Ann Thorac Surg 2007;83:1152-1157
© 2007 The Society of Thoracic Surgeons
Original Articles: General Thoracic
Tracheal Compression With "Hairpin" Right Aortic Arch: Management by Aortic Division and Aortopexy by Right Thoracotomy Guided by Intraoperative Bronchoscopy
Hermes C. Grillo, MD*,
Cameron D. Wright, MD*
Thoracic Surgical Division, Surgical Services, Massachusetts General Hospital and the Department of Surgery, Harvard Medical School, Boston, Massachusetts
Accepted for publication November 1, 2006.
* Address correspondence to Dr Wright, Thoracic Surgery, Massachusetts General Hospital, Blake 1570, 55 Fruit St, Boston, MA 02114 (Email: wright.cameron{at}mgh.harvard.edu).
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Abstract
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Background: Four patients with severe tracheal obstruction due to right aortic arch, aberrant left subclavian artery, diverticulum of Kommerell, ligamentum or ductus arteriosum, and, additionally, right descending aorta, mild pectus excavatum, and high aortic arch apex, with narrow space between the ascending and descending aortic limbs, underwent division of ligamentum, excision of diverticulum and division (and reimplantation) of aberrant subclavian, either in multiple or single operations, but failed to achieve relief of obstruction.
Methods: In addition to the procedures noted, fabric sling aortopexy of ascending and descending aortic limbs around adjacent ribs, with or without aortic division after prosthetic graft between ascending and descending aortic limbs was required, all performed through a right thoracotomy and adjunctive cervical incision, and with flexible bronchoscopic monitoring of each step.
Results: Three patients obtained full relief of airway obstruction, which has persisted in follow-up from eight to over 12 years. One who had persistent severe tracheal malacia after prior tracheal resection and resultant chronic pulmonary sepsis died from these complications.
Conclusions: In this unusual subset of a rare vascular ring anomaly, radical methods were necessary for correction of airway obstruction after failure of prior conventional procedures.
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Introduction
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Obstruction of the trachea and esophagus in the usual patient with right aortic arch and aberrant left subclavian artery (ALSA) responds to division of the vascular ring (left ligamentum arteriosum and ALSA), and further to excision of a diverticulum of Kommerell when present [1]. The aberrant subclavian artery is best anastomosed to the side of the left carotid artery (LCC). If an atretic left arch is present it is divided.
Three patients were treated, who failed to respond to varied prior divisions of ligamentum arteriosum or ductus, atretic arch, ALSA, and excision of aortic diverticulum (Kommerell). Two patients, with very severe airway obstruction, had also undergone subsequent tracheal resection in futile attempts to obtain relief. In one additional patient, who had not previously been operated upon, the vascular steps listed were carried out serially under bronchoscopic monitoring until demonstrable airway relief was achieved by aortopexy. Aortopexy was required in every case, and division of the aortic arch in three of the four. In the three patients operated upon previously, their obstructive symptoms had worsened after all prior procedures. Both patients who had previously undergone tracheal resection arrived with T-Y silicone stents in their airways.
All four patients had right aortic arch, ligamentum arteriosum, ALSA, and diverticulum of Kommerell (Fig 1A). In addition, all showed a degree of pectus excavatum with anteroposterior narrowing of the thorax, high apex of the aortic arch, and right descending aorta. This gave the aortic arch a "hairpin"-like configuration with little space between the two aortic limbs (Fig 2). Airway obstruction hence persisted there despite division of the vascular ring, including ALSA, and excision of the diverticulum. Sling aortopexy was necessary in all four patients, with aortic division in the three who had been operated upon previously.
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Fig 1. (A) Anatomic features. (AA = ascending aorta, right arch; DA = descending aorta, right side behind AA; DK = diverticulum of Kommerell; E = esophagus; LA = ligamentum arteriosum; LCC = left common carotid; LS = anomalous left subclavian; PA = pulmonary artery; RCC = right common carotid; RS = right subclavian; T = trachea.) (B) Surgical correction. Aortic arch divided beyond RS; lower suture line on DA at site of excision of DK; alternatively DK excision encompassed with excision of short segment of DA; Gore-Tex sling around AA limb and second rib; LS implanted into LCC; LA divided; Dacron graft from AA to DA below hilum.
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Fig 2. (A) Angiogram (anterior-posterior view) of patient 2 demonstrating right aortic arch, diverticulum of Kommerell (DK) and aberrant left subclavian artery. (B) Angiogram (lateral view) of patient (2) demonstrating "hairpin" aorta at apex of chest and location of DK.
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Patients and Methods
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Patient details are listed in Table 1. All four had suffered respiratory symptoms, including dyspnea on effort, low exercise tolerance, wheezing, and frequent respiratory infections; three also reported dysphagia initially. Two (patients 1 and 4) had severe respiratory limitation and two (patients 2 and 3) were completely incapacitated. As noted, three had undergone multiple operations previously without relief. These procedures are listed in Table 1.
Anatomical configurations were carefully outlined with computed tomographic scans in all patients (Fig 3). Three had aortograms (Fig 2). Bronchoscopy showed pulsatile compression of the lower third of the trachea on the right anterior wall of up to 80% to 90% of the cross-sectional area by the ascending right aortic arch. Nearer the carina and at the takeoff of the right main bronchus the airway was also compressed posterolaterally on the right, with strong pulsations visible, by the diverticulum of Kommerell in the descending arch. The lumen between the two points of compression was "teardrop" shaped, with the wide portion to the left (Fig 4). The airway narrowed even further on expiration. Cartilaginous rings, although deformed, were identified in the affected tracheal segment in all except the eight-year-old patient who had undergone prior tracheal resection. Access for tracheal resection in this patient had been obtained by temporary division of the aortic arch. His lower trachea was now malacic and required stenting throughout his subsequent course.
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Fig 3. (A) Chest computed tomography (CT) (axial image) of patient 2 demonstrating markedly compressed and displaced trachea from the aortic arch anomaly. (B) Chest CT (axial image) of patient 2 after operation demonstrating midline trachea of normal diameter. (T = trachea, E = esophagus.)
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Fig 4. Chest computed tomography (axial image) of patient 4 demonstrating tracheal compression (teardrop configuration) from the aortic arch anomaly and the diverticulum of Kommerell (DK). The "teardrop" is reversed from what is seen bronchoscopically.
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Operations
Ventilatory anesthesia was provided by a single lumen flexible armored endotracheal tube placed bronchoscopically in the left main bronchus. A bronchial blocker was positioned in the proximal right main bronchus. Changes in the degree of airway occlusion were directly observed serially at each stage of operation by withdrawing the endotracheal tube into the upper trachea and inserting a flexible bronchoscope through a sealing adaptor. The endotracheal tube was then readvanced into the left main bronchus after each bronchoscopic observation.
The major portion of the procedure was performed through right thoracotomy by the bed of the shingled fifth rib. The azygos vein was divided and doubly ligated. Anatomic structures were fully dissected for exact confirmation of the anomalies, carefully preserving vagus, recurrent laryngeal, and phrenic nerves. If the ligamentum arteriosum had not been previously divided this was done. Ascending and descending aorta were dissected, opening the pericardium as necessary, and these structures were encircled with tapes; the loop around the ascending limb being passed beneath the superior vena cava. The lower portion of the intrathoracic descending aorta was also dissected when it was necessary to divide the aorta. Need for aortic division was determined by retracting the aortic limbs with tapes during direct bronchoscopic visualization of the trachea. All intercostal arteries were preserved in each case in order to avoid any possible threat to the origin of the anterior spinal artery.
The diverticulum of Kommerell was resected with a side-biting clamp and oversewn in two layers. The aberrant left subclavian artery was divided, tentatively sutured closed, and dissected well up into the mediastinum in the thoracic outlet. Aortopexy was performed by encircling the ascending or, in one patient both ascending and descending aortic limbs, with a broad sling of Gore-Tex (W.L. Gore, Flagstaff, AZ) folded on itself longitudinally in two thicknesses, in order not to threaten late vascular erosion. A sling about the ascending aorta was passed around the adjacent (anterior) segment of the second rib near the chondral junction (Fig 1B), and, in one case, a second sling around the descending aortic limb encircling the eighth rib posteriorly. The Gore-Tex loop was completed with two rows of heavy Prolene (Ethicon, Somerville, NJ) sutures (Fig 5). In the first patient fascia lata was used instead of Gore-Tex.
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Fig 5. Chest computed tomography (axial image) of patient 4 after operation, demonstrating relief of tracheal compression and Gore-Tex sling around the ascending aorta and the second rib. (GS = Gore-Tex sling.)
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In the first three patients, who had undergone multiple prior operations, aortic limb traction alone was insufficient to "spring" the arch widely enough to relieve airway compression, observed bronchoscopically. Hence, aortic division was necessary usually just beyond the right subclavian artery. This was preceded by infrahilar bypass with a synthetic Dacron graft (Hemashield; Meadox Medicals Inc, Oakland, NJ) from the ascending to the descending aorta, using side-biting clamps with appropriate heparinization. In two patients, short segments of aortic arch were resected at the time of division of the arch; in one (patient 2) including the diverticulum of Kommerell. In patient 4, who had not been subject to prior surgery, suspension of the ascending aortic limb around the second rib was sufficient to open the airway after successive division of ligamentum, the ALSA and excision of diverticulum failed to provide adequate correction on bronchoscopic assessment.
After thoracotomy, dissection of the ALSA was completed and it was anastomosed to the side of the LCC through a cervical or cervical-mediastinal incision (Fig 1B). The latter was limited to division of the upper sternum only.
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Results
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Relief of vascular compression of the airway, both lower trachea and the origin of the right main bronchus, was achieved in all patients. The three patients without evident malacia of airways were symptomatically improved at once. Their anteroposterior lower tracheal diameters increased from 2 to 5 mm before operation, to 8 to 10 mm postoperatively.
Postoperative complications (Table 1) included bleeding at the site of harvesting of fascia lata in patient 1, chyle leak from an accessory duct along the aortic adventitia in patients 2 and 3, intrathoracic bleeding from unidentified site in patient 4, and persistent pulmonary problems in patient 3, who required continued silicone airway stenting for severe tracheobronchomalacia.
Patient 4, who received complete correction at an initial operation, is well and fully active over eight years later. She has had three successful pregnancies. Patient 2 completed nursing school in England, works professionally full-time, and participates in sports. She is now well over twelve years postoperative. Patient 1 has some limitations on exercise, believed by her physicians in the Netherlands to be related to cardiac function. Her flow volume curve showed considerable improvement one year after surgery as did the curve in patient 2.
The eight-year old who had undergone so many and such extensive procedures (patient 3), including ill-advised tracheal resection, and whose trachea already required stenting for malacia remained malacic. It was hoped that growth might lead to improvement in the trachea in time, but continued stenting was essential even though the vascular bulge and pulsations were now absent. The already septic changes in lungs and bronchi (chronic pneumonia and bronchiectasis) progressed. Death occurred from pulmonary sepsis combined with nutritional failure over the subsequent three years.
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Comment
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Langlois and colleagues [1] emphasized that in cases of vascular ring due to right aortic arch and left ligamentum that it was also necessary to excise a diverticulum of Kommerell and to divide and reimplant the aberrant left subclavian artery. These techniques had been previously advised either in whole or in part to manage this abnormality [2, 3]. Backer and colleagues [4] reported relief of recurrent symptoms of obstruction after prior division of ligamentum in eight children by further resection of Kommerell’s diverticulum and reimplantation of ALSA. They now advise this as primary treatment [5]. The appearance of initial symptoms in adults from this particular anomaly has also been noted [6–9] and attributed to aortic enlargement or to aneurysm. Excision of diverticula in children has hence been advocated [10].
The four patients here appear to represent further complexity in this particular variant of vascular ring (right aortic arch, left ligamentum, diverticulum of Kommerell, ALSA, and right descending aorta). With ascending and descending aortic limbs aligned with one another on the right and with anteroposterior narrowing of the thorax (mild pectus excavatum), the space between the aortic limbs is markedly diminished. The aorta is high and a "hairpin" configuration results. The trachea is deformed in its lower third anteriorly from the right by the ascending aorta and posteriorly along with right main bronchus by the diverticulum of Kommerell in the descending aorta. This appears to be a rare subset of an already uncommon anomaly.
Three of our patients had not been relieved of obstructive airway symptoms by division of ligamentum and one of these patients was not relieved by subsequent excision of a diverticulum and division of ALSA. Only one had had no prior operations (Table 1). Their surgical treatment by us was monitored at each step of operation by serial intraoperative flexible bronchoscopy until airway obstruction was seen to be relieved. Langlois and colleagues [1] emphasized the diagnostic accuracy and importance of flexible bronchoscopy in determining the efficacy of correction in such surgery. After elimination of ligamentum, diverticulum, and ALSA in each of our patients, aortopexy and (or) aortic division was required for relief. The value of intraoperative bronchoscopy at each step of these procedures cannot be stressed enough.
In three patients, traction on loops around the aorta (after ligamentum and ALSA had been divided and diverticulum excised) failed to relieve obstruction. Division of the aortic arch alone also did not sufficiently spring the aorta. Anatomical factors did not provide a suitable site for suture pexy of the aorta, even assuming that such pexy might be permanent. Therefore, we utilized a broad sling that seemed unlikely to erode the aorta in the long term and looped it around the adjacent second rib near the chondral junction. The tension used was guided by intraoperative bronchoscopy to assess the effect on the airway. Initially fascia lata was used but a broad strip of Gore-Tex folded longitudinally and sutured to itself seemed preferable in that an added incision was avoided and the sling would not be subject to tissue remodeling or attenuation over time. The sling has remained effective and free of complications over the long term in all of these patients.
Right thoracotomy with additional cervical or cervical mediastinal incision for anastomosis of ALSA to LCC provided easy access for excision of diverticulum, division of ligamentum, division and reimplantation of ALSA, and for aortic bypass graft with aortic arch division and aortopexy.
The usual approach to a right arch and ALSA with Kommerell’s diverticulum is by a left thoracotomy. Our first patient had a previous left thoracotomy with division of the ALSA and resection of the diverticulum, yet she remained symptomatic with airway compression. It was an easy decision, in her, to operate through the right chest to move the aorta off the right side of the airway. Indeed, it would be impossible to do this from the left chest. This experience gave us confidence that we could control and divide the ALSA and the diverticulum from the right side. Cina and colleagues [11] came to the same conclusion in removing an aneurysmal right-sided aortic arch involving a diverticulum after a preceding cervical incision. Our next patient (patient 2) had such severe airway compression we believed that division of the ALSA and resection of the diverticulum would not be enough to relieve the pressure on the airway. Intraoperative bronchoscopy proved that to be correct. Being on the right side of the chest thus allowed both division of the ALSA and resection of the diverticulum, and the ability either to divide the aorta or to perform aortopexy. A right thoracotomy provides surprisingly good access to the origin of the ALSA at Kommerell’s diverticulum. It does require performing an ALSA to carotid anastomosis through a small left cervical incision after completing the chest portion of the operation. When we were confronted with the subsequent two similar patients, we correctly surmised that they would be best handled from a right-sided approach. The distinguishing characteristics of this small subset of patients who required this unusual approach appear to be the combination of the pectus deformity, the tightly spaced hairpin aorta, and the extreme severity of the airway compression. We have indeed studied two additional adult patients with right arch, right descending aorta, ALSA, and diverticulum, but without pectus excavatum deformity, who had moderate tracheal compression insufficient to require treatment.
Robotin and colleagues [12] noted one patient with dextroposed ascending aorta, right-sided arch, and right descending aorta, whose right main bronchus was narrowed in the reduced subaortic space, and who was relieved by "posterior aortopexy." Konstantinov and Puga [13] described esophageal compression by a combination of right aortic arch, ALSA, and diverticulum of Kommerell, but with the arch descending promptly to the left. This patient underwent excision of diverticulum and subclavian anastomosis to the descending aorta. Complete relief of obstruction, however, required aortic bypass graft and division of the arch beyond the RSA.
In three patients, permanent relief was obtained over the long term (8 to 12 years). In one, however, a lengthy series of prior procedures, which had included two pectus repairs and extensive tracheal resection by aortic division and reanastomosis, led to irreversible tracheal malacia and chronic pulmonary sepsis. Experience with a single previously unoperated patient suggests that sling aortopexy without aortic division might be a sufficient adjunct to division of the ligamentum, excision of diverticulum, and reimplantation of ALSA, where scarring from prior surgery is absent. Only further experience will determine this.
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Footnotes
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* Dr Grillo died on Oct 14, 2006. 
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References
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- Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery J Thorac Cardiovasc Surg 2006;129:1339-1347.
- Wong PHC, Lee JWT. Right aortic arch and Kommerell’s diverticulum Thorax 1983;38:553-555.[Free Full Text]
- Georges JL, DeBrux J-L, Langlois J, et al. Les anomalies des arcs aortiques chez l’adulteUne cause rare de compression trachéo-oesophagienne. A propos de deux cas. Ann Chir: Chir Thorac Cardio-vasc 1989;43:99-104.
- Kieffer E, Bahnimi A, Koskas F. Aberrant subclavian artery: surgical treatment in thirty-three adult patients J Vasc Surg 1994;19:100-111.[Medline]
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- Chun K, Colombani PM, Dudgeon DL, Haller Jr JA. Diagnosis and management of congenital vascular rings: a 22-year experience Ann Thorac Surg 1992;53:597-603.[Abstract]
- Cina CS, Arena GO, Bruin G, Clase CM. Kommerell’s diverticulum and aneurysmal right-sided aortic arch J Vasc Surg 2000;32:1208-1214.[Medline]
- Robotin MC, Bruniaux J, Serraf A, et al. Unusual forms of tracheobronchial compression in infants with congenital heart disease J Thorac Cardiovasc Surg 1996;112:415-423.[Abstract/Free Full Text]
- Konstantinov IE, Puga FJ. Surgical treatment of persistent esophageal compression by an unusual form of right aortic arch Ann Thorac Surg 2001;72:2121-2123.[Abstract/Free Full Text]
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Abstract
Introduction
