[Double aortic arch and aortotracheal fistula as cause of fatal massive bleeding: a rare and dangerous association]. / Doble arco aórtico y fistula aortotraqueal como causa de hemorragia masiva fatal: una asociación rara y peligrosa.

Vascular rings are unusual congenital malformations. Among them, double aortic arch (DAA) is often difficult to diagnose due to its low incidence of symptoms. DAA can be associated with tracheal or esophageal compression and, in severe cases, could require tracheal intubation or chronic use of a nasogastric tube. This scenario favors the development of aortotracheal fistulas (ATF) or aortoe-sophageal fistulas (AEF). OBJECTIVE: To present a clinical case with an unusual association of DAA with ATF and to reinforce the importance of maintaining high diagnostic suspicion in patients with massive aerodigestive bleeding without an obvious source. CLINICAL CASE: A 32-week preterm newborn who required prolonged mechanical ventilation and presented intermittent episodes of massive oropharyngeal bleeding with hemodynamic compromise associated with lower airway obstruction without pulmonary hemorrhage. The patient underwent upper endoscopy and exploratory laparotomy without evidence of bleeding. Flexible nasopharyngolaryngoscopy and direct laryngoscopy also showed no abnormalities. A CT angiography showed complete DAA with indentation of the left dominant arch over the trachea, without severe stenosis or evidence of a fistula. AEF was suspected, so exploratory surgery was considered. However, the patient died before surgery due to a massive pulmonary hemorrhage. The autopsy revealed the presence of ATF. CONCLUSIONS: In patients with massive aerodigestive bleeding without an obvious source, the presence of DAA and possible AEF/ ATF should be considered. Imaging studies have a poor performance for this diagnosis, so surgery should be considered for diagnosis and treatment in these patients.

Prenatal diagnosis of right aortic arch: associated anomalies and fetal prognosis according to different subtypes.

OBJECTIVES: Right aortic arch (RAA) is a rare anomaly with an incidence of 0.1 % in the adult population and low-risk fetuses. Our aim in this study was to evaluate associated anomalies and conditions according to subtypes. METHODS: This was a retrospective study examining consecutive pregnancies diagnosed with RAA in our hospital between 2018 and 2022. Fetuses with RAA were divided into three groups, RAA with right-sided ductus arteriosus (RAA-RDA), RAA with left-sided ductus arteriosus (RAA-LDA), and RAA with a double aortic arch (RAA-DAA). RESULTS: A total of 81 fetuses were diagnosed as having RAA during the study period. The rate of cardiac anomalies (82.8 %) in the RAA-RDA group was higher than in the RAA-LDA (17.6 %) and RAA-DAA (22.2 %) groups (p<0.001). No statistically significant difference was found between the groups in terms of maternal age, diagnosis week, pregnancy outcome, extracardiac anomalies, and genetic anomalies. Three (8 %) of 36 fetuses with isolated RAA who resulted in live birth developed symptoms related to the vascular ring, and one (2.7 %) newborn with RAA-DAA underwent surgery. CONCLUSIONS: The incidence of cardiac anomalies is high in fetuses with RAA-RDA. Ultrasound examinations should be performed for cardiac anomalies and additional structural anomalies. Vascular ring formation is a rare but important complication due to compression risk to the trachea and esophagus.

Management of esophageal cancer in patients with a right aortic arch or double aortic arch: a case series of 34 cases.

BACKGROUND: Few cases describing patients with a right aortic arch (RAA) or double aortic arch (DAA) and esophageal cancer (EC) have been reported. METHODS: We analyzed RAA and DAA cases treated with esophagectomy in our center's database and reported in English-language studies until April 1, 2023. Our study assessed the malformation characteristics and surgical details of EC patients with RAA and DAA. RESULTS: We extracted data of 24 EC patients with RAAs and 10 EC patients with DAAs. In both groups, the patients were more likely to be Japanese and male, to have squamous cell carcinoma and to have tumors located in the upper thoracic esophagus or middle thoracic esophagus. Left thoracotomy was commonly applied for RAA patients. For DAA patients, the proportions of left-sided and right-sided approaches were similar. Esophagectomy under video-assisted thoracoscopic surgery (VATS) in RAA or DAA patients had been performed on a routine basis in recent years. There were two anastomotic leakages in each group. Specifically, Kommerell diverticulum rupture occurred in 1 RAA patient; gastric tube dilation occurred in 1 DAA patient; and recurrent laryngeal nerve (RLN) injury occurred in 2 RAA patients. The postoperative course was uneventful for most patients in both cohorts. CONCLUSIONS: Esophageal carcinoma is rarely seen in patients with an RAA or DAA. To adequately dissect superior mediastinal LNs, an auxiliary incision (such as sternotomy), the left door open method or a preceding cervical procedure should be used appropriately. Esophagectomy, whether via thoracotomy or thoracoscopic surgery, can be performed safely for both RAA and DAA.

Incomplete vascular ring of the aortic arch presenting with dysphagia in an adult: case report.

Anomalies of the arterial branches of the arch of the aorta are rare, with the aberrant right subclavian artery being the most common of this anomaly. Majority of the anomalies are asymptomatic and often discovered as incidental findings. In the great majority of the symptomatic cases, the presentation may be either with breathlessness or dysphagia or both. This is in addition to the nature of the intrinsic arterial disease of the aberrant vessel, especially in adult patients; and unless borne in mind, the diagnosis is often missed leading to delays and wrong treatment. In this report we present a case of dysphagia in an adult male Nigerian initially diagnosed as œsophageal stricture from herbal potion ingestion but review of his imaging investigations gave a final diagnosis of dysphagia lusoria from an aberrant right subclavian artery. The difficulty in making a diagnosis and the need for a multidisciplinary review of the imaging investigations are highlighted. The patient was successfully treated by a combined trans-thoracic and cervical approach with division and re-implantation of the aberrant vessel unto the right common carotid artery. He has remained symptom-free for 2 years after surgery. Although the great majority of these anomalies are often asymptomatic, it is important they are borne in mind both in imaging investigations as well as in procedures involving structures in the upper visceral mediastinum. Various surgical approaches have been documented in the management of symptomatic ones; it is however recommended that options that ensure revascularization of the affected limb be selected.

COMMD5 is involved in the mechanisms of hypotension after parathyroidectomy in patients receiving hemodialysis.

BACKGROUND: Secondary hyperparathyroidism (SHPT) is a common complication of end-stage renal disease. Parathyroidectomy (PTx) is often employed for treatment of severe SHPT. However, PTx may cause hypotension via unknown mechanisms. COMM domain-containing protein 5 (COMMD5) in the parathyroid glands has been linked to blood pressure regulation of spontaneously hypertensive rats. OBJECTIVE: To explore the relationship between COMMD5 levels and reduced BP after PTx in patients receiving hemodialysis (HD). METHODS AND RESULTS: (1) The study cohort included 31 patients receiving HD who underwent PTx. Serum COMMD5 levels were higher post-PTx vs. pre-PTx. (2) Sprague-Dawley rats (n = 22) were assigned to a 5/6 nephrectomy group or sham surgery group, vascular rings of the thoracic aorta from rats with CKD were incubated with COMMD5, and changes in vascular tension were compared. COMMD5 inhibited vasoconstriction of vascular rings with intact endothelium, but had no effect on vascular rings without the endothelium. (3) Human umbilical vein endothelial cells were stimulated with COMMD5 or small interfering RNA (siRNA). The expression levels of atrial natriuretic peptide (ANP) and endothelial nitric oxide synthase (eNOS) were up-regulated and down-regulated, respectively. CONCLUSIONS: Serum COMMD5 levels were increased after PTx in SHPT patients. COMMD5 promoted high expression of ANP and eNOS in endothelial cells, leading to vasodilation and resulting in hypotension.

Subclavian Steal Phenomenon Associated With Vascular Ring in an Infant Who Had a Prenatally Diagnosed Right Aortic Arch With an Atretic Aberrant Left Subclavian Artery.

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.

Concurrent double aortic arch and circumflex aorta repair in a symptomatic child: a case report.

Double aortic arch with circumflex aorta is a rare type of vascular ring. Symptoms result from external compression of the trachea and esophagus. The best surgical approach for patients with double arch and circumflex aorta is debated, and options include the highly complex aortic uncrossing procedure. Herein we describe the surgical treatment of a patient with concurrent double aortic arch and circumflex aorta by division of the non-dominant arch and ligamentum arteriosum, plication and posterior tacking of the diverticulum of Kommerell. This left thoracotomy approach provided complete symptom resolution.

Paediatric tracheobronchomalacia: Incidence, patient characteristics, and predictors of surgical intervention.

OBJECTIVES: Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients. METHODS: We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis. RESULTS: 249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention. CONCLUSIONS: TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention.

Asociación del complejo ring-sling con pig bronchus: a propósito de un caso pediátrico / Ring-sling complex association with pig bronchus: a pediatric case report

El complejo ring-sling es una asociación entre el sling de la arteria pulmonar y la estenosis traqueal congénita por anillos traqueales completos. El sling de la arteria pulmonar es una forma rara de anillo vascular dentro de las cardiopatías congénitas. Se presenta el caso clínico de un niño con estridor laríngeo asociado a dificultad respiratoria evaluado en otro centro, donde se realizó endoscopia respiratoria y se observó compresión traqueal extrínseca. Ante la sospecha clínica de anillo vascular, se solicitó angiotomografía computada (angioTC) y se confirmó diagnóstico de sling de arteria pulmonar. Fue derivado a nuestro hospital y durante la intervención quirúrgica se realizó nueva endoscopia respiratoria y traqueobroncografía. Se llegó al diagnóstico de estenosis traqueal congénita con bronquio derecho accesorio (pig bronchus) y anillos vasculares completos, ambos reparados en el mismo acto quirúrgico.

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act.

[Ring-sling complex association with pig bronchus: a pediatric case report]. / Asociación del complejo ring-sling con pig bronchus: a propósito de un caso pediátrico.

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act.

El complejo ring-sling es una asociación entre el sling de la arteria pulmonar y la estenosis traqueal congénita por anillos traqueales completos. El sling de la arteria pulmonar es una forma rara de anillo vascular dentro de las cardiopatías congénitas. Se presenta el caso clínico de un niño con estridor laríngeo asociado a dificultad respiratoria evaluado en otro centro, donde se realizó endoscopia respiratoria y se observó compresión traqueal extrínseca. Ante la sospecha clínica de anillo vascular, se solicitó angiotomografía computada (angioTC) y se confirmó diagnóstico de sling de arteria pulmonar. Fue derivado a nuestro hospital y durante la intervención quirúrgica se realizó nueva endoscopia respiratoria y traqueobroncografía. Se llegó al diagnóstico de estenosis traqueal congénita con bronquio derecho accesorio (pig bronchus) y anillos vasculares completos, ambos reparados en el mismo acto quirúrgico.

Prenatal Diagnosis of the Right Aortic Arch: Change in Detection Rate, the Status of Associated Anomalies, and Perinatal Outcomes in 137 Fetuses.

To evaluate prenatal findings of the right aortic arch (RAA), associated cardiac, extracardiac, and genetic anomalies, its perinatal outcomes and the need for postnatal interventions in cases of isolated RAA with a view to facilitating appropriate counseling. This was a multicenter, cohort study, that was undertaken in two international major cardiac centers between 2009 and 2020. The study subjects were prenatally diagnosed RAA cases with and without other structural cardiac defects. A RAA was identified in 137 fetuses. There were 84 cases of isolated RAA. Associations with additional intracardiac malformations were found in 53 (38.7%) cases. An extracardiac anomaly was observed in 26/137 (18.9%) fetuses, 11/84 (13.0%) fetuses with isolated RAA, and 15/53 (28.3%) fetuses with an additional intracardiac anomaly. The incidence of extracardiac and chromosomal anomalies was significantly higher in cases of RAA with abnormal intracardiac anatomy (28.3-18.8%, respectively), compared with RAA with normal intracardiac anatomy (13.0-5.9%, respectively) (p < 0.05). 22q11.2 microdeletion was found higher in RAA with CHD (4/18 fetuses) than isolated RAA (2/24 fetuses) (22.2% vs. 8.3% respectively). ALSA was present in 19.3% of cases. ALSA was more frequently observed in cases of isolated RAA (23.6%), than in RAA with structural CHD (7.6%) (p < 0.05). The pregnancy was interrupted in six fetuses, and one died in utero. The mortality rate was higher in fetuses with intracardiac anomaly than RAA without cardiac anomaly (11/49 (22.4%) vs. 2/81 (2.4%). Vascular ring formation was revealed in 21/98 cases. The RAA caused symptoms of a vascular ring in only one patient (0.7%) requiring surgery in the follow-up. Overall survival after initial diagnosis in the total cohort was 85.4% with 38 of 53 (71%) RAA with CHD cases and 79 of 84 (94.0%) isolated RAA cases. Chromosomal and extracardiac anomalies are lower in isolated RAA but not negligible hence amniocentesis should be routinely offered in all cases. The requirement for postnatal intervention in the immediate neonatal period is remote, therefore delivery of these fetuses need not be undertaken at a cardiac or surgical center.

Esophageal cancer with a double aortic arch: a case report and literature review.

BACKGROUND: Double aortic arch (DAA) is an extremely rare vascular malformation, even more so when coexisting with esophageal cancer. METHODS: We report a new case of DAA with esophageal cancer recently seen at our Thoracic Tumor Clinic and review cases of DAA coexisting with esophageal cancer reported in the literature of English language from 2010 to 2020. The purposes of our literature review were to explore how to best achieve radical esophagectomy while reducing postoperative complications. The clinical manifestations, diagnostic method, surgical approach, reconstruction route, and the extent of lymphadenectomy of esophageal cancer with DAA were analyzed in detail. RESULTS AND CONCLUSION: For such patients, 3D computed tomography is necessary for preoperative diagnosis. The surgical approach should consider factors such as the location of the tumor in the esophagus and whether the tumor is surrounded by DAA, as well as the position of the descending aorta and the requirements for the surgical field for lymphadenectomy. If esophageal reconstruction is required, the retrosternal route is preferred. We recommend that only patients with positive results of intraoperative frozen biopsy of recurrent laryngeal nerve lymph nodes should undergo three-field lymphadenectomy, which may be the best method to achieve radical esophagectomy for middle and lower esophageal cancers with DAA while minimizing postoperative complications.

Prenatal diagnosis of right aortic arch and aberrant left subclavian artery in association with bilateral ductus arteriosus by two-and four-dimensional echocardiography: a case of rare vascular ring and review of literature.

The incidence of bilateral ductus arteriosus is sporadically reported in the uterus, and right aortic arch with aberrant of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. We describe a case of right aortic arch and aberrant left subclavian artery associated with bilateral ductus arteriosus. Scanning around the three-vessel and trachea view to search for an "O" shape and a "U" shape vascular ring is essential for the diagnosis, which reveals vascular structures coursing around the trachea. Four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation technique illustrates the spatial relationships of these vessels and the trachea, which should be considered as a complementary modality in fetal cardiac examinations.

Cardiovascular causes of tracheobronchial compression: a decade experience in a Paediatric Congenital Heart Centre.

BACKGROUND: Vascular compression of the airway often complicates CHD management. This study evaluated the use of CT in determining cardiovascular causes, clinical manifestations, and outcome of tracheobronchial compression among children with CHD. METHODS: A retrospective review of clinical records of all patients with CT scan evidence of tracheobronchial compression from January 2007 to December 2017 at National Heart Institute. Cardiovascular causes of tracheobronchial compression were divided into three groups; group I: vascular ring/pulmonary artery sling, II: abnormally enlarged or malposition cardiovascular structure due to CHD, III: post-CHD surgery. RESULTS: Vascular tracheobronchial compression was found in 81 out of 810 (10%) patients who underwent CT scan. Group I lesions were the leading causes of vascular tracheobronchial compression (55.5%), followed by group II (34.6%) and group III (9.9%). The median age of diagnosis in groups I, II, and III were 16.8 months, 3 months, and 15.6 months, respectively. Half of group I patients are manifested with stridor and one-third with recurrent chest infections. Persistent respiratory symptoms, lung atelectasis, or prolonged respiratory support requirement were clues in groups II and III. Higher morbidity and mortality in younger infants with severe obstructive airway symptoms, associated airway abnormalities, and underlying complex cyanotic CHD. CONCLUSIONS: Vascular ring/pulmonary artery sling and abnormally enlarged or malposition cardiovascular structure were the leading causes of cardiovascular airway compression. A high index of suspicion is needed for early detection due to its non-specific presentation. The outcome often depends on the severity of airway obstruction and complexity of cardiac lesions.

Long-term outcomes in children undergoing vascular ring division: a multi-institution experience.

OBJECTIVES: Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division. METHODS: A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed. RESULTS: The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up. CONCLUSIONS: The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.

Surgical treatment of persistent right aortic arch with combined ligamentum arteriosum transection and esophageal diverticulum resection in three dogs.

OBJECTIVE: To describe the treatment of persistent right aortic arch (PRAA) in dogs with combined ligamentum arteriosum (LA) transection and esophageal diverticulum resection. ANIMALS: Three client owned dogs. STUDY DESIGN: Short case series. METHODS: Medical records were reviewed for clinical signs, diagnostic procedures, surgical treatment, post-operative therapies including medications and feeding regime, outcomes, and follow-up imaging. RESULTS: Esophageal resection was performed using a thoracoabdominal (TA) stapler with suture overlay. All dogs recovered well from surgery and did not experience any peri- or post-operative complications. The last follow-up was performed between 64 and 1004 days post-operatively. In all cases, regurgitation resolved and did not recur in any dogs. No dogs required medical therapy or dietary modifications. In two cases, follow-up imaging was performed that revealed marked improvement of esophageal dilation. CONCLUSION: Resection of esophageal diverticulum secondary to PRAA utilizing a TA stapler with suture overlay was technically feasible and did not seem associated with early or late complications.