Vascular Rings with Dr. Carl Backer
With Dr. Carl Backer · hosted by Dr. Tom Bash & Dr. Brittany Levy · StayCurrentMD
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
A vascular ring is a group of blood vessels that encircle and compress the trachea and the esophagus, with a wide variety of types.
The double aortic arch is the classic vascular ring and was the first one ever repaired by Doctor Gross at Boston Children's in the 1940s.
In embryologic development, there are two aortic arch systems (right and left); in most humans the right arch involutes leaving a normal anatomic left arch anterior and to the left of the trachea.
In patients with double aortic arch, the right arch persists so both left and right arches encircle the trachea and esophagus, starting anterior, splitting right and left, then joining posteriorly.
Right aortic arch with left ligamentum creates a vascular ring when the right arch wraps around the trachea and esophagus like a hook and the left ligamentum (connecting to left pulmonary artery) completes the ring.
Double aortic arch tends to be a tighter ring with pulsatile blood on all sides, while right aortic arch tends to be less pathologic with symptoms developing later in life.
Pulmonary artery sling was first described by Willis Potts in Chicago; the left pulmonary artery originates from the right pulmonary artery and passes posterior to the trachea but anterior to the esophagus, compressing the trachea and right main stem bronchus.
About two-thirds of pulmonary artery sling patients have associated tracheal stenosis from complete tracheal rings rather than normal U-shaped rings with posterior membranous septum.
Pulmonary artery sling patients can present very ill in the newborn period with combination of left pulmonary artery compression and stenosis of distal trachea and bronchus from complete tracheal rings.
In innominate artery compression, the innominate artery takes off further along the aortic arch (more to the left), then wraps in front of the trachea and compresses it anteriorly when crossing to the right.
Almost all patients operated on for innominate artery compression syndrome have been less than one year of age.
At a major freestanding children's hospital in Chicago over 60 years, approximately 400 vascular ring patients were treated, averaging about 8 patients per year, increasing to 10-12 per year in the most recent decade.
Vascular rings require high index of suspicion; many patients with noisy breathing, stridor, cough, or dysphagia are previously misdiagnosed as having asthma and croup and treated with albuterol and inhalers.
There is an association of 22Q11 deletion and DiGeorge syndrome with vascular rings, identified more easily with modern genetic microarrays.
The most common presentation of vascular rings is in infancy with respiratory symptoms: noisy breathing, stridor, a seal-barking cough, retractions, head bobbing, and neck hyperextension to flatten and straighten the trachea.
Dysphagia tends to be more of a symptom in older children, either when they start taking solid food or in those with Kommerell diverticulum which can enlarge over time and compress the esophagus.
Pulmonary artery sling patients can present with severe respiratory distress, desaturation, severe retractions requiring intubation, and hyperinflation of the right lung from ball-valve effect where air enters but cannot exit.
Several pulmonary artery sling patients were referred for quote 'absence of the left lung' because the hyperinflated right lung pushed all structures to the left; after repair the left lung was normal.
Innominate artery compression patients present with severe stridor and noisy breathing but not swallowing trouble; they can present with apneic episodes.
In pulmonary artery sling, the esophagus is compressed anteriorly so a bolus of food compresses the trachea more and causes apnea; the same mechanism occurs in innominate artery compression syndrome.
Plain chest X-ray is the first diagnostic step; in double aortic arch the normal aortic knob on the left side is lost, and pulmonary artery sling shows hyperinflation of the right lung.
Thirty years ago barium swallow was the next diagnostic procedure with subtle esophageal compression signs; radiologists before cross-sectional imaging were good at interpreting these to identify right arch or double arch.
MRI is preferred by some institutions to avoid radiation, but it takes longer and often requires sedation or general anesthesia which should be avoided in children and babies.
Fetal diagnosis is increasingly possible when screening ultrasound shows the aortic arch in an abnormal location on the tracheal view; the recommended paradigm is echocardiogram at birth (which can identify right arch, double arch, or pulmonary artery sling), then CT timing based on symptoms.
If the baby is symptomatic, get CT scan and operate before hospital discharge; if not symptomatic, obtain CT imaging between 2 and 4 months of age when they can be fed and swaddled.
For symptomatic vascular rings, operation is recommended in essentially all patients because outcomes are so good and patients do so well; it is a very gratifying operation to perform.
For double aortic arch, the standard operation is to divide the smaller of the two arches; because the right arch is typically dominant, most patients are operated through a left posterolateral muscle-sparing thoracotomy in the 3rd or 4th intercostal space.
Double lumen endotracheal tubes are not used in pediatric vascular ring surgery because the patients are too small; the lung is pushed anteriorly instead.
The ligamentum should be divided in all double aortic arch cases, and the smaller arch (usually left) is divided between vascular clamps with the two stumps oversewn with proline suture.
In the past, standard treatment was just to divide the ligamentum with vascular clamps and oversewing or with ligation and division using silk ligature and proline purse string suture.
Many patients have aberrant origin of the left subclavian from the descending thoracic aorta coming out of a Kommerell diverticulum; a number of these patients returned with recurrent symptoms as the diverticulum enlarged.
For about 15 to 20 years, Dr. Backer has been almost routinely resecting the Kommerell diverticulum and reimplanting or transferring the left subclavian artery to the left carotid artery; this is a larger operation but has had essentially no serious complications, complete relief of dysphagia, and next to no recurrence.
Kommerell diverticulum can enlarge over time into an aneurysm with all the complications an aneurysm can have: dissection, rupture, etc.
Pulmonary artery sling is operated through median sternotomy with cardiopulmonary bypass; the left pulmonary artery is disconnected from the right PA (stump oversewn), brought through the space posterior to trachea/anterior to esophagus up into the left mediastinum, and anastomosed to the main pulmonary artery at the ligamentum site.
If tracheal stenosis is present in pulmonary artery sling, it is typically repaired at the same time with slide tracheoplasty.
For innominate artery compression, one strategy is right anterior lateral thoracotomy with pledgeted sutures to suspend the innominate artery to the back of the sternum; the other technique is median sternotomy to transfer the innominate artery to a spot more right where it is not compressing the trachea.
Thoracoscopic intervention for vascular rings has been around for a long time and is fine for a very small isolated number of patients, but pulmonary artery sling will not be done thoracoscopically.
There is danger in doing double aortic arch thoracoscopically because if the staple line slips or comes apart, the aorta hemorrhages directly into the left chest.
Most vascular ring patients have immediate improvement in their breathing postoperatively.
Patients with severe compression and a component of tracheomalacia can take 6 months to 1 year before they return to normal.
A very small percentage of patients continue to have symptoms after vascular ring surgery, but most are substantially improved by the operation.
There is a group of patients who did not have their Kommerell diverticulum addressed and required reoperation; Dr. Backer wrote a paper about reoperations after vascular ring surgery.
Dr. Backer reports one child spent over 300 days in the hospital being treated for asthma and croup before diagnosis of double aortic arch; after division the child was not hospitalized anymore.
The great majority of vascular ring patients do not have known genetic syndromes and do not tend to have other congenital heart defects, with the exception that tracheal stenosis is very frequently associated with pulmonary artery slings.
Pulmonary artery sling patients have a higher incidence of congenital heart defects like atrial septal defects, tetralogy of Fallot, or VSDs.
About one-third of patients with tetralogy of Fallot or common arterial trunk have right aortic arches, but these tend to have mirror image branching and do not have vascular rings.
Kommerell diverticulum refers to the bulbous configuration of the origin of the aberrant left subclavian artery in the setting of right-sided aortic arch; dilation results in compression causing dysphagia, dyspnea, stridor, wheezing, cough, recurrent pneumonia, obstructive emphysema, or chest pain.
CT scan with contrast is now the easiest study that instantly identifies whether there is a vascular ring and what type; current scanning protocols have very low radiation doses.
According to Dr. Backer, there are not very many patients that are actually good candidates for thoracoscopic intervention; the thoracoscopic approach might send patients home on day one post-op but they often stay till day 2, so not much time is saved.
It is important to set expectations for patients and families that it may be a few months before complete improvement in symptoms is seen.