Congenital Vascular Ring Research Articles

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.

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.

Diagnosis of congenital thoracic arterial vascular rings in infants and young children using multi-slice spiral CT and MRI.

Diagnosis of congenital thoracic arterial vascular rings in infants and young children using multi-slice spiral CT and MRI.

Congenital vascular rings as a cause of respiratory and esophageal problems in children

Congenital anomalies of the aortic arch include a wide range of anatomic pathologies, which may be completely asymptomatic or accompanied by severe respiratory and/or esophageal symptoms, especially in the case of complete vascular rings (VR). Purpose - to draw the attention of pediatricians and family phisicians to possible clinical presentations of aortic arch anomalies, namely VR, to increase their awareness of this pathology, that will contribute to differential diagnosis. VR, are anomalies of the aortic arch that result from abnormal development of the embryonic aortic arches. The prevalence of VR is approximately 1%, of which 55% are double aortic arch. This congenital heart defect can be either isolated or combined with other congenital heart defects. Associated cardiac pathology occurs in 12.6%, and includes ventricular septal defect and Tetralogy of Fallot. Sometimes this defect is associated with DiGeorge’s and Turner’s syndrome. The timing of appearance of clinical symptoms varies from the early neonatal period to adulthood. However, most patients develop clinical symptoms in the first months after birth, and they indicate the need of cardiac surgery during the first year of life. Given the serious consequences of this anomaly, VR should be included in the list of differential diagnoses of wheezing, stridor, difficulty swallowing and bronchial obstruction syndrome in children, especially when these symptoms appear since birth, are persistent and respond poorly to standard therapeutical approaches. Therefore, it is important for pediatricians to be alert for a congenital heart defect such as VR as one of the possible causes of congenital stridor, wheezing, and swallowing disorders in infants. Contrast-enhanced computed tomography and magnetic resonance imaging of the heart are the diagnostic modalities of choice for VR diagnosis. These diagnostic methods provide accurate location, nature of branching and dominance of the aortic arch as well as the degree of compression of the airway and esophagus. Three-dimensional reconstruction helps to plan future surgical intervention. No conflict of interests was declared by the authors.

Coexisting Occult Congenital Anomalies: Case Presentation & Literature Review of a Vascular Ring in Beckwith-Wiedemann Syndrome

Beckwith-Wiedemann Syndrome (BWS) is a pediatric overgrowth disorder commonly associated with macroglossia. Speech/feeding difficulties and airway compromise often warrant surgical intervention through reduction glossectomy. Given the complexity of pediatric patients with congenital anomalies, concurrent diagnoses of coexisting conditions may be delayed. We present a pediatric patient with BWS who presented with chronic respiratory distress and stridor in the setting of obstructive sleep apnea (OSA) and macroglossia. We seek to highlight the concurrent presence and treatment approach of a congenital vascular ring and macroglossia as there are no reported cases of these coexisting anomalies in the literature.

Un suono che inganna: quando sospettare il doppio arco aortico

The article describes a case of a 10-month-old male infant presenting with respiratory distress, laryngotracheitis and double aortic arch (DAA). Tracheal compression by vascular structures in infants is uncommon and may be masked by nonspecific respiratory symptoms. DAA is the most common vascular ring. Doctors should think about a congenital vascular ring due to DAA in children with repeated episodes of laryngitis, hissing breath, cough and dyspnoea when other common aetiologies are excluded. A precise diagnosis can be made through the contrast-enhanced chest CT scan.

Congenital Aortic Vascular Ring: In-Utero Sonographic Assessment ofTracheal Patency and Postnatal Outcome.

Congenital aortic vascular ring may present after birth with variable degrees of respiratory distress due to tracheal compression. The aim of this study was to prospectively evaluate in utero tracheal patency in correlation with postnatal outcome. During an eight-year period, fetuses with aortic arch abnormality encircling the trachea and forming a complete ring were recruited for the study. Tracheal patency was classified as: no compression, partial compression, or complete compression. Postnatal MRI/CTangio studies were performed and outcome data was retrospectively analyzed from the medical records. Among the 46 fetuses recruited to the study, 38 had right aortic arch (RAA), and 8 presented with double aortic arch (DAA). In the RAA group 35 (92.1 %) of the fetuses presented no compression and 34 (97.1 %) of them were asymptomatic in the long-term follow-up. Three fetuses (7.9 %) in this group presented in utero compression: one was terminated at 16 weeks of gestation due to associated ominous findings, and the other two had mild respiratory symptoms around 12months of age and underwent surgery with a good outcome. In the DAA group, all fetuses presented in utero with tracheal compression. Seven showed partial and one complete compression. Among the seven with partial compression, six were symptomatic and underwent surgery. The case with severe airway occlusion had emergency tube insertion in the delivery room and underwent surgery at 7 days but died from severe respiratory complications. This is the first study to evaluate in utero tracheal patency in cases with vascular ring. It allows better prenatal and postnatal workup and follow-up including potentially life-threatening respiratory failure.

Congenital malformations potentially affecting respiratory function: multidisciplinary approach and follow-up.

Background and aim.Congenital malformations such as oesophageal atresia (OA) and tracheoesophageal fistula (TOF), congenital pulmonary airway malformations (CPAMs), congenital diaphragmatic hernia (CDH) and vascular rings (VRs) can affect lung development and respiratory function. This observational study describes our multidisciplinary approach and respiratory follow-up of children with such congenital malformations.Methods.Clinical data of children followed at the Pediatric Respiratory Unit of Parma University Hospital (Italy) between January 2015 and January 2020 were collected.Results.Twenty-three patients with congenital malformation affecting lung development were identified. Almost half of our patients were diagnosed with fetal ultrasound. Children attended the clinic at a mean age of 3 (3.7) years and follow-up visits were scheduled every 6 months average. More than half of our patients were hospitalized for lower respiratory tract infections. Six out of 9 children able to perform spirometry showed anomalies in lung function. Chest physiotherapy was recommended especially in children with OA.Conclusions.Children with congenital malformations affecting lung development are at risk of short and long-term respiratory complications, especially in the first years of life. OA was the malformation more associated to respiratory problems. Multidisciplinary approach and appropriate personalized follow-up are recommended for the best management of these children. (www.actabiomedica.it)

Diagnosis and management of congenital vascular rings: Efficacy of prenatal sonography and enhanced-computed tomography

Diagnosis and management of congenital vascular rings: Efficacy of prenatal sonography and enhanced-computed tomography

Diagnosis and surgical repair of congenital double aortic arch in infants

ObjectivesDouble aortic arch (DAA) is a rare congenital vascular malformation. This study aims to summarize the experience of diagnosis and surgical treatment for congenital double aortic arch.MethodsThe clinical data of 24 cases with double aortic arch (DAA) from January 2008 to January 2018 in our hospital was reviewed retrospectively.ResultsA total of 24 cases, including 12 patients with isolated DAA and 12 patients with DAA and associated intracardiac defects were identified. There were 14 males and 10 females, with an average age of 11 months. The associated intracardiac malformations included ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), pulmonary stenosis (PS), and patent foramen ovale (PFO). Of the 12 patients with DAA and intracardiac malformations, 7 patients underwent intracardiac repair simultaneously, however, 3 patients underwent isolated double aortic arch correction. One patient with DAA and TGA underwent surgical correction of congenital vascular ring at the first stage, and the arterial switch operation was performed at the second stage. The clinical outcomes of 23 patients were promising, however, in one patient, parents decided not to do the surgery due to personal reasons. The average follow-up time was 35 months.ConclusionsTracheal and esophageal compression are commonly seen in patients with DAA, however could be relieved significantly after surgery. In particular cases, the simultaneous intracardiac defects repair could be performed. Misdiagnosis was easily established with isolated echocardiography. Fortunately, the correct diagnosis of DAA and associated intracardiac defects could be established with the use of combined chest computed tomography.

The long-term outcome of an isolated vascular ring - A single-center experience.

The aim of this study is to document the long-term outcome of patients with a vascular ring. A single-center retrospective review of clinical symptoms was conducted in all patients born between 1980 and 2013, diagnosed with a complete vascular ring and at least 2 years of follow-up. Data were extracted from patient files and clinical symptoms were assessed by questionnaires sent to the parents. Age and diagnostic tools, type of surgery, postoperative complications, and the prevalence of clinical symptoms (stridor at rest, stridor with exercise, cough, exercise intolerance, dysphagia, and frequent respiratory infections) were reviewed. Fifty-one patients were included. The diagnosis was made before the age of 2 in 35/51. Surgery was performed in 41/51 patients with a limited number of reversible complications. The median follow-up was 8 (IQR 5-12) years. After 2 years, 21/51 patients were asymptomatic: 2/7 asymptomatic patients at diagnosis became symptomatic and symptoms resolved in 16/44 initially symptomatic patients. Consequently, 30/51 patients still had symptoms after 2 years. Of these, 16 had partial improvement, 5 stable symptoms, and 9 aggravation of symptoms. After 10 years, 26/36 patients were free of complaints. The most frequent long-term symptom was stridor. Surgical treatment of a congenital vascular ring is safe and mostly performed in patients with a double aortic arch. Conservative treatment for patients with little symptoms seems to be justifiable. A considerable number of patients have residual long-term symptoms after surgical relief of the vascular ring.

The Natural and Unnatural History of Congenital Aortic Arch Abnormalities Evaluated in an Adult Survival Cohort

BackgroundThis study describes the different types of congenital vascular rings according to their anatomy, symptoms, and age at clinical onset and reports the surgical outcomes. MethodsA retrospective observational database study was conducted, reviewing the medical charts of 69 adult survivors with a history of a vascular ring, identified from the Dutch Congenital Cor vitia database. ResultsMedian age at presentation was 8.5 years (0-53.0 years). Thirty patients (43.5%) had a “left aortic arch with aberrant right subclavian artery,” 21 patients (30.4%) a “double aortic arch,” and 16 patients (23.2%) a “right aortic arch with aberrant left subclavian artery.” The main symptomatology at presentation comprised respiratory symptoms (82.9%). Almost three-quarters of patients were also diagnosed with asthma/bronchial hyperreactivity. Patients with a double aortic arch had more symptoms than patients with a left aortic arch with aberrant right subclavian artery and right aortic arch with aberrant left subclavian artery (P < 0.001), requiring surgery most often (P < 0.001). In patients with childhood onset of symptoms, preoperative spirometry (ie, peak expiratory flows) was more often abnormal as compared with adult patients (P = 0.007). Surgery was performed in 42.0% of all patients at a median age of 17 years (0-63.0 years). Twenty-four (92.3%) of the operated patients showed improvement or complete relief of symptoms shortly after surgery. Of 26 asymptomatic nonoperated patients, 3 patients (11.5%) eventually developed symptoms. ConclusionsThe low incidence of vascular rings, their anatomic heterogeneity, and a wide range of common symptoms often lead to misdiagnosis. Clinical awareness is warranted as a large subset of patients could benefit from surgery, even at an adult age.

Asthma-like symptoms: is it always a pulmonary issue?

Background: Double aortic arch is a rare congenital and complete vascular ring around trachea and esophagus. It is usually diagnosed during infancy. The symptoms are generally related to respiratory and gastroesophageal tracts. Case presentation: A 20-year-old female patient was referred to our outpatient clinic for persistent dry cough. She had a history of an episode of inhalation of food bolus as an infant and recurrent bronchitis, anorexia and allergic bronchial asthma since the childhood. Since the beginning, an intrathoracic obstruction was suspected at pulmonary function tests. After 1 month of complete asthma treatment, the cough was unchanged and the spirometry confirmed the presence of an intrathoracic obstruction. Then, she underwent a chest CT with contrast medium, a contrast transthoracic echocardiography, a fiberbronchoscopy and an esophageal radiography with contrast medium. The final diagnosis was made and a double aortic arch was found. Conclusion: A careful observation of the flow/volume curve should always be guaranteed and the presence of congenital vascular anomalies should be suspected in case of difficult-to-treat asthma.

Asthma-like symptoms: is it always a pulmonary issue?

BackgroundDouble aortic arch is a rare congenital and complete vascular ring around trachea and esophagus. It is usually diagnosed during infancy. The symptoms are generally related to respiratory and gastroesophageal tracts.Case presentationA 20-year-old female patient was referred to our outpatient clinic for persistent dry cough. She had a history of an episode of inhalation of food bolus as an infant and recurrent bronchitis, anorexia and allergic bronchial asthma since the childhood. Since the beginning, an intrathoracic obstruction was suspected at pulmonary function tests. After 1 month of complete asthma treatment, the cough was unchanged and the spirometry confirmed the presence of an intrathoracic obstruction. Then, she underwent a chest CT with contrast medium, a contrast transthoracic echocardiography, a fiberbronchoscopy and an esophageal radiography with contrast medium. The final diagnosis was made and a double aortic arch was found.ConclusionA careful observation of the flow/volume curve should always be guaranteed and the presence of congenital vascular anomalies should be suspected in case of difficult-to-treat asthma.

Quantified evaluation of tracheal compression in pediatric complex congenital vascular ring by computed tomography

Clinically, early diagnosis and treatment is important for survival of pediatric with vascular ring (VR) associated with congenital heart disease (CHD), and accurate evaluation of VR is a prerequisite for repair surgical. The study aimed to assess the quantitative characteristics of tracheal compression in pediatrics with VR and CHD using dual-source computed tomography (DSCT), and further provided effective information for surgical decisions. A total of 49 VR patients with CHD and 56 controls were enrolled. The tracheal quantitative measurements (short diameter, long diameter, tracheal area and tracheal length) were obtained, and the degree of tracheal compression was assessed. Our results indicated that VR associated with CHD may cause more serious and complex symptoms, and the greater tracheal compression were found on DSCT when more severe symptoms were present (r = 0.84). The degree of tracheal compression was significantly different within the VR group between those with and without surgery (P = 0.002). Finally, there were good agreement among (1-long diameter ratio), (1-short diameter ratio) and (1-area ratio) in patients and controls, respectively. This study indicated that DSCT enables provides accurate quantitative tracheal compression information for VR pediatrics associated with CHD, and evaluation of the degree of tracheal compression by 1-area ratio may contribute to the repair surgical of VR.

Comparative study between multi-detector computed tomography and echocardiography in evaluation of congenital vascular rings

Vascular rings are unusual anomalies represent less than 1% of all cardiac anomalies, it is abnormal development of aortic arch complex leading to formation of a ring formed by vessels that encircle both the trachea and esophagus, echocardiography had limited acoustic window which leads to inadequate evaluation of great vessels, Multi-detector computed tomography (MDCT) is one of the most important non invasive diagnostic tool for detection the vascular ring anomalies. The AimTo evaluate role of MDCT Scanning for diagnosis of congenital vascular rings anomalies compared to echocardiography. MethodsThis is a prospective study of 21 children suspected to have vascular ring anomalies. All patients underwent chest radiography, echocardiography examinations and MDCT Scanning using a 128-row CT scanner with 3D reconstruction. ResultsTwenty-one patients (11 male and 10 female), mean age (14 months) were diagnosed as vascular ring anomalies by MDCT then confirmed by surgical results MDCT diagnosed 14 patients with (double aortic arch), 4 patients (right sided aortic arch with aberrant left subclavian), 2 patients (left sided aortic arch with aberrant right subclavian artery) and one patient with pulmonary sling. MDCT also diagnosed seven patients with tracheobronchial stenosis. Echocardiography succeeded only in nine patients with DAA and failed in five and succeeded in diagnosis of two cases of (right aortic arch with aberrant left subclavian). ConclusionMDCT is an excellent diagnostic tool for optimum detection of vascular ring anomalies and other extra cardiac lesions compared to echocardiography.

Report of One Case with Congenital Vascular Ring

Congenital vascular rings is an unusual congenital condition and severely affects the survival and life quality of the patients. A 23-year-old gravid woman was referred for counseling at 24 weeks of gestation because of abnormal ultrasound findings of fetal congenital vascular ring. Fetal echocardiography showed a complete vascular ring with a right aortic arch (RAA), left ductus arteriosus (LDA) and left intracardia echogenicfocus. Conventional cytogenetic analysis revealed an apparent balanced reciprocal translocation between the distal segment of the long arm of a chromosome 5 and the long arm of chromosome 2: 46, XY, t (2;5) (q3.5; q31.1). This abnormal karyotype was detected in gravid woman. However, the microarray analysis on amniocytes using HumanCytoSNP-12 array detected 2.57-Mb deletion at 22q11.21. Metaphase fluorescence in situ hybridization (FISH) analysis on cultured amniocytes confirmed an interstitial 22q11.2 deletion. The fetus was died owing to breathing and feeding difficulties. Our study highlights the clinical value of genetic detection and prenatal diagnosis of Congenital vascular rings by karyotype analysis coupled with SNP array.

Early and late outcomes after surgical management of congenital vascular rings

Early and late outcomes after surgical management of congenital vascular rings

Advances in early diagnosis and treatment of congenital vascular rings with airway stenosis

Vascular ring is the congenital abnormality of the aortic arch and its branches, around the trachea and / or esophagus, resulting in a series of compression symptoms.The clinical prognosis depends on the degree of airway stenosis.Due to easiness to be misdiagnosed, early correct diagnosis and timely surgical treatment are the key to survival.This article discusses the early diagnosis and treatment progress of vascular ring with airway stenosis, aiming to improve the physician′s skills of diagnosis and treatment. Key words: Vascular rings; Airway stenosis; Early diagnosis; Treatment

Early and late outcomes after surgical management of congenital vascular rings.

Surgical relief of tracheoesophageal compression is commonly effective in vascular ring anomalies. Respiratory symptoms necessitating chronic inhalation therapy only persist in a minority of children. Patients with double aortic arch are at increased risk to remain symptomatic, particularly with infectious exacerbations.