Journal of Vascular Surgery
Volume 50, Issue 4 , Pages 903-906, October 2009

Hybrid endovascular treatment of aneurysm degeneration in a rare right-aortic arch anomaly with Kommerell diverticulum

  • Paolo Frigatti, MD
    • ,
  • Franco Grego, MD
    • ,
  • Giovanni P. Deriu, MD
    • ,
  • Sandro Lepidi, MD

      Affiliations

    • Corresponding Author InformationCorrespondence: Sandro Lepidi, MD, Division of Vascular and Endovascular Surgery, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School Via Giustiniani 2, 35128, Padova, Italy

Division of Vascular and Endovascular Surgery, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy

Received 11 March 2009; accepted 24 April 2009. published online 06 July 2009.

Article Outline

We present a hybrid endovascular approach to a 6.5-cm aneurysm of the right-sided aortic arch with an aberrant left subclavian artery arising from a Kommerell diverticulum, connected to the left pulmonary artery through the ligamentum arteriosum. The two-step procedure consisted of a bilateral carotid-subclavian bypass, followed by an ascending aorta-bicarotid bypass and completed by an endovascular exclusion of the aneurysms by covering the whole aortic arch and its branches. The patient had no complications and is asymptomatic 21 months after surgery. Hybrid procedures may be helpful in complex aortic arch pathologies, reducing complications of challenging open surgery.

 

We present a two-step hybrid endovascular approach to a 6.5-cm aneurysm of the right-sided aortic arch. Imaging showed the patient had an aberrant left subclavian artery (LSA) arising from a Kommerell diverticulum and connected to the left pulmonary artery through the ligamentum arteriosum.

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Case report 

A 51-year-old healthy asymptomatic man was referred to our department after a routine chest radiograph showed enlargement of the upper mediastinum at the jugular level, indicating the presence of an arch anomaly. A barium swallow (Fig 1, A) revealed an indentation of the esophagus on its right aspect. Magnetic resonance angiography and computed tomography angiography (CTA) scan showed the presence of a right-sided aortic arch, with the right and left carotid artery originating at the same level as first branches, followed by the right subclavian artery (RSA) and left subclavian artery (LSA) arising from a Kommerell diverticulum (KD; Fig 1, B-D). The aberrant LSA was connected to the pulmonary artery through the ligamentum arteriosum. At the level of the RSA, the arch became aneurysmatic, reaching the diameter of 6.5 cm at the level of the KD and presented an acute angulation with the ascending aorta (Fig 1, E).

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  • Fig 1. 

    A, Barium swallow shows enlargement of the superior mediastinum at the jugular level and a right-sided indentation of the esophagus (black arrow). B, Preoperative MIP reconstruction of magnetic resonance angiography (MRA) shows an anterior (A) view of the right-sided aortic arch, with the right and left carotid arteries (RCA and LCA) originating as first branches at the same level, followed by the right subclavian artery (RSA) and the Kommerell diverticulum (KD). S, Superior; I, inferior; R, right; L, left. C, Preoperative MIP reconstruction of MRA showing a left (L) lateral view. The aberrant left subclavian artery (LSA) originates from the KD, connected to the pulmonary artery through the ligamentum arteriosum (LA). A, Anterior; P, posterior. D, Preoperative MIP reconstruction of MRA shows a right (R) lateral view. The LA is connected to the left pulmonary artery (LPA). E, A CT scan cross-section at the level of the KD shows a large aortic aneurysm and an acute-angulated arch. ATA, ascending thoracic aorta.

To avoid an aortic graft replacement and cardiopulmonary bypass, the patient was offered a less invasive two-step hybrid endovascular procedure. A landing zone distal to the carotid arteries origin was considered inadequate to guarantee the aneurysm exclusion due to the acute angle and the short distance of about 1.5 cm between the carotid arteries and the RSA. Therefore a more extended landing zone was chosen proximal to the carotid arteries.

Step 1 

With the patient under general anesthesia and in the supine position, two J-stick supraclavicular neck incisions were used to perform a bilateral carotid-subclavian bypass using an 8-mm Gore-Tex graft (W. L. Gore & Associates, Flagstaff, Ariz). The RSA was tied just proximal to the origin of the vertebral artery. The same procedure was not feasible for the LSA, and an endovascular occlusion was planned.

Step 2 

The next day, through a midline sternotomy and repeat neck incisions, the ascending aorta was clamped tangentially to perform a bypass to the cervical right carotid artery using a 10-mm Gore-Tex graft. A bypass to the cervical left carotid artery was then performed using an 8-mm Gore-Tex graft originating in the thorax from the 10-mm Gore-Tex graft. This solution allowed a less bulky intrathoracic reconstruction and a more anatomic bypass compared with a bifurcated graft.

Through a cutdown on the left common femoral artery, a 10F sheath was inserted. A hydrophilic 0.035-inch guidewire (Terumo Medical Corp, Tokyo, Japan) was advanced into the ascending aorta, and a wire exchange was made for a stiff, 0.035-inch Lunderquist wire (CookEurope, Bjaeverskov, Denmark) through a pigtail catheter. The left axillary artery was punctured, a 5F sheath was inserted, and a pigtail catheter was advanced into the ascending aorta for angiography of the arch and its branches. Through the femoral access, a Cook TX2 endograft (ZTEG 2P 34-140; Cook, Bloomington, Ind) was used for the proximal landing zone 1 cm distal to the carotid bypass, followed by a second Cook TX2 (ZTEG 2PT 38-152) that had been fitted to land in the proximal thoracic aorta with an overlapping of two stents between endografts. A TRILOBE balloon (W. L. Gore & Associates) was used to align the two grafts onto the wall of the arch.

From the left axillary access, several Tornado embolization coils (Cook) and two (12- and 14- × 8-mm) vascular plugs (Amplatzer, AGA Medical Corp, Golden Valley, Minn) were deployed in the KD and the LSA. A completion angiography showed the excellent apposition to the vessel wall and the complete exclusion of the aneurysm and KD with no endoleaks (Fig 2). Wounds were closed in layers, leaving two drains in the thorax, one drain in the neck, and one in the femoral wound.

The patient had no postoperative complications and was discharged 6 days later. The patient remained asymptomatic during the 21-month follow-up. Fig 3 shows the correct graft apposition and complete aneurysms exclusion at the CTA control 18 months after treatment.

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  • Fig 3. 

    Follow-up CT scan control 18 months after the procedure shows (A) a frontal view of the right-sided arch with the origin of the bypass and the proximal endograft and (B) a posterolateral view of the correct endograft apposition with the complete aneurysm exclusion in the acute-angulated arch.

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Discussion 

A right-sided aortic arch is a rare congenital defect of the aorta. The frequency of occurrence is about 0.1%.1, 2 Several variations of the aortic arch system are known to occur in association with a right aortic arch or right descending aorta. Atherosclerotic degeneration of these anomalies in adulthood, like aneurysms or dissections, may pose challenging problems to their treatment. Two major variations of right aortic arch can be distinguished. Type I presents with “mirror-image branching,” in which a left innominate artery arises as the first branch off the aortic arch and then divides into a left common carotid and LSA. Type II presents with an aberrant LSA that arises as a fourth branch off the aortic arch.3

The patient presented here had a type II anomaly, which represents 39.5% of all right-sided aortic arches.4 A left ligamentum (or ductus) arteriosum usually joins the root of the aberrant LSA to the left pulmonary artery and thus completes a vascular ring. The aorta is often dilated into a conical diverticulum at the point of origin of the LSA, producing a retroesophageal impression.1 However, patients with type II anomaly usually have no symptoms of tracheoesophageal compression.5

In 1936 Kommerell6 first reported an aberrant RSA originating from the descending thoracic aorta of a left-sided arch and associated with persistence of a remnant of the right dorsal aorta. The latter appeared as a diverticulum from which the aberrant RSA originated. An aberrant RSA with a left-sided aortic arch is the most common of the anomalies involving the subclavian artery.

In a review of the literature of Kommerell aneurysms associated with an aberrant RSA or LSA, Austin7 reported that 19% of affected patients presented with rupture, and all of them died. In the series and review of the literature of aneurysms associated with a right-sided arch reported by Cinà et al,8 53% of the 32 cases collected presented with rupture or dissection. Even if the size at which these aneurysms will rupture cannot be predicted, the authors suggested an aggressive treatment for aneurysms of ≥3 cm in diameter in good-risk patients.

A number of open surgical procedures were reported, most often using circulatory support, such as partial or total cardiopulmonary bypass with deep hypothermia and circulatory arrest, or a temporary brachial-femoral bypass. In 25 surgically treated patients, Cinà et al8 reported 3 deaths (12%) and one patient with paraplegia (4%). In the review presented by Austin,7 the operative mortality for elective treatment of Kommerell aneurysm was 16.6%.

Several reports have been published on hybrid endovascular treatment of aneurysms of an aberrant RSA and left-sided aortic arch using covered stents in the descending aorta over the origin of the LSA associated with extra-anatomic right or bilateral carotid-subclavian bypass/transposition.9, 10, 11, 12, 13, 14 In the same fashion, endovascular repair of a descending thoracic aortic aneurysm in a right-sided aortic arch with aberrant LSA has been recently reported.15, 16 We present here a more extended hybrid endovascular treatment for a right-sided arch aneurysm associated with an aberrant LSA, due to the more complex anatomy of the vessels anomaly.

The patient was offered a hybrid two-step procedure consisting of a total aortic debranching and an endograft aneurysm exclusion covering the entire aortic arch with all its trunks. Recent comprehensive reviews on complications, durability, and efficacy of these hybrid procedures have been published.17, 18 Hybrid procedures may be helpful in complex aortic arch pathologies, reducing the complications of a challenging open approach.

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References 

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  2. Shuford WH, Sybers RG, Gordon IJ, Baron MG, Carson GC. Circumflex retroesophageal right aortic arch simulating mediastinal tumor or dissecting aneurysm. AJR Am J Roentgenol. 1986;146:491–496
  3. Edwards JE. Anomalies of the derivatives of the aortic arch system. Med Clin North Am. 1948;32:925–948
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  16. Klonaris C, Avgerinos ED, Katsargyris A, Matthaiou A, Georgopoulos S, Psarros V, et al. Endovascular repair of a right-sided descending thoracic aortic aneurysm associated with a right aortic arch and a left subclavian artery arising from a Kommerell's diverticulum. Cardiovasc Intervent Radiol. 2008;[Epub ahead of print; doi: 10.1007/s00270-008-9469-3]
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 Competition of interest: none.

PII: S0741-5214(09)01002-7

doi:10.1016/j.jvs.2009.04.065

Journal of Vascular Surgery
Volume 50, Issue 4 , Pages 903-906, October 2009

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