Congenital Aortic Arch Anomalies Research Articles

Accuracy and image quality of wide-detector revolution CT angiography combined with prospective ECG-triggered CT angiography in the diagnosis of congenital aortic arch anomalies in Chinese children.

To explore the accuracy and image quality of wide-detector revolution CT angiography combined with prospective ECG-triggered CT angiography in the diagnosis of congenital aortic arch anomalies in Chinese children. From January 2020 to July 2022, the clinical data of 57 children with congenital aortic arch anomalies confirmed by surgery were collected. All patients underwent CT angiography (CTA) with Revolution CT and transthoracic echocardiography (TTE) before the operation. The accuracy of CTA and TTE in the diagnosis of aortic arch anomalies was compared with the surgical results. The diagnostic sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of CTA and TTE for congenital aortic arch anomalies (including intracardiac and extracardiac structural abnormalities) were 92.2% and 85.5%, 99.4%, and 99.1%, 97.4% and 95.6%, 98.1% and 96.9%, and 97.2% and 95.2%, respectively. Regarding extracardiac structural malformations, the sensitivity of CTA was 100%, whereas that of TTE was 78.6% (P < 0.001). Regarding intracardiac structural malformations, the sensitivity of CTA was 84.5%, whereas that of TTE was 92.5% (P < 0.001). Regarding satisfaction with the images in aortic arch anomalies, surgeons noted that the CTA images were more useful for diagnosis and operation planning compared with TTE. Wide-detector revolution CT angiography combined with prospective ECG triggering can be routinely used to assess congenital aortic arch anomalies, providing adequate image quality and high diagnostic accuracy. However, limitations in the identification of intracardiac structural abnormalities are noted.

S2630 Massive Hematemesis: A Case of Esophageal-Subclavian Fistula

Introduction: Esophageal-subclavian fistulae are rare and are most commonly seen as a complication of congenital aortic arch anomalies. We report a case of an esophageal-subclavian fistula in a patient with a right aortic arch with an aberrant left subclavian artery (ALSA), an uncommon anatomical variant that occurs in approximately 1% of the general population. Case Description/Methods: A 76-year-old man with atrial fibrillation, prior pulmonary embolism, and hyperthyroidism with a multinodular goiter presented to an outside hospital (OSH) after being found down at home. He was intubated for acute hypoxic respiratory failure at the OSH, and transferred to our center for resection of the multinodular goiter causing airway compression. His postoperative course was complicated by multiple infections and prolonged intubation. CT imaging incidentally noted a four vessel aortic arch with retroesophageal ALSA. Two months into the hospitalization, he had large volume hematemesis and subsequent cardiac arrest requiring multiple rounds of resuscitation and Blakemore tube placement into the esophagus. Upon deflation of the Blakemore, he coded again and was started on massive transfusion protocol before successfully deflating the device. Emergent endoscopy was notable for clotted blood in the stomach and active, pulsatile bleeding of the proximal esophagus (25 cm), concerning for arterio-esophageal fistula. Thoracic surgery, cardiac surgery, and vascular surgery were consulted; however, given overall poor prognosis and high risk with invasive surgery, no operative intervention was offered. He was ultimately transitioned to comfort care (Figure 1). Discussion: While arterial-esophageal fistulae are rare, it is important to recognize these cases early as they can lead to fatal hemorrhage if not surgically intervened upon. In the case of ALSA, the vessel takes a retroesophageal course and often forms a dilated segment at the proximal end, called a diverticulum of Kommerell, which compresses the esophagus posteriorly. The vascular pressure on the esophagus can ultimately create a fistula. Prolonged use of indwelling devices, such as nasogastric tubes (NGT) and endotracheal tubes (ETT), may create pressure necrosis and erosion of the posterior esophageal wall, thus contributing to fistula formation. While there are currently no guidelines for screening for aortic arch abnormalities, it has been suggested that those with aortic arch abnormalities noted on CT imaging may benefit from avoidance of prolonged use of NGT or ETT.Figure 1.: Endoscopy image showing upper third of the esophagus with profuse, active bleeding.

Congenital Anomalies of Aortic Arch: A Pictorial Essay.

Aortic abnormalities account for 15 to 20% of all congenital cardiovascular diseases. The purpose of this pictorial essay is to illustrate various congenital anomalies of the aortic arch, which influence patient management and outcome.

A Case of Right Subclavian Artery Agenesis.

The authors present a 12-year-old male with an asymptomatic absence of the proximal right subclavian artery. On physical examination, his right brachial, radial, and ulnar pulses were diminished compared with the left side. Computed tomographic angiography revealed that the right subclavian artery was supplied from the anterior right internal mammary artery. A description of the acquired and congenital aortic arch anomalies and thoracic outlet syndrome differential diagnoses is provided.

Vascular rings

Vascular rings are congenital aortic arch anomalies that lead to compression of the trachea or esophagus. The goal of this review is to summarize our current recommendations for the management of patients with a diagnosis of a vascular ring. We review the history, classification methods, and epidemiology of the various types of vascular rings. We then propose a management strategy for the relatively new paradigm of fetal diagnosis, including the management of asymptomatic vascular rings. Finally, we finish with a review of the operative techniques and outcomes for the four main categories of vascular rings.

Aortic arch anomalies, embryology and their relevance in neuro-interventional surgery and stroke: A review.

Congenital aortic arch anomalies are commonly encountered during neurointerventional procedures. While some anomalies are identified at an early age, many are incidentally discovered later in adulthood during endovascular evaluations or interventions. Proper understanding of the normal arch anatomy and its variants is pivotal to safely navigate normal aortic arch branches and to negotiate the catheter through anomalies during neurointerventional procedures. This is particularly relevant in the increasingly "transradial first" culture of neurointerventional surgery. Moreover, some of these anomalies have a peculiar predilection for complications including aneurysm formation, dissection, and rupture during the procedure. Therefore, an understanding of these anomalies, their underlying embryological basis and associations, and pattern of circulation will help endovascular neurosurgeons and interventional radiologists navigate with confidence and consider relevant pathologic associations that may inform risk of cerebrovascular disease. Here, we present a brief review of the basic embryology of the common anomalies of the aortic arch along with their neurological significances and discuss, through illustrative cases, the association of aortic arch anomalies with cerebral vascular pathology. Understanding the aortic arch anomalies and its embryological basis is essential to safely navigate the cerebral vascular system during neurointerventional surgeries.

Anomalous subaortic left brachiocephalic vein: Prevalence and associated anomalies

Introduction: Thoracic venous anomalies are very rare with an estimated prevalence of 0.7% in the general population. One of the rarest such anomaly is a subaortic course of the left brachiocephalic vein (LBV) commonly associated with congenital cardiac or aortic arch anomalies. Material and Methods: We retrospectively analyzed chest computed tomography scans of 710 participants and found incidentally two cases of anomalous subaortic course of the LBV in two females. Results: The observed prevalence of subaortic LBV is 0.28% (2/710 cases). Isolated subaortic LBV without any associated cardiac or aortic arch anomaly was observed in a female patient. Rare bilateral ectopic origin of bronchial arteries from corresponding subclavian arteries was also noted in this patient. In another female patient, right aortic arch anomaly was associated with subaortic LBV. The right-sided aortic arch had an aberrant retroesophageal left subclavian artery arising from a Kommerell's diverticulum as the last branch. Discussion and Conclusion: Extensive literature search has yielded only 15 cases of isolated anomalous subaortic LBV in subjects without any cardiac and aortic arch anomaly. Although this condition is asymptomatic, its presence, when detected, should alert the clinician to the possible presence of associated congenital cardiac and aortic arch anomalies. Accurate knowledge of these rare anomalies will enhance the diagnostic accuracy and proper interpretation of radiological images. Such thorough knowledge will avoid interpreting the anomalous vein as an enlarged lymph node, enlarged left superior intercostal vein, and reduce the chances of surgical complications.

Clues and techniques of prenatal ultrasonographic diagnosis for fetal aortic arch anomalies

Objective To explore the ultrasonographic features, diagnostic clues and methods of fetal aortic arch anomalies, and to improve the diagnosis rate of prenatal ultrasonography for fetal aortic arch anomalies. Methods Fetuses with aortic arch anomalies diagnosed by prenatal ultrasound were selected as the study objects. The prenatal ultrasonographic features of the three views of the aortic arch were carefully observed. The three views included the followings: the transverse view(the view of three vessels and trachea), the sagittal view (the longitudinal view of the aortic arch), and the coronal view (the coronal view of the descending aorta). The prenatal diagnostic clues and diagnostic techniques of aortic arch anomalies were summarized and analyzed. Results Eighty-one cases with aortic arch anomalies were followed up. Fifteen cases were left-sided aortic arch and left-sided ductus arteriosus with aberrant right subclavian artery, 22 cases with right aortic arch and left-sided ductus arteriosus with aberrant left subclavian artery, 4 cases were mirror right aortic arch and left-sided ductus arteriosus, 2 cases were mirror right aortic arch and right-sided ductus arteriosus, 11 cases were double aortic arch, 23 cases were coarctation of the aorta, and 4 cases were interruption of aortic arch. Among them, 27 cases were complicated with other cardiac and extra-cardiac malformations and 13 cases with chromosomal abnormalities. Prenatal ultrasonographic clues for diagnosis of coarctation of the aorta included the three vessels and trachea view showing small diameter and disproportion to the diameter of the ductus arteriosus. And the longitudinal view of the aortic arch was helpful for showing the position and the extent of coarctation of the aorta. Prenatal ultrasonographic clues for diagnosis of interruption of aortic arch were that the three vessels and trachea view and the longitudinal view of the aortic arch showed the interruption between aortic arch and descending aorta. And the abnormal combination cases of the aortic arch segments also had their corresponding diagnostic clues. These clues included that the three vessels and trachea view showed abnormal position, arrangement and running of the branches of the aortic arch. And the coronal view of the descending aorta also showed abnormal changes in the branches of aortic arch. Conclusions Various types of congenital aortic arch anomalies have characteristic ultrasonography and their corresponding diagnostic clues and methods. The combined use of the three views of the aortic arch is effective in prenatal diagnosis for aortic arch anomalies. Key words: Ultrasonography, prenatal; Aortic arch anomalies; Diagnostic clues

Complex Vascular Ring Diagnosed on Cardiovascular MR in a 3-Day-Old Infant.

Prenatal ultrasonography in the early third trimester showed an unusual branching pattern of the right aortic arch. Echocardiography performed 4 h after birth showed the right aortic arch with mirror-image branching, patent ductus arteriosus, and patent foramen ovale. Because the location of the ductus arteriosus was unclear on echocardiography, cardiovascular magnetic resonance imaging was performed 3 days after birth. Advanced techniques including contrast-enhanced time-resolved magnetic resonance angiography and 3D time-of-flight magnetic resonance angiography allowed accurate diagnosis of a vascular ring comprising ascending and descending aorta, right aortic arch with mirror-image branching, and diverticulum of Kommerell giving rise to a left ligamentum arteriosum. The infant had hiccups, but no other symptoms. The esophagram was negative for obstruction. The infant was closely monitored; however, she developed esophageal obstruction at 7 months of age because of the vascular ring. She underwent lysis of the left ligamentum arteriosum followed by aortopexy for relief of esophageal obstruction. This report shows the utility of neonatal cardiovascular magnetic resonance imaging to evaluate complex congenital aortic arch anomalies.

Usefulness of intraoperative bronchoscopy during surgical repair of a congenital cardiac anomaly with possible airway obstruction: three cases report.

Compression of the airway is relatively common in pediatric patients, although it is often an unrecognized complication of congenital cardiac and aortic arch anomalies. Aortopexy has been established as a surgical treatment for tracheobronchial obstruction associated with vascular anomaly, aortic arch anomaly, esophageal atresia, and tracheoesophageal fistula. The tissue-to-tissue arch repair technique could result in severe airway complication such as compression of the left main bronchus which was not a problem before the correction. We report three cases of corrective open heart surgery monitored by intraoperative bronchoscopy performed during prebypass, and performed immediately before weaning from bypass, to evaluate tracheobronchial obstruction caused by congenital, complex cardiac anomalies in the operating room.

Surgical treatment of congenital anomalies of the aortic arch : Long-term results

Congenital anomalies of the aortic arch gather a wide spectrum of malformations concerning the aortic arch or some of its branches and the pulmonary artery. These malformations are rare (1 in 1000 live births) and sometimes responsible of complete (vascular ring) or partial (vascular sling) encirclement of the oesophagus, trachea or bronchi. In cases of symptomatic airways obstruction or oesophagus compression the surgical treatment is mandatory and curative. In this paper we describe the experience of Children's Hospital of Toulouse on the surgical treatment of congenital anomalies of aortic arch.

Аномалии дуги аорты: проблемы пренатальной и постнатальной тактики

Аномалии дуги аорты: проблемы пренатальной и постнатальной тактики

Evaluation of Aortic Arch Anomalies by Echocardiography and CT Angiography, Could CT be the Primary Method of Diagnosis?

Aims: In our study we highlight the importance of noninvasive imaging, stressing the role of CTA over echocardiography in the diagnosis of congenital aortic arch anomalies. Study Design:Prospective design.

Congenital Cardiac, Aortic Arch, and Vascular Bed Anomalies in PHACE Syndrome (from the International PHACE Syndrome Registry)

PHACE syndrome represents the association of large infantile hemangiomas of the head and neck with brain, cerebrovascular, cardiac, ocular, and ventral or midline defects. Cardiac and cerebrovascular anomalies are the most common extracutaneous features of PHACE, and they also constitute the greatest source of potential morbidity. Congenital heart disease in PHACE is incompletely described, and this study was conducted to better characterize its features. This study of the International PHACE Syndrome Registry represents the largest central review of clinical, radiologic, and histopathologic data for cardiovascular anomalies in patients with PHACE to date. Sixty-two (41%) of 150 subjects had intracardiac, aortic arch, or brachiocephalic vessel anomalies. Aberrant origin of a subclavian artery was the most common cardiovascular anomaly (present in 31 (21%) of150 subjects). Coarctation was the second most common anomaly, identified in 28 (19%) of 150 subjects, and can be missed clinically in patients with PHACE because of the frequent association of arch obstruction with aberrant subclavian origin. Twenty-three (37%) of 62 subjects with cardiovascular anomalies required procedural intervention. A greater percentage of hemangiomas were located on the left side of the head and neck in patients with coarctation (46% vs 39%); however, hemangioma distribution did not predict the presence of cardiovascular anomalies overall. In conclusion, PHACE is associated with a high risk of congenital heart disease. Cardiac and aortic arch imaging with detailed assessment of arch patency and brachiocephalic origins is essential for any patient suspected of having PHACE. Longitudinal investigation is needed to determine the long-term outcomes of cardiovascular anomalies in PHACE.

Diagnosis of congenital aortic arch anomalies in chinese children by multi-detector computed tomography angiography

The purpose of this study was to evaluate the value of multi-detector computed tomography (MDCT) angiography for the diagnosis of congenital aortic arch anomalies and present the radiological images of congenital aortic arch anomalies in Chinese children. MDCT angiography and transthoracic echocardiography (TTE) were applied for the diagnosis of congenital aortic arch anomalies in 362 Chinese children between May 2006 and December 2011 (age ranges from 5 days to 12 years; mean age, 3.3 years). Surgery and/or catheter angiography (CA) were conducted in all patients to confirm the final diagnosis. In the 362 Chinese children with congenital heart anomalies, congenital aortic arch anomalies were definitely diagnosed in 198 children and 164 children ruled out by operation and/or (CA). Among the 198 children with anomalies, coarctation of aorta (CoA), interruption of aortic arch (IAA), right aortic arch, aberrant right subclavian artery and double aortic arch were diagnosed in 134, 32, 20, 10 and 2 children respectively, and there were 6 cases with uncommon congenital aortic arch anomalies: 2 had double aortic arch including 1 with five branches of the aortic arch, 2 had isolation of the right subclavian artery with two patent ductus arteriosus (PDA), 1 had an isolation of the common carotid artery with a PDA, and 1 had double PDA with a single ventricle and pulmonary artery atresia. Among the 32 children with IAA, 28 were of type A, and 4 were of type B. The diagnostic sensitivity, specificity and accuracy of MDCT angiography for congenital aortic arch anomalies were 100% (198/198), 98% (161/164) and 99% (359/362), respectively. The diagnostic sensitivity, specificity and accuracy of TTE were 92% (182/198), 81% (133/164) and 87% (315/362), respectively. In conclusion, MDCT angiography is a reliable, noninvasive imaging technique for the diagnosis of congenital aortic arch anomalies in children. Sometimes, even more information can be obtained from this technique than from conventional angiography.

Intrathoracic subclavian artery aneurysm repair in the thoracic endovascular aortic repair era

Intrathoracic subclavian artery aneurysms (SAAs) are rare aneurysms that often occur in association with congenital aortic arch anomalies and/or concomitant thoracic aortic pathology. The advent of thoracic endovascular aortic repair (TEVAR) methods may complement or replace conventional open SAA repair. Herein, we describe our experience with SAA repair in the TEVAR era. A retrospective review was performed of all intrathoracic SAAs repaired at a single institution since United States Food and Drug Administration approval of TEVAR in 2005. Nineteen patients underwent 20 operations to repair 22 (13 native, nine aberrant) SAAs with an intrathoracic component. Mean SAA diameter was 3.1 cm (range, 1.6-6.0 cm). Mean patient age was 57 years (range, 24-80 years). Twenty-one percent (n = 4) of patients had a connective tissue disorder (two Loeys-Dietz, two Marfan). Thirty-six percent (n = 8) of SAAs were repaired by open techniques and 64% (n = 14) via a TEVAR-based approach. All TEVAR cases required proximal landing zone in the aortic arch (zone 0-2), and revascularization of at least one arch vessel was required in 83% (10/12) of patients. Concomitant repair of associated aortic pathology was performed in 50% (n = 10) of operations. Thirty-day/in-hospital rates of death, stroke, and permanent paraplegia/paraparesis were 5% (n = 1), 5% (n = 1), and 0%, respectively. Over mean (standard deviation) follow-up of 24 (21) months, 16% (n = 3) of patients required reintervention for subclavian artery bypass graft revision (n = 2) or type II endoleak (n = 1). This is the largest single-institution series to date of TEVAR for SAA repair. Modern endovascular techniques expand SAA repair options with excellent results. The majority of SAAs and nearly all aberrant SAAs (Kommerell's diverticulum) can now be repaired using a TEVAR-based approach without the need for sternotomy or thoracotomy.

Developmental Aortic Arch Anomalies in Infants and Children Assessed With CT Angiography

The purpose of this article is to review the normal anatomy of the aortic arch and the most common variations of congenital aortic arch anomalies using low-radiation-dose, defined as a dose-length product of 7-15 mGy × cm, MDCT angiography. Radiologists should be prepared to fully describe congenital aortic arch anomalies; recognize them on CT angiography, especially the presence or absence of vascular rings or aberrant arteries; and explain their association with the trachea and esophagus.

Changes of Brain Magnetic Resonance Imaging Findings After Congenital Aortic Arch Anomaly Repair Using Regional Cerebral Perfusion in Neonates and Young Infants

The objective of this prospective study is to compare magnetic resonance imaging (MRI) findings before and after surgery for repairing congenital aortic arch anomalies using regional cerebral perfusion. Neurologic examinations that included brain MRI, brain sonography, and electroencephalograms were performed before and after surgery for congenital aortic arch anomalies and the accompanying intracardiac anomalies using regional cerebral perfusion in 11 neonates and young infants. The median age at operation was 11 days (range, 5 to 46). The diagnoses included coarctation of the aorta with accompanying intracardiac anomalies (n = 10) and interruption of the aortic arch (n = 1). Aortic arch repair was performed using regional cerebral perfusion through the right innominate artery (regional perfusion time: 25.6 ± 6.0 minutes) without cardiac arrest. Two patients had new postoperative lesions on postoperative brain MRI, and these were acute focal subdural hemorrhage (n = 1) and acute focal infarction (n = 1). However, they were without clinical significance. Periventricular leukomalacia was not observed on brain MRI. There was no significant change between the preoperative and postoperative findings on brain sonography and electroencephalograms. All the patients showed normal neurologic growth for a mean follow-up duration of 175.3 days (range: 25 to 497 days). There were newly developed lesions on the postoperative brain MRI in 2 of 11 patients, even though these patients showed normal brain sonography and electroencephalogram findings and normal neurologic development. Our regional cerebral perfusion protocol for aortic arch repair showed tolerable neurologic outcomes, and it did not induce periventricular leukomalacia.

P15.11: Trends in prenasal thickness, nasal bone length and nuchal fold thickness measurements in second and third trimester Down syndrome fetuses

Objective: To analyze the methods of prenatal sonographic diagnosis of all kinds of aortic arch anomalies and to improve prenatal sonographic detection rate of aortic arch anomalies. Methods: 73 fetuses with congenital aortic arch anomalies were studied. The results from prenatal ultrasound examination, postnatal examination were compared. Results: 73 cases were with congenital aortic arch anomalies, 33 cases with malposition of aortic arch, 9 cases with normal left aortic arch with aberrant left subclavian artery, 5 cases with interruption of aortic arch, 26 cases with coarctation of aortic arch. The accuracy rate of prenatal sonographic diagnosis was 87.7%. Conclusions: Aortic arch anomalies have typical sonographic image. If we grasp typical sonographic images, we could make accurate diagnosis.

P15.13: A case series of unilateral empty renal fossa not caused by renal agenesis

Objective: To analyze the methods of prenatal sonographic diagnosis of all kinds of aortic arch anomalies and to improve prenatal sonographic detection rate of aortic arch anomalies. Methods: 73 fetuses with congenital aortic arch anomalies were studied. The results from prenatal ultrasound examination, postnatal examination were compared. Results: 73 cases were with congenital aortic arch anomalies, 33 cases with malposition of aortic arch, 9 cases with normal left aortic arch with aberrant left subclavian artery, 5 cases with interruption of aortic arch, 26 cases with coarctation of aortic arch. The accuracy rate of prenatal sonographic diagnosis was 87.7%. Conclusions: Aortic arch anomalies have typical sonographic image. If we grasp typical sonographic images, we could make accurate diagnosis.