left subclavian artery (24/28vs 53/78, p = 0.04) and associated cardiac pathology (18/28vs 33/78, p = 0.05). The rate of genetic anomalies was overall higher than previously reported (34%) and did not differ between groups (11/28vs 25/78, p = 0.48). Those with a prenatal diagnosis were less likely to require cross-sectional imaging (9/28vs 48/78, p <0.01), modifying the vascular ring subtype diagnosis in 2 patients. Surgical intervention was common and did not differ between groups (24/28vs 66/78, p = 0.89). In conclusion, prenatal detection of vascular rings has increased. Despite differences in vascular ring subtype and associated cardiac pathology, the incidence of genetic anomalies and need for surgical intervention is not associated with timing of diagnosis. Genetic counseling should be universally offered. The diagnostic accuracy of echocardiography suggests additional imaging may not be routinely required.
Topics: Abnormalities, Multiple; Adolescent; Bronchoscopy; Cardiac Surgical Procedures; Child; Child, Preschool; Disease Management; Echocardiography; Female; Follow-Up Studies; Heart Defects, Congenital; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging, Cine; Male; Pregnancy; Retrospective Studies; Ultrasonography, Prenatal; Vascular Ring
PubMed: 30876659
DOI: 10.1016/j.amjcard.2019.02.030