ELIZABETH STEPHENS: My name is Elizabeth Stephens. I am a pediatric and congenital heart surgeon at Mayo Clinic. In this video, we illustrate the operative management of adult vascular rings. Specifically, right arch with aberrant left subclavian artery. We will show two cases with operative footage for examples.

This illustrates the anatomy. We are looking from the left chest and can see the airway esophagus and the aorta heading behind the airway and esophagus into the right chest. In the foreground, we see the ligamentum and aberrant left subclavian artery with a Kommerell's diverticulum causing compression.

In terms of repair, we will be showing the technique developed by Doctors Bower and Pochettino in which the left subclavian artery is transferred onto the left common carotid artery via a supraclavicular incision. And then in the second stage, a the portion of the aorta with the Kommerell's diverticulum is replaced with a tube graft via a right thoracotomy on partial cardiopulmonary bypass.

In this first case, the patient is a 21-year-old female competitive basketball player who was noted to have a murmur which prompted an Echo. This Echo demonstrated abnormal branching of the head vessels, which led to a CT scan that demonstrated a right arch with aberrant left subclavian artery and Kommerell's diverticulum. Upon further inquiry, she noted to have longstanding respiratory difficulties and dysphagia.

Here is a 3D reconstruction of a CT scan demonstrating her anatomy. You can appreciate the aberrant subclavian and Kommerell's diverticulum. Now we are looking down the barrel, and you can see the ring narrowing the airway and esophagus.

In this first stage of the repair, the head is at the top of the screen and we are making a small left supraclavicular incision. We are now dissecting free the left common carotid. Here, we're putting a vessel loop around the carotid. Now, we are dissecting free the subclavian artery.

We are marking where the anastomosis with the subclavian will go, which is relatively lateral on the carotid. Here, the anastomosis is being completed. The other end of the subclavian has been oversewn. Here is the completed anastomosis.

This diagram illustrates the second stage of the repair through a right thoracotomy. The anatomy is labeled with the Kommerell's diverticulum coming off the aorta, the right subclavian, which will need to be clamped during the repair, and then the azygous vein and superior vena cava.

This next diagram shows the arterial anatomy as viewed from a right thoracotomy. You can appreciate the Kommerell's diverticulum again, the right subclavian artery, and the left subclavian carotid anastomosis performed in the neck during stage one.

This is the intraoperative anatomy. The head is still left on the screen, and we have now performed a right thoracotomy, having notched one rib above and below the thoracotomy. You can see the descending aorta, the azygous vein crossing, and the superior vena cava. The azygous vein is being divided for exposure, and the proximal azygous can be used for cannulation into the superior vena cava.

We are now dissecting out the aortic arch. A blue vessel loop is around the right subclavian artery, and that is being dissected free. The aorta is now being sized, and a 20-millimeter graft was chosen. We use a Seldinger technique for descending aortic cannulation with an 18-French Fem-Flex cannula.

Here, we're putting in the venous cannula in the superior vena cava. This is a pediatric 26-French angled cannula, and the purse string can be on the proximal azygous or the SVC itself without need to enter the pericardial cavity. Now we're putting a clamp on the right subclavian artery. Now we are clamping the descending aorta. And now, clamping the aortic arch.

The superior portion of the graft is now being sutured in place using 4.0 purling. We are now suturing the anterior wall. The clamp on the arch has been moved to the graft, and felted mattress repair sutures have been placed as needed.

Here is the remnant aorta, and you can get a sense of the pouch of the Kommerell's diverticulum extending posteriorly, as also shown in the still frame. Now we are doing the inferior aortic anastomosis. Here's the front wall of the graft anastomosis. Here is the final result and the patient's post-operative CT scan.

The second case is that of a 62-year-old gentleman with worsening upper airway symptoms and dysphagia. A CT scan demonstrated the same vascular ring anatomy. Namely, right-sided arch with aberrant left subclavian and Kommerell's diverticulum.

Here is the CT scan demonstrating a severely dilated Kommerell's diverticulum and aneurysmal dilation of his descending aorta. Here is a 3D reconstruction of that CT scan. An esophagram demonstrated narrowing and deviation of the esophagus to the left.

Here, we are already on partial bypass, and we are transecting the proximal descending aorta. You can appreciate the large Kommerell's diverticulum extending posteriorly. Given the aneurysm dilation of the descending aorta of this patient, a more extensive aortic replacement was performed, down to distal aorta with more normal diameter and tissue quality. The proximal and distal anastomoses are performed as in the last case, and here is the final result. Here is the post-operative 3D reconstruction of the CT scan.

This is a short summary of a complex operation of an unusual anomaly that we at Mayo have obtained substantial experience in. We hope that you have found this video helpful. Please visit our other Mayo YouTube videos as well as our website for further information.