A double aortic arch (DAA) is a congenital anomaly that belongs to the group of "vascular rings", ie. abnormalities of the aortic arch that result in complete encircling of the trachea and the esophagus by blood vessels arising from the heart [1] [2] [3] [4]. Vascular rings comprise approximately 1-2% of all congenital abnormalities of the heart and aorta [5], making them a rare occurrence in clinical practice. A DAA is distinguished by the division of the ascending aorta into two separate branches (one going anteriorly to the trachea and the esophagus, while the other going posteriorly) and then rejoining to form the descending aorta [1] [2] [4]. Signs and symptoms stem from compression of the trachea or the esophagus, and their onset is most commonly reported in infancy and early childhood [5]. Dysphagia, together with feeding difficulties, is the main gastrointestinal symptom [3] [4] [6]. Conversely, stridor, dyspnea, wheezing and breathing difficulties are notable signs related to the respiratory tract [3] [5]. Recurrent respiratory infections are also reported in a number of cases [7]. On the other hand, asymptomatic patients in whom the diagnosis was discovered incidentally have been documented, the reason being atresia of one of the two encircling branches of the aorta that put less pressure on the trachea and esophagus [4] [7].The presence of additional cardiac anomalies (any forms of cyanotic congenital heart disease) that manifest with severe respiratory insufficiency and cyanosis may delay the diagnosis of a DAA [6].

• Epilepsy

  Epilepsy in cerebral hydatidosis Epilepsia. 15: 45-54. PMID 4207595 0.01 1973 Arseni C, Panoza G, Gontea L, Marinescu V. The Schneider centromedullary suppression syndrome | SINDROMUL DE SUPRESIUNE CENTROMEDULARA SCHNEIDER Chirurgia. 22: 481-486. [neurotree.org]

• Stridor

  It did not show any evidence of common causes of stridor, like lobar emphysema, bronchogenic cyst or mediastinal mass. [annalspc.com]

  Children's Memorial Hospital Abstract 7 month old female with stridor Keywords Double aortic arch, aorta, vascular ring, plain film, CT, MR, 963 Publication Date: 2005-09-09 History 7 month old female with stridor Discussion A double aortic arch, a true [mirc.luriechildrens.org]

  A 7-year-old boy had a history of repeated pneumonia and stridor. He was not relieved of the symptoms by routine treatment. [annals.in]

  After weaning from mechanical ventilation, he had the persistence of mild stridor despite improvement of respiratory symptoms. [ncbi.nlm.nih.gov]

  Patients may be discovered incidentally, while difficulties in swallowing, dyspnea, stridor, and wheezing are main symptoms, usually appearing in the first years of life. [symptoma.com]

• Pneumonia

  A 7-year-old boy had a history of repeated pneumonia and stridor. He was not relieved of the symptoms by routine treatment. [annals.in]

  One patient died in hospital from severe pneumonia. The intubation time ranged from 20 to 68 h and the intensive care unit stay ranged from one day to five days. [ncbi.nlm.nih.gov]

  Valentina Di Spirito, Carmine Guarino, Giovanni Rossi, Nadia Corcione, Livia De Pietro, Serafino Antonio Marsico European Respiratory Journal 2011 38: p2138; DOI: Abstract A twenty-one year old female with a history of recurrent pneumonia and airway [erj.ersjournals.com]

  He had suffered recurrent pneumonia in childhood. Beside an irregular pulse, the physical examination was normal. [link.springer.com]

  A7-year-old girl presented with severe dyspnea and pneumonia, which required endotracheal intubation, mechanical ventilation, and intravenous antibiotics. She had a history of several severe airway infections with inspiratory stridor. [circ.ahajournals.org]

• Respiratory Distress

  The symptoms are related to tracheal or esophageal compression, i.e. respiratory distress, wheezing, tachypnea, or difficulty swallowing, vomiting, choking, or aspiration. [mirc.luriechildrens.org]

  We present the interesting images of a child of respiratory distress because of tracheal compression from DAA. [ncbi.nlm.nih.gov]

  […] from nonspecific complaints to life threatening respiratory distress. [ctsnet.org]

• Respiratory Insufficiency

  insufficiency and cyanosis may delay the diagnosis of a DAA. [symptoma.com]

• Inspiratory Stridor

  She had a history of several severe airway infections with inspiratory stridor. After she recovered, a bronchoscopy and an esophagogram (Figure 1 ) were performed to screen for a vascular ring. An echocardiogram revealed a double aortic arch. [circ.ahajournals.org]

• Dysphagia

  Here, we report the case of a symptomatic adult with severe dysphagia associated with a DAA. CASE REPORT A 60-year old man visited our hospital because of severe dysphagia. [icvts.oxfordjournals.org]

  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. [ncbi.nlm.nih.gov]

  Chiaki Kondo Shin Takabayashi Yoichiro Miyake Koji Onoda Hideto Shimpo Isao Yada Abstract We present the case of a 39-year-old male who had complaints of dysphagia and throat pain. [link.springer.com]

  American College of Cardiology Foundation Monica Fernandez-Valls, MD ⁎, Javier Arnaiz, MD †, Dickson Lui, MD ‡, Maria Elena Arnaiz-Garcia, MD §, Ana Canga, MD † and Rafael Martin-Duran, PhD ⁎ ⁎ † ‡ § A 62-year-old woman presented with breathlessness and dysphagia [onlinejacc.org]

• Choking

  The symptoms are related to tracheal or esophageal compression, i.e. respiratory distress, wheezing, tachypnea, or difficulty swallowing, vomiting, choking, or aspiration. [mirc.luriechildrens.org]

  Apollo has been choking and vomiting 2-3 times a week and his stridor has returned. {Update December 2012} Apollo had a second heart surgery October 30, 2012. [littleearthlingblog.com]

  Your baby may have: Problems breathing Lung infections Poor feeding, such as choking Trouble swallowing Vomiting It may be noticed in infancy, but it is often found later. You will be asked about your child’s symptoms and health history. [health.cvs.com]

  Symptoms Your baby may have: Problems breathing Lung infections Poor feeding, such as choking Trouble swallowing Vomiting Diagnosis It may be noticed in infancy, but it is often found later. [winchesterhospital.org]

• Fracture

  […] radiculopathy ) Narrowing of the spinal column ( spinal stenosis ) Abnormal wearing on the bones and cartilage in the spine (spondylitis) Other abnormal results may be due to: Ankylosing spondylitis, a type of arthritis Bone infection Cauda equina syndrome Fractures [mclarenhealthplan.org]

• Headache

  We evaluated a 32-year-old woman with history of depression, migraines without aura, and cannabis use who presented with a thunderclap headache unresponsive to triptans. [ncbi.nlm.nih.gov]

The physician's role in raising clinical suspicion toward a congenital anomaly of the heart is crucial for diagnosing a double aortic arch. For this reason, a detailed history obtained from the patient (or the parent in the case of young children and infants) must cover the course and progression of symptoms. Because the DAA produces no murmurs, cardiac auscultation does not reveal any pathological findings, but the potential coexistence of a congenital heart disease illustrates the importance of a properly conducted physical examination. Imaging studies, however, are the cornerstone in detecting a double aortic arch. Plain radiography (showing tracheal deviation) or even transthoracic ultrasonography are of limited use in the assessment of vascular rings [5] [6], thus more advanced techniques, such as computed tomography (CT) and magnetic resonance imaging (MRI), need to be employed [5] [6]. CT angiography is recommended as the gold-standard procedure in order to make a definite diagnosis, which can clearly visualize the two vessels and their structure [5]. In the majority of cases, the posterior arch is dominant (approximately 75% of cases) [2] [3], and it is not uncommon for the less dominant arch to exhibit signs of atresia [2].

The following case presentation details an effective treatment approach in cerebrovascular stenting in a patient with variant aortic arch anatomy. [ncbi.nlm.nih.gov]

Surgical resection of one arch is the only curative treatment to relieve the compressive features. [annals.in]

Treatment and prognosis Surgical intervention with tracheostomy from the side of descending aorta and division of the minor (smaller) arch and ligamentum arteriosum is indicated for symptomatic patients. [radiopaedia.org]

(Outcomes/Resolutions) If Double Aortic Arch is managed adequately and maintained under control, the prognosis for this heart defect is typically good. [dovemed.com]

This condition is usually diagnosed in childhood, and surgical correction has a good prognosis. There have been sporadic reports in adulthood, for which the most common presenting complaints are dysphagia and asthma-like symptoms. [revespcardiol.org]

In most cases, this abnormality is diagnosed in childhood because of symptoms related to esophageal or tracheal obstruction, and surgical correction usually yields a good prognosis. [circ.ahajournals.org]

The case emphasizes the suspicion for vascular ring due to DAA in children with repeated pneumonia, stridor, dysphagia and aspiration when other common etiologies were excluded. [annals.in]

(Etiology) Double Aortic Arch is a congenital birth defect; individuals that exhibit this defect are born with the condition The reason why the aorta undergoes abnormal development is currently unknown What are the Signs and Symptoms of Double Aortic [dovemed.com]

Their etiology is related to abnormal development of the embryonic aortic arches. When the abnormal blood vessels form a ring around the trachea and esophagus, it is termed vascular ring. These rings may be complete or incomplete. [hawaii.edu]

The epidemiology and genetics of congenital heart disease. Clin Perinatol 200; 28 :1-10. Google Scholar Copyright information © Bohn Stafleu van Loghum 2007 Authors and Affiliations R. Nijveldt 1 Email author T. Germans A. M. Beek M. J. W. Götte A. [link.springer.com]

Journal Journal ID (nlm-ta): Pan Afr Med J Journal ID (iso-abbrev): Pan Afr Med J Journal ID (publisher-id): PAMJ Title: The Pan African Medical Journal Publisher: The African Field Epidemiology Network ISSN (Electronic): 1937-8688 Publication date (Electronic [scienceopen.com]

Epidemiology [ edit ] Complete vascular rings represent about 0.5-1% of all congenital cardiovascular malformations. The majority of these are double aortic arches. [en.wikipedia.org]

Epidemiologic technics in tuberculosis | Ancheta epidemiologicǎ în tuberculozǎ Viata Medicala; Revista De Informare Profesionala Si Stiintifica a Cadrelor Medii Sanitare. 29: 19-20. PMID 6784327 0.01 1979 Dumitrache S, Marinescu V. [neurotree.org]

Edwards was the first to describe embryonic pathophysiologic mechanisms of aortic arch development in 1948. The classic vascular rings will result from disruption at 4 points in the normal development. The 1st point is at the right dorsal aorta. [hawaii.edu]

In addition to the importance of early diagnosis and prompt surgery for DAA, appropriate preoperative respiratory management is emphasized to prevent similar occurrences in the future. [ncbi.nlm.nih.gov]

Prevention Double aortic arch can’t be prevented. [winchesterhospital.org]

Prevention There is no known way to prevent this condition. References Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 20th ed. [pennstatehershey.adam.com]

1. Ito H, Konishi A, Kon-Nai T, Ishibashi T, Takahashi S. Double aortic arch with atresia, tapering and aneurysm of the left arch. Br J Radiol. 2006;79(944):e71-74.
2. Ramos-Duran L, Nance JW Jr, Schoepf UJ, Henzler T, Apfaltrer P, Hlavacek AM. Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol. 2012;198(5):W466-47.
3. Kimura-Hayama ET, Meléndez G, Mendizábal AL, Meave-González A, Zambrana GF, Corona-Villalobos CP. Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 2010;30(1):79-98.
4. Hardin RE, Brevetti GR, Sanusi M, et al. Treatment of Symptomatic Vascular Rings in the Elderly. Tex Heart Inst J. 2005;32(3):411-415.
5. Noguchi K, Hori D, Nomura Y, Tanaka H. Double aortic arch in an adult. Interactive Cardiovascular and Thoracic Surgery. 2012;14(6):900-902
6. Lee M-L. Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations. Yonsei Med J. 2007;48(5):818-826.
7. Türkvatan A, Büyükbayraktar FG, Ölçer T, Cumhur T. Congenital Anomalies of the Aortic Arch: Evaluation with the Use of Multidetector Computed Tomography. Korean J Radiol. 2009;10(2):176-184.