The Unlucky Triad: Vascular Ring Due to Extremely Rare Right Sided Aortic Arch With Ductus Diverticulum and Retro-Esophageal Ligamentum Causing Severe Dysphagia

A 49-year-old African American male patient with no past medical history was admitted because of 3 months of difficulty swallowing solid and liquid foods. He had constant retrosternal discomfort and appeared malnourished. The chest radiograph revealed a right sided aortic arch with tracheal deviation to the left. A swallow study confirmed a fixed esophageal narrowing at the level of T6. Contrast enhanced Computed Tomography (CT) angiogram of the chest and neck revealed a mirror image right aortic arch with a left sided cardiac apex and a prominent ductus diverticulum (measuring 1.7 × 1.8 cm). This structure extended posterior to and indented the mid esophagus. A left posterolateral thoracotomy was performed and the ductus diverticulum was resected. A retroesophageal ligamentum arteriosum was found during surgery and divided. This rare combination of congenital anatomical aberrations led to severe dysphagia in our patient. Successful surgical correction in the form of resection of the ductus diverticulum and division of the retroesophageal ligamentum arteriosum led to complete resolution of our patient’s symptoms.

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Vascular ring surgery – can we do better?

European Heart Journal ◽

10.1093/ehjci/ehaa946.3210 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

G Amir ◽

N Soffair ◽

G Frenkel ◽

E Bruckheimer ◽

E Nachum ◽

...

Keyword(s):

Aortic Arch ◽

Respiratory Symptoms ◽

Ductus Arteriosus ◽

Vascular Ring ◽

Symptomatic Relief ◽

Mirror Image ◽

Vascular Rings ◽

Number Of Patients ◽

Age At Surgery

Abstract Background Vascular anomalies of the Aortic arch can cause respiratory symptoms in children due to tracheal compression. Treatment consists of division of the vascular rings, nevertheless data regarding mid- term results is scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. Methods Between2007–2014, 85 children underwent vascular ring surgery. 51 had Double Aortic Arch (DAA, 60%), 31 Right Arch with Aberrant Subclavian Artery (RAA & ALSA, 36.5%) and 3 had RAA, mirror image branching & left ductus arteriosus (3.5%). Mean age and weight at operation were 12.4±13months and 8.6±4.1 kg respectively. Mid-term follow-up included clinical follow up by a pulmonologist (38 patients, 44.7%) and a telephone questionnaire (71 patients, 83%) performed 57±25.7 months after surgery. Results In most patients, symptomatic relief occurred in less than 6 months (table 1). Mid –term follow up revealed that although most parents described a significant improvement in their child's respiratory symptoms (95%), a significant number of patients described some residual respiratory symptoms (table 2). We did not find any significant association between age at surgery (under 6 month), or vascular ring anatomy (DAA vs. RAA&ASA) and the presence of residual symptoms at follow up Conclusions Surgical division of vascular rings results in a significant clinical improvement within one year, nevertheless many patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms in mid- term follow up. Further evaluation whether a more aggressive surgical approach is warranted in order to decrease the incidence long-term symptoms. Funding Acknowledgement Type of funding source: None

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Double aortic arch: an elusive diagnosis in a child with CHD

Cardiology in the Young ◽

10.1017/s1047951121004856 ◽

2021 ◽

pp. 1-3

Author(s):

Mariana Lemos ◽

Miguel Fogaça da Mata ◽

Ana Coutinho Santos

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Aortic Arch ◽

Ct Angiography ◽

Septal Defect ◽

Vascular Ring ◽

Pulmonary Atresia ◽

Double Aortic Arch ◽

Shunt Surgery

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

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Complete Vascular Ring Detected by Barium Esophagography

Ear Nose & Throat Journal ◽

10.1177/014556130208100816 ◽

2002 ◽

Vol 81 (8) ◽

pp. 554-555 ◽

Cited By ~ 3

Author(s):

Liam J. Skinner ◽

Stephanie Ryan ◽

John D. Russell

Keyword(s):

Aortic Arch ◽

Vascular Ring ◽

Double Aortic Arch ◽

Vascular Rings ◽

Barium Esophagography

The diagnosis of a vascular ring can be made on the basis of characteristic findings on barium esophagography. We report a case of a double aortic arch in a 9-month-old girl that was diagnosed in this manner, and we briefly review the anatomic characteristics of vascular rings.

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Use of Contrast-Enhanced MR Angiography (CE-MRA) for the Diagnosis of a Vascular Ring Anomaly in a Dog

Case Reports in Veterinary Medicine ◽

10.1155/2012/316530 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4

Author(s):

Silke Hecht ◽

April M. Durant ◽

William H. Adams ◽

Gordon A. Conklin

Keyword(s):

Magnetic Resonance ◽

Aortic Arch ◽

Vascular Ring ◽

Ligamentum Arteriosum ◽

University Of Tennessee ◽

Contrast Enhanced ◽

Dry Food ◽

Magnetic Resonance Imaging Mri ◽

The Right ◽

Band Crossing

A 4-month-old female mixed breed dog was presented to the University of Tennessee College of Veterinary Medicine with a history of regurgitation and cachexia. Thoracic radiographs revealed focal megaesophagus cranial to the heart base. Magnetic resonance imaging (MRI) was performed. True fast imaging with steady-state precession (TrueFISP), fast low angle shot (FLASH), and short tau inversion recovery (STIR) sequences were acquired prior to contrast medium administration. Contrast-enhanced magnetic resonance angiography (CE-MRA) demonstrated focal megaesophagus and position of the aortic arch to the right of the esophagus. A small ductus diverticulum and an indistinct linear soft tissue band crossing the esophagus were also noted. Surgical exploration confirmed MR diagnosis of a persistent right aortic arch (PRAA) with left ligamentum arteriosum. The dog improved following surgery but was unable to be transitioned to dry food. To our knowledge this is the first report describing the use of CE-MRA for preoperative diagnosis and guided surgical treatment of a vascular ring anomaly in a dog.

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Diverticulum of Kommerell with Micro Aneurysm, an Aberrant Right Subclavian Artery and Ventricular Septal Defect in a 39 Years Old Female: Case Report

10.37191/mapsci-jccr-2(3)-038 ◽

2021 ◽

Author(s):

Xiaoming Bian

Keyword(s):

Ventricular Septal Defect ◽

Aortic Arch ◽

Subclavian Artery ◽

Septal Defect ◽

Aortic Rupture ◽

Vascular Ring ◽

Vascular Lesion ◽

Aberrant Right Subclavian Artery ◽

Successful Operation ◽

Diverticulum Of Kommerell

Diverticulum of Kommerell is a congenital anomaly in the development of the primitive embryonic aortic vasculature resulting in either an aberrant left subclavian artery (ALSA) from a right-sided aortic arch (RSAA) or an aberrant right subclavian artery (ARSA) from a left-sided aortic arch (LSAA). Kommerell’s diverticulum (KD), is extremely rare in the general population having prevalence of 0.7–2.0 %. KD with true aneurysmal dilatation like any vascular lesion of the thoracic aorta weakens the vasculature and increases the risk of aortic rupture. Timely surgical intervention is of paramount importance in most of these cases. Variable clinical presentations in patients with KD are either a result of compression of some mediastinal organs or strangulation by the vascular ring from the aberrant subclavian artery (ASA). We report a case of a 39 years old Chinese female with a rare co-existence of a huge KD with a microaneurysm, an aberrant right subclavian artery (ARSA), and ventricular septal defect (VSD). She underwent a successful operation and all her pre-surgical symptoms disappeared.

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Video-Assisted Thoracoscopic Division of Vascular Rings in Pediatric Patients

The American Surgeon ◽

10.1177/000313480507100403 ◽

2005 ◽

Vol 71 (4) ◽

pp. 289-291 ◽

Cited By ~ 8

Author(s):

Curt S. Koontz ◽

Amina Bhatia ◽

Joe Forbess ◽

Mark L. Wulkan

Keyword(s):

Aortic Arch ◽

Pediatric Patients ◽

Vascular Ring ◽

Operating Time ◽

Surgical Techniques ◽

Retrospective Chart Review ◽

Ligamentum Arteriosum ◽

Vascular Rings ◽

Computed Tomographic ◽

Video Assisted

Vascular rings are usually repaired via left thoracotomy. We report our series of pediatric patients with vascular rings that were repaired thoracoscopically. From February 2002 to September 2004, 13 patients underwent video-assisted thoracoscopic surgical techniques (VATS) division of their vascular ring. Chest magnetic resonance arterography (MRA) and/or computed tomographic arteriography (CTA) were used to evaluate the vascular ring in most patients. Patients were chosen for VATS repair based on surgeon's choice and type of vascular ring. Data are expressed as mean ± SD. The Children's Healthcare of Atlanta Institutional Review Board approved this retrospective chart review. Age and weight was 1.5 ± 1.8 years (range: 4 months–17 years) and 16.0 ± 12.5 kg (range: 6.0–22.1 kg), respectively (n = 13). Associated diseases included congenital heart disease (n = 2). Symptoms included respiratory complaints (n = 6), dysphagia (n = 2), dysphagia and shortness of breath (n = 1), pneumonia (n = 2), tracheal deviation (n = 1), and one patient was asymptomatic. Vascular ring types included double aortic arch (n = 4) and right aortic arch with an aberrant left subclavian artery and a left ligamentum arteriosum (n = 9). Operating time was 70 ± 20 minutes (range: 46–122 minutes). One patient had to be opened because of a large arch. Length of stay was 1.9 ± 0.9 days (range: 1–3 days). There were no complications, and all patients improved clinically at follow-up. Thoracoscopic repair of certain types of vascular rings seems to be safe and effective in children. More patients, however, need to be studied.

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P725 An unexpected direction: a case report of a double aortic arch in an asymptomatic woman in adulthood

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.397 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

I Campos ◽

C Vieira ◽

N Salome ◽

V H Pereira ◽

A Costeira Pereira ◽

...

Keyword(s):

Case Report ◽

Aortic Arch ◽

Ct Angiography ◽

Vascular Ring ◽

Double Aortic Arch ◽

Posterior Arch ◽

Vascular Rings ◽

Thoracic Ct ◽

Extrinsic Compression ◽

The Right

Abstract Introduction Complete vascular rings represent about 0.5-2% of all congenital cardiovascular malformations, with the double aortic arch (DAA) being the most common of the complete vascular rings, causing tracheoesophageal compression. The right (posterior) arch is usually dominant (70%), although the two arches can have the same size (5%). The left (anterior) arch is dominant in only approximately 25% of cases. In most cases, this anomaly is diagnosed during childhood due to symptoms caused by oesophageal or tracheal compression. For this reason, case reports of adults are rare. This report describes a case of a 61-year-old woman with DAA with dominant left arch, diagnosed accidentallyby thoracic CT angiography. Case Report Description A 61 years old woman with a previous story of hypertension and type 1 diabetes presented to the emergency service with dyspnoea and thoracic pain. She also referred a history of intermittent dysphagia and cough with at least 12 years of progression. All the parameters of the physical examination were within normal limits. The electrocardiogram showed a normal sinus rhythm with no evidence of acute ischemia and her blood analyses did not show any abnormalitie. She also performed a thoracic CT angiography, which excluded signs of pulmonary embolism, but revealed a vascular ring suggesting a double aortic arch with permeability in both right and left arches as well as their collaterals. The Cardiac MRI was performed with the purpose of excluding ischemia, confirming the double aortic arch with left dominance. The right arch, posterior to the oesophagus and trachea, and the left arch, in an anterior position, showed an anatomic compression of the oesophagus as well as the proximal trachea, capable of eliciting the symptoms mentioned. Other congenital anomalies were excluded. The echocardiography did not demonstrate any additional cardiac malformation. Endoscopy shows a pulsatile extrinsic compression of the esophagus (aortic ring). The patient is currently being studied and closely monitored in the Cardiology consultation. Discussion The most common type of complete vascular ring is the double aortic arch, which accounts for 70% of the complete rings. In most cases, there are two permeable arches, usually with right dominance (70% of the cases). Rarely, both arches are symmetrical. Symptoms usually appear in the fifth month of life. In most cases, only supportive treatment is required. Conclusion This case illustrates the atypical features of this congenital malformation, namely the diagnosis during adulthood as well as the left dominance. Abstract P725 Figure. A double aortic arch

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Right aortic arch with an aberrant left subclavian artery: sonographic features by HDlive Flow imaging

Cardiology in the Young ◽

10.1017/s1047951120001079 ◽

2020 ◽

Vol 30 (6) ◽

pp. 892-893

Author(s):

Toyohide Endo ◽

Daigo Ochiai ◽

Mamoru Tanaka

Keyword(s):

Aortic Arch ◽

Subclavian Artery ◽

Left Subclavian Artery ◽

Vascular Ring ◽

Vascular Anomaly ◽

Right Aortic Arch ◽

Flow Imaging ◽

Branching Pattern ◽

Aberrant Left Subclavian Artery ◽

The Right

AbstractThe right aortic arch is a congenital vascular anomaly, which may form a vascular ring. However, prenatal identification of the branching pattern of brachiocephalic vessels is often limited. In this paper, we clearly demonstrated the branching pattern of brachiocephalic vessels in a case of right aortic arch with an aberrant left subclavian artery using HDlive Flow imaging.

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Anomalous origin of a pulmonary artery in a teenager with tetralogy of Fallot with associated double aortic arch and coronary arteriovenous fistula

Cardiology in the Young ◽

10.1017/s1047951119000933 ◽

2019 ◽

Vol 29 (5) ◽

pp. 727-729

Author(s):

Vishal Agrawal ◽

Parth Solanki ◽

Ritesh Shah ◽

Divyakant Parmar ◽

Amit Mishra

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Tetralogy Of Fallot ◽

Vascular Ring ◽

Right Ventricular Outflow Tract ◽

Left Pulmonary Artery ◽

Ventricular Outflow Tract ◽

Double Aortic Arch ◽

Anomalous Origin ◽

Tract Fistula

AbstractWe report the case of a 14-year-old female who had tetralogy of Fallot along with anomalous origin of the left pulmonary artery from the ascending aorta with co-dominant double aortic arch forming a complete vascular ring compressing the oesophagus along with a left main coronary artery to right ventricular outflow tract fistula. She underwent surgical correction without conduit placement.

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