Medical Care

Medical care prior to surgical repair of double aortic arch depends on the clinical presentation. In most patients, only supportive care is required.

Catheter interventions are not used in the management of double aortic arch.

Aside from analgesic medications, pharmacologic therapy is not usually required after removal from mechanical ventilation. Relief from the pain of the thoracotomy incision may be achieved with age-appropriate narcotic and nonsteroidal anti-inflammatory medications.

After postoperative stabilization in the intensive care unit and removal from mechanical ventilatory support, the patient may be transferred to the regular inpatient care area for advancement of feedings and additional postoperative care.

Further inpatient care

Routine postthoracotomy care is provided following repair of double aortic arch.

Patients are removed from mechanical ventilation as soon as possible, and the chest tube usually is discontinued on the first postoperative day.

The remainder of the inpatient stay is focused on determining and managing any residual symptomatology, providing sufficient enteral nutrition, transitioning the patient to enteral analgesics, and educating the parents.

In patients with residual or recurrent obstruction of the airways, aortopexy may relieve the compression and associated symptoms.^[12]

Consultations

Unless specific associated anomalies or problems are identified, consultations usually are not necessary. As noted above, chromosome 22q11 deletion is present in a substantial proportion of patients with double aortic arch.

Consultation with a geneticist is indicated in patients with other characteristic features of the chromosome 22q11 deletion syndrome and may be appropriate in young infants, in whom typical features of the syndrome may not yet be evident.

Surgical Care

The first description of surgical relief of tracheal obstruction caused by vascular ring was reported by Gross in 1945.^[13]

Indications

Surgical division of the vascular ring is indicated in any patient with symptoms of airway or esophageal compression and in patients undergoing surgery for repair of associated cardiovascular or thoracic anomalies.

Techniques

The fundamental principle of surgical management of double aortic arch is division of the ring to relieve compression of the trachea and esophagus. In general, this is achieved by dividing the minor arch through an ipsilateral thoracotomy. Note the following:

When the minor arch is atretic, the atretic segment is ligated or clipped and then divided. When the minor arch is patent, it is usually ligated and divided between the subclavian artery and descending aorta. The ligamentum arteriosum, which is almost always left sided, is ligated and divided as well.
Dissection should be carried down to the trachea and esophagus to ensure that no constricting fibrous bands remain.
If necessary to minimize residual posterior compression of the trachea/esophagus, arteriopexy may be performed by suturing the retroesophageal aortic segment to the prevertebral fascia.
Video-assisted thoracoscopic division of vascular rings is performed at several centers and appears to be an effective approach in most cases, but data on outcomes are limited. Robotically assisted division of vascular rings has also been reported.
When associated intracardiac anomalies require surgery through a median sternotomy, division of the double arch is performed during the same procedure.

Results

In the current era, essentially no operative mortality is associated with repair of isolated double aortic arch.^[14]Outcomes in patients with associated anomalies depend on the coexisting condition.

Postoperative care and precautions

Postoperative care after division of double aortic arch is similar to that for patients undergoing other cardiovascular procedures through a thoracotomy. Most patients experience immediate relief after surgery, although persistent respiratory symptoms and signs may be present, especially in very young infants with severe preoperative symptoms. Except in patients undergoing concurrent repair of associated anomalies, cardiopulmonary bypass is not used during the repair; therefore, postoperative cardiac function typically is not a problem.

Early postoperative complications

Complications in the early postoperative period are uncommon after division of a vascular ring.

The major issue is persistent respiratory symptoms, especially in neonates who are more susceptible to tracheomalacia.

Other rare operative complications include chylothorax resulting from injury to the thoracic duct, diaphragmatic paresis/paralysis secondary to phrenic nerve injury, and, following repair through a left thoracotomy (ie, in patients with a right-dominant double arch), vocal cord paresis/paralysis resulting from injury to the recurrent laryngeal nerve.

Diet

No special dietary considerations are indicated in patients with double aortic arch other than those dictated by associated conditions. Postoperatively, enteral feeding is resumed as soon as possible.

In patients with dysphagia or emesis as a presenting symptom, adequate oral intake should be verified, and feeding therapy should be instituted if necessary. In patients with chromosome 22q11 deletion, velopharyngeal insufficiency or cleft palate frequently is present; oral feedings should be resumed with the aid of feeding specialists.

Activity

Patients with double aortic arch are not subject to specific restrictions on activity. Prior to repair, activity may be limited by symptoms.

Following repair, any persistent respiratory symptoms resulting from tracheomalacia should dictate activity limitations.

Long-Term Monitoring

For the early posthospital period, educate parents concerning the possible persistence of symptoms, the potential benefit of prone positioning in patients with tracheomalacia, signs and symptoms of aspiration, and management of the thoracotomy incision.

Close follow-up observation should be maintained after repair of double aortic arch, especially in young children with persistent tracheomalacia or other respiratory symptoms.

Additional imaging or bronchoscopic evaluation is not indicated unless residual symptoms persist or other mitigating circumstances are present.

No dietary or activity restrictions are indicated after repair of an isolated vascular ring.

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Media Gallery

Double Aortic Arch. Schematic diagram (left) of the primitive pharyngeal arch system shows the left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries, fourth (IV) and sixth (VI) pharyngeal arches, distal pulmonary arterial segments (PA), dorsal aortas (DA), and seventh intersegmental arteries (VII). The proximal (p) sixth arches develop into the proximal pulmonary arteries and the distal (d) sixth arches become the arterial ducts. The seventh intersegmental arteries develop into the subclavian arteries. Schematic diagram (right) shows the segments of the pharyngeal arch system that regress (shown in black) in the normal formation of the thoracic great arteries. Left pulmonary artery (LPA); ductus arteriosus (PDA); right pulmonary artery (RPA); subclavian artery (SCA).
Double Aortic Arch. Schematic diagram (left) depicts the segments of the pharyngeal arch system that regress (shown in black) so that the mature vascular anatomy of a double aortic arch can develop. The dominant and minor arches can vary in laterality and specific patterns of branching and segmental hypoplasia/atresia. (These variables are not specified in this diagram.) Left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries; fourth (IV) and sixth (VI) pharyngeal arches; distal pulmonary arterial segments (PA); dorsal aortas (DA); seventh intersegmental arteries (VII); proximal (p) sixth arches; distal (d) sixth arches. Mature anatomy (right) of a double aortic arch with a dominant right arch and patent minor left arch. In most patients, a single left-sided ductus arteriosus or ligamentum arteriosum is present. Left pulmonary artery (LPA); ductus arteriosus (PDA); right pulmonary artery (RPA); subclavian artery (SCA).
Double Aortic Arch. Transverse MRI images in a patient with double aortic arch. Both arches are patent; the right arch is dominant. Images A-F are arranged in a caudad to cephalad order. (A) Transverse image at the level of the pulmonary valve. The ascending aorta (AAo) and descending aorta (DAo), cephalad to the junction of the left and right arches, can be seen. (B) At the level of the pulmonary artery (PA) bifurcation, the distal confluence of the left and right arches forming the single descending aorta is depicted. (C) The distal portions of the left (L) and right (R) arches can be seen posterior and to the left and right sides of the trachea. Note the anteroposterior compression of the tracheal carina (anterior to and between the arches). (D) Moving cephalad, the dominance of the right arch can be seen. (E) At the level of the proximal/transverse aortic arches, the origin of the left and right arches from the rightward ascending aorta can be seen. (F) The left and right common carotid and subclavian arteries arise from the left and right arches, respectively. The common carotid arteries are the dark round structures anterior to and to either side of the trachea. The subclavian arteries are the dark round structures posterior to and to either side of the trachea.
Double Aortic Arch. Coronal spin-echo MRI images in a patient with a double aortic arch. Both arches are patent, with the right (R) slightly larger in caliber than the left (L). Compression of the trachea (T) between the two arches can be seen (left). The confluence of the arches and the descending aorta (D) are shown (right).