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Double Aortic Arch

A double aortic arch is a congenital anomaly in which the ascending aorta that arises from the heart splits into two branches that completely encircle the trachea and the esophagus, eventually joining to form a descending aorta. Patients may be discovered incidentally, while difficulties in swallowing, dyspnea, stridor, and wheezing are main symptoms, usually appearing in the first years of life. Imaging studies, in the form of computed tomography angiography or magnetic resonance imaging, are necessary to make the diagnosis.


Presentation

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].

Cyanotic Congenital Heart Disease
  • congenital heart disease) that manifest with severe respiratory insufficiency and cyanosis may delay the diagnosis of a DAA.[symptoma.com]
  • TOF is one of common cyanotic congenital heart disease that occurs in approximately 1 in 3600 live births and accounts for 3.5% of infants born with congenital heart disease.[cardiothoracicsurgery.biomedcentral.com]
Falling
  • This may not always work, because in small children the amount of contast may be too small to trigger or due to breathing the cursor may fall to the lungs. If bolus tracking does not trigger, start the scan at 15 seconds.[radiologyassistant.nl]
Tracheal Deviation
  • Plain radiography (showing tracheal deviation) or even transthoracic ultrasonography are of limited use in the assessment of vascular rings, thus more advanced techniques, such as computed tomography (CT) and magnetic resonance imaging (MRI), need to[symptoma.com]
  • There might be evidence of tracheal deviation and/or compression. Sometimes patients present with radiologic findings of pneumonia.[en.wikipedia.org]
Progressive Dysphagia
  • We describe the case of a 69-year-old male who presented with a double aortic arch, right arch dominant, left arch patent, experiencing progressive dysphagia since childhood.[ncbi.nlm.nih.gov]
Left Ventricular Dysfunction
  • We report the case of a 2-month-old baby with a double aortic arch, type C atresia of the left arch and severe hypoplasia of the right aortic arch between the right carotid and subclavian arteries, resulting in systemic obstruction, left ventricular dysfunction[ncbi.nlm.nih.gov]
Fidgeting
  • Before that surgery, an adult portion of any restaurant meal would spoil in the fridge before she could finish the whole thing; she’d sit at a restaurant, fidgeting and chatting, the bite of tofu speared on her fork going cold.[swallowmysunshine.com]
Aura
  • 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]
Dizziness
  • Samenvatting A 58-year-old male was referred to our outpatient cardiology clinic for evaluation of atrial fibrillation, fatigue, dizziness and exertional dyspnoea. He had suffered recurrent pneumonia in childhood.[link.springer.com]

Workup

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].

Right Bundle Branch Block
  • Electrocardiography showed atrial fibrillation (ventricular response of 60 to 70 beats/min), a right bundle branch block and nonspecific repolarisation abnormalities. Pulmonary vascular redistribution was visible on chest X-ray (figure 1).[link.springer.com]

Treatment

  • Asthma that is poorly controlled despite adequate treatment should be investigated for medication compliance, treatment adherence, environmental control, asthma mimics, and comorbidities especially in infants and young children.[ncbi.nlm.nih.gov]
  • Anatomic details of this rare type of complete vascular ring demonstrated on MDCT facilitated appropriate surgical treatment.[ncbi.nlm.nih.gov]
  • Double aortic arch should be considered in the differential diagnosis of chronic asthma when a patient fails to respond to medical treatment.[ncbi.nlm.nih.gov]
  • Aortopexy proved effective and safe in improving symptoms and provides a simple treatment option for children with severe malacia or tracheal compression after DAA repair.[ncbi.nlm.nih.gov]
  • One case of late diagnosis of this congenital malformation and long-term consequences of late surgical treatment with persistent tracheo-broncomalacia and dynamic airway obstruction is reported.[ncbi.nlm.nih.gov]

Prognosis

  • (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]
  • 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]
  • 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]

Etiology

  • (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]

Epidemiology

  • 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]
  • Double aortic arch can’t be prevented. Double aortic arch. Johns Hopkins University, Cove Point Foundation website. Available at: ...(Click grey area to select URL) Updated January 24, 2017. Accessed June 28, 2018. Vascular rings and slings.[cvshealthresources.com]

References

Article

  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.

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Last updated: 2019-06-28 11:30