Serie de casos de arco aórtico derecho: aproximación diagnóstica prenatal / Case series of right aortic arch: prenatal diagnostic approach

RESUMEN El arco aórtico derecho es producto de la involución anormal de los arcos vasculares embrionarios. En los últimos años, el diagnóstico fetal se ha hecho más frecuente con la utilización de la ultrasonografía rutinaria del corazón y los grandes vasos. El hallazgo de un arco aórtico derecho involucra muchos aspectos que pueden afectar el pronóstico del feto; por ello, el estudio exhaustivo debe ser sistematizado. A continuación, presentamos una serie de seis casos de diagnóstico prenatal y sugerimos un algoritmo de evaluación.

ABSTRACT The right aortic arch is a product of abnormal involution of the embryonic vascular arches. In recent years, fetal diagnosis has become more frequent with the use of routine ultrasonography of the heart and great vessels. The finding of a right aortic arch involves many aspects that may affect the prognosis of the fetus; therefore, the exhaustive study must be systematized. Below, we present a series of six cases of prenatal diagnosis and suggest an evaluation algorithm.

Prenatal ultrasound diagnosis of fetal right aortic arch / 中国医师进修杂志

Objective:To explore the prenatal ultrasound manifestations of fetal congenital right aortic arch and its diagnostic significance.Methods:The characteristics of prenatal ultrasound images (focusing on the three vessel trachea section) and clinical data of 128 cases of fetal congenital right aortic arch diagnosed in Dalian Women and Children′s Medical Center(Group) from January 2014 to January 2019 were analyzed retrospectively.Results:Among 128 cases of fetal congenital right aortic arch, 111 cases were right aortic arch and leftductus arteriosus with aberrant left subclavian artery (RAA-LDA-ALSA), 1 case was diagnosed as left aortic arch atresia of double aortic arch by operation after birth; 9 cases were right aortic arch and right ductus arteriosus (RAA-RDA); 6 cases were mirror-image right active aortic arch and left ductus arteriosus connected to descending aorta (RAA-BAMB-LDA-DAO); 2 cases were mirror-image right aortic arch and left ductus arteriosus connected to the left innominate artery (RAA-BAMB-LDA-LINA). The three vessels and trachea view (3VT) had characteristic sonographic features.If necessary, coronary section of the upper thoracic aorta and / or stic were added - HD live flow technology assisted the diagnosis of 3vt section.Conclusions:Three vessel trachea view is a sensitive and effective view for prenatal detection and diagnosis of congenital right aortic arch. Upper thoracic aorta coronal section and combined stic - HD live flow technology can make up for its shortcomings. Prenatal ultrasound diagnosis of right aortic arch hasguiding significance for prenatal prognosis consultation.

Diagnóstico prenatal de arco aórtico derecho fetal con anillo vascular / Prenatal diagnosis of fetal right aortic arch with a vascular ring

Resumen Las anomalías del arco aórtico se refieren a malformaciones congénitas de la posición o patrón de ramificación. Se pueden detectar mediante la visualización dirigida del corte de los tres vasos o de tres vasos y tráquea, con o sin Doppler de color. El diagnóstico prenatal debe conllevar un examen detallado de ultrasonido cardiaco y extra-cardiaco. La identificación de un arco aórtico derecho aislado es crucial para distinguir esta afección del arco aórtico doble. Se describe un caso de diagnóstico prenatal de arco aórtico derecho fetal con anillo vascular a las 29 semanas de gestación, quien fue referido por posible anomalía cardíaca en la ecografía de rutina. El ultrasonido demostró posición anormal de la aorta descendente, situada en el lado derecho de la columna vertebral en el plano tetracameral. La aorta se conectaba con la arteria pulmonar izquierda a través del ductus arterioso, configurando un anillo vascular. La detección prenatal de esta anomalía congénita vascular, ya sea de forma aislada o en asociación con otras malformaciones cardiacas o no cardiacas, podría conducir a un asesoramiento más adecuado.

Abstract Aortic arch anomalies refers to congenital malformations of the position or branching pattern. They can be detected by targeted visualization of the three-vessel slice or three-vessel and trachea, with or without color Doppler. Prenatal diagnosis should involve a detailed cardiac and extra-cardiac ultrasound examination. The identification of an isolated right aortic arch is crucial to distinguish this condition from a double aortic arch. We describe a case of prenatal diagnosis of fetal right aortic arch with vascular ring at 29 weeks gestation who was referred for possible cardiac anomaly on routine ultrasound. Sonography demonstrated abnormal position of the descending aorta, located on the right side of the spine in the tetra chamber plane. The aorta was connected to the left pulmonary artery through the ductus arteriosus, configuring a vascular ring. Prenatal detection of this congenital vascular anomaly, either in isolation or in association with other cardiac or noncardiac malformations, could lead to more appropriate counseling.

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.

A Case of a Super-Elderly Patient Who Underwent Total Arch Replacement Using the Frozen Elephant Trunk Technique for a Thoracic Aortic Aneurysm with a Right-Sided Aortic Arch / 日本心臓血管外科学会雑誌

The case is that of a 90-years-old man. A previous doctor performed abdominal graft replacement for an abdominal aortic aneurysm 5 years earlier and continued outpatient CT follow-up. Follow-up CT showed the right aortic arch and dilation of the thoracic aortic aneurysm, and the patient was referred to our hospital. Contrast-enhanced CT showed an aortic arch aneurysm ; the aneurysm diameter was 62 mm in major axis and 60 mm in minor axis, which was judged to be suitable for surgery. It was a rare right-sided aortic arch with no congenital heart malformation and no situs inversus. Endovascular treatment was considered because he was 90 years old and very elderly, but there were concerns about the risk of embolism, irregular manipulation and central landing. For the surgical method, we selected total arch replacement using a frozen elephant trunk technique. We succeeded in avoiding serious complications by selecting an appropriate treatment method through careful evaluation.

A Case Report after Two Years of Total Debranching and Endovascular Repair for Kommerell Diverticulum / 日本心臓血管外科学会雑誌

A 78-year-old woman with abnormal shadows on computed tomography (CT) was given a diagnosis of right-sided aortic arch and Kommerell diverticulum (KD), accompanied by aberrant left subclavian artery. Although no symptoms were observed, the maximum diameter of the aneurysm was 63 mm, and surgical intervention was chosen because of the possibility of rupture. At first, a 4-branched blood vessel prosthesis with a side branch was anastomosed to the ascending aorta. Next, after reconstructing the cervical branches, a Conformable GORE® TAG® (W.L. Gore and Associates, 34 mm×200 mm) was inserted from the side branch and expanded in the range of Zones 0 to Th 7. Finally, ALSA coil embolization was performed. She was discharged on postoperative day 36, and at her 2-year follow-up, she was doing well, with shrinkage of Kommerell diverticulum.

Endovascular treatment to cerebral infarction combined with right aortic arch and Kommerell's diverticulum: case report / 中国医师杂志

Objective To investigate the effect of endovascular treatment of cerebral infarction with right aortic arch and Kommerell's diverticulum.Methods Retrospective analysis was done to assess the treatment effect of a case from vascular surgery,Xuanwu Hospital of Capital Medical University.This case was diagnosed as cerebral infarction with right aortic arch and Kommerell's diverticulum.Results One month after the treatment of cerebral infarction,we successfully used the thoracic aortic stent to isolate the Kommerell's diverticulum.There were no operative complications occurred.The stent had good shape and smooth blood flow was seen in the stent.Conclusions Endovascular treatment to cerebral infarction with right aortic arch and Kommerell's diverticulum is safe and feasible,and the clinical outcome requires further long-term follow-up.

Endovascular treatment to cerebral infarction combined with right aortic arch and Kommerell 's diverticulum: case report / 中国医师杂志

Objective@#To investigate the effect of endovascular treatment of cerebral infarction with right aortic arch and Kommerell's diverticulum.@*Methods@#Retrospective analysis was done to assess the treatment effect of a case from vascular surgery, Xuanwu Hospital of Capital Medical University. This case was diagnosed as cerebral infarction with right aortic arch and Kommerell's diverticulum.@*Results@#One month after the treatment of cerebral infarction, we successfully used the thoracic aortic stent to isolate the Kommerell's diverticulum. There were no operative complications occurred. The stent had good shape and smooth blood flow was seen in the stent.@*Conclusions@#Endovascular treatment to cerebral infarction with right aortic arch and Kommerell's diverticulum is safe and feasible, and the clinical outcome requires further long-term follow-up.

Hybrid Repair in a Patient with Kommerell Diverticulum and Right Aortic Arch / 日本心臓血管外科学会雑誌

We experienced a hybrid repair for Kommerell diverticulum and right aortic arch. A 62-year-old man with dyspnea and dysphagia was referred to our hospital. He underwent atrium septum defect closure when he was 15 years old and was found to have ventricular septal defect and severe aortic regurgitation at the referring hospital. Preoperative computed tomography incidentally showed right aortic arch and Kommerell diverticulum with aberrant left subclavian artery. First, we performed ventricular septal defect closure and aortic valve replacement. Three months later, we performed one-stage hybrid repair of Kommerell diverticulum that included left common carotid-subclavian artery bypass, left subclavian artery plug occlusion and descending aortic replacement via a right thoracotomy. This hybrid strategy did not require in-situ reconstruction of the aberrant subclavian artery and minimized the risk of bleeding, injuries of esophagus and recurrent laryngeal nerve. The postoperative course was uneventful. This hybrid repair is a safe and effective procedure for Kommerell diverticulum with aberrant subclavian artery.

Alterações congênitas do coração e dos grandes vasos em cães / Congenital abnormalities of the heart and large vessels of dogs

As alterações congênitas do coração e dos grandes vasos (ACCGV) são definidas como defeitos morfológicos associados ao nascimento e são as anomalias congênitas mais frequentes dos animais domésticos. O objetivo deste trabalho foi determinar a frequência de diagnósticos de ACCGV em cães no Rio Grande do Sul, com ênfase na Região Metropolitana de Porto Alegre, no período de janeiro de 2000 a dezembro de 2016. Do total de 7.903 necropsias de cães, 27 morreram espontaneamente ou foram submetidos à eutanásia devido às ACCGV, representando 0,3% dos casos. Em 11,1% dos cães apresentaram dois distúrbios congênitos no coração ou nos grandes vasos, totalizando 30 alterações. A idade variou de um dia a 12 anos, com a idade mediana de quatro meses. Em 81,5% acometeu cães com raça, e 18,5% sem raça definida. Em relação ao sexo, 51,8% dos cães eram machos e 48,2%, fêmeas. Estenose subaórtica foi a alteração mais frequente, seguido por defeito do septo atrial, persistência do arco aórtico direito, persistência do ducto arterioso, estenose pulmonar e defeito do septo interventricular, e fibroelastose endocárdica. Dos casos múltiplos, as combinações encontradas foram: persistência do ducto arterioso associado com defeito do septo atrial, estenose subaórtica com defeito do septo interventricular, e defeito do septo atrial e ventricular.(AU)

Congenital alterations of the heart and large vessels (CAHLV) are defined as morphological defects associated with birth and are the most frequent congenital anomalies of domestic animals. The aim of this study was to determine the frequency of CAHLV in dogs in Rio Grande do Sul, with emphasis in the Metropolitan Region of Porto Alegre, from January 2000 to December 2016. Of the 7,903 necropsied dogs, 27 died spontaneously or were submitted to euthanasia due to CAHLV, representing 0.3% of the cases. In 11.1% of the dogs, there were two congenital disorders in the heart or in the great vessels, totaling 30 changes. The age of affected dogs ranged from one day to 12 years, with the median age of four months. In 81.5% it affected dogs with breed, and 18.5%, without breed defined. In 51.8% were males, and 48.2%, females. Subaortic stenosis was the most frequent alteration, followed by atrial septal defect, persistent right aortic arch, patent ductus arteriosus, pulmonic stenosis, ventricular septal defect, and endocardial fibroelastosis. Of the multiple cases, the combinations found were: patent ductus arteriosus associated with atrial septal defect, subaortic stenosis with ventricular septal defect, and atrial and ventricular septal defect.(AU)

Prenatal echocardiographic diagnosis of right aortic arch and its branch type / 中华超声影像学杂志

Objective To investigate the value of prenatal echocardiographic diagnosis of anomalies of the right aortic arch ( RAA ) and its branch type ,and provide rapid diagnostic methods . Methods A retrospective analysis of 55 cases of RAA of prenatal ultrasound diagnosis results ,and their imaging characteristics were summarized . Results There were 55 cases of anomalies of the RAA ,included 42 cases of RAA with aberrant left subclavian artery ( RAA-ALSA ) and 13 cases of RAA with mirror image branching pattern( RAA-MB) ,1 case of double aortic arch(DAA) was misdiagnosed as RAA . The prenatal echocardiographic characteristics of RAA-ALSA :in three-vessel-aortic arch view ,the first branch of the aortic arch was the left carotid artery ,which ran in front of the trachea ,and ALSA ran behind the trachea and esophagus . The prenatal echocardiographic characteristics of RAA-MB :the first branch of the aortic arch was the brachiocephalic trunk ,which ran in front of the trachea and was divided to the left carotid artery and the left subclavian artery . Conclusions Prenatal ultrasound can qunickly and accurately diagnose anomalies of the right aortic arch and its branch type according to the direction ,thickness and secondary branches of the first branch of the aortic arch . The location of arterial duct is helpful to judge the formation of vascular ring .

Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular / Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.

Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

Right aortic arch with aberrant left innominate artery arising from Kommerell's diverticulum / Arco aórtico à direita associado a artéria inominada esquerda aberrante originada de divertículo de Kommerell

Abstract We report a case of an uncommon thoracic aorta anomaly-right aortic arch with aberrant left innominate artery arising from Kommerell's diverticulum-that went undiagnosed until adulthood.

Resumo É apresentado o caso de uma rara anomalia da aorta torácica - arco aórtico à direita associado a artéria inominada esquerda aberrante com origem em divertículo de Kommerell -, com diagnóstico apenas na vida adulta.

Prenatal diagnosis of isolated mirror-image right aortic arch with vascular ring by fetal echocardiography / 中华超声影像学杂志

Objective To improve the prenatal echocardiographic diagnostic accuracy for fetus with isolated mirror-image right aortic arch which combined with vascular ring by analyzing and accumulating its echocardiographic features.Methods Echocardiographic signs were analyzed retrospectively and fetal echocardiographic features were accumulated in 16 cases with prenatal diagnosis of isolated mirror-image right aortic arch with left-side ductus arteriosus which formed vascular ring between January 2014 and February 2016.All cases were confirmed by neonatal echocardiography.Results The fetal echocardiography characters:① In 3-vessel and trachea view,fetal right aortic arch was demonstrated a Ushaped appearance of the great vessels,the right arm of the ‘ U’ represented the aortic arch and its left branch with ductal continuation.② In 3-vessel and trachea view or upper axial mediastinal transverse view,left innominate artery passed straightly to the left of the trachea.Left innominate artery and right-side ductus arteriosus were overlapped and the distance of two vessels was very close.③ In double arch view and longitudinal view for ductus,Left innominate artery did not connect to descending aorta or ductus arteriosus and did not involve in the formation of vascular ring.④ Color Doppler played an important role in diagnosis of this disease.Power Doppler or high-definition imaging easily showed color overflow and wrongly displayed the crossed relationship of left innominate artery with descending aorta as the anatomical connection,and caused the misdiagnosis of this disease as double aortic arch.Conclusions Isolated mirrorimage right aortic arch with vascular ring is not rare in the fetal period.Because ductus arteriosus is large and patent during the prenatal period,it is easily mistaken for the crossed relationship of left innominate artery with descending aorta as the anatomical connection and finally misdiagnoses this anomaly as double aortic arch.The keys to accurate diagnosis of the disease are clearly visualization of the innominate artery and its relationship with descending aorta and ductus arteriosus by scanning different views.

Persistent left cranial vena cava with congenital heart defect in two dogs

The purpose of this report is to introduce persistent left cranial vena cava (PLCVC) with persistent right aortic arch (PRAA) and patent ductus arteriosus (PDA). Case 1 was a Cocker Spaniel with PRAA and case 2 was a Maltese with PDA. PLCVC was enclosed at the sites of PRAA and PDA surgery; therefore, it was lifted dorsally during PDA and PRAA surgery. Surgery to repair congenital heart defects including PRAA and PDA is recommended for dogs that do not die of PLCVC at a young age.

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.

Debranching and Endovascular Repair for Kommerell's Diverticulum Involving Right-Sided Aortic Arch with Mirror Image Branching / 日本心臓血管外科学会雑誌

A 71-year-old man with an abnormal shadow on chest x-ray was given a diagnosis of Kommerell's diverticulum involving the right-sided aortic arch with mirror image branching. Furthermore, mild funnel chest had been seen on CT scan more than 10 years earlier. The patient was followed up because there were no symptoms ; the Kommerell's diverticulum expanded to reach 63 mm in diameter. To eliminate the risk of rupture, we performed thoracic endovascular aortic repair (TEVAR) with a commercially available device, consisting of bypass grafting of the supra-aortic branches. The patient was discharged from the hospital in good clinical condition, with no signs of endoleak and currently shows no indications of device migration. We thus concluded that debranching TEVAR for Kommerell's diverticulum with right-sided aortic arch is minimally invasive, safe, and effective. Availability of this device that has a new performance feature is expected to improve treatment results and lead to advances in minimally invasive endovascular repair.

Endovascular Repair with a Fenestrated Stent Graft for Kommerell's Diverticulum with a Right Aortic Arch / 日本心臓血管外科学会雑誌

A 77-year-old man with an abnormal shadow on chest x-ray film, was found to have Kommerell's diverticulum associated with a right aortic arch 2 years previously. During the period of follow-up, the Kommerell's diverticulum was expanded to 5.3 cm in diameter. CT revealed a right aortic arch with mirror-image branching and Kommerell's diverticulum. To eliminate the risk of rupture, the Kommerell's diverticulum was excluded by deployment of a handmade fenestrated stent-graft using the brachial wire traction technique via the right femoral artery and left brachial artery. At 30-months of follow-up the patient is doing well, with no signs of endoleak or migration. Endovascular repair of Kommerell's diverticulum with a right aortic arch is feasible, safe and effective. This is a rare case of a right aortic arch with Kommerell's diverticulum and without left aberrant subclavian artery.

Surgical Repair of an Aneurysm of the Right Aortic Arch with a Retroesophageal Aortic Segment and Mirror-image Branching / 日本心臓血管外科学会雑誌

A 79-year old man presented with hoarseness and we diagnosed an aortic arch aneurysm, 60 mm in diameter. The aortic arch was right-sided and traversed posterior to the esophagus and trachea, and the arch vessels were mirror-imaged. Total arch replacement was performed under hypothermic circulation arrest using selective cerebral perfusion through a median sternotomy. Three cervical vessels were reconstructed, a 24-mm Hemashield was passed anterior to the trachea and esophagus, and an additional right thoracotomy was not necessary. The patient was uneventfully discharged on the 26th postoperative day.

Ventilatory compromise in an infant with Tetralagy of fallot and right aortic arch after insertion of transesophageal echocardiography probe: A case report / 대한마취과학회지

Right aortic arch, a congenital malformation of the great vessels, can cause compression of trachea and/or esophagus. We experienced a case of ventilatory compromise in an infant with tetralogy of Fallot and right aortic arch immediately after insertion of transesophageal echocardiography probe during cardiac surgery. Although intraoperative transesophageal echocardiography can be safely performed in infants with congenital heart disease, it should be done with caution in patient with similar vascular malformation.