Medical Care
Medical care before surgical repair depends on the clinical presentation. In most cases, only supportive management is required.
Catheter interventions are not used in the management of vascular ring formed by a right aortic arch.
Routine postthoracotomy care is provided following division of a vascular ring. Patients are removed from mechanical ventilation as soon as possible, and tube thoracostomy is generally discontinued on the first postoperative day. The remainder of the inpatient stay is focused on determining and managing any residual symptoms, providing sufficient enteral nutrition, transitioning the patient to enteral analgesics, and educating the parents.
Aside from analgesic medications, pharmacologic therapy is generally not 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.
Consultations
Unless specific associated anomalies or problems are identified, consultations are generally not necessary. As noted above, band 22q11 deletion is present in a substantial proportion of patients with vascular rings.
A consultation with a geneticist is indicated in patients with other characteristic features of the band 22q11 deletion syndrome and may be appropriate in young infants, in whom typical features of the syndrome may not yet be evident.
Transfer
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.
Surgical Care
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. [13]
Techniques
The fundamental principle of surgical management of vascular rings is division of the ring to relieve compression of the trachea and esophagus.
In patients with all forms of vascular ring with a right aortic arch and a left-sided ductus arteriosus or ligamentum arteriosum, this is achieved by dividing the ductus/ligamentum through a left thoracotomy. Dissection should be carried down to the trachea and esophagus to ensure that no constricting fibrous bands remain. In patients with an aberrant subclavian artery in whom residual posterior compression of the trachea or esophagus is a concern, arteriopexy may be performed by suturing the retroesophageal diverticulum to the prevertebral fascia.
Video-assisted thoracoscopic division of vascular rings is performed at several centers and seems to be an effective approach in most cases, but data on outcomes are limited. Robotically assisted division of vascular rings has also been reported. However, this technique would not be applicable if there is a need to resect a large diverticulum of Kommerel and reimplant the subclavian artery.
When associated intracardiac anomalies require surgery through a median sternotomy, division of the ductus/ligamentum is performed during the same procedure.
Results
In the current era, essentially no operative mortality is associated with repair of isolated vascular rings. [14] Outcomes in patients with associated anomalies depend on the coexisting condition.
Postoperative care and precautions
Postoperative care after division of a vascular ring 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 symptoms preoperatively. Except in patients undergoing concurrent repair of associated anomalies, cardiopulmonary bypass is not used during the repair; therefore, postoperative cardiac function is generally not a problem.
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. Occasionally, the tracheomalacia is severe. However, this tends to improve with relief of the vascular ring over time.
Other rare operative complications include chylothorax due to injury to the thoracic duct, diaphragmatic paresis/paralysis secondary to phrenic nerve injury, and vocal cord paresis/paralysis resulting from injury to the recurrent laryngeal nerve.
Diet
No special dietary considerations are indicated in patients with a vascular ring 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 instituted if necessary.
In patients with band 22q11 deletion, velopharyngeal insufficiency or cleft palate is frequently present; oral feedings should be resumed with the aid of feeding specialists.
Activity
Patients with vascular rings are not subject to specific restrictions on activity.
Prior to repair, their activity may be limited by symptoms. Following repair, any persistent respiratory symptoms due to tracheomalacia should dictate activity limitations.
Long-Term Monitoring
Close follow-up should be maintained after division of a vascular ring, especially in young children with persistent tracheomalacia or other respiratory symptoms. Additional imaging or bronchoscopic evaluation is not indicated unless persistent residual symptoms or other mitigating circumstances are present.
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Left: Schematic diagram of the primitive pharyngeal arch system showing the left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries, the 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. Right: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for normal development of the great arteries and their thoracic branches (common carotid artery [CCA], left pulmonary artery [LPA], ductus arteriosus [PDA], right pulmonary artery [RPA], subclavian artery [SCA]).
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Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with aberrant left subclavian artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with an aberrant left subclavian artery arising from a retroesophageal diverticulum with a left-sided ligamentum arteriosum to the left pulmonary artery.
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Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with mirror-image branching of the brachiocephalic vessels and a left-sided ductus arteriosus from the descending aorta to the left pulmonary artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with mirror-image branching of the brachiocephalic vessels and a left-sided ductus arteriosus from the descending aorta to the left pulmonary artery.
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Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with aberrant retroesophageal left innominate artery. Abbreviations are as in the first image. Right: Mature anatomy of a vascular ring formed by a right aortic arch with an aberrant retroesophageal left innominate artery with a left-sided ligamentum arteriosum to the left pulmonary artery.
