Minimally invasive surgical technique for the treatment of congenital double aortic arch / 中国胸心血管外科临床杂志

@#Objective    To summarize the treatment results of double aortic arch (DAA) by minimally invasive surgical technique. Methods    We retrospectively analyzed the clinical data of DAA patients who underwent minimally invasive surgeries in our center between October 2016 and August 2021. Results    There were 11 males and 4 females with a mean age of 3-61 (20.00±18.80) years. There were 8 patients of DAA and 7 patients of DAA complicated with distal left-sided aortic arch atresia and ligamentum connection. All patients received operations through minimal subaxillary incision, 13 patients were through left side and 2 patients were through right side. One patient with ventricular septal defect was performed operations concurrently under the cardiopulmonary bypass through right minimal subaxillary incision. All patients had symptom improvement without surgery related complications or death in postoperative period. The duration of operation was 30-192 (61.93±40.19) min and mechanical ventilation time was 2-9 (5.33±2.53) h. The length of ICU stay was 18-124 (51.00±38.07) h and hospital stay time was 8-21 (12.67±3.42) d. All patients had sympto-matic relief with good growth and exercise tolerance during the follow-up of 6 (3, 9) months. Conclusion    Minimally invasive surgical technique is a safe, effective and cosmetic approach with good results for DAA treatment.

Advances in clinical diagnosis and treatment of double aortic arch / 国际儿科学杂志

Double aortic arch is a congenital aortic arch malformation, which is caused by abnormal development of the fourth aortic arches during embryonic development.The patient with double aortic arch has no specific symptoms.It often encircles the trachea and esophagus.In severe cases, it causes dyspnea and dysphagia, and even endangers life.Currently, double aortic arch is diagnosed mainly by imaging techniques, such as echocardiography, computed tomography angiography, magnetic resonance angiography, cardiac catheterization.The main treatment is surgery.Therefore early diagnosis and treatment are very important to avoid tracheomalacia and improve the prognosis.This article will briefly review the research progress of embryonic development mechanism, classification, clinical diagnosis, treatment and prognosis of double aortic arch, aiming to improve the physician′s skills of diagnosis and treatment.

Hemorrhagic shock caused by double aortic arch and aortoesophageal fistula in a newborn: a case report and literature review / 中国新生儿科杂志

Objective:To study the clinical characteristics of double aortic arch (DAA) combined with aortoesophageal fistula (AEF), and summarize the diagnosis and treatment experience.Methods:Retrospective analysis was performed on the diagnosis and treatment of a newborn with hemorrhagic shock caused by DAA combined with AEF in the Guangzhou Women and Children's Medical Center. The key searching words included "double aortic arch", "aortoesophageal fistula", "vascular ring", "newborn or neonate", and "infant, newborn". The relevant reports were retrieved from databases of CNKI, Wanfang, VIP, PubMed, Springer Link, Google Scholar, Web of Science, Embase, Cochrane Library and OVID, to summarize the clinical features, diagnosis and treatment experience of neonates with DAA and AEF. The retrieval deadline was December 31, 2020.Results:A full-term female newborn was hospitalized for dyspnea immediately after birth, and failed to evacuate from the ventilator for several times. The patient was fed with nasogastric tube and transferred to our hospital because of hemorrhagic shock occurring in 32 days after birth, and gastrointestinal bleeding occurred repeatedly with the maximum bleeding volume reaching 200 ml/time. DAA was diagnosed by cardiac ultrasound and CT, AEF hemorrhage was finally confirmed by gastroscopy, aortography and operation. DAA correction and esophagus repair were successfully performed, and the infant recovered well after the operation. At 9-month old, the infant grew and developed well. At present, no reports of DAA combined with AEF neonates have yet to be published in medical literatures in China. Seven English language literatures included 7 cases of AEF complicated with DAA in neonatal period, 5 cases survived and 2 cases died have so far been reported. All patients have a long history of gastric tube indwelling.Conclusions:The incidence of DAA combined with AEF is rare in the newborn with respiratory and swallowing difficulties as the first manifestation. The disease symptoms progressed rapidly, and life-threatening digestive tract hemorrhage may occur, which often requires surgical treatment. Prolonged gastric tube retention should be avoided in DAA children to prevent the occurrence of AEF.

Clinical analysis of surgical correction for congenital vascular ring in children in a single center / 中国胸心血管外科临床杂志

@#Objective    To investigate the best anatomical classification, surgical timing, procedure and clinical outcomes of congenital vascular ring. Methods    The clinical data of 58 patients who underwent congenital vascular ring surgery in Pediatric Surgery Center, Fuwai Hospital between 2014 and 2019 were retrospectively analyzed. There were 32 (55.2%) males and 26 (44.8%) females with a median age of 16.5 (2-73) months. Preoperative symptoms, imaging examinations, anatomical classifications, surgical procedures and postoperative recovery were assessed. Results    There were 20 (34.5%) patients of double aortic arch, 22 (37.9%) patients of right aortic arch with left arterial duct or ligament, 15 (25.9%) patients of left aortic arch with aberrant right subclavian artery, and 1 (1.7%) patient of circumflex aorta with cervical aorta arch. The median ventilator supporting time was 6.0 (0-648) h, and the median hospital stay time was 14.5 (7-104) d. One patient with coarctation of aorta died of severe pulmonary infection during perioperative period, and the others survived without symptoms and reoperation after discharge. The median follow-up time was 7.0 (1-62) months. Conclusion    For children with unexplained dyspnea and dysphagia, or with right aortic arch, preoperative imaging examinations such as computed tomography or magnetic resonance imaging are required to confirm the diagnosis of vascular ring. Surgical correction of congenital vascular ring is safe and reliable, and can effectively relieve symptoms. The mortality rate and reoperation rate are low, and the follow-up results are satisfactory.

Anillos vasculares: Serie de 28 pacientes atendidos en un Servicio de Endoscopía Respiratoria Pediátrica / Vascular rings: Series of 28 patients of a Pediatrie Respiratory Endoscopy Department

Los anillos vasculares tienen una incidencia del 1 % entre las enfermedades cardiovasculares congénitas. Constituyen una malformación embriológica en la que el arco aórtico, sus ramas o las arterias pulmonares provocan presión sobre la tráquea y/o el esófago. Anatómicamente, se dividen en dos grupos, dependiendo de cómo rodean la tráquea y el esófago: completo o incompleto. La sintomatología suele iniciarse en los primeros meses de vida con estridor bifásico o espiratorio que aumenta con el llanto y la alimentación, tos traqueal de tonalidad metálica, infecciones respiratorias recurrentes, episodios de apnea refleja y cianosis, sibilancias, hiperextensión cervical, retracción esternal e intercostal, y dificultad en la alimentación. Se realizó un análisis retrospectivo de 28 pacientes con diagnóstico de anillo vascular que concurrieron a la consulta en el Servicio de Endoscopía Respiratoria del Hospital Garrahan entre enero de 2015 y septiembre de 2017.

Vascular rings account for 1 % of the congenital cardiovascular diseases. They constitute an embryological malformation in which the aortic arch, its branches, or the pulmonary arteries cause pressure on the trachea and/or oesophagus. Anatomically, they are divided into two groups -complete or incomplete- depending on how they surround the trachea and/or the oesophagus. Symptom onset is usually in the first months of life with biphasic or expiratory stridor that increases with crying and feeding, a metallic tracheal cough, recurrent respiratory infections, episodes of apnea and cyanosis, wheezing, cervical hyperextension, sternal and intercostal retraction, and feeding difficulties. A retrospective analysis of 28 patients with vascular rings seen at the Department of Respiratory Endoscopy at Garrahan Paediatric Hospital between January 2015 and September 2017 is presented.

Unrecognized double aortic arch in a preterm infant: Diagnostic challenges and perioperative management

Vascular rings are relatively rare, and are frequently overlooked as a potential cause of neonatal respiratory distress in non specialized centres. This might lead to delay in diagnosis during which the child is subjected to repeated respiratory insults. Procrastination in prompt diagnosis and management can lead to significant comorbidities or even death. We highlight the case report of a preterm infant with an unrecognized double aortic arch who presented with severe respiratory morbidity and the subsequent management.

Prenatal echocardiography in the diagnosis of fetal double aortic arch / 中华超声影像学杂志

To explore the way of prenatal echocardiography in the diagnosis of fetal double aortic arch . Methods T he data of fetuses diagnosed as double aortic arch in 6 prenatal centers in Hunan in echocardiograms performed at 20－36 weeks of gestation from 2013 to 2018 were reviewed . T he characteristics of echocardiographic with double aortic arch , and the associated malformations were observed ,the clinical outcome were analyzed . Results T he main echocardiographic features of the double aortic arch were three‐vessel‐tracheal view s ,which showed a bifurcation of the ascending aorta and a ring consisted of aortic right and left arch . From this retrospective analysis , 29 double aortic arches were identified ,which 8 cases ( 28% ) combined with cardiac defect and extracardiac abnormalities , 1 case with 22q11 deletion . Among them ,5 cases were confirmed by autopsy ,24 cases were diagnosed by computed tomography angiography ( 8 cases were confirmed by operation ) . Conclusions Systematic prenatal echocardiography in the diagnosis of fetal double aortic arch has significant clinical value in the cliagnose of double aortic arch ,w hether it is associated with other malformations and is important for assessing fetal prognosis .

Clinical characteristics and treatment of congenital vascular rings in 54 infants / 中华胸心血管外科杂志

Objective To summarize the clinical characteristics and therapeutic effect of congenital vascular rings in in-fants. Methods The clinical data of 54 children with vascular rings were retrospectively analyzed from August 2011 to June 2017 at our center for surgical treatment. Age 2 days to 11 months,the median age was 1 months;weight 1. 4 - 9. 4 kg,the average weight of(4. 52 ± 1. 89)kg. The type of vascular ring includes 21 cases of double aortic arch,right aortic arch with left aortic duct / ligament with or without vagal left subclavian artery in 14 cases,9 cases of pulmonary artery suture,left aortic arch with right aortic duct / ligament with or without 5 cases of oblique right subclavian artery,3 cases of nameless arterial com-pression syndrome,2 cases of other vascular rings. All cases underwent echocardiography,chest radiography,and CT scan of the heart,and 42 cases were examined before operation or intraoperative bronchoscopy. 39 cases(72％)had tracheal stenosis. Results Twenty-six patients underwent extracorporeal circulation underwent vascular anteroposterior surgery with an average extracorporeal circulation time of( 160 ± 61)min and 28 patients underwent extracorporeal circulation. Postoperative mechani-cal ventilation 1 to 22 days,the median number of days 2 days;hospitalization time 7 to 62 days,the median of 19 days,hos-pital death in 2 cases(3. 7％). Discharge patients were followed up for 2 to 74 months,respiratory symptoms to varying degrees to ease or disappear,discharged in 3 cases. Conclusion Airway stenosis is a serious complication of the vascular ring. CT is the best way to determine the vascular ring. To avoid serious airway complications,the vascular ring should be operated as soon as possible,and the airway stenosis and intracardiac malformation Children suggested concurrent treatment,this can improve the survival rate of surgery and improve the prognosis of children.

Sugical repair of double aortic arch associated with complex cardiac anomalies / 中华胸心血管外科杂志

Objective To evaluate the clinical characteristics,diagnosis and surgical repair methods of double aortic arch (DAA) associated with complex cardiac anomalies.Methods Retrospectively analyzed the clinical data of the 5 DAA associated with complex cardiac anomalies patients,4 males and 1 female,age from 41 days to 19 years old,weight 4.3-56.0 kg.Accompanied cardiac malformites including 2 cases with TOF,2 cases with DORV,and 1 case with d-TGA.4 cases were diagnosed DAA via MSCT and were surgical treated combined with cardiac malformations at same stage.1 case of DAA associated with d-TGA experienced stubborn pulmonary infection with increased airway resistance and could not tolerate ventilator weaning after aterial switch operation.Then he was diagnosed DAA with left arch atresia through MSCT.So,the patient underwent another surgery to excise the left aortic arch.Results One case worsened postoperatively by the cause of vomiting and aspiration and then died.The other four were survival and discharged with no DAA repair relating complication.Conclusion Double aortic arch can be cured by surgical repair and DAA with cardiac defects can be operated in one stage via median sternotomy.Congenital vascular ring should be considered in patients with stubborn and unexplained respiratory symptoms,who should receive timely imaging examinations for accurate diagnosis and surgical treatment at the early stage.

Double aortic arch as a source of airway obstruction in a child.

Double aortic arch (DAA) is a congenital vascular anomaly. The diagnosis was difficult till the child was symptomatic, and other causes were ruled out. We present the interesting images of a child of respiratory distress because of tracheal compression from DAA.

Clinical and Imaging Findings of Symptomatic Right Aortic Arch and Double Aortic Arch: Case Report.

Aim: Anomalies of aortic arch are uncommon, accounting for only 1-3% of all congenital cardiac diseases. A vascular ring results from the abnormal development of an aortic arch complex. Generally, it manifests with tracheoesophageal compression symptoms during infancy. We aimed to discuss clinical and imaging findings of two cases of vascular ring based on existing literature. Cases: This article presents two cases of a 4-year-old child diagnosed with right sided aortic arch and a new-born child diagnosed with double aortic arch in neonatal unit. The first patient presented with complaints of coughing, wheezing and shortness of breath, the second patient suffered from respiratory distress. In the both cases, diagnosis were established using multi-detector computed tomography (MDCT). Both patients were diagnosed with vascular ring, considering their existing findings, and referred for surgery. Discussion: Apparent airway obstructions lead to critical complaints at early ages. Moderately severe symptoms and the non-existence of anatomic compression may cause resulting diseases to be diagnosed in a later period of life. A vascular ring can be diagnosed using chest radiography, barium oesophagus graphy, transthoracic echocardiography, MDCT, magnetic resonance imaging and angiography. However, pathologies of the studied cases (right aortic arch, double aortic arch, and tracheal compression) are effectively observed with MDCT and using the post-processing images of MDCT. Conclusion: A vascular ring should be considered in the event of recurrent lung infections, unexplained coughs, wheezing and stridor during childhood as well as in the case of unexplained respiratory distress in new-born children. MDCT is an excellent modality for patients suspected of having a vascular ring.

Multi-Detector Row Computed Tomographic Evaluation of a Rare Type of Complete Vascular Ring: Double Aortic Arch with Atretic Left Arch Distal to the Origin of Left Subclavian Artery

Double aortic arch with an atretic left arch distal to the origin of left subclavian artery was diagnosed with multi-detector row computed tomography (MDCT) in two children with dysphagia. This rare type of complete vascular ring is clinically important because it may be confused with right aortic arch in mirror imaging. Anatomic details of this rare type of complete vascular ring demonstrated on MDCT facilitated appropriate surgical treatment.

Una causa poco usual de sintomas respiratorios persistentes: Anillo vascular tipo doble arco aórtico, reporte de caso

Los anomalías congénitas del arco pórtico pueden presentar múltiples manifestaciones clínicas y causar síntomas respiratorios persistentes y de difícil control, a edades muy tempranas de la vida, su asociación con otros defectos congénitos no es infrecuente, principalmente, principalmente cardíacos. Representa un reto para el clínico, sospechar el diagnóstico, corroborarlo y además conocer el mejor abordaje imagenológico para su evaluación ; ya que a pesar de la morbilidad de la patología, la detección temprana de un defecto importante aunado a la resolución quirúrgica oportuna, mejora grandemente la sobrevida del paciente. Presentamos un caso peculiar, el primero reportado como variante Doble Arco Aórtico en Panamá, de un producto gemela que presentó síntomas respiratorios de difícil manejo , y que a través del uso de diferentes técnicas diagnósticas y a la corrección quirúrgica temprana, pudo mejorarse su calidad de vida, con escasos síntomas residuales.

Congenital anomalies of the aortic arch may multiple clinical manifestations and cause persistent respiratory symptoms and difficult to manage even at very early ages of life, its association with other birth defects are not uncommon, especially cardiac defects. It could be a challenge for the clinician to suspect the diagnosis and know the best imaging approach; because the morbidity of the disease, early detection of major anomaly coupled with timely surgical resolution, greatly improves patient survival. We preset a peculiar case, the first variant reported to double aortic arch in Panama, of a twin that showed early respiratory symptoms which were difficult to manage and through the use of several imaging techniques and surgical correction , we could improve her quality of life with just few residual symptoms.

A Case of Balanced Type Double Aortic Arch Diagnosed Incidentally by Transthoracic Echocardiography in an Asymptomatic Adult Patient

A 36-year-old male patient with no remarkable medical history was admitted to our hospital for a health check up. On chest radiography, bilateral aortic notches at the level of aortic arch were shown suggesting aortic arch anomaly without any clinical symptoms. Two aortic arches were almost same-in-size on suprasternal view of transthoracic echocardiography. In addition, multidetector computed tomography showed balanced type double aortic arch forming a complete vascular ring which encircled the trachea and esophagus. The trachea was slightly compressed by the vascular ring whereas the esophagus was intact. Nevertheless, the pulmonary function test was normal. The patient was discharged from hospital with instructions for periodic follow-up.

The role of echocardiography in diagnosis and management of double aortic arch / 中华超声影像学杂志

Objective To evaluate the role of echocardiography in diagnosis and post-operative evaluation of infants and children with double aortic arch. Methods Echocardiography was performed in 6 patients with double aortic arch from January 2006 to June 2008. The outcome of postoperative follow-up was also evaluated. Results Six patients ranged in age from 3 months to 4 years and averaged 18 months. There were 2 boys and 4 girls. Echocardiography diagnosed double aortic arch in 4 patients, one balanced arch and 3 right arch dominant, and was confirmed by surgical observation in each patient. The size of the two arches were almost same in balanced arch,and the diameter of right arch was bigger than that of left arch in right arch dominant. Echocardiography failed to diagnose in 2 patients with balanced arch. Three patients had associated heart defect, such as patent ductus arteriosus and ventricular septal defect. Five of the 6 patients underwent surgical division of the left arch in order to relieve esophageal and tracheal compression. At about 2 years postoperative follow-up,echocardiography can clearly show the flow of aortic arch and it's branches. Conclusions It is an important clue to double aortic arch if only two branches of aortic arch was shown on supersternal view by echocardiography. As a non-invasive techniqe, echocardiography is useful for diagnosing and post-operative evaluating of patients with double aortic arch.

Congenital Cardiopulmonary Anomalies in Infants with Recurrent Stridor and/or Respiratory Distress: Report of 3 Cases / 소아알레르기및호흡기학회지

Although laryngomalacia is the most common cause of congenital stridor in infancy, it is a benign, self-limited disease which usually resolves by the age of 18 months with no long- term sequelae. Nevertheless, infants who suffered from recurrent stridor and respiratory distress should be evaluated for other causes of stridor such as cardiopulmonary anomalies. We report 3 cases of infants who had recurrent stridor and respiratory distress from their early infancy. Case 1 had a double aortic arch and a tracheomalacia, case 2 had a hypoplasia of the right lung, and case 3 had a horseshoe lung as well as scimitar syndrome. Physicians should be alert for the possibility of the congenital cardiopulmonary abnormalities in infants with recurrent stridor and/or respiratory distress.

Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations

PURPOSE: The clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases. MATERIALS AND METHODS: From July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n=28), hemitruncus arteriosus (HTA) (n=3), type I truncus arteriosus (TA) (n=4), and the aortopulmonary window (APW) (n=3), were comparatively reviewed. RESULTS: All 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnositic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology. CONCLUSION: In case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

Anillo vascular por doble arco aórtico simétrico. Reporte de un caso / Double symmetrical aortic arch. Case report

Describimos un paciente de 6 meses de edad con doble arco aórtico simétrico y sintomatología obstructiva traqueal y esofágica, tratado quirúrgicamente. Se realizó una correlación entre las imágenes diagnósticas obtenidas con esofagograma, ecococardiografía y angiografía con un espécimen anatómico, dando énfasis a la utilidad del ecocardiograma como estudio inicial.

We report a six-month-old patient with a double symmetrical aortic arch with tracheal and esophageal obstructive symptoms, who was treated surgically. The diagnostic images consisting of esophagogram, echocardiography and angiography were correlated with an anatomical specimen; the usefulness of the echocardiogram as an initial test is emphasized. (Arch Cardiol Mex 2005; 75: 178-181).

An Autopsy Case of Double Aortic Arch

Double aortic arch is the most common type of symptomatic vascular ring. In most patients, the symptoms are manifested at birth or in early infancy. Double aortic arch usually has more severe symptoms than other types of complete vascular rings. We experienced one case of complete duplicated double aortic arch with left descending aorta and left patent ductus arteriosus. A one-day-old female neonate was transferred to our hospital because of mild dyspnea and stridor. She showed intractable CO2 retention and respiratory difficulty with time in spite of ventilator therapy. She died of respiratory failure 23 hours after birth. On autopsy, we found that the diameter of the vascular ring was 0.7cm in maximum extent. It was ovoid and contained trachea and esophagus. The trachea was flattened due to allowing 0.1x0.2cm. The compression level of the trachea was approximately 1cm from the tracheal bifurcation.