Symptomatic Vascular Ring Research Articles

Computational fluid dynamics investigation on aortic hemodynamics in double aortic arch before and after ligation surgery

Double aortic arch (DAA) malformation is one of the reasons for symptomatic vascular rings, the hemodynamics of which is still poorly understood. This study aims to investigate the blood flow characteristics in patient-specific double aortic arches using computational fluid dynamics (CFD). Seven cases of infantile patients with DAA were collected and their computed tomography images were used to reconstruct 3D computational models. A modified Carreau model was used to consider the non-Newtonian effect of blood and a three-element Windkessel model taking the effect of the age of patients into account was applied to reproduce physiological pressure waveforms. Numerical results show that blood flow distribution and energy loss of DAA depends on relative sizes of the two aortic arches and their angles with the ascending aorta. Ligation of either aortic arch increases the energy loss of blood in the DAA, leading to the increase in cardiac workload. Generally, the rising rate of energy loss before and after the surgery is almost linear with the area ratio between the aortic arch without ligation and the ascending aorta.

The multiform sonographic spectrum of arterial duct in right aortic arch

To study the different characteristics of arterial duct (AD) in a series of prenatally detected right aortic arch (RAA). Out of 832 congenital heart diseases (CHD) referred to a tertiary center, 98 cases had RAA. Based on anatomical landmarks we identified 7 types of AD: type 1 left-sided, transverse; type 2 left-sided, vertical; type 3 from the underside of aortic arch (AA), vertical; type 4 right-sided, mirror-image “V”, transverse; type 5 right-sided, “H” shaped, transverse; type 6 bilateral; type 7 absent or unidentifiable. For each type of AD the incidence of associated major CHD was calculated and chi-square test was applied to verify the null hypothesis with significance level of p < 0.05. Type 1 occurred in 43% of cases including 4 with CHD and no cases with pulmonary outflow obstruction (POO). Symptoms of vascular ring were present in 41% of survivors. Type 2, 3 and 7 AD were associated with tetralogy of Fallot (TOF) or equivalents. No type 5 AD with CHD had POO and 3 isolated cases had asymptomatic hypoplasia of left pulmonary artery (LPA). Two type 6 AD had disconnection of LPA. Type 1 occurred more often as an isolated finding (p < 0.001), whereas types 2 (p = 0.0026), 3 (p = 0.0045), 4 (p = 0.0325) and 7 (p = 0.0001) were frequently associated with major CHD. In RAA, type 1 (U-shaped) is usually an isolated finding (p < 0.001) which includes all symptomatic vascular rings. POO is always present when the AD is vertical or absent but not when it lies on a transverse plane. Bilateral AD is rare and brings the risk of functionary loss of left lung if not identified.

A Ten-year Single-center Surgical Experience With Symptomatic Complete Vascular Rings

Limited data exist on the management of complete vascular rings (CVR) in adults. We reviewed our institution's surgical experienceinthe managementof these patients. Between 2010 and 2019, all adult patients that underwent a thoracotomy for a CVR repair were identified. We performed a retrospective medical record review of these patients to characterize their demographics and outcomes. Among the 5 patients identified (3 females, 2 males; Mean age 50 ± 9 years), anatomic variants were right arch and Kommerell diverticulum (KD) in 3 (60%) and double aortic arch in 2 (40%) patients. Indications for operation included dysphagia in 4 (80%), respiratory symptoms in 3 (60%) and aneurysmal KD in 1 (20%) patient. Two right aortic arch exclusion, 1 ligamentum arteriosum (LA) division, 1 LA division combined with a KD resection and 2 aortic reconstructions with interposition Dacron graft under partial cardiopulmonary bypass, were performed. Two carotid-subclavian artery transpositions prior to the thoracotomy were done. The postoperative length of stay was 10.0 (IQR 7.3-14.8) days. One reoperation for chylothorax and 1 for symptoms recurrence were performed for the same patient. Over a follow-up period of 1.4 (IQR 0.4-7.0) years, no mortality or majorpostoperative complications occurred. At their last follow-up visit, all patients reported no related remaining symptoms, except for persisting mild asthma in 1 patient. Open repair of CVR in adults can be performed safely with low complication rate. Symptoms improved in all patients after definitive repair.

Symptomatic vascular rings: a case misdiagnosed as difficult asthma in a school-age girl.

Symptomatic vascular rings: a case misdiagnosed as difficult asthma in a school-age girl.

A novel symptomatic vascular ring in a neonate

Introduction: Vascular rings are congenital anomalies of the aortic arch system characterized by a vascular anomalies of mediastinal vessels that encircle and compress the esophagus and trachea. Aortic arch anomalies may be associated with other congenital cardiac defects or chromosomal anomalies, such as microdeletion of chromosome 22q11. In neonatal/pediatric life, clinical presentation shows stridor and difficulty in swallowing or dysphagia.

Congenital vascular ring.

A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. With the development of new technologies, multi-detector computed tomography (MDCT) has become a good diagnostic modality for pre- and postoperative evaluation. MDCT provides an excellent image of aortic arch abnormalities and the related anatomy, as well as the tracheal pathology. For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.

Right Aortic Arch, Kommerell Diverticulum, and Symptomatic Retro-Esophageal Vascular Ring in an Adult (Case Presentation and Review)

A 24 year-old Hispanic man presented with progressive retrosternal chest pain, shortness of breath, and dysphagia. Chest X-ray, computed tomography angiography, and magnetic resonance (MRA) studies were compatible with a right aortic arch and a 3.5-cm descending right aortic Kommerell diverticulum. Barium esophagogram and esophagoscopy demonstrated a constricting band crossing the esophagus at the level of the diverticulum. After discussion with other cardiothoracic surgeons, with a combined 300 years of experience, they reported only 1 other possible such case. Kommerell's diverticula are usually repaired in children and rarely are seen in the adult. Kommerell diverticular treatment options include endovascular stenting, cardiopulmonary bypass with resection and grafting, right thoracotomy, and a cervical approach. Because of the patient's findings of a right arch, a Kommerell diverticulum, and a constricting band causing esophageal compression and respiratory distress, it was felt stenting alone would not relieve his symptoms. Therefore, we used a left third posterior intercostal thoracotomy approach to resect the constricting esophageal band connecting the Kommerell diverticulum to the left subclavian artery and to perform the Kommerell diverticulectomy. Releasing the esophageal and tracheal compressing band and resection of the diverticulum provided complete symptom relief with elimination of the dysphagia, dyspnea, wheezing, and chest pain, using a single surgical procedure.

Identification and management of fetal isolated right-sided aortic arch in an unselected population.

Inclusion of the three vessels and trachea view in the routine assessment of the fetal heart at the 18-20-week morphology scan improves recognition of a right-sided aortic arch (RAA). We report our experience of RAA diagnosed in an unselected population of pregnant women attending for a routine morphology scan. The obstetric imaging databases of two ultrasound centers were reviewed retrospectively to identify all routine fetal morphology scans performed at 18-22 weeks' gestation between January 2011 and December 2014. A review of postnatal charts was conducted to ascertain findings at birth, neonatal complications and the anatomical findings at any neonatal echocardiographic or surgical procedure. Parents of older infants were contacted by phone to assess their wellbeing and identify any respiratory or feeding difficulties. In the 48-month study period, 43 083 routine anomaly scans were performed. Twenty-three cases of isolated RAA were identified, a prevalence of 0.05%. Nineteen (83%) cases of isolated RAA had a left-sided arterial duct and four (17%) had a right-sided duct. Postnatal follow-up data were obtained in all cases. The prevalence of a symptomatic vascular ring due to a double aortic arch was 13% (3/23). One (4%) case was diagnosed with DiGeorge syndrome. RAA can be identified easily on a routine fetal anomaly scan, however the prevalence of RAA is low in an unselected population. Antenatally diagnosed cases should be referred for detailed fetal echocardiography and the patient should be made aware of the association with DiGeorge syndrome and the symptoms associated with a vascular ring. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Assessment of children with vascular ring.

Vascular rings may cause pressure on the trachea and/or esophagus of varying degree, resulting in symptoms. This study assessed the presentation symptoms, diagnostic methods and treatment results after surgery in children with vascular ring. Symptomatic vascular ring patients undergoing surgery between January 2010 and August 2014 at Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, were retrospectively assessed. The presentation symptoms, demographic characteristics, and diagnostic tests were evaluated. Operative data and postoperative follow up, complications and problems were also examined in detail. Twenty-one patients underwent surgery, 13 (63%) of whom were male. Median age was 12 months (range, 1 month-8 years). Among these patients, 62% (n = 13) had double aortic arch, 24% (n = 5) had right-sided aortic arch + ligamentum arteriosum, 10% (n = 2) had left-sided aortic arch + aberrant right subclavian artery, and 4% (n = 1) had pulmonary sling abnormality. Computed tomography (CT) angiography was performed in 17/21 patients. All the patients were successfully operated on. In the early postoperative period, two patients had chylothorax and one had nerve paralysis of nervus laryngeus recurrens. One patient died in the early period, and two patients had ongoing stridor on follow up. CT angiography is effective for the differential diagnosis and visualization of vascular ring abnormality. In such cases, full recovery can be assured with early diagnosis and surgery.

Successful Robotic-Assisted Division of Symptomatic Vascular Ring

Successful Robotic-Assisted Division of Symptomatic Vascular Ring

Imaging a Boa Constrictor—The Incomplete Double Aortic Arch Syndrome

Incomplete double aortic arch is a rare anomaly resulting from atresia rather than complete involution in the distal left arch resulting in a non-patent fibrous cord between the left arch and descending thoracic aorta. This anatomic anomaly may cause symptomatic vascular rings, leading to stridor, wheezing, or dysphagia, requiring surgical transection of the fibrous cord. Herein, we describe an asymptomatic 59 year-old man presenting for contrast-enhanced CT angiography to assess cardiac anatomy prior to radiofrequency ablation, who was incidentally found to have an incomplete double aortic arch with hypoplasia of the left arch segment and an aortic diverticulum. Recognition of this abnormality by imaging is important to inform both corrective surgery in symptomatic patients, as well as assist in the planning of percutaneous coronary and vascular interventions.

Primary Translocation of Aberrant Left Subclavian Artery for Children With Symptomatic Vascular Ring

The standard operation for vascular ring with right aortic arch and aberrant left subclavian artery is ligamentum arteriosum division. A new surgical approach with primary translocation of the aberrant left subclavian artery to the left carotid artery, removal of the Kommerell diverticulum, and division of the ligamentum through left thoracotomy was recently applied. This study assessed the early outcomes of this approach. This is a retrospective review of all patients having an operation for right aortic arch with aberrant left subclavian artery and the Kommerell diverticulum between January 2001 and April 2011 at a single institution. Eight patients had a division of the ligamentum and 10 had a primary translocation of an aberrant left subclavian artery with diverticulum removal and ligamentum division. The median operative age and weight for each surgical technique group were, respectively, 2.2 and 1.8 years (p = 0.56) and 10.7 and 12.6 kg (p = 0.30). All patients were symptomatic preoperatively. No deaths occurred. One patient from each group had chylous effusion that was medically treated. The median hospital stay for each group was 4 and 5 days (p = 0.45). During the median follow-up of 47 months, 3 patients in the division of ligamentum group required albuterol aerosol or oral antiinflammatory medication for respiratory symptoms. All patients in the primary translocation group remained asymptomatic and required no medication (p = 0.069). Primary translocation of aberrant left subclavian artery with removal of the diverticulum and division of the ligamentum had excellent early outcomes and can potentially eliminate residual symptoms and late complications.

Right aortic arch and its variants

A number of congenital anomalies of the aortic arch complex can occur, ranging from asymptomatic normal variations in arch vessel branch pattern to symptomatic vascular rings, stenoses, and arch interruptions with a frequency ranging from 0.5% to 3.0%. A right aortic arch is present in 0.1% of the population and can occur in isolation or be associated with congenital heart disease. Patients may present at any age with signs and symptoms of airway or esophageal compression, heart failure, or abnormal chest imaging studies. This pictorial essay will show the computed tomography and appearances of congenital variations of the right aortic arch and use Edwards' hypothetical embryonic double aortic arch model to explain the cause of some of these variants.

Unusual Manifestations of Vascular Rings

Vascular rings are a relatively rare entity caused by abnormal development of the fetal aortic arches. Most patients with vascular rings present in infancy or early childhood with respiratory (inspiratory) symptoms. The treatment of patients with symptomatic vascular rings is generally straight-forward, focusing on surgical division of the ring. The majority of patients are "cured" by this simple procedure. However, a small percentage of patients do not follow this typical course. The purpose of this manuscript is to review and discuss three patients who demonstrated unusual manifestations of vascular rings. Two patients required reoperation after double aortic arch repair and one had successful treatment of an aberrant right subclavian artery.

Late-onset dysphagia lusoria assessed by 3-dimensional computed tomography of an aortic arch abnormality

Symptomatic vascular rings causing late-onset dysphagia (dysphagia lusoria) are quite unusual in adults. We present a 42-year-old woman with a 1-year history of dysphagia from a right-sided aortic arch with an aberrant left subclavian artery and left-sided ligamentum arteriosum. Using a helical CT scan of the chest and a three-dimensional reconstruction software, her anatomic abnormality was completely delineated. Arch aortogram confirmed the anomaly. The patient underwent a left posteriolateral thoracotomy. Intraoperative findings correlated precisely with the preoperative reconstruction data. Division of the ligamentum resulted in a complete decompression of the esophagus and resolution of the patient's symptoms.

Vascular anomalies causing tracheoesophageal compression: a 20-year experience in diagnosis and management.

Vascular rings and other congenital aortic arch anomalies may be major causes of tracheoesophageal obstruction in children. In this report, our diagnostic approach, surgical treatment, and early and late results for 30 patients are reported. During a 20-year period (1982-2002), 30 children underwent surgery for tracheoesophageal compression caused by aortic arch anomalies. The median age at operation was 8 months (range, 36 days to 94 months), and the median patient weight was 8 kg (range, 2.4-16 kg). At 53.3% of cases, double aortic arch was by far the most common encountered cause of compression. Patients were admitted with respiratory distress, stridor, apnea, dysphagia, or recurrent respiratory tract infections. Diagnosis was established by barium esophagogram, computed tomography, magnetic resonance imaging, and angiography. The operative approaches were through a left thoracotomy or a median sternotomy. Operative mortality rate was 3.3%. Follow-up data from 2 months to 10 years (mean follow-up, 34 weeks) were available for all 30 patients. Twenty-six patients (86.7%) were essentially free of symptoms, 3 patients (10%) had residual respiratory problems, and 1 patient (3.3 %) had a gastroesophageal reflux problem. These results suggest that surgical correction of symptomatic vascular rings can be performed with low mortality and morbidity rates.

Late-onset dysphagia lusoria

Symptomatic vascular rings are uncommon in adults. We report a case of dysphagia in a 48-year-old woman from a right-sided aortic arch with aberrant left subclavian artery and left-sided ligamentum arteriosum. Through a left posterolateral thoracotomy, the ligamentum was divided, relieving the patient’s dysphagia. Twenty-three cases of adult-onset dysphagia lusoria attributable to this anatomy have been reported. We recommend a left thoracotomy with division of the ligamentum as treatment.

Symptomatic Vascular Rings in Adulthood: An Uncommon Mimic of Asthma

Symptomatic thoracic vascular rings presenting in adulthood are thought to be rare. During a 3-year time period, we diagnosed four cases of symptomatic vascular rings, which had been treated unsuccessfully for suspected asthma. Spirometry was characterized by normal forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1), and FEV1/FVC, decreased peak expiratory flow (PEF), and truncation of the expiratory flow volume loop. Chest radiographs revealed a right aortic arch in each case with computed tomography (CT) or magnetic resonance imaging (MRI) confirming the diagnosis of a vascular ring. The specific abnormalities consisted of right aortic arch with mirror branching of the main arteries and persistent ligamentum arteriosum; right aortic arch with diverticulum and a fibrous embryonic left arch; right aortic arch with aberrant left subclavian artery arising from a diverticulum of Kommerell; and a right aortic arch with persistent ligamentum arteriosum. Although they are uncommon, vascular rings first presenting in adulthood as a mimic of asthma are not rare. This diagnosis should be considered in adults when abnormal truncation of the flow-volume loop occurs or when radiographic aortic arch abnormalities are found.

Right aortic arch with aberrant subclavian arteries: a cause of esophageal compression

We report a case of symptomatic partial vascular ring that to our knowledge has not been previously described. This anomaly includes a right aortic arch with an aberrant left subclavian artery and an atypical origin of the right subclavian artery.

Vascular Rings of the Thoracic Aorta in Adults

Vascular rings have been well documented to cause respiratory and gastrointestinal symptoms in infants and children. Few reports document symptomatic vascular rings in adults, and most have emphasized dysphagia as the predominant symptom. We present the case of a 36-year-old white male with a double aortic arch and progressive dyspnea on exertion. This led us to review previous reports of vascular rings in adults. Criteria for review consisted of anatomically complete vascular rings of the aortic arch in adults age 18 years or older. We identified 25 prior cases for review and included our recent patient. The most common vascular ring anomalies in our review of adults is double aortic arch (n = 12; 46%) followed by right aortic arch with aberrant left subclavian artery and ligamentum arteriosum (n = 8; 30%). Of 24 patients (66%), 16 were symptomatic. Reported symptoms involving the respiratory tract (n = 10 of 24; 42%) included dyspnea on exertion (n = 5), bronchitis (n = 2), recurrent pneumonia, stridor, and unspecified respiratory ailment (n = 1 each). Dysphagia was less common, occurring in eight patients (33%). Previously proposed mechanisms for respiratory tract symptoms include tracheomalacia, static or dynamic compression of the airways, intravascular volume infusion, and aspiration. We also propose exercise-induced dilatation of the aortic arch and age-dependent changes in thoracic compliance as potential mechanisms of dyspnea.