Vascular Ring Research Articles

Middle Lobe Syndrome: A Case Report and Literature Review

Background: Middle lobe syndrome (MLS) is a distinct clinical and radiographic entity characterized by recurrent or chronic collapse of the middle lobe of the right lung, but it can also involve the lingula of the left lung. Case Study: This study presents a rare case of MLS caused by a vascular ring never described in the literature until now and provides physicians with the clinical and instrumental tools in order to early recognize and promptly treat this condition. The case report was reported according to CARE guidelines. A literature research on PubMed/MEDLINE was also performed using the MeSH terms “Middle lobe syndrome OR MLS AND double aortic arch” “Middle lobe syndrome OR MLS AND vascular rings”. No case described in the literature was found. In most cases, MLS presents non-specific respiratory symptoms, which unfortunately is responsible for the diagnostic delay that patients with this pathology often suffer. The diagnostic delay is estimated to be 8 months (range 3 to 36 months). A history of dysphagia and regurgitation can be indirect signs of a vascular compression, such as vascular rings, which can cause MLS. Conclusion: To date, the reported case is the only case in the literature of MLS caused by double aortic arch. The key point for the diagnosis of MLS is diagnostic suspicion. Early recognition of MLS is essential to quickly start a targeted therapeutic program avoiding the persistence of vicious circle atelectasis-recurrent respiratory infections, and this could significantly improve the long-term outcome of these patients.

Double innominate vein in a case with double aortic arch: a case report

BackgroundDouble aortic arch (DAA) is a type of vascular ring, causing stridor and dysphagia owing to compression of the trachea and esophagus. In contrast, double innominate veins, which is a relatively rare venous anomaly, usually does not cause symptoms or require intervention.Case presentationA 2 month-old boy presented with mild stridor and a hoarse voice while crying, and was diagnosed as having DAA, small atrial septal defects and double innominate vein. The double innominate veins, crossing over and underneath the ascending aorta, did not contribute to airway compression. To release the narrow vascular ring caused by the DAA, surgical resection of the atretic side of the arch was performed by lateral thoracostomy at 4 months of age, before the appearance of signs of bronchomalacia. The double innominate veins were left untouched, and open-heart surgery was avoided.ConclusionsDAA should be suspected if airway symptoms are present in early infancy, particularly if a right aortic arch is noted. It is important not to hesitate to perform diagnostic imaging and release the DAA, as this might prevent or minimize airway complications. However, for double innominate veins, operative release is not necessary unless it interferes with surgical procedures or is causing symptoms.

Salidroside ameliorates hypoxic pulmonary hypertension by regulating the two-pore domain potassium TASK-1 channel

BackgroundHypoxic pulmonary vasoconstriction (HPV) is a reflex constriction of vascular smooth muscle. This study aims to investigate the role of Salidroside (Sal) in pulmonary arterial dilatation and the potential mechanism of Sal regulating hypoxic pulmonary hypertension in vitro and in vivo. MethodsA rat model of hypoxic pulmonary hypertension (HPH) was constructed using hypoxic chamber. The effect of Sal on HPH were evaluated using vascular ring, whole cell patch-clamp, WGA staining, HE staining, and Sirius Scarlet staining assays. ResultsSal treatment alleviated the injury of acute hypoxia on pulmonary circulation in SD rats. Meanwhile, Sal treatment reduced the pulmonary vascular tone of acute hypoxia in a concentration-dependent manner, which was involved in the TWIK-related acid-sensitive potassium channel 1 (TASK-1) mediating diastolic effect. We found that Sal treatment significantly increased the TASK-1 current of pulmonary artery smooth muscle cells (PASMCs) in a concentration-dependent manner, as well as reversed the inhibitory effect of acute hypoxia on the TASK-1 current. Moreover, Sal treatment improved the TASK-1 current density, suppressed the proliferation, and enhanced the apoptosis of PASMCs in SD rats under continuous hypoxic condition. In addition, we found that the electrophysiological remodeling and pulmonary vascular remodeling of PASMCs were improved by the treatment of Sal through the regulation of TASK-1 channel. ConclusionsSal could alleviate HPH by restoring the function of TASK-1 channel, which may provide a novel method for the treatment of HPH.

Recent Demographic Trends and Clinical Insights in Pediatric Vascular Rings and Slings: A Retrospective Case Series Analysis

Recent Demographic Trends and Clinical Insights in Pediatric Vascular Rings and Slings: A Retrospective Case Series Analysis

Retinoic Acid Improves Vascular Endothelial Dysfunction by Inhibiting PI3K/AKT/YAP-Mediated Ferroptosis in Diabetes Mellitus.

Vascular endothelial dysfunction is the initial factor involved in cardiovascular injury in patients with diabetes. Retinoic acid is involved in improving vascular complications in patients with diabetes, but its protective mechanism is still unclear. This study aimed to evaluate the effect and mechanism of All-Trans Retinoic Acid (ATRA) on endothelial dysfunction induced by diabetes. In the present study, streptozotocin (STZ)-induced diabetic rats and high glucose (HG)-induced human umbilical vein endothelial cells (HUVECs) were observed, and the effects of ATRA on HG-induced endothelial dysfunction and ferroptosis were evaluated. ATRA treatment improved impaired vasorelaxation in diabetic aortas in an endothelium-dependent manner, and this effect was accompanied by an increase in the NO concentration and eNOS expression. Ferroptosis, characterized by lipid peroxidation and iron overload induced by HG, was improved by ATRA administration, and a ferroptosis inhibitor (ferrostatin-1, Fer-1) improved endothelial function to a similar extent as ATRA. In addition, the inactivation of phosphoinositol-3-kinase (PI3K)/protein kinases B (AKT) and Yes-Associated Protein (YAP) nuclear localization induced by HG were reversed by ATRA administration. Vascular ring relaxation experiments showed that PI3K/AKT activation and YAP inhibition had similar effects on ferroptosis and endothelial function. However, the vasodilative effect of retinoic acid was affected by PI3K/AKT inhibition, and the inhibitory effects of ATRA on ferroptosis and the improvement of endothelial function were dependent on the retinoic acid receptor. ATRA could improve vascular endothelial dysfunction by inhibiting PI3K/AKT/YAP-mediated ferroptosis induced by HG, which provides a new idea for the treatment of vascular lesions in diabetes.

Anatomic and functional evidence for renal autonomic innervation in normotensive and hypertensive rats.

Renal denervation (RDN) has been used for treating resistant hypertension. A few recent studies have shown vagal innervation of kidneys causing confusion. This study aimed to provide anatomical and functional evidence for renal autonomic innervation. Experiments were performed in male Wistar-Kyoto rats (WKY) and spontaneously hypertensive rats (SHR). Pseudorabies virus (PRV) in the paraventricular nucleus and rostral ventrolateral medulla was prevented by bilateral RDN, but not subdiaphragmatic vagotomy. PRV did not appear in the dorsal motor nucleus of the vagus and nucleus tractus solitarii 72 h after renal injection of PRV. Adrenergic fibers were approximately seven times more than cholinergic fibers in the main renal artery (MRA) and its first (1RA) and second grade (2RA) branches. Adrenergic fibers in 1RA were more than those in MRA and 2RA. Tyrosine hydroxylase immunoreactivity in these arteries was higher in SHR than in WKY. Norepinephrine (NE) increased and α-receptor antagonist reduced vascular ring tension of renal arteries. The effect of NE was greater in 1RA and 2RA than in MRA, which was prevented by α-receptor antagonist. Acetylcholine (ACh) or blockage of β-receptors, M receptors, or N receptors had no significant effects on vascular ring tension and the effect of NE. Renal blood flow was reduced by electrical stimulation of renal nerves but not affected by stimulation of the subdiaphragmatic vagus. These results provide anatomical and functional evidence that kidneys are innervated and renal blood flow is regulated by renal sympathetic nerves rather than the vagus. Renal vasoconstriction is regulated by NE and adrenergic fibers rather than ACh or cholinergic fibers in WKY and SHR.NEW & NOTEWORTHY Kidneys are innervated by renal nerves rather than the vagus. Adrenergic fibers in renal arteries are about seven times more than cholinergic fibers. Renal vasoconstriction is regulated by norepinephrine and adrenergic fibers rather than acetylcholine or cholinergic fibers. Renal blood flow is regulated by renal sympathetic nerves and is not affected by the vagus. These findings provide anatomical and functional evidence for renal autonomic innervation in normotensive and hypertensive rats.

Mailuoning oral liquid ameliorates vasculitis in thromboangiitis obliterans rats via inactivating cGAS-STING-IRF3 and TLR4-MAPKs/NF-κB signaling pathways

Ethnopharmacological relevanceMailuoning oral liquid (MLN O), one traditional Chinese patent medicine, has a good therapeutic effect on thromboangiitis obliterans (TAO) in clinical practice. However, the underlying mechanism remains unclear. Aim of the studyThis study aimed to explore the effects and potential mechanisms of MLN O against TAO based on network pharmacology and experimental verification. Materials and methodsNetwork pharmacology was used to identify the intersectional targets and signaling pathways of MLN O and TAO. In vivo, the TAO model was established by injecting sodium laurate and dihydrotestosterone (DHT) into the femoral arteries of Wistar rats. Rats were given the indicated drugs by intragastric administration (i.g.), intravenous injection (i.v.), or subcutaneous injection (s.c.) per day for 21 days since a week before surgery. In vitro, HUVECs, RAW264.7, and THP-1 cells were stimulated by LPS and DHT to simulate the pathological changes of TAO. The anti-inflammatory, anticoagulant, and immunomodulatory effects of MLN O were evaluated by histological observation, blood biochemical indexes detection, H&E staining, immunohistochemistry, enzyme-linked immunosorbent assay (ELISA), qRT-PCR, western blotting and immunofluorescence assays. Furthermore, the vascular ring test was applied to explore the vasodilatory activity of MLN O. ResultsMLN O significantly improved the pathological signs in TAO rats through its excellent anti-inflammatory, anticoagulant, immunomodulatory, and vasodilatory effects. Specifically, MLN O alleviated the gangrene and reduced the thrombosis in TAO rats, meanwhile, suppressed the expressions of inflammatory factors and clotting factors, which is related to the inactivations of cGAS-STING-IRF3 and TLR4-MAPKs/NF-κB signaling pathways. However, the superphysiological dose of DHT deteriorated the pathological development of TAO in vitro and in vivo. Moreover, the results of network pharmacology are consistent with the experimental verification. ConclusionCollectively, this study indicates for the first time that MLN O could alleviate TAO by inhibiting cGAS-STING-IRF3 and TLR4-MAPKs/NF-κB signaling pathways, which sheds light on a novel clinical therapeutic strategy for TAO.

Basal release and relaxation responses to 6-nitrodopamine in swine carotid, coronary, femoral, and renal arteries

Mammalian and reptilian vascular tissues present basal release of 6-nitrodopamine, which is reduced when the tissues are pre-incubated with the NO synthase inhibitor L-NG-Nitro arginine methyl ester (L-NAME), or when the endothelium is mechanically removed. 6-Nitrodopamine induces vasorelaxation in pre-contracted vascular rings by antagonizing the dopaminergic D2-like receptor. Here it was investigated whether male swine vessels (including carotid, left descendent coronary, renal, and femoral arteries) release 6-nitrodopamine, dopamine, noradrenaline, and adrenaline, as measured by liquid chromatography coupled to tandem mass spectrometry. The in vitro vasorelaxant action of 6-nitrodopamine was evaluated in carotid, coronary, renal, and femoral arteries precontracted by U-46619 (3 nM), and compared to that induced by the dopamine D2-receptor antagonist L-741,626. Expression of tyrosine hydroxylase and the neuromaker calretinin was investigated by immunohistochemistry. All vascular tissues presented basal release of endothelium-derived catecholamines. The relaxation induced by 6-nitrodopamine was not affected by preincubation of the tissues with either L-NAME (100 μM, 30-min preincubation) or the heme-site inhibitor of soluble guanylyl cyclase ODQ (100 μM, 30-min preincubation). Electrical field stimulation (EFS)-induced contractions were significantly potentiated by previous incubation with L-NAME, but unaffected by ODQ preincubation. The contractions induced by EFS were reduced by preincubation with either 6-nitrodopamine or L-741,626. Immunohistochemistry in all arteries revealed the presence of tyrosine hydroxylase in the endothelium, whereas immunoreactivity for calretinin was negative. Swine vessels present basal release of endothelium-derived catecholamines and expression of tyrosine hydroxylase in the endothelium. The vasodilation induced by 6-nitrodopamine is due to blockade of dopaminergic D2-like receptors.

Relationship Between Quantitative Tracheal Geometry and Clinical Course in Various Types of Vascular Ring.

To investigate the relationship between quantitative tracheal geometry and clinical course among various types of vascular ring and to identify factors correlating with symptom presentation. Patients with vascular ring diagnosed between April 2010 and December 2022 were included. All the patients were classified as type 1 (complete double aortic arch); type 2 (incomplete double aortic arch); type 3 (circumflex aorta); type 4 (right aortic arch and aberrant left subclavian artery with a left retroesophageal diverticulum of Kommerell); or type 5 (mirror-imaged right aortic arch with retroesophageal aortic diverticulum). Their clinical characteristics and quantitative variables on computed tomography (CT) were compared. Of the 50 patients enrolled, those with type 1 tended to have a smaller luminal tracheal diameter at the level of the ring. The median symptom-free survival time was shortest in this group (16.0days [95% confidence interval (CI): 9.4-51.0]), followed by type 3 (138.0days [95% CI: 0.0-851.4]). Type 1 (hazard ratio [HR]: 9.0; 95% CI: 2.3-35.0; P = 0.001), type 3 (HR: 4.2; 95% CI: 1.4-13.2; P = 0.013), and the percentage of tracheal narrowing in the anteroposterior projection (HR: 0.87; 95% CI: 0.78-0.96; P = 0.008) were significantly associated with symptom presentation in the time-dependent course. The aortic arch encircling the entire circumference in type 1 and high-pressure vasculature in front of the vertebral body in types 1 and 3 may contribute to raising the risk of symptom presentation.

Vascular Ring Surgical Repair: Re-Implantation of the Left Subclavian Artery into the Left Carotid Artery in a Pediatric Patient.

Vascular rings are a rare congenital anomaly of the aortic arch, in which a ring-shaped structure forms, surrounding the trachea and/or esophagus, potentially causing compression. We describe the case of a 14-month-old female patient with failure to thrive secondary to dysphagia, and a vascular ring formed by a right aortic arch, an aberrant left subclavian artery, and a left ligamentum arteriosum. Surgical repair involved ligamentum arteriosum division, Kommerell's diverticulum obliteration, and left subclavian artery re-implantation into the left carotid artery. Endoscopy and EndoFLIPTM evaluated the intraoperative improvement in esophageal narrowing and impedance, respectively. The postoperative period was uneventful, and follow-up visits demonstrated dysphagia resolution and a patent re-implanted left subclavian artery.

Circumflex left aortic arch: a new vascular ring anomaly in dogs.

To describe the circumflex left aortic arch as a new form of canine congenital vascular ring anomaly leading to esophageal constriction. 3 large-breed dogs of variable age: a 9-month-old entire male German Shepherd Dog-Rottweiler cross, a 17-week-old entire male German Shepherd Dog, and a 9-year-old neutered female Labrador Retriever, seen between April 2022 and May 2023. Dogs presented for chronic postprandial regurgitation and poor growth. One dog presented for a hard mass on the caudal mandibular region. Computed tomography revealed a normal leftward-oriented cranial half and an abnormal caudal half of the aortic arch crossing toward the right dorsal hemithorax leading to esophageal constriction in all dogs. In addition, 1 dog presented with a right patent ductus arteriosus and 1 with an aberrant right subclavian artery. Circumflex left aortic arch is a newly discovered rare type of congenital vascular ring anomaly in dogs, which presents a similar anatomical derangement to the human form. Clinical signs include postprandial regurgitation and poor growth. Circumflex left aortic arch vascular anomaly cannot be surgically corrected via the left thoracotomy that is commonly used for the more common vascular ring anomalies in dogs. Computed tomography is a useful diagnostic modality for the diagnosis of this condition, detection of concomitant congenital vascular malformations, evaluation of associated pathologies, and surgical planning.

Stridor, Snoring and Sleep Apnea in a Three-Year-Old Girl: Report of a Patient with Follicular Lymphoid Hyperplasia

The present study describes a three-year-old girl who was referred to us due to stridor and snoring from one week of age and progressive worsening of sleep apnea two months before our visit. Gastroesophageal reflux disease was ruled out due to being unresponsiveness to treatment. Laryngomalacia was also ruled out because the symptoms and signs were aggravated by passing the time. The presence of a vascular ring was also overruled by echocardiography. Direct laryngoscopy and bronchoscopy were performed to investigate the vocal cord paralysis, glottic stenosis, airway foreign body, adenotonsillar hypertrophy, nasopharyngeal mass, and tracheal stenosis as the etiology. A tonsillar mass was found by them which was confirmed by CT scan. The surgical excision of the mass resulted in the complete resolution of the signs and symptoms. The pathologic studies of the resected mass found it to be follicular lymphoid hyperplasia (FLH), the most common benign lymphoid reaction of the nasopharynx area.

Successful case report of congenital long-segment complex tracheal stenosis treated with sliding tracheoplasty associated with pulmonary artery vascular ring

BackgroundCongenital tracheal stenosis is a rare structural obstructive disorder affecting a segment of the trachea. It is often caused by complete or nearly complete cartilaginous rings narrowing the airway. Approximately 50% of cases are associated with abnormal left pulmonary artery rings.Case presentationWe present the case of a 4-month-old female infant with complex congenital tracheal stenosis associated with a left pulmonary artery vascular ring and congenital heart disease. The patient underwent successful surgical intervention involving repair of the congenital heart defect and reimplantation of the pulmonary artery, followed by sliding tracheoplasty in one surgery procedure. Postoperative outcomes were favorable, with successful extubating and resolution of respiratory symptoms.ConclusionsSliding tracheoplasty proved an effective treatment option in this complex case, highlighting its reproducibility and favorable outcomes in managing congenital tracheal stenosis.

Smooth Muscle Cells-Expressed Bmal1 Regulates Vascular Calcification Independent of the Canonical Circadian Pathway.

Vascular calcification is a major cardiovascular issue that increases morbidity and mortality in diabetes patients. While dysregulation of the circadian master regulator Basic Helix-Loop-Helix ARNT-Like Protein 1 (Bmal1) in vascular smooth muscle cells (VSMC) under diabetic conditions has been suggested, its role in vascular calcification is unclear. In VSMC, Bmal1 was upregulated under high glucose treatment and in aortic tissues from a diabetic mouse model. RNA sequencing from isolated VSMC between Bmal1 deletion and wildtype mice indicated Bmal1's pro-calcification role. Indeed, reduced levels of the osteogenic master regulator, Runt-Related Transcription Factor 2 (Runx2), were found in Bmal1 deletion VSMC under diabetic conditions. Alizarin red staining showed reduced calcification in Bmal1 deletion VSMC in vitro and vascular rings ex vivo . Furthermore, in a diabetic mouse model, SMC-Bmal1 deletion showed reduced calcium deposition in aortas. Collectively, diabetes-upregulated circadian regulator Bmal1 in VSMC contributes to vascular calcification. Maintaining normal circadian regulation may improve vascular health in diabetes.

Salidroside Reduced Ca2+-CaM-CAMKII-Dependent eNOS/NO Activation to Decrease Endothelial Cell Injury Induced by Cold Combined with Hypoxia.

To investigate vascular endothelium damage in rats exposed to hypoxic and cold and the effect of salidroside in protecting against this damage. A rat isolated aortic ring hypoxia/cold model was established to simulate exposure to hypoxic and cold. The levels of endothelial cell injury markers were measured by ELISA. TEM was performed to observe the ultrastructure of vascular ring endothelial cells. In vitro assays were performed to verify the effect of salidroside on endothelial cells. CCK-8 and flow cytometry were performed to analyze endothelial cell survival and apoptosis, respectively. Ca2+ concentrations were measured by Flow cytometry, and the expressions of NOS/NO pathway-related proteins were measured by WB. Endothelial cell damage, mitochondrial swelling, autophagy, and apoptosis were increased in the hypoxia group and hypoxia/hypothermia group. All of these effects were inhibited by salidroside. Moreover, exposure to cold combined with hypoxia reduced the NO levels, Ca2+ concentrations and NOS/NO pathway-related protein expression in the hypoxia group and hypoxia/hypothermia group. Salidroside treatment reversed these changes. Salidroside protected against endothelial cell injury induced by cold and hypoxia through reduction of Ca2+-CaM-CAMKII-dependent eNOS/NO activation, thereby preventing mitochondrial damage, reducing ROS levels, and inhibiting apoptosis.

Surgical approach with minimally invasive intervention in lusoria dysphagia.

The lusoria artery has a prevalence of 0.5-2% in the general population. The abnormal development of the aortic arch forms vascular rings around the trachea and esophagus, causing pressure on them and leading to characteristic symptoms such as chest pain, difficulty breathing, and/or swallowing. Conventionally, only the subclavian artery was severed to release the esophagus, as done in neonates. However, this can lead to long-term hypotrophy of the thoracic limb. The surgical intervention remains controversial, with limitations, and it is only to be performed when the patient presents with symptoms. In the following two cases, a two-stage minimally invasive approach is described: first, a left lateral minithoracotomy for lusoria artery sectioning, and second, a supraclavicular approach for reimplantation into the right carotid artery.

Respiratory Follow-up in a Cohort of Children with Congenital Malformations Affecting Lung Development: A Cohort Study

Background; Congenital malformations like oesophageal atresia (OA) and tracheo-esophageal fistula (TOF), congenital pulmonary airway malformations (CPAMs), congenital diaphragmatic hernia (CDH) and vascular rings (VRs) can influence lung development and respiratory function with significant impact on individuals, families, and health care system. This observational study outlines our multidisciplinary approach and respiratory follow-up for children with these congenital malformations. Methods; We collected clinical data of children followed at the Pediatric Respiratory Unit of Parma University Hospital (Italy) between January 2015 and May 2023. Results; Thirty-five patients have been included. The most common anomalies were AE (n = 12) and CHD (n = 9), followed by CPAMs (n = 9) and VRs (n = 5). In 50% of patients, the diagnosis was made through prenatal ultrasound, particularly in almost all patients with CPAMs (88.8%) and CDH (77.7%), contrary to OE, diagnosed postnatally in the majority of patients (83%). Children underwent their first respiratory visit at an average age of 2.5 years, follow-up was conducted on average every 6 months. More than half of patients (54%) was hospitalized for lower respiratory tract infections, particularly those with OA and those aged <3 years. Eight out of the 16 children capable of performing spirometry showed abnormalities in lung function. Conclusions; Children with congenital malformations are at risk of short and long-term respiratory complications. A personalized follow-up with close collaboration between pediatric pulmonologist, surgeon, neonatologist, physiotherapist is essential to optimize their management and improve their respiratory function.

Implications of right aortic arch prenatal diagnosis: the multicentric nationwide ARCADE cohort

ObjectivesThis study aims to describe the various presentations of the prenatally diagnosed isolated right aortic arch (RAA), that is, without associated congenital heart defect and to evaluate the impact of...

The role of Plaur-miR1-5p encoded within the urokinase receptor gene (Plaur) in angiogenesis

Angiogenesis plays a crucial role in tissue and organ regeneration by supplying essential nutrients and oxygen through the development of new blood vessels. Mesenchymal stem/stromal cells release extracellular vesicles that actively contribute to angiogenesis by carrying pro-angiogenic growth factors and microRNAs. MicroRNAs, small non-coding RNA molecules, are central players in angiogenesis, affecting endothelial cell proliferation, specialization, migration, apoptosis, and post-transcriptional gene expression.In the present study, we investigated the impact of extracellular vesicles containing Plaur-miR1- 5p microRNAs on angiogenesis, specifically focusing on its initial stages: vascular cell migration and the formation of capillary-like structures. Recently we discovered Plaur-miR1-5p, which is encoded within the urokinase receptor gene (Plaur). However, the functions of this microRNA remain largely unexplored. Using a vascular ring model embedded in Matrigel, we demonstrate that Plaur-miR1-5p is encapsulated within extracellular vesicles and plays a regulatory role in capillary-like structure formation. Moreover, applying bioinformatic analysis, we have identified potential target genes of Plaur-miR1-5p that participate in the regulation of angiogenesis.This study advances our comprehension of the fundamental processes governing angiogenesis, particularly the involvement of extracellular vesicles and microRNAs. Moreover, it sheds light on the functional aspects ofthe Plaur gene, contributing to a more profound understanding of its role in regulation of angiogenesis.

Outcomes of surgical treatment of tracheobronchomalacia in children.

Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptomsas well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student'st-test for continuous data. Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.