Double aortic arch
Double aortic arch | |
---|---|
Other names | DAA |
Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an
The symptoms are related to the compression of the trachea, esophagus or both by the complete vascular ring. Diagnosis can often be suspected or made by
Signs and symptoms
Symptoms are caused by vascular compression of the airway, esophagus or both. Presentation is often within the first month (neonatal period) and usually within the first 6 months of life. Starting at birth an inspiratory and expiratory
Secondary to compression of the esophagus babies often feed poorly. They may have difficulties in swallowing liquids with choking or regurgitating and increased respiratory obstruction during feeding. Older patients might refuse to take solid food, although most infants with severe symptoms nowadays are operated upon before they are offered solid food. Occasionally patients with double aortic arches present late (during later childhood or adulthood). Symptoms may mimic asthma.[citation needed]
Causes
Little is known regarding the exact causes of aortic arch anomalies. However, the association with
Diagnosis
This section needs more primary sources. (January 2021) |
Prenatal diagnosis (fetal ultrasound): Today the diagnosis of double aortic arch can be obtained in-utero in experienced centers.[5] Scheduled repair soon after birth in symptomatic patients can relieve tracheal compression early and therefore potentially prevent the development of severe tracheomalacia.
Chest X-ray: Plain chest x-rays of patients with double aortic arch may appear normal (often) or show a dominant right aortic arch or two aortic arches . There might be evidence of tracheal deviation and/or compression. Sometimes patients present with radiologic findings of pneumonia.
Barium swallow (esophagraphy): Historically the esophagram used to be the gold standard for diagnosis of double aortic arch. In patients with double aortic arch the esophagus shows left- and right-sided indentations from the vascular compression. Due to the blood-pressure related movement of the aorta and the two arches, moving images of the barium-filled esophagus can demonstrate the typical pulsatile nature of the obstruction. The indentation from a dominant right arch is usually deeper and higher compared to the dent from the left arch.
Bronchoscopy: Although bronchoscopy is not routinely done in patients with suspected or confirmed double aortic arch, it can visualize sites and severity of pulsatile tracheal compression.
Echocardiography: In babies under the age of 12 months, echocardiography is considered to be sensitive and specific in making the diagnosis of double aortic arch when both arches are open. Non-perfused elements of other types of vascular rings (e.g. left arch with atretic (closed) end) or the ligamentum arteriosum might be difficult to visualize by echocardiography.
Computed tomography (CT):
Magnetic resonance imaging (MRI): Magnetic resonance imaging provides excellent images of the trachea and surrounding vascular structures and has the advantage of not using radiation for imaging compared to Computed tomography.
Cardiac catherization/aortography: Today patients with double aortic arch usually only undergo cardiac catherization to evaluate the hemodynamics and anatomy of associated congenital cardiac defects. Through a catheter in the ascending aorta contrast media is injected and the resulting aortography may be used to delineate the anatomy of the double aortic arch including sites of narrowing in the left aortic arch. Aortography can also be used to visualize the origin of all head and arm vessels originating from the two arches.
Classification
Double aortic arch is a subtype of complete vascular ring. There are three types of double aortic arch:
- Right dominant arch (about 70% of surgical cases)
- Balanced or codominant (about 5%): both arches are of equal size
- Left dominant (about 25%)
Double aortic arch with right dominant arch: Normally there is only one (left) aortic arch. In patients with double aortic arch the ascending aorta arises normally from the left ventricle but then divides into two arches, a left and a right aortic arch which join posteriorly to become the descending aorta.
The smaller left arch passes
The right aortic arch is completing the vascular ring by passing to the right and then behind the esophagus and trachea to join the usually left-sided descending aorta. The first vessel coming off the right arch is usually the right common carotid artery followed by the right subclavian artery.
Double aortic arch with left dominant arch: In this less common condition, as the name indicates, the left arch is the larger of the two aortic arches. The origins of the arm and head vessels are identical to the anatomy of double aortic arch with right dominant arch
Balanced or codominant double aortic arch: In this rare condition both aortic arches are of the same diameter.
Treatment
Surgical correction is indicated in all double aortic arch patients with obstructive symptoms (stridor, wheezing, pulmonary infections, poor feeding with choking). If symptoms are absent a conservative approach (watchful waiting) can be reasonable. Children with very mild symptoms may outgrow their symptoms but need regular follow-up.[citation needed]
Anesthesia and intraoperative monitoring
The procedure is performed in general anesthesia. It is useful to place
Open division of vascular ring
Isolated double aortic arch without associated intracardiac defects is a vascular anomaly that can be corrected without the support of cardiopulmonary bypass.[citation needed]
For surgical division of the narrower left aortic arch in a typical double arch patient with a dominant right arch, the patient is placed on the right side. After prepping and draping of the left chest a posterolateral
After insertion of a chest tube to prevent hemothorax and/or pneumothorax, the fourth and fifth rib are approximated by an absorbable suture. The surgery is completed by closure of the left thoracotomy wound in layers.[citation needed]
In most centers, the
Postoperative care
After the surgery, some patients require intubation and mechanical ventilation for several days to allow adequate tracheal toilet, but most patients can have the tubes removed soon after the surgery. The obstructive airway symptoms may be worse in the first postoperative weeks. Only a few patients have immediate relief of stridor, but many obtain immediate relief of problems with swallowing (dysphagia). After extubation, it might be necessary to maintain positive airway pressure by appropriate flows of a humidified oxygen/air mixture.[citation needed]
Epidemiology
Complete vascular rings represent about 0.5-1% of all congenital cardiovascular malformations. The majority of these are double aortic arches. There is no known gender preference, i.e. males and females are about equally affected. There is also no known ethnic or geographic disposition.[citation needed]
Associated cardiovascular anomalies are found in 10-15% of patients. These include:[citation needed]
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Patent ductus arteriosus (PDA)
- Tetralogy of Fallot (ToF)
- Transposition of the great arteries(D-TGA)
History
With the use of barium esophagography it became possible to diagnose aortic arch anomalies during life in the 1930s. The first open surgical correction via thoracotomy was performed by
Certain types of double aortic arch with a left arch that is small in diameter (less than 2 or 3mm patent) or atretic might be suitable for a so-called minimally invasive video-assisted thoracoscopic surgery (VATS) approach.[10]
References
- Congenital Heart Disease, London, Arnold, 2004, p. 497
- Cardiac Surgery, 3rd ed., New York, Churchill Livingstone, 2003, p. 1415
- ^ Momma K, Matsuoka R, Takao A. Aortic arch anomalies associated with chromosome 22q11 deletion. Pediatr Cardiol. 1999 Mar-Apr;20(2):97-102
- PMID 2738813.
- vascular rings. Am J Cardiol. 2009 Feb 1;103(3):416-9
- ^ Hommell L. Observationes anatomicae de arcu aortae bifido du dueto thoracico duplica, et de carstidum atque subclaviarum. Holdomas 1737;21:161
- ^ von Siebold CT. Ringfoermiger Aortenbogen bei einem neugeborenen blausuechtigen Kinde. J Geburtsh Frauenzimmer-Kinderkrank 1837;16:294
- N Engl J Med1945;233:586
- ^ Neuhauser EB. The roentgen diagnosis of double aortic arch and other anomalies of the great vessels. Am J Roentgenol Radium Ther Nucl Med 1946;56:1
- ^ Burke RP, Rosenfeld HM, Wernovsky G, Jonas RA.Video-assisted thoracoscopic vascular ring division in infants and children. J Am Coll Cardiol. 1995;25:943